Donna Falvo, RN, PhD, CRC [622688]

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Donna Falvo, RN, PhD, CRC [622688]

Byadmin ianuarie 1, 2024

Third Edition
Donna Falvo, RN, PhD, CRC
Adjunct Professor
Rehabilitation Psychology and Counseling
Allied Health Sciences, School of Medicine
The University of North Carolina
at Chapel HillMedical and
Psychosocial Aspects
of Chronic Illness
and Disability

Copyright © 2005 by Jones and Bartlett Publishers, Inc.All rights reserved. No part of the material protected by this copyright may be reproduced orutilized in any form, electronic or mechanical, including photocopying, recording, or by anyinformation storage and retrieval system, without written permission from the copyright owner.Library of Congress Cataloging-in-Publication DataFalvo, Donna R.Medical and psychosocial aspects of chronic illness and disability / Donna Falvo.—3rd ed. p. ; cm.Includes bibliographical references and index.ISBN 0-7637-3166-8 (casebound)1. Chronic diseases. 2. Chronically ill—Rehabilitation. 3. Chronic diseases—Social aspects.4. Chronic diseases—Psychological aspects.[DNLM: 1. Chronic Disease. 2. Disabled Persons—psychology.3. Disabled Persons—rehabilitation. 4. Social Adjustment. WT 500 F197m 2005] I. Title. RC108.F35 2005616'.044–dc222004017494Production CreditsAcquisitions Editor: Kevin SullivanProduction Director: Amy RoseAssociate Production Editor: Tracey ChapmanAssociate Editor: Amy SibleyAssociate Marketing Manager: Emily EkleCover Design: Anne SpencerManufacturing Buyer: Amy BacusComposition: Bill Noss Graphic DesignPrinting and Binding: Malloy, Inc.Cover Printing: Malloy, Inc.Printed in the United States of America09 08 07 06 05 10 9 8 7 6 5 4 3 2 1World HeadquartersJones and Bartlett Publishers40 Tall Pine DriveSudbury, MA [anonimizat] and Bartlett PublishersCanada2406 Nikanna RoadMississauga, ON L5C 2W6CANADAJones and Bartlett PublishersInternationalBarb House, Barb MewsLondon W6 7PAUK

Dedication
This book is dedicated to the memory of
Dr. Alan Woolf,
A man of science, presence, integrity, strength, and honor

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About the Author
Donna Falvo, R.N., Ph.D., CRC is Adjunct Professor at the University of North Carolina
at Chapel Hill School of Medicine, Division of Rehabilitation Psychology andCounseling. She is a Registered Nurse, Licensed Psychologist, and Certifed RehabilitationCounselor. She has over 30 years serving as teacher, clinician, and researcher. A formerProfessor and Coordinator of Rehabilitation Counseling, Rehabilitation Institute,Southern Illinois University, she was named a Mary Switzer Scholar in 1986, and elect-ed to Sigma XI National Scientiﬁc Research Society in 1995. She was elected Presidentof the American Rehabilitation Counseling Association in 1998 and currently serveson the Editorial Board of the Rehabilitation Counseling Bulletin . She is the author of over
40 articles and book chapters and, in addition to authoring the two previous editionsof Medical and Psychosocial Aspects of Chronic Illness and Disability, she is author of the
book Effective Patient Education: A Guide to Increased Compliance, also in its third edition.
v

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Eileen Burker, PhD, CRCAssociate ProfessorDepartment of Allied Health
Science
Divison of Rehabilitation
Psychology andCounseling
School of MedicineThe University of North
Carolina at Chapel Hill
Catherine T. Calvert, PhD,
CRC
Rehabilitation CounselorNorth Carolina Jaycee Burn
Center
University of North
Carolina Hospitals
Chapel Hill, North CarolinaPatrick P. Carone, MDPsychiatrist Carolina Rehabilitation and
Surgical Associates
Cary, North CarolinaStacy Carone, EdD, CRCAssistant ProfessorDepartment of Allied Health
Science
Division of Rehabilitation
Psychology andCounseling
School of MedicineThe University of North
Carolina at Chapel Hill
Richard E. Falvo, PhDAdjunct ProfessorCell & Molecular PhysiologySchool of MedicineThe University of North
Carolina at Chapel Hill
Ernest Grant, RN, MSNOutreach CoordinatorNorth Carolina Jaycee Burn
Center
University of North
Carolina Hospitals
Chapel Hill, North CarolinaDawn E. Kleinman, MDDermatologistAlamance Skin CenterBurlington, North CarolinaFred Price, RN, MBA (c)Nurse ManagerNorth Carolina Jaycee Burn
Center
University of North
Carolina Hospitals
Chapel Hill, North CarolinaDianne Rawdanowicz Rehabilitation CounselorN.C. Division Vocational
Rehabilitation Services
Department of Health and
Human Services
Raleigh, North CarolinaStephanie J. Sjoblad, AuDClinic DirectorAudiologist/ Assistant
Professor
Allied Health SciencesDivision of Speech/HearingSchool of MedicineThe University of North
Carolina at Chapel Hill
viiSpecial thanks to the following people who generously volunteered their time to read,
review, critique, and discuss various sections of the book. Their dedication and com-mitment to individuals with chronic illness and disability is greatly appreciated by theauthor as well as by the individuals they serve. AcknowledgmentsCHAPTER 1

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In its third edition, Medical and Psycho-
social Aspects of Chronic Illness and Disa-bility has been revised and updated.
Certain sections, such as those on condi-tions of the nervous system have beensubstantially expanded. Added to theend of each chapter are brief case studiesto stimulate discussion. Cases are hypo-thetical and not based on any speciﬁc caseor individual.
This book is designed for nonmedical
professionals and students who have lit-tle prior medical knowledge but who workwith individuals with chronic illness and
disability and need to have an understand-
ing of medical conditions, their implica-tions, and need to have an understandingof medical terms. It is designed as a refer-ence book for professionals in the ﬁeld aswell as a textbook for students. The bookcontinues to use a functional approach tounderstanding a number of medical con-ditions. In an attempt to reinforce thisapproach, an Appendix on FunctionalLimitations has been added (Appendix E).
Chronic illness and disability impact all
areas of individual’s and their family’slives. Only by understanding an individ-ual’s total experience with chronic illnessand disability and how all areas of theirlife are affected are professionals fully ableto help them reach their goals. The focusof the book is to help professionals andstudents understand medical and psycho-social aspects of chronic illness and dis-ability and how they affect an individual’sfunctioning in all areas of life, includingpsychological and social impact, impacton activities of daily living, and on voca-tional function. — DRF
ixPrefaceCHAPTER 1

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CHAPTER 1 PSYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC
ILLNESS AND DISABILITY ………………………………………………………..1Impact of Chronic Illness and Disability ………………………………………..1Disease and Illness ……………………………………………………………………….2Impairment, Disability, and Handicap ……………………………………………2Stress in Chronic Illness and Disability…………………………………………..3Coping Style and Strategies …………………………………………………………..4
Denial………………………………………………………………………………5Regression ………………………………………………………………………..5Compensation ………………………………………………………………….5Rationalization………………………………………………………………….6Diversion of Feelings …………………………………………………………6
Emotional Reactions to Chronic Illness or Disability……………………….6
Grief ………………………………………………………………………………..6Fear and Anxiety……………………………………………………………….7Anger……………………………………………………………………………….7Depression ……………………………………………………………………….7Guilt………………………………………………………………………………..7
Chronic Illness and Disability Through the Life Cycle …………………….8
Chronic Illness or Disability in Childhood ………………………….9Chronic Illness or Disability in Adolescence ………………………10Chronic Illness or Disability in Young Adulthood ………………10Chronic Illness or Disability in Middle Age ……………………….11Chronic Illness or Disability in Older Adulthood ……………….11
Other Issues in Chronic Illness and Disability……………………………….12
Self-Concept and Self-Esteem……………………………………………12Body Image …………………………………………………………………….12Stigma ……………………………………………………………………………13
The Impact of Uncertainty ………………………………………………………….13Invisible Disabilities ……………………………………………………………………14Sexuality ……………………………………………………………………………………14Family Adaptation to Chronic Illness and Disability ……………………..15Quality of Life ……………………………………………………………………………16Adherence to Prescribed Treatment and Recommendations ……………17Patient (Client and Family) Education………………………………………….19Stages of Adaptation and Adjustment…………………………………………..19Functional Aspects of Chronic Illness and Disability ……………………..20
Psychological Issues in Chronic Illness and Disability…………20
xiTable of ContentsCHAPTER 1

Lifestyle Issues in Chronic Illness and Disability ………………..20Social Issues in Chronic Illness and Disability ……………………21Vocational Issues in Chronic Illness and Disability …………….21
CHAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM: PART I
CONDITIONS OF THE BRAIN ………………………………………………….25Normal Structure and Function of the Nervous System………………….25
Nerve Cells……………………………………………………………………..26The Central Nervous System…………………………………………….26The Brain ……………………………………………………………………….28
Conditions Affecting the Brain…………………………………………………….30
Traumatic and Atraumatic Brain Damage…………………………..30Right-Sided Versus Left-Sided Brain Damage………………………37Functional Consequences of Brain Damage ……………………….38Treatment and Management of Brain Damage …………………..46Functional Implications of Brain Damage………………………….49Cerebral Palsy …………………………………………………………………55Epilepsy………………………………………………………………………….60
Diagnostic Procedures Used for Conditions of the Nervous System…67
Skull Roentgenography (X-ray)…………………………………………67Computed Tomography (CT Scan, CAT Scan)…………………….67Magnetic Resonance Imaging (MRI) ………………………………….68Brain Scan (Brain Nuclear Scan)………………………………………..68Positron Emission Transaxial Tomography (PET Scan) ………..69Cerebral Angiography………………………………………………………69Lumbar Puncture (Cerebrospinal Fluid Analysis, Spinal Tap) .69Electroencephalography (EEG)………………………………………….69Neuropsychological Tests …………………………………………………70
Psychosocial Issues in Nervous System Conditions
Involving the Brain……………………………………………………………….70
Case Studies……………………………………………………………………………….70
CHAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM: PART II
CONDITIONS OF THE SPINAL CORD AND PERIPHERALNERVOUS SYSTEM AND NEUROMUSCULAR CONDITIONS ………73Normal Structure and Function of the Spinal Cord and
Peripheral Nervous System …………………………………………………….73
The Spinal Cord………………………………………………………………73The Peripheral Nervous System…………………………………………74
Conditions Affecting the Spinal Cord…………………………………………..76
Spinal Cord Injuries…………………………………………………………76Spina Biﬁda…………………………………………………………………….87Poliomyelitis and Post-Polio Syndrome……………………………..90
Neuromuscular Conditions………………………………………………………….94
Parkinson’s Disease………………………………………………………….94Huntington’s Disease (Huntington’s Chorea)……………………..98XII MEDICAL AND PSYCHOSOCIAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Amyotrophic Lateral Sclerosis (ALS; Lou Gehrig’s Disease)…100Guillain-Barré Syndrome………………………………………………..102Myasthenia Gravis…………………………………………………………104Muscular Dystrophy ………………………………………………………104
Other Conditions of the Nervous System ……………………………………104
Multiple Sclerosis…………………………………………………………..104Central Sleep Apnea ………………………………………………………110Narcolepsy ……………………………………………………………………110Lyme Disease ………………………………………………………………..111Bell’s Palsy…………………………………………………………………….111
Diagnostic Procedures in Conditions of the Spinal Cord or
Neuromuscular or Peripheral Nervous System………………………..111
Spine Roentgenography (X-ray)………………………………………111
Electromyography (EMG) and Nerve Conduction
Velocity Studies………………………………………………………..111
General Issues in Nervous System Conditions……………………………..112
Psychosocial Issues in Conditions of the Nervous System….112Vocational Issues in Conditions of the Nervous System…….117
Case Studies……………………………………………………………………………..118
CHAPTER 4 CONDITIONS OF THE EYE AND BLINDNESS ………………………….123
Normal Structure and Function of the Eye ………………………………….123Measuring Vision ……………………………………………………………………..125Types of Visual Impairments ……………………………………………………..126Conditions Causing Visual Impairment or Blindness……………………126
Refractive Errors…………………………………………………………….126Difﬁculty with Coordination of the Eyes …………………………127Opacities of the Eye……………………………………………………….127Injuries to the Eyes………………………………………………………..128Inﬂammation and Infections of the Eye…………………………..129Glaucoma……………………………………………………………………..129Retinopathy ………………………………………………………………….131Retinal Detachment……………………………………………………….132Retinitis Pigmentosa………………………………………………………132Macular Degeneration ……………………………………………………132
Diagnostic Procedures for Conditions of the Eye …………………………133
Comprehensive Eye Exam………………………………………………133Tonometry ……………………………………………………………………133Gonioscopy…………………………………………………………………..133Ophthalmoscopic Examination ………………………………………133Fluorescein Angiography………………………………………………..134
Treatment and Management of Conditions of the Eye
and Blindness……………………………………………………………………..134
Eyeglasses and Contact Lenses………………………………………..134Refractive Eye Surgery ……………………………………………………134Prosthetic Devices and Eye Replacement………………………….135Table of Contents xiii

Assistive Devices and Low-Vision Aids …………………………….135Orientation and Mobility Training ………………………………….136
Psychosocial Issues in Conditions of the Eye and Blindness………….137
Special Issues for Individuals Who Are Partially Sighted ……137Psychological Issues in Conditions of the Eye
and Blindness…………………………………………………………..138
Lifestyle Issues in Conditions of the Eye and Blindness …….139Social Issues for Individuals with Visual Conditions
or Blindness …………………………………………………………….140
Vocational Issues for Individuals with Conditions of the Eye
or Blindness ……………………………………………………………………….140
Case Studies……………………………………………………………………………..141
CHAPTER 5 HEARING LOSS AND DEAFNESS…………………………………………….143
Normal Structure and Function of the Ear…………………………………..143
The Outer Ear ……………………………………………………………….143The Middle Ear ……………………………………………………………..143The Inner Ear………………………………………………………………..144
Hearing Loss and Deafness ………………………………………………………..145
Frequency and Intensity of Sound…………………………………..145Deﬁnition and Classiﬁcation of Hearing Loss …………………..145Causes of Hearing Loss…………………………………………………..148Conditions of the Ear Contributing to Hearing Loss …………148
Conditions of the Vestibular System…………………………………………..151Diagnostic Procedures……………………………………………………………….151
Identiﬁcation of Hearing Loss…………………………………………151Evaluation of the Vestibular System (Disorders of Balance)..156
Treatment of Hearing Loss and Deafness …………………………………….156
Surgical Procedures………………………………………………………..156Devices and Aids for Hearing Loss…………………………………..157
Psychosocial Issues in Hearing Loss ……………………………………………164
Deafness and Deaf Culture……………………………………………..164Psychological Issues in Hearing Loss ……………………………….166Lifestyle Issues in Hearing Loss……………………………………….167Social Issues in Hearing Loss…………………………………………..167
Vocational Issues in Hearing Loss……………………………………………….169Case Studies……………………………………………………………………………..170
CHAPTER 6 PSYCHIATRIC DISABILITIES………………………………………………….173
Deﬁning Psychiatric Disability …………………………………………………..173The Diagnostic and Statistical Manual of Mental Disorders…………..173Common Psychiatric Disabilities………………………………………………..175
Conditions Diagnosed in Infancy, Childhood,
or Adolescence …………………………………………………………175
Delirium and Dementia………………………………………………….179Schizophrenia ……………………………………………………………….182XIV MEDICAL AND PSYCHOSOCIAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Mood Disorders …………………………………………………………….185Anxiety Disorders ………………………………………………………….186Somatoform Disorders……………………………………………………188Factitious Disorders ……………………………………………………….189Dissociative Disorders…………………………………………………….189Personality Disorders……………………………………………………..189
Diagnostic Procedures in Psychiatric Disability ……………………………190
Uses of Diagnostic Psychological Testing …………………………190Intelligence Tests …………………………………………………………..190Mental Status Examination and Assessment
Through Interviews ………………………………………………….190
Personality Assessment…………………………………………………..191Neuropsychological Testing ……………………………………………191Behavioral Assessment……………………………………………………192
General Treatment of Psychiatric Disability…………………………………192
Psychiatric Rehabilitation……………………………………………….192Nonpharmacologic Approaches to Treatment of
Psychiatric Disability ………………………………………………..193
Pharmacologic Approaches to Treatment of
Psychiatric Disability ………………………………………………..194
Electroconvulsive Therapy ……………………………………………..197
Psychosocial and Vocational Issues in Psychiatric Disability …………197
Psychological Issues……………………………………………………….197Lifestyle Issues ………………………………………………………………198Social Issues ………………………………………………………………….198Vocational Issues …………………………………………………………..199
Case Studies……………………………………………………………………………..200
CHAPTER 7 CONDITIONS RELATED TO SUBSTANCE USE …………………………205
Deﬁning Substance Use Disorders ………………………………………………205Substance Abuse and Dependence ……………………………………………..205
Intoxication ………………………………………………………………….206Withdrawal …………………………………………………………………..206Addiction ……………………………………………………………………..207
Substance Use and Chronic Illness and Disability………………………..207Physical Effects of Alcohol Abuse and Dependence ……………………..208
Treatment of Alcohol Dependence ………………………………….209Alcohol-Related Medical Illness ………………………………………209
Use Disorders Involving Other Substances…………………………………..213
Caffeine and Nicotine ……………………………………………………213Sedatives ………………………………………………………………………214Opioids…………………………………………………………………………215Stimulants…………………………………………………………………….215Cannabis ………………………………………………………………………217Hallucinogens ……………………………………………………………….218Inhalants………………………………………………………………………218Table of Contents xv

Medical Consequences of Abuse of Other Drugs and Substances …..219
Drug-Related Illness……………………………………………………….219
Diagnostic Procedures……………………………………………………………….221
Screening Instruments……………………………………………………222Direct Drug Screening ……………………………………………………222Medical Evaluation………………………………………………………..222Behavioral and Psychological Screening ………………………….223
Treatment of Substance Use Disorders ………………………………………..223Psychosocial and Vocational Issues in Substance Abuse………………..224
Psychological Issues……………………………………………………….224Lifestyle Issues ………………………………………………………………224Social Issues ………………………………………………………………….225Vocational Issues …………………………………………………………..226
Case Studies……………………………………………………………………………..227
CHAPTER 8 CONDITIONS OF THE BLOOD AND IMMUNE SYSTEM……………231
Normal Structure and Function………………………………………………….231
Normal Structure and Function of Red Blood Cells …………..231Normal Structure and Function of White Blood Cells and
Immunity………………………………………………………………..232
Normal Structure and Function of Platelets
and Coagulation ………………………………………………………234
Conditions Affecting the Blood or Immune System……………………..234
Anemia…………………………………………………………………………234Thalassemia…………………………………………………………………..236Polycythemia ………………………………………………………………..236Agranulocytosis (Neutropenia)………………………………………..236Pupura………………………………………………………………………….237Leukemia………………………………………………………………………237Hemophilia …………………………………………………………………..237Sickle Cell Disease………………………………………………………….240Human Immunodeﬁciency Virus (HIV) Infection……………..244
Diagnostic Procedures for Conditions Affecting the Blood
or Immune System………………………………………………………………250
Standard Blood Tests ……………………………………………………..250Bleeding Time Test…………………………………………………………250Prothrombin Time (PT, Pro Time) Test …………………………….251Partial Thromboplastin Time (PTT) Test …………………………..251Bone Marrow Aspiration ………………………………………………..251ELISA and Western Blot………………………………………………….251Hemoglobin Electrophoresis …………………………………………..251Sickle Cell Prep ……………………………………………………………..251
General Treatment for Conditions Affecting the Blood
or Immune System………………………………………………………………252
Transfusion …………………………………………………………………..252Bone Marrow Transplant………………………………………………..252XVI MEDICAL AND PSYCHOSOCIAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Psychosocial Issues in Conditions Affecting the Blood
or Immune System………………………………………………………………252
Psychological Issues……………………………………………………….252Lifestyle Issues ………………………………………………………………253Social Issues ………………………………………………………………….254
Vocational Issues in Conditions Affecting the Blood
or Immune System………………………………………………………………254
Case Studies……………………………………………………………………………..255
CHAPTER 9 ENDOCRINE CONDITIONS ……………………………………………………259
Normal Structure and Function of the Endocrine System……………..259Conditions of the Endocrine System…………………………………………..261
Hyperthyroidism (Graves’ Disease, Throtoxicosis)…………….261Hypothyroidism (Myxedema) …………………………………………262Cushing’s Syndrome (Adrenal Cortex Hyperfunction) ………262Addison’s Disease (Adrenocortical Insufﬁciency) ………………263Diabetes Insipidus………………………………………………………….263Diabetes Mellitus …………………………………………………………..263
Diagnostic Procedures for Conditions of the Endocrine System…….272
Blood Tests for Thyroid Function ……………………………………272Blood Tests for Diabetes Mellitus …………………………………….272
General Treatment of Endocrine Conditions……………………………….272Psychosocial and Vocational Issues in Endocrine Conditions………..273
Psychological Issues……………………………………………………….273Lifestyle Issues ………………………………………………………………273Social Issues ………………………………………………………………….273
Vocational Issues in Endocrine Conditions …………………………………273Case Studies……………………………………………………………………………..274
CHAPTER 10 CONDITIONS OF THE GASTROINTESTINAL SYSTEM………………277
Normal Structure and Function of the Gastrointestinal System …….277Conditions of the Gastrointestinal System ………………………………….279
Conditions of the Mouth ……………………………………………….279Conditions of the Esophagus ………………………………………….280Conditions of the Stomach…………………………………………….282Conditions of the Intestine…………………………………………….285Conditions of the Accessory Organs of the
Gastrointestinal System…………………………………………….291
General Diagnostic Procedures for Conditions of
the Gastrointestinal System………………………………………………….294
Barium Swallow (Upper Gastrointestinal Series) ……………….294Barium Enema (Lower Gastrointestinal Series)………………….294Esophageal Manoscopy (Manometry)………………………………294Endoscopy (Gastroscopy) ……………………………………………….295Proctoscopy, Colonoscopy, and Sigmoidoscopy………………..295Cholecystography………………………………………………………….295Table of Contents xvii

Cholangiography…………………………………………………………..295Ultrasonography (Abdominal Sonography)………………………295Computer Tomography (CT Scan, CAT Scan) …………………..295Radionuclide Imaging ……………………………………………………296Biopsy…………………………………………………………………………..296Abdominal Paracentesis………………………………………………….296Laparoscopy………………………………………………………………….296
General Treatment for Conditions of the Gastrointestinal System…296
Medications ………………………………………………………………….296Hyperalimentation (Total Parenteral Nutrition)………………..297Stress Management………………………………………………………..297
Psychosocial Issues in Conditions of the Gastrointestinal System….297
Psychological Issues……………………………………………………….297Lifestyle Issues ………………………………………………………………298Social Issues ………………………………………………………………….299
Vocational Issues in Conditions of the Gastrointestinal System…….299Case Studies……………………………………………………………………………..300
CHAPTER 11 CARDIOVASCULAR CONDITIONS………………………………………….303
Normal Structure and Function of the Cardiovascular System ………303Cardiovascular Conditions ………………………………………………………..305
Arteriosclerosis (Atherosclerosis)……………………………………..305Aneurysm……………………………………………………………………..306Endocarditis ………………………………………………………………….306Pericarditis ……………………………………………………………………307Rheumatic Heart Disease………………………………………………..307Hypertension ………………………………………………………………..307Coronary Artery Disease: Angina Pectoris
and Myocardial Infarction…………………………………………309
Cardiac Arrhythmia……………………………………………………….312Valvular Heart Conditions ……………………………………………..315Congestive Heart Failure ………………………………………………..316Peripheral Vascular Conditions……………………………………….317
Diagnostic Procedures in Cardiovascular Conditions……………………320
Chest Roentgenography (X-ray)………………………………………320Electrocardiography……………………………………………………….320Holter Monitor ……………………………………………………………..320Cardiac Stress Test …………………………………………………………320Angiography …………………………………………………………………321Echocardiography………………………………………………………….321Radionuclide Imaging ……………………………………………………321Cardiac Catheterization………………………………………………….321
General Treatment of Cardiovascular Conditions ………………………..322
Medical Treatment…………………………………………………………322Surgical Treatment…………………………………………………………322XVIII MEDICAL AND PSYCHOSOCIAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Cardiac Rehabilitation……………………………………………………325
Psychosocial Issues in Cardiovascular Conditions………………………..326
Psychological Issues……………………………………………………….326Lifestyle Issues ………………………………………………………………327Social Issues ………………………………………………………………….327
Vocational Issues in Cardiovascular Conditions…………………………..328Case Studies……………………………………………………………………………..328
CHAPTER 12 CONDITIONS OF THE RESPIRATORY (PULMONARY) SYSTEM…331
Normal Structure and Function of the Respiratory System ……………331Conditions of the Respiratory System…………………………………………333
Infections of the Respiratory System ……………………………….333Chronic Lung Diseases …………………………………………………..337Occupational Lung Diseases (Pneumoconiosis; Asbestosis;
Silicosis; Berylliosis; Byssinosis; Occupational Asthma) ..346
Other Conditions Affecting Respiratory Function………………………..348
Restrictive Pulmonary Disease ………………………………………..348Bronchiectasis……………………………………………………………….348Cystic Fibrosis……………………………………………………………….348Apnea…………………………………………………………………………..351Chest Injuries………………………………………………………………..352
Diagnostic Procedures for Respiratory Conditions ……………………….353
Chest Roentgenography (X-ray)………………………………………353Bronchoscopy ……………………………………………………………….353Laryngoscopy………………………………………………………………..353Pulmonary Angiography ………………………………………………..353Pulmonary Function Tests………………………………………………353Ventilation/Perfusion Scan (Lung Scan) …………………………..354
General Treatment for Respiratory Conditions…………………………….354Psychosocial Issues in Respiratory Conditions……………………………..355
Psychological Issues……………………………………………………….355Lifestyle Issues ………………………………………………………………356Social Issues ………………………………………………………………….357
Vocational Issues in Respiratory Conditions………………………………..358Case Studies……………………………………………………………………………..359
CHAPTER 13 URINARY TRACT AND RENAL CONDITIONS………………………….363
Normal Structure and Function of the Urinary Tract ……………………363Urinary Tract and Renal Conditions …………………………………………..365
Cystitis (Lower Urinary Tract Infection) …………………………..365Pyelonephritis……………………………………………………………….365Urinary or Renal Calculi (Kidney Stones;
Nephrolithiasis; Urolithiasis)……………………………………..366
Hydronephrosis …………………………………………………………….367Glomerulonephritis ……………………………………………………….367Table of Contents xix

Nephrosis (Nephrotic Syndrome)…………………………………….368Polycystic Kidney Disease……………………………………………….368Nephrectomy ………………………………………………………………..368Renal Failure …………………………………………………………………369
Diagnostic Procedures for Renal and Urinary Tract Conditions……..383
Urinalysis ……………………………………………………………………..383Urine Culture………………………………………………………………..383Blood Urea Nitrogen………………………………………………………383Serum Creatinine…………………………………………………………..383Creatinine Clearance Test……………………………………………….383Kidney, Ureter, and Bladder Roentgenography (KUB)………..384Intravenous Pyelogram…………………………………………………..384Cystoscopy……………………………………………………………………384Retrograde Pyelography………………………………………………….384Renal Biopsy …………………………………………………………………384Renal Angiography ………………………………………………………..385
Psychosocial Issues in Renal and Urinary Tract Conditions…………..385
Psychological Issues……………………………………………………….385Lifestyle Issues ………………………………………………………………385Social Issues ………………………………………………………………….386
Vocational Issues in Renal and Urinary Tract Conditions……………..386Case Studies……………………………………………………………………………..387
CHAPTER 14 CONDITIONS OF THE MUSCULOSKELETAL SYSTEM………………389
Normal Structure and Function of the Musculoskeletal System …….389
The Skeletal System ……………………………………………………….389The Muscular System……………………………………………………..392
Conditions of the Musculoskeletal System ………………………………….392
Trauma …………………………………………………………………………392Overuse and Repetitive Motion Injuries…………………………..395Degenerative Conditions………………………………………………..396Back Pain………………………………………………………………………398Chronic Pain…………………………………………………………………402Amputation…………………………………………………………………..406Rheumatoid and Autoimmune Conditions………………………411Other Conditions of the Musculoskeletal System ……………..420
Diagnostic Procedures for Conditions of the
Musculoskeletal System ……………………………………………………….422
Roentgenography (Radiography, X-rays) ………………………….422Arthrography ………………………………………………………………..422Diskography and Myelography……………………………………….422Arthroscopy ………………………………………………………………….422Arthrocentesis……………………………………………………………….422Bone Scan …………………………………………………………………….423Magnetic Resonance Imaging (MRI) ………………………………..423XX MEDICAL AND PSYCHOSOCIAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Computer Tomography (Computed Axial Tomography,
CAT Scan, CT Scan) ………………………………………………….423
Blood Tests……………………………………………………………………424
General Treatments for Conditions of the Musculoskeletal System..424
Medications ………………………………………………………………….424Hyperbaric Oxygen Therapy …………………………………………..425Physical Therapy……………………………………………………………425Casts…………………………………………………………………………….426Assistive Devices ……………………………………………………………426Orthoses……………………………………………………………………….426Traction………………………………………………………………………..427Surgical Treatment…………………………………………………………427
Psychosocial Issues in Conditions of the Musculoskeletal System….428
Psychological Issues……………………………………………………….428Lifestyle Issues ………………………………………………………………429Social Issues ………………………………………………………………….430
Vocational Issues in Conditions of the Musculoskeletal System…….431Case Studies……………………………………………………………………………..432
CHAPTER 15 SKIN CONDITIONS AND BURNS ……………………………………………437
Normal Structure and Function of the Skin…………………………………437Psychological, Social, and Vocational Impact of Skin Conditions ….438Skin Conditions ……………………………………………………………………….439
Dermatitis …………………………………………………………………….439Allergic Reactions ………………………………………………………….439Psoriasis………………………………………………………………………..440Infections of the Skin …………………………………………………….442Acne …………………………………………………………………………….442Herpes Zoster (Shingles) …………………………………………………442Skin Cancers …………………………………………………………………443
General Diagnostic Procedures for Conditions of the Skin ……………443
Biopsy…………………………………………………………………………..443Scrapings, Cultures, and Smears ……………………………………..443Patch Tests ……………………………………………………………………443
General Treatment of Conditions of the Skin………………………………443
Medications ………………………………………………………………….443Dressings and Therapeutic Baths or Soaks………………………..444Light Treatment (Phototherapy) ……………………………………..444Dermabrasion ……………………………………………………………….444Chemical Face Peeling……………………………………………………444Plastic and Reconstructive Surgery ………………………………….444
Burns……………………………………………………………………………………….445
Types of Burn Injury………………………………………………………445Burn Depth …………………………………………………………………..446Burn Severity ………………………………………………………………..447Table of Contents xxi

Burn Treatment……………………………………………………………..448Psychosocial Issues in Burn Injury…………………………………..451Vocational Issues in Burn Injury……………………………………..454
Psychosocial and Vocational Issues in Conditions of the Skin ………455
Psychological Issues……………………………………………………….455Lifestyle Issues ………………………………………………………………455Social Issues ………………………………………………………………….455Vocational Issues …………………………………………………………..456
Case Studies……………………………………………………………………………..456
CHAPTER 16 CANCERS……………………………………………………………………………..459
Normal Structure and Function of the Cell …………………………………459Development of Cancer…………………………………………………………….459Causes of Cancer………………………………………………………………………460Types of Cancer………………………………………………………………………..461Staging and Grading of Cancer ………………………………………………….461General Diagnostic Procedures in Cancer ……………………………………462
Radiographic Procedures (X-ray)……………………………………..462Diagnostic Surgery…………………………………………………………462Cytology ………………………………………………………………………462Endoscopy…………………………………………………………………….463Nuclear Medicine…………………………………………………………..463Laboratory Tests…………………………………………………………….463
General Treatment of Cancer……………………………………………………..463
Surgical Procedures………………………………………………………..464Chemotherapy………………………………………………………………464Radiation Therapy …………………………………………………………465Biological Therapies……………………………………………………….466
Common Cancers and Speciﬁc Treatments………………………………….467
Cancer of the Gastrointestinal Tract………………………………..467Cancer of the Larynx……………………………………………………..468Cancer of the Lung………………………………………………………..471Cancer of the Musculoskeletal System……………………………..471Cancer of the Urinary System…………………………………………471Cancer of the Brain or Spinal Cord …………………………………473Lymphomas ………………………………………………………………….473Multiple Myeloma …………………………………………………………474Leukemia………………………………………………………………………474Cancer of the Breast ………………………………………………………475Gynecological Cancer…………………………………………………….477Cancer of the Prostate ……………………………………………………477Skin Cancers …………………………………………………………………478
Psychosocial Issues in Cancer…………………………………………………….478
Psychological Issues……………………………………………………….478Lifestyle Issues ………………………………………………………………480Social Issues ………………………………………………………………….480XXII MEDICAL AND PSYCHOSOCIAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Vocational Issues in Cancer ……………………………………………………….481Case Studies……………………………………………………………………………..482
CHAPTER 17 ASSISTIVE DEVICES………………………………………………………………485
Deﬁning Assistive Technology……………………………………………………485Individual Assessment……………………………………………………………….486Types of Assistive Devices ………………………………………………………….487
Devices for Activities of Daily Living……………………………….487Mobility Aids ………………………………………………………………..488Sensory Devices …………………………………………………………….488Communication Devices………………………………………………..488Cognitive Memory Aids …………………………………………………489Adaptive Computer Aids ………………………………………………..489Controls and Switches……………………………………………………489Environmental Modiﬁcations …………………………………………489
Other Types of Assistive Devices ………………………………………………..489Service Animals ………………………………………………………………………..490Assistive Devices for Recreation………………………………………………….490Assistive Devices in the Workplace……………………………………………..490Appraisal of Assistive Devices and Alternatives ……………………………491
CHAPTER 18 MANAGED CARE AND CHRONIC ILLNESS AND DISABILITY ….493
The Concept of Managed Care…………………………………………………..493Deﬁning Managed Care …………………………………………………………….493Organizational Models of Managed Care…………………………………….494Additional Concepts in Managed Care ……………………………………….494Clinical Practice Guidelines……………………………………………………….495Ethical Issues in Managed Care ………………………………………………….495Impact of Managed Care on Individuals with Chronic Illness
or Disability………………………………………………………………………..496
Future Issues Facing Managed Care…………………………………………….497
APPENDIX A MEDICAL TERMINOLOGY…………………………………………………….499APPENDIX B GLOSSARY OF MEDICAL TERMS……………………………………………503APPENDIX C MEDICATIONS ……………………………………………………………………..525APPENDIX D GLOSSARY OF DIAGNOSTIC PROCEDURES ……………………………529APPENDIX E FUNCTIONAL LIMITATIONS………………………………………………….535
INDEX ………………………………………………………………………………………………………537Table of Contents xxiii

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IMPACT OF CHRONIC ILLNESSAND DISABILITY
The impact of chronic illness and dis-
ability is far-reaching, extending beyondthe individual to all those with whom theindividual has contact. Chronic illnessand disability affect all facets of life,including social and family relationships,economic well-being, activities of daily liv-ing, and recreational and vocational activ-ities. Although several factors inﬂuencethe extent of impact, every chronic illnessor disability requires some alteration andadjustment in daily life. The extent ofimpact is dependent on:
• the nature of the condition
• individuals’ pre-illness/disability per-
sonality
• the meaning of the illness or disabil-
ity to individuals
• individuals’ current life circumstances • the degree of family and social sup-
port
Reactions to chronic illness and disabil-
ity vary considerably. Some individuals with
chronic illness or disability place less im-portance on the condition and associ
ated
limitations than do able-bodied membersof society. Social groups establish theirown standards with regard to idealized
physical and emotional traits, roles, and re-
sponsibilities. Individuals with chronic ill-
ness or disability who do not ﬁt the socially
determined norm may ﬁnd that, regard-less of their strengths and abilities, theycontinue to be regarded in the context ofsocietal views rather than their own.
People vary in their tolerance to symp-
toms, their functional limitations, andtheir general ability to cope with chronicillness and disability. Consequently, onemust consider the effect of the diagnosis,symptoms, and treatment on all aspects ofindividuals’ lives, speciﬁcally on theircapacity to function within their environ-ment.
Functional capacity goes beyond specif-
ic tasks and activities. It also includes sig-niﬁcant events and relationships withfamily, friends, employers, and casual ac-quaintances. No relationship exists in iso-lation. Just as individuals’ reactions toillness or disability inﬂuence the reactionsof others, so the reactions of others affectindividuals’ self-concept and perception oftheir own strengths and abilities.
Participation in family, social, and work
activities assumes interaction and thecapacity to perform a variety of activities.As interactions or capacities change, or asPsychosocial and Functional
Aspects of Chronic Illness
and DisabilityCHAPTER 1
1

they become limited or restricted, rolesand relationships also change. Althoughsome changes and adjustments may bemade with relative ease, others can haverepercussions in many areas of daily life.The meaning and importance that indi-viduals and their families attribute to asso-ciated changes inﬂuence the ability toaccept the condition and to make neces-sary adjustments. The medical conditionitself is only one factor that determines
individuals’ ability to function effectively.
DISEASE AND ILLNESS
Words are powerful conveyers of con-
cepts (Smart, 2001). Using a standard def-inition of terms facilitates communicationand understanding of what each termimplies. The term disease is derived from
themedical model , which refers to changes
in the structure or function of body sys-tems. The medical model focuses on thetreatment and elimination of symptoms.The term illness refers to individuals’ per-
ception of their symptoms and how theyand their families respond to these symp-toms (Morof Lubkin & Larsen, 2002). It isimportant to understand both concepts.Professionals working with individualswith chronic illness or disability mustunderstand the symptoms, limitations,and progression of a condition in order tofacilitate individuals’ adaptation to theircondition and to maximize their potentialfor functioning. Insight into the medicalnature of a condition helps guide profes-sionals in assessments and interventions,as well as in understanding the physicalconsequences the individual is experienc-ing (Dudgeon, Gerrard, Jensen, Rhodes, &Tyler, 2002). It is also important for pro-fessionals to have insights into individu-als’ perception of their condition and thepersonal relevance and meaning it has forthem so that interventions can be direct-ed toward meeting speciﬁc needs (Shaw,Segal, Polatajko, & Harburn, 2002). Theremust be an understanding of individuals’strengths, resources, and abilities as wellas of the symptoms and limitations asso-ciated with the condition if one is to effec-tively assess the impact of the conditionon their daily lives and goals in relation-ship to the tasks they perform at home, atwork, and in their social environment.
Other terms helpful to understanding
the impact of chronic illness or disabilityon individuals are acute and chronic .
Acute refers to the sudden onset of symp-
toms that are short term and that incapac-itate individuals for only a short time.Chronic refers to symptoms that last
indeﬁnitely and that have a cause thatmay or may not be identiﬁable. Some con-ditions that begin acutely but are notresolved become ongoing and chronic. Achronic condition requires individuals toreorient their overall lifestyle to accommo-date manifestations of the condition. Itrequires them to adapt to the realizationthat life as they previously knew it haschanged. They are then faced with thetask of reorienting values, beliefs, behav-iors, and goals to adapt to that reality.
The course of an illness over time,
including the actions taken by individu-als, their families, and health profession-als working with them to manage or shapethe course of the condition, is called a tra-
jectory (Corbin, 2001). The concept is
important to professionals working withindividuals with chronic illness and dis-ability because it implies a continuum andemphasizes the social and environmentalimpact on the condition.
IMPAIRMENT, DISABILITY,AND HANDICAP
Although sometimes used interchange-
ably, the terms impairment , disability , and2 CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Stress in Chronic Illness and Disability 3
handicap have separate meanings and de-
scribe different concepts. To promote theappropriate use of these terms, in 1980 theWorld Health Organization establishedthe International Classification of Im-
pairment, Disability, and Handicap , which
deﬁned these concepts.
•Impairment refers to the loss or
abnormality of psychological, physi-cal, or anatomical structure or func-tion at the system or organ level thatmay or may not be permanent andthat may or may not result in disa-bility.
•Disability refers to an individual
limitation or restriction of an activi-ty as the result of an impairment.
•Handicap refers to the disadvantage
to the individual resulting from animpairment or disability that presentsa barrier to fulﬁlling a role or reach-ing a goal (World Health Organi-zation, 1980).
Although impairments cause some de-
gree of disability in most people (e.g.,spinal cord injury), the degree to whichthey result in disability is also determinedby individual circumstances. What mayappear to be a relatively minor disruptionof function may actually have major con-sequence for the life of the individualaffected. For example, loss of a little ﬁn-ger may be more disabling for a concertpianist than it would be for a heavy equip-ment operator. Spinal cord injury result-ing in paraplegia has a different impact forsomeone who is an accountant than itwould have for someone who is a labor-er. Determining the extent of disabilityand resulting handicaps includes consid-ering the condition in the context of eachindividual’s life and particular circum-stances without imposing preconceivedideas about how disabling or handicap-ping the condition is. STRESS IN CHRONIC ILLNESSAND DISABILITY
Change is an unavoidable part of life.
Change of job, change of home, changeof family composition, or changesbrought about through the normal agingprocess are all common experiences. De-pending on individuals’ perception andthe circumstances involved, change maybe positive or negative, but it always re-quires some adjustment or adaptation andthus produces a certain degree of stress.
Chronic illness and disability produce
signiﬁcant change and consequently stressbecause individuals must deal with achange of customary lifestyle, loss of con-trol, disruption of physiological process-es, pain or discomfort, and potential lossof role, status, independence, and ﬁnan-cial stability. When individuals have con-ﬁdence in their ability to maintain controlover their destiny and when they believethat changes, although inevitable, aremanageable, stress is less pronounced.When their perceptions of the changesassociated with chronic illness or disa-bility seem insurmountable or beyondtheir ability to cope, stress can be over-whelming.
The degree of stress associated with
chronic illness or disability often is relat-ed to the degree of threat it represents toindividuals. Potential threats of chronicillness or disability include:
• threats to life and physical well-being• threats to body integrity and comfort
as a result of the illness or disabilityitself, the diagnostic procedures, ortreatment
• threats to independence, privacy,
autonomy, and control
• threats to self-concept and fulﬁllment
of customary roles
• threats to life goals and future plans

• threats to relationships with family,
friends, and colleagues
• threats to the ability to remain in
familiar surroundings
• threats to economic well-being
The response to the stresses imposed
by the threat of chronic illness or disa-bility depends on perceptions of theimpact the condition has on variousareas of life, as well as on individuals’capacity to cope.
Stress cannot be easily quantiﬁed, but it
can be interpreted from the behaviorsexhibited by those experiencing chronicillness or disability. When demandsexceed psychological, social, or ﬁnancialresources, stress may be manifested in avariety of ways, such as noncompliancewith recommended treatment, self-destructive behaviors such as substanceabuse, hostility, depression, or otherharmful responses.
Individuals in the same situation do not
necessarily experience the same degree ofstress, and the amount of change oradjustment required is not necessarily anindicator of the amount of stress per-ceived. Those who are able to adapt andcope effectively and mobilize resources aremore successful in managing stress andachieving more stable outcomes.
COPING STYLE AND STRATEGIES
Coping is a constellation of many acts
rather than a single act, is constantlychanging, and is highly individualized.Coping mechanisms are learned and de-veloped over time. Individuals use themto manage, tolerate, or reduce the stressassociated with signiﬁcant life events andto attempt to restore psychological equi-librium after a stressful or traumaticevent. Everyone has developed a varietyof coping mechanisms through his or herlife experiences, and each individual hasa predominant coping style to reduce anx-iety and restore equilibrium when con-fronted with a stressful situation. Copingis manifested through behavior. Copingbehavior is effective and adaptive when it
helps individuals reduce stress and attaintheir fullest potential. It is ineffective andmaladaptive when it inhibits growth and
potential or contributes to physical ormental deterioration.
Coping may be required not only for
dealing with the initial diagnosis, but alsofor subsequent events. Conditions that areprogressive with compounding limitationsnecessitate ongoing coping and adjust-ment to incorporate additional changesinto daily life.
Individuals cope with illness and dis-
ability in different ways. Some activelyconfront their condition, learning newskills or actively engaging in treatment tocontrol or manage the condition. Othersdefend themselves from stress and therealities of the diagnosis by denying itsseriousness, ignoring treatment recom-mendations, or refusing to learn new skillsor behaviors associated with the condi-tion. Still others cope by engaging in self-destructive behavior, actively continuingbehavior that has detrimental effects ontheir physical condition.
Effective coping must be viewed in the
context of each individual’s personalbackground and experiences, life situa-tion, and perception of circumstances.Individuals tend to use coping strategiesthat have worked successfully for them inthe past. When old strategies are no longereffective or are not appropriate to the newsituation, new coping strategies must beimplemented to neutralize events sur-rounding the chronic illness or disabilityand to adjust to any associated limitations.Effective coping enables individuals toattain emotional equilibrium, to achieve4
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Coping Style and Strategies 5
a positive mental outlook, and to avoidincapacitation from fear, anxiety, anger, ordepression. However, coping does notoccur in a vacuum. The social milieu inwhich individuals ﬁnd themselves canfacilitate or discourage effective coping. Ingeneral, an optimum environment is onethat helps individuals gain a sense of con-trol by actively participating in decisionmaking and taking responsibility for theirown destiny as much as possible.
Coping strategies are subconscious
mechanisms that individuals use to copewith stress. All individuals have predom-inant coping strategies to reduce anxietyand restore equilibrium when confrontedwith stress. The strategies they used in thepast are often those employed when theyare confronted with the stress of chronicillness or disability. The use of copingstrategies reduces anxiety, helping individ-uals assume balance and productivity intheir lives. Although these strategies canbe helpful, overuse can be detrimental.
Denial
The diagnosis of chronic illness or dis-
ability and the associated implications canbe devastating and anxiety provoking.Denial is a coping strategy some individ-
uals use to negate the reality of a situation.In the case of chronic illness or disability,individuals may deny that they have thecondition by avoiding recommendedtreatment or by denying implications ofthe condition. In the early stages of adjust-ment, denial may be beneﬁcial in that itenables individuals to adjust to the painfulreality of their situation at their own pace,preventing excessive anxiety. When denialcontinues, however, it can prevent indi-viduals from following medical recom-mendations or from learning new skillsthat would help them reach their maxi-mum potential.Denial of the chronic illness or disabil-
ity can have far-reaching effects on oth-ers if, by denying the condition, indi-viduals place others at risk. For example,proper precautions can greatly reduce thespread of some contagious diseases, suchas tuberculosis or HIV infection. Indi-viduals in active denial of their tubercu-losis or its ramiﬁcations may neglect totake tuberculosis medications regularly,and those with HIV infection may haveunprotected sex, putting others in jeop-ardy. Some individuals may put others atrisk by denying their limitations, such asindividuals who are legally blind but con-tinue to drive even though driving hasbeen prohibited.
Regression
In regression, individuals revert to an
earlier stage of development and becomemore dependent, behave more passively,or exhibit more emotionality than wouldnormally be expected at their develop-mental level. In the early stages of chron-ic illness or disability, returning to thestate of dependency experienced in an ear-lier stage of development can be therapeu-tic, especially if treatment of the conditionrequires rest and inactivity. When individ-uals continue in a regressive mode, how-ever, it can interfere with adjustment andthe attainment of a level of independencethat would allow them to reach maximumfunctional capacity.
Compensation
Individuals using compensation as a
coping strategy learn to counteract func-tional limitations in one area by becom-ing stronger or more proﬁcient in another.Compensatory behavior is generally high-ly constructive when new behaviors aredirected toward positive goals and out-

comes. For example, someone who is un-able to maintain his or her level of phys-ical activity because of limitations associ-ated with his or her condition may turnto creative writing or other means of self-
expression. Compensation as a coping strat-
egy can be detrimental, however, whenthe new behaviors are self-destructive orsocially unacceptable. For example, some-one who experiences disﬁgurement as aresult of his or her disability may becomepromiscuous as a way of compensating for
the perception of physical unattractiveness.
Rationalization
As a coping strategy, rationalization
enables individuals to ﬁnd socially accept-able reasons for their behavior or to excusethemselves for not reaching goals or notaccomplishing tasks. Although rationali-zation can soften the disappointment ofdreams unrealized or goals unreached, itcan also produce negative effects if itbecomes a barrier to adjustment, preventsindividuals from reaching their full poten-tial, or interferes with effective manage-ment of the medical condition itself.
Diversion of Feelings
One of the most positive and construc-
tive of all coping strategies can be thediversion of unacceptable feelings or ideasinto socially acceptable behaviors. Thosewith chronic illness or disability may haveparticularly strong feelings of anger orhostility about their diagnosis or the cir-cumstances surrounding their condition.If their emotional energy can be redeﬁnedand diverted into positive activity, theresults can be beneﬁcial, making virtue outof necessity and transforming deﬁcit intogain. As with all coping strategies, diver-sion of feelings can have negative effectsif feelings of anger or hostility are chan-neled into negative behaviors or sociallyunacceptable activities.
EMOTIONAL REACTIONS TOCHRONIC ILLNESS OR DISABILITY
Sudden, unexpected, or life-threatening
chronic illness or disability engenders avariety of reactions. How individuals viewtheir condition, its causes, and its conse-quences greatly affects what they do in theface of it. They may view their conditionas a challenge, an enemy to be fought, apunishment, a sign of weakness, a relief,a strategy for gaining attention, an irrep-arable loss, or an uplifting spiritual expe-rience. Although emotional reactionsvary, the following are common.
Grief
Grief is a normal reaction to loss. Indi-
viduals with chronic illness and disability
may experience loss of a body part, loss of
function, role, or social status, or other per-ceived losses that lead to a reaction of grief.
Although the grieving and the progression
through stages of grief vary from person to
person, a common initial reaction is shock,
disbelief, or numbness during which thediagnosis or its seriousness may be deniedor disputed. As individuals acknowledgethe reality of the situation, the grief reac-tion may become more pronounced.
After repeated confrontations with ele-
ments of loss, normal adaptation resultsin a gradual change in emphasis and focusthat enables individuals to accept the lossemotionally and to make the adjustmentsand adaptations that are necessary to re-establish their place within the everydayworld. When the grief reaction is pro-longed, individuals may develop a patho-logical grief reaction, which may becomemore disabling than the chronic illness ordisability itself. 6
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Emotional Reactions to Chronic Illness or Disability 7
Fear and Anxiety
Individuals normally become anxious
when confronted with threat. A chronicillness or disability can pose a threat be-cause of the potential loss of function,love, independence, or ﬁnancial security.Threat causes anxiety. Some individualsfear the unknown or unpredictability of acondition, which provokes anxiety. Forothers, hospitalizations that immersethem in a strange and unfamiliar environ-ment away from home, family, and thesecurity of routine produce anxiety. Whenconditions are life-threatening, fear andanxiety may be associated not only withloss of function, but also with loss of life.Fear and anxiety associated with chronicillness or disability can place individualsin a state of panic, rendering them psy-chologically immobile and unable to act.Helping them regain a sense of controlover their situation through informationand shared decision making can be animportant step in reducing anxiety andfacilitating rehabilitation.
Anger
Individuals with chronic illness or dis-
ability may experience anger at them-selves or others for perceived injustices orthe losses associated with their condition.They may believe that their chronic illnessor disability was caused by negligence orthat their condition was avoidable. If theyperceive themselves as victims, angermay be directed toward the persons or cir-cumstances they blame for the conditionor situation. If they believe that their ownactions were partly to blame for the chron-ic illness or disability, anger may be direct-ed inward.
Anger can also be the result of frustra-
tion. Individuals may vent frustration andanger by showing hostility toward thosewho have no relationship to the develop-ment of the chronic illness or disabilityand no inﬂuence over its outcome. Angermay also be an expression of the realiza-tion of the seriousness of the situation andits associated feelings of helplessness. Attimes, anger may not be openly expressedbut rather expressed through quarreling,arguing, complaining, or being excessive-ly demanding in an attempt to gain somecontrol. Helping individuals express angerin appropriate ways and enabling them toexperience a sense of control over their sit-uation can help to resolve anger, whichcould otherwise be detrimental to success-ful rehabilitation.
Depression
With the realization of the reality,
seriousness, and implications of thechronic illness or disability, individualsmay experience feelings of depression,helplessness and hopelessness, apathy,and/or dejection and discouragement.Signs of depression include sleep distur-bances, changes in appetite, difﬁculty con-centrating, and withdrawal from activity.Not all individuals with chronic illness ordisability experience signiﬁcant depres-sion, and, in those who do, depressionmay not be prolonged. The extent towhich depression is experienced variesfrom person to person. Prolonged orunresolved depression can result in self-destructive behaviors, such as substanceabuse or attempted suicide. Individualswith prolonged depression should bereferred for mental health evaluation andtreatment.
Guilt
Guilt can be described as self-criticism
or blame. Individuals or family membersmay feel guilt if they believe they con-

tributed to, or in some way caused, thechronic illness or disability. Those whodevelop lung cancer or emphysema afteryears of tobacco use, or those who receivea spinal cord injury from an accident thatoccurred because they were driving whileintoxicated, may experience guilt becauseof the role they played. In other instances,they may experience guilt because theyfeel their chronic illness or disabilityplaces a burden on their family or becausethey are unable to fulﬁll former roles.
Family members may experience guilt
because of anger or resentment they havetoward the individual with a disability.Guilt may also be associated with blame.Family members may actively demon-strate scorn or contempt toward the indi-vidual with chronic illness or disability,causing him or her to feel more guilty.
Guilt may be expressed or unexpressed
and can occur in varying dimensions. Itcan be an obstacle to the successful ad-justment to the condition and its limita-tions. Self-blame or blame ascribed byothers is detrimental not only to the indi-vidual’s self-concept, but also to rehabili-tative efforts as a whole. Guilt that affectsrehabilitation potential or well-being is anindication that referral to appropriate pro-fessionals for evaluation and treatmentmay be appropriate.
CHRONIC ILLNESS AND DISABILITYTHROUGH THE LIFE CYCLE
Development is not static or ﬁnite. It is
a continual process from infancy to oldage and death. Each developmental stageis associated with certain age-appropriatebehaviors, skills, and developmental tasksthat allow psychological and cognitivetransition from one stage to another.Individuals’ age and developmental stageinﬂuence their reactions to chronic illnessor disability and the problems and conse-quences they experience.
Each developmental stage of life has its
own particular stresses or demands, apartfrom those experienced as a result of ill-ness or disability. Chronic illness and dis-ability at various stages of developmentcan affect the independence, self-control,and life skills associated with these differ-ent developmental stages. Since the needs,responsibilities, and resources of adultsdiffer from those of children, the impactof chronic illness or disability in later years
differs from its impact in young adulthood.
Family members and others generally
adjust their behavior to accommodate andto interact appropriately with individualsas they pass from one developmental stageto the next. When individuals experiencechronic illness or disability, however,others may modify expectations of age-appropriate behavior, and these modiﬁedexpectations may interfere with the indi-vidual’s mastery of the normal skillsrequired to meet the challenges of futuredevelopmental stages.
All aspects of development are related.
Each developmental stage must be under-stood within the context of the individ-ual’s past and current experience. Thosewith chronic illness or disability must beconsidered in the context of their devel-opmental stage and the way in which thechanges and limitations associated withtheir condition inﬂuence the attitudes,perceptions, actions, and behaviors char-acteristic of that stage. Stages of develop-ment serve as a guideline not only inassessing individuals’ functional capacity,but also in determining potential stressorsand reactions.
Problems and stresses at different devel-
opmental stages are similar whether indi-viduals have a chronic illness or disabilityor not. Although there are no clear linesof demarcation between life stages and all8
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Chronic Illness and Disability Through the Life Cycle 9
individuals develop at different rates,there are some commonalities associatedwith different life stages.
Ideally, those with chronic illness or dis-
ability should be encouraged to progressthrough each stage of development as nor-mally as possible, despite their condition.Those whose emotional, social, education-al, or occupational development has beenthwarted may be more handicapped bytheir inability to cope with the subsequentchallenges of life than by any limitationsexperienced because of the illness or dis-ability per se.
Chronic Illness or Disability inChildhood
Although the majority of children with
chronic illness or disability and their fam-ilies adapt successfully, these children areat increased risk of emotional and behav-ioral disorders (Gledhill, Rangel, &Garralda, 2000). In early life, childrendevelop a sense of trust in others, a senseof autonomy, and an awareness and mas-tery of their environment. During theseyears, they begin to learn communicationand social skills that enable them to inter-act effectively with others. They also learnthat limits are set on their explorations,expressions of autonomy, and behaviors.Important to their development is a bal-ance between encouraging initiative andsetting limits consistently.
Chronic illness or disability can impede
the attainment of normal developmentalgoals. Repeated or prolonged hospitaliza-tions may deprive children of nurturingby a consistent and loving caregiver. Thephysical limitations of the condition ortreatment may prevent normal activities,socialization, and exploration of the envi-ronment. In some cases, overly protectivefamily members may restrict activities orprohibit the child from expressing emo-tions normally. In other instances, overlysympathetic parents may condone inappro –
priate behaviors rather than correct them.
Conditions affecting the development
of communication skills may also affectchildren’s interaction with the environ-ment, as well as their future development.Congenital conditions (conditions present at
birth) or conditions that occur in earlychildhood require adjustments through-out the life cycle. These limitations mustbe confronted and compensated for withevery new aspect of normal development.Awareness of normal developmentalneeds enables professionals working withthese children to facilitate experiencesthat foster normal development and toenhance children’s ability to reach theirfull potential.
For most children, entering school ex-
pands their world beyond the scope of
their family. Before children attend school,
the values, rules, and expectations theyexperience are, for the most part, largelythose expressed within the family. As theyenter school, however, they are exposed toa larger social environment. Not only dothey learn social relationships and coop-erative interactions, but they also begin todevelop a sense of initiative and industry.Children gradually become aware of theirspecial strengths. As new skills begin todevelop, school-age children gain thecapacity for sustained effort that eventu-ally results in the ability to follow throughwith tasks to completion. Approval andencouragement by others and acceptanceby peers help them build self-conﬁdence,further enhancing development.
When children with chronic illness or
disability enter school, they may not needspeciﬁc special education placement, butthey may require coordinated school in-terventions to maximize attendance andfacilitate educational and social growth.School-related problems may be reﬂectedin these children’s psychological well-

being, their interaction with other chil-dren, or their academic performance.When physical or cognitive limitationsaffect their ability to perform the skillsnormally valued at their developmentalstage, acceptance by peers may be affect-ed. School attendance may be disruptedby the need for repeated absences, result-ing in the inability to interact on a con-sistent basis within the peer group, whichmay diminish social interactions.
In an attempt to shield the child from
hurt and emotional pain, family membersmay further isolate the child from socialinteractions, creating the potential forreduced self-conﬁdence. The reluctance ofsympathetic family members to allow thechild to participate in activities in whichthere may be failure can interfere with thechild’s ability to accurately evaluate his orher potential. Encouragement of socialinteractions and activities to the degreepossible enables the child to develop theskills and abilities that are needed for lat-er integration into the larger world.
Chronic Illness or Disability inAdolescence
Perceptions of and interactions with
peers become increasingly important asadolescents further deﬁne their identityapart from membership in their family.With the need to establish independence,adolescents begin to emancipate them-selves from their parents and may rebelagainst authority in general. Physicalmaturation brings about a strong preoccu-pation with the body and appearance.Adolescents’ need to be attractive to oth-ers often becomes paramount. Awarenessof and experimentation with sexual feel-ings present a new dimension with whichthe adolescent must learn to cope. Datingand expression of sexuality are importantaspects of maturation. Thus any alterationin physical appearance caused by a chron-ic illness or disability can inﬂuence ado-lescents’ perception of body image andself-concept, thwarting the expression ofsexual feelings.
Adolescents with physical disabilities
may also be at risk for secondary disabil-ities associated with psychosocial factors(Anderson & Klarke, 1982; Stevens, Steele,Jutai, Kalnins, Bortolussi, & Biggar, 1996).An illness or disability during adolescencecan disrupt relationships with peers,resulting in delayed social and emotional
development. Limitations imposed by the
condition, its treatment, or the sympathe-tic and protective reactions by familymembers may become barriers to theattainment of independence and individ-ual identity. Parents may be overprotectiveto the point of infantilizing the adoles-cent, thus decreasing self-esteem and self-conﬁdence.
In some instances, certain characteristics
of normal adolescent development, suchas rebellion against authority or the needto be accepted by a peer group, can inter-fere with the treatment necessitated by achronic illness or disability. If adolescentsdeny the limitations associated with their
disability or ignore treatment recommenda –
tions, there can be further detrimental ef-fects on physical and functional capacity.
Chronic Illness or Disability in YoungAdulthood
In young adulthood, individuals estab-
lish themselves as productive members ofsociety, integrating vocational goals,developing the capacity for intimate rela-tionships, and accepting social responsi-bility. When a chronic illness or disabilitydevelops, its associated limitations, ratherthan the individual’s interests or abilities,may deﬁne his or her social, vocational,and occupational goals. 10
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Chronic Illness and Disability Through the Life Cycle 11
Physical limitations may also inhibit
individuals’ efforts to build intimate rela-tionships or to maintain the relationshipsthey have already established. At thisdevelopmental stage, established relation-ships are likely to be recent, and the lev-
el of commitment and willingness to make
necessary sacriﬁces may vary. Dependingon the nature of the condition, procre-ation may be difﬁcult or impossible, or, ifthe individual already has young children,child-care issues may be the source ofadditional concerns in light of the func-tional limitations inherent in a speciﬁcchronic illness or disability. Young adultswho have not fully gained independenceor left their family of origin by the timeof the onset of their chronic illness or dis-ability may ﬁnd gaining independencemore difﬁcult. In some cases the family’soverprotectiveness may prevent themfrom having experiences appropriate totheir own age group.
Chronic Illness or Disability inMiddle Age
Individuals in middle age are generally
established in their career, have a commit-ted relationship, and are often providingguidance to their own children as theyleave the family to establish their owncareers and families. At the same time,middle-aged individuals may be assuminggreater responsibility for their own elder-ly parents, who may be becoming increas-ingly fragile and dependent. Duringmiddle age, individuals may begin toreassess their goals and relationships asthey begin to recognize their own mortal-ity and limited remaining time.
Illness or disability during middle age
can interfere with further occupationaldevelopment and may even result in ear-ly retirement. Such changes can have asigniﬁcant impact on the economic well-being of individuals and their families, aswell as on their identity, self-concept, andself-esteem. It may be necessary to alterestablished roles and associated responsi-bilities within the family. At the sametime, individuals’ partners, even when therelationship is long term, may be reeval-uating their own life goals. They may per-ceive chronic illness or disability as aviolation of their own well-being, andthey may choose to leave the relationship.Responsibilities for children and agingparents provide additional ﬁnancial andemotional stress to that experienced as aresult of illness or disability.
Chronic Illness or Disability inOlder Adulthood
Ideally, older adults have adapted to the
triumphs and disappointments of life andhave accepted their own life and immi-nent death. Although physical limitationsassociated with normal aging are variable,older adults often experience diminishedphysical strength and stamina, as well aslosses of visual and hearing acuity. Illnessor disability during older adulthood canpose physical or cognitive limitations in
addition to those due to aging. The spouse
or signiﬁcant others of the same age groupmay also have decreased physical stami-na, making physical care of individualswith chronic illness or disability more dif-ﬁcult. When older adults with chronic ill-ness or disability are unable to attend totheir own needs or when care in the homeis unmanageable, they may ﬁnd it neces-sary to surrender their own lifestyle and
move to another environment for care and
supervision. Many individuals in the older
age group live on a ﬁxed retirement in-come, and the additional expenses associ-ated with chronic illness or disability placesigniﬁcant strain on an already tight
bud-
get. Not all older individuals, of course, have

retirement beneﬁts, savings, or other re-
sources to draw on in time of ﬁnancial need.
OTHER ISSUES IN CHRONICILLNESS AND DISABILITYSelf-Concept and Self-Esteem
Self-concept is tied to self-esteem and per-
sonal identity. It can be deﬁned as individ-uals’ perceptions and beliefs about theirown strengths and weaknesses, as well asothers’ perceptions of them. Self-esteem
can be deﬁned as “the evaluative compo-nent of an individual’s self concept”(Corwyn, 2000, p. 357). It is often thoughtof as the assessment of one’s own self-worth with regard to attained qualitiesand performance (Gledhill et al., 2000).
Self-concept inﬂuences the perceptions
of others about an individual. A negativeself-concept can produce negative re-sponses in others, just as a positive self-concept can increase the likelihood thatothers will react in a positive manner.Individuals’ self-esteem is related to theirself-concept and how others respond tothem. Consequently, self-concept has asigniﬁcant impact on interactions withothers and on the psychological well-being of the individual.
Body Image
Body image, an important part of self-
concept, involves individuals’ mental view
of their body with regard to appearanceand ability to perform various physicaltasks. It is inﬂuenced by bodily sensations,social and cultural expectations, and reac-tions of and experiences with others(White, 2000). Body image also changesover time as one’s appearance, capabilities,and functional status change over the lifecycle. It is inﬂuenced by each individual’spersonal conception of attractiveness,which is also determined by social andcultural inﬂuences. Body image is relatedto both self-concept and self-esteem.
Chronic illness or disability forces an
individual to alter his or her self-image toaccommodate associated changes. Factors
inﬂuencing the degree of alteration include:
• the visibility of change• the functional signiﬁcance of the
change
•
the speed with which change occurred
• the importance of physical change or
associated functional limitations tothe individual reactions of others(Moore et al., 2000).
The degree to which an altered self-
image is perceived by the individual in anegative way inﬂuences social and intra-personal interactions, functional capacity,and success or failure in the workplace(Cusack, 2000).
The extent to which individuals incor-
porate change into their body image isalso dependent on the meaning and sig-niﬁcance of the change to the individual.The degree of physical change or disﬁgure-ment is not always proportional to thereaction it provokes. Change consideredminimal by one individual may be con-sidered catastrophic by another.
Changes do not have to be visible in or-
der to alter body image. Burn scars onparts of the body normally covered byclothing or the introduction of an artiﬁ-cial opening or stoma such as with colo-stomy may cause signiﬁcant alteration inbody image even though physical changesare not readily apparent to others.
The concept of body image is complex
and individually determined. Body imageis not only the way individuals perceivethemselves, but also the way they perceiveothers as seeing them. Negative views ofone’s body can be a barrier to psycholog-ical well-being, social interactions, func-12
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

The Impact of Uncertainty 13
tional capacity, and workplace adjust-ment. Consequently, the ultimate goal isto help individuals adapt to changes
brought about by chronic illness or disabil-
ity, integrating those changes into a restruc –
tured body image that can be assimilatedand incorporated into daily life.
Stigma
Stigma is a signiﬁcant factor in many
chronic illnesses and disabilities. Despiteefforts to create a heightened awareness ofthe negative impact of prejudice andstereotypes, and despite changes in socialand public policy that have helped toreduce the stigma associated with chron-ic illness or disability, it still exists formany individuals with chronic or dis-abling conditions.
Acceptable standards of appearance,
activities, and roles are socially deter-mined. Individuals who deviate fromsocietal expectations of what is acceptableare often labeled as different from themajority and, thus, often stigmatized. Thedegree of stigma varies from setting to set-ting, from disability to disability, and fromperson to person. Conditions that are par-ticularly anxiety provoking or threateningare likely to have more stigma attached.Stigma results in discrimination, social iso-lation, disregard, depreciation, devalua-tion, and, in some instances, threats tosafety and well-being. Gender and/or raceor ethnic background can be additionalsources of prejudice and subsequent stig-ma, causing additional stress and creatingadditional barriers to effective functioning(Nosek & Hughes, 2003).
Stigma can have a profound impact on
the ability to regain and maintain func-tional capacity and on acceptance of one’sillness or disability. Stigma not only affectsself-concept and self-esteem, but it alsoproduces barriers that prohibit individu-als from reaching their full potential. Inan effort to avoid stigma, individuals maydeny, minimize, or ignore their conditionand/or treatment recommendations, eventhough it is detrimental to their welfare.Although efforts to reduce or obliteratestigma in society should continue, stigmais most likely to be overcome throughindividual effort. It is possible to reducethe negative impact of societal stigma byhelping individuals establish a sense oftheir own intrinsic worth, despite thecharacteristics of their medical condition.
THE IMPACT OF UNCERTAINTY
Uncertainty in the lives of individuals
with chronic illness and disability canexist for a variety of reasons, but it is oftenrelated to concerns about an unknownfuture, erratic symptoms, the unpredicta-bility of the progression of the disease, orambiguous symptoms. Some chronic ill-nesses and disabilities have an immediateand permanent impact on functionalcapacity, whereas in others the course ofthe illness or disability is more variable.Deterioration may occur slowly over thespan of several years or rapidly withinmonths. Some conditions have periods ofremission, when symptoms become lessnoticeable or almost nonexistent, only tobe followed by periods of unpredictableexacerbation, when symptoms becomeworse. In some cases, the same conditionprogresses at different rates for differentindividuals, rapidly for some and slowlyfor others. In some conditions, it is difﬁ-cult to determine when or if the conditionwill reach the point of severe disability orwhether a dramatic change of functionalcapacity will take place.
Uncertainty of prognosis or progression
of the condition can make planning andprediction of the future difﬁcult. This un-

predictability can be frustrating for affect-ed individuals as well as for those aroundthem. There may be reluctance to plan forthe future at all, so that inability to pre-dict the future becomes more disablingthan the actual physical consequences ofthe condition itself. In other instances,given the unpredictability of their condi-tion, individuals may elect to follow a dif-ferent life course than they would haveotherwise chosen. Decisions not to havechildren, to cut down on the number ofhours spent in the work environment, orto suddenly relocate to a different part ofthe country may be misinterpreted bythose unaware of the individual’s condi-tion or its associated unpredictability. Forthose conditions in which symptoms orresidual effects are unapparent to others,such decisions may be met with misunder-standing or criticism. Criticisms of suchdecisions may be particularly distressingto individuals who do not wish to discloseor share intimate details of their conditionwith the casual observer.
Insecurity about the course of the con-
dition may also cause those closest to theindividual to withdraw emotional interac-tions or support in an attempt to protectthemselves from potential future loss.Thus uncertainty poses particular chal-lenges for individuals and their familiesand can be a source of stress. Living in thepresent, rather than dwelling on eventsthat may or may not occur, can help toreduce stress and anxiety and enhance thequality of life.
INVISIBLE DISABILITIES
Some chronic illnesses or disabilities
have associated physical changes that canbe objectively assessed by others or have
functional limitations that necessitate the
use of adaptive devices. The visibility of a
condition has often been associated withstigmatization and marginality (Livneh &Wilson, 2003). Other conditions, such as
diabetes or cardiac conditions, have no out-
ward signs that alert casual observers to anindividual’s condition. The term invisible
disability refers to these latter conditions.
Because there are no outward physicalsigns or other cues to indicate limitationsassociated with chronic illness or disabil-ity, others have no basis on which to altertheir expectations of the individual’sfunctional capacity. Although this lack ofreaction can be positive (in the sense thatit prevents others from acting out of prej-
udice or stereotypes), it can also be negative
in the sense that it can enable individu-als to deny or avoid acceptance of theircondition and its associated implications.
The degree to which a condition remains
invisible may be a function of the close-ness of the observer’s association with theindividual. Although casual acquaintances
may not notice limitations, those more close –
ly involved with the individual in day-to-day activities may more readily observe
them. However, some conditions under nor-
mal circumstances may offer no visiblesigns or cues, no matter how close anoth-er person is with the affected individual.
The unapparent aspect of the limitation
in invisible disability may be a unique ele-
ment related to individuals’ adjustment and
acceptance of their limitation. Without
environmental feedback to create a tangi-
ble reality of the condition, individuals with
invisible disability may postpone adapta-tion or ignore the medical treatment orrecommendations necessary to control thecondition and prevent further disability.
SEXUALITY
Human sexuality is more than genital
acts or sexual function. It is intrinsic to aperson’s sense of self (Hordern & Currow,2003). It is an ever changing, lived expe-14
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Family Adaptation to Chronic Illness and Disability 15
rience, affecting the way individuals viewthemselves and their body (Hordern,2000). Sexuality encompasses the wholeperson and is reﬂected in all that individ-uals say and do. It is an important part ofidentity, self-image, and self-concept.Each person is a sexual being with a needfor intimacy, physical contact, and love.Chronic illness or disability can havemany effects on sexuality and can inﬂu-ence all phases of sexual response(McInnes, 2003).
The expression of sexual urges is one
form of sexuality. Chronic illness or dis-ability can affect sexual expression be-cause of physical limitations, depression,lack of energy, pain, alterations in self-image, or the reactions of others. In someconditions, the main barrier to sexualexpression may be problems with self-con-cept and body-image; in other condi-tions, physical changes may present phys-ical barriers that affect sexual functiondirectly.
Regardless of the type of limitations
associated with chronic illness or disabil-ity, sexual expression continues to be animportant function that should beaddressed (McBride & Rines, 2000). Insome instances, it may be necessary tohelp individuals overcome their own mis-perceptions and fears in order to establisha means for sexual expression. In otherinstances, individuals may need assistanceto overcome barriers or to learn methodsof sexual expression different from thoseused previously. In any case, sexual adjust-ment is a signiﬁcant element in therestoration of an individual’s maximalfunctional capacity.
FAMILY ADAPTATION TO CHRONICILLNESS AND DISABILITY
The family is the social network from
which individuals derive identity and withwhom they feel strong psychologicalbonds. “Family” has different meaningsfor different people and is not alwaysbased on blood or law. Family providesprotection, socialization, physical care,support, and love. Each individual with-in the family structure plays some rolethat is incorporated into everyday familylife.
Chronic illness or disability has an emo-
tional and economic impact on families aswell as on individuals. Family reactions tochronic illness and disability may be sim-ilar to those experienced by the individ-ual and may include shock, denial, anger,guilt, anxiety, and depression. Familiesmust make adaptations, adjustments, androle changes both as a unit and as individ-ual family members. The way in whichfamilies react and adapt to chronic illnessand disability affects an individual’s sub-sequent adjustment. Whether familiesfoster independence or dependence, showacceptance or rejection, or encourage orsabotage compliance with restrictionsand recommended treatments has a pro-found effect on individuals’ ultimatefunctional capacity.
When a family member is no longer
able to perform certain functions, familiesmay react in various ways. There may bea strong desire to be a “normal” familyagain. Family members with prior ex-pectations for the individual’s future or“what he or she might have been” mayexperience anger, resentment, or disap-pointment if they see chronic illness ordisability interfering with the achieve-ment of their expectations.
Family members can also act as advo-
cates for the individual. They may need tobecome more involved with health profes-sionals and service agencies or may needto be increasingly assertive to obtain nec-essary services. If individuals with chron-ic illness or disability require signiﬁcant

care or therapies to be administered athome, family members may becomefatigued because of the extra responsibil-ity and work required, especially if respiteservices are limited.
Families, like individuals, have differing
resources, depending on their life circum-stances, previous experiences, and the per-sonalities involved. Individual familymembers may be called upon to providenot only emotional support but alsophysical care, supervision, transporta-tion, or a variety of other services neces-sitated by the individual’s condition. Inaddition, changes in roles or ﬁnancial cir-cumstances due to chronic illness or dis-ability may alter the goals and plans ofother family members, such as a sibling’splans for college or a parent’s retirementplans. The amount of care and attentionrequired by individuals with chronic ill-ness or disability may create emotionalstrain among family members, resulting infeelings of resentment, antagonism, andfrustration. Role change and ambiguitymay make it necessary to redeﬁne familyrelationships as new and unaccustomedduties and responsibilities arise.
QUALITY OF LIFE
Successful rehabilitation means more
than helping individuals reach their maxi-mum functional capacity. It also meanshelping them achieve and enhance theirquality of life. Quality of life is subjective
in nature and has no universal meaning.No two people deﬁne the term in quite thesame way. Although some see it as opti-mal functioning at the highest level ofindependence, others may place greateremphasis on life itself, regardless of levelof function. Only the individual can deter-mine the personal meaning of this term.Individual value systems, cultural back-grounds, spiritual perspectives, and theattitudes and reactions of those within theenvironment all inﬂuence the interpreta-tion of quality of life.
As already stated, perceptions of the
same condition and its impact vary fromindividual to individual (Burker, Carels,Thompson, Rodgers, & Egan, 2000; Crews,Jefferson, Broshek, Barth, & Robbins,2000). People with similar conditions,symptoms, and limitations may perceivetheir condition in totally different man-ners. Determining factors are the charac-teristics of the condition and its treat-ment, the age and developmental stage ofthe individual, the degree of limitationand the extent of disability experienced,and how characteristics of the conditionaffect the individual’s deﬁnition of qual-ity of life. Symptoms or limitations thatone individual accepts and adapts to maybe overwhelming and intolerable toanother.
Because of the ambiguous nature of the
concept, it is difﬁcult to assess quality oflife (Bishop & Feist-Price, 2001). Attemptsto discover and accurately measure qual-ity of life have caused considerable con-fusion and resulted in the creation ofmultiple indicators, ranging from physi-ologic parameters to the ability to returnto work to the ability to participate insocial activities and the number of psy-chological problems experienced by theindividual. In addition, studies of qualityof life have often identiﬁed discrepanciesbetween the judgment of service providersand that of the consumer regarding qual-ity-of-life outcomes (Leplege & Hunt,1997).
Individuals’ perception of quality of life
is among the main determinants ofdemand for services, compliance withtreatment, and satisfaction with treatmentand services provided. How some individ-uals assess the impact of their conditionon their quality of life is determined by16
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Adherence to Prescribed Treatment and Recommendations 17
the degree to which they feel control overtheir life circumstances or destiny.Accurate knowledge about their conditionand treatment, together with active par-ticipation in decision making about themanagement of the condition, can enableindividuals with chronic illness and dis-ability to make judgments that will enablethem to enhance their quality of life interms of their own needs, goals, and cir-cumstances.
ADHERENCE TO PRESCRIBEDTREATMENT ANDRECOMMENDATIONS
Most chronic illnesses or disabling con-
ditions require ongoing treatment, med-ical supervision, or restrictions on activityto control the condition or to preventcomplications. However, many individu-als with chronic illness or disability fail tofollow the recommendations prescribed,imperiling their own well-being (Dunbar-Jacob et al., 2000; Graham, 2003).Neglecting to take medications as pre-scribed, resisting restriction of activities, orengaging in behaviors that are likely tocause complications can significantlyinﬂuence individuals’ medical prognosisand functional capacity (Dolder, Lacro,Leckband, & Jeste, 2003; Schmaling, Afari,& Blume, 2000; Vergouwen, Bakker,Katon, Verheij, & Koerselman, 2003;Zygmunt, Olfson, Boyer, & Mechanic,2002). The best rehabilitation plan is of lit-tle value if individuals do not follow thetreatments designed to control theirsymptoms or disease or to prevent com-plications or progression of the disease(Kovac, Patel, Peterson, & Kimmel, 2002;Loghman-Adham, 2003).
Although individuals who purposely
behave in a way that makes their condi-tion worse seem irrational, there are anumber of explanations for nonadherentbehavior. Illness or disability elicits manyresponses from individuals and their fam-ilies. Different reactions, experiences, andmotives direct behavior and can help orhinder adherence with treatment recom-mendations.
Individuals’ lives are guided by a set of
norms and values—expressed or un-expressed. Each individual has a person-al, unique perspective on health, illness,and medical care itself. Thus each indi-vidual understands the meaning of illness,the consequences ascribed to adherence torecommendations, and treatment recom-mendations and their implications differ-ently. Whereas some individuals reactmildly to a condition that may devastateanother, others display considerable emo-tional and physical discomfort with con-ditions that most people consider minor.Obviously, various psychosocial factorsdetermine individuals’ reactions to illnessand, consequently, their reactions to therecommendations and advice given.
Chronic illness or disability disrupts the
way individuals view themselves and theworld, and it can produce distortions inthinking. Most individuals initially expe-rience a feeling of vulnerability and a shat-tering of the magical belief that they areimmune from illness, injury, or evendeath. With this realization, they may losetheir sense of security and cohesiveness.Life may seem a maze of inconveniences,hazards, and restrictions. Nonadherenceto recommendations may be an attemptto exert self-determination, to regain asense of autonomy and control, and toclaim some mastery over their individualdestiny. In other instances, resistance totreatment recommendations may be adenial of the condition itself.
Nonadherence can also be a reﬂection
of an individual’s feelings about his or herlife circumstances. For some individuals,having a chronic illness or disability is not

a positive role; for others, it may be farpreferable to the social role they held pre-viously. Some may vacillate between thewish to be independent and the wish toremain dependent. Chronic illness or dis-ability can be a means of legitimizingdependency, as well as a means of in-creasing the amount of attention re-ceived. Subsequently, individuals may bereluctant to return to former roles andobligations. Motivation to retain the sickrole is at times greater than the motivationto gain optimal function. As a result, ulti-mate rehabilitation is hampered.
Failure to adhere to recommendations
can also be a response to guilt that hasbeen incorporated into the reaction to orbeliefs about illness or disability. If healthand well-being are perceived as rewards fora life well lived, illness or disability maybe viewed as punishment for real or imag-ined actions of the past. Adherence tomedical advice may be perceived as inter-ference with a punishment believed to bedeserved. In other instances, individualsmay feel guilty because they believe thatthe illness or disability is a direct result oftheir own negligence or overt actions.Guilt or shame at being different may alsohinder adherence to treatment recom-mendations. Some individuals mayattempt to hide their condition from oth-ers and, thus, fail to follow recommenda-tions that they fear may call attention totheir condition.
The impact of chronic illness or disabil-
ity on individuals’ general economic well-being can also affect their ability andwillingness to follow treatment recom-mendations. Many occupations offerfringe beneﬁts, such as paid sick days oreven time off with pay in which to seekmedical care, but other occupations pro-vide no such beneﬁts. In the latter in-stances, days taken off from work becauseof illness or medical appointments candecrease income. The economic conse-quences of chronic illness or disabilitymay also cause a reverse reaction. If anindividual is receiving disability beneﬁtsand has little opportunity for satisfactoryemployment, he or she may not followrecommendations that would increase hisor her capacity to return to work andthereby decrease or eliminate beneﬁts.
Finally, as already discussed, quality of
life is a relative concept, uniquely deﬁnedby each individual. If treatment recom-mendations result in pain, discomfort, orinconvenience greater than the beneﬁtperceived by the individual in terms of hisor her own subjective deﬁnition of thequality of life, compliance with pre-scribed recommendations may not be per-ceived as worth the psychological, social,or physical cost. Treatment can some-times, but not always, be adjusted to makeadhering to recommendations more palat-able. Individuals’ right to self-determina-tion must be carefully balanced with theassurance that the choice of nonadher-ence is based on information and fullunderstanding of the consequences.
Some individuals readily adjust to the
challenges, limitations, and associated be-havioral changes necessitated by chronicillness or disability. Many individuals,however, actively sabotage treatment andrecommendations, to their own detri-ment. In such instances, professionals’goals should be to attempt to understandthese individuals’ underlying problemsand motivations and to help them makenecessary adjustments and adaptations.Rather than criticize them for disinterest,lack of motivation, or failure to follow rec-ommendations, it is important to identi-fy the barriers that prohibit adherence andto recognize that such reactions may indi-cate difﬁculty in accepting the conditionor adapting recommendations to theirown unique way of life.18
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Stages of Adaptation and Adjustment 19
PATIENT (CLIENT AND FAMILY)EDUCATION
Although medical care, support, and
auxiliary services are important aspects ofhelping individuals reach their maxi-mum potential, successful management ofchronic disease or disability requires con-siderable individual and family effort.Regardless of the complexity of the con-dition, many individuals are now expect-ed to carry out treatments in their homerather than depend on medical personnelin health care settings. Individuals’ under-standing of their condition and treatmentis one of the basic components of self-determination and responsible care. Notonly must they understand how to inte-grate regimens into daily routines andhow to carry out daily care activities, butthey must also understand preventivehealth care measures to retain functionand prevent further disability or healthproblems (Falvo, 2004). Because of increas-ing public awareness of the need for indi-viduals to accept this greater responsibilityand self-determination, a number of pro-grams and counseling services have beenestablished to help clients and their fam-ilies reach this goal.
STAGES OF ADAPTATION ANDADJUSTMENT
A host of personal, social, and environ-
mental experiences, demands, supportsand resources, and coping strategies inter-act to inﬂuence adaptation outcomes(Livneh, 2001). The process of adjustmentincludes a search for meaning in the expe-rience and an attempt to regain controland self-determination over events thataffect one’s life. Most individuals withchronic illness and disability experiencesome loss, either a direct physical loss ora more indirect loss of the ability to par-ticipate in some previously performedactivity. Regardless of the nature of theloss, a variety of reactions may take placewhile individuals attempt to make neces-sary adaptations and changes.
Stages of adjustment are individual
and varied. The shock of diagnosis and itsconsequent implications may have anumbing effect, so initially individualsmay demonstrate little emotional reac-tion. As the reality of the situation be-comes clear, they may experience a senseof hopelessness and despair, mourning fora self, a role, or a function that is lost.They may experience feelings of anger,which alternate with depression. Manyindividuals go through a period of mourn-ing and bereavement similar to that expe-rienced when a loved one is lost.Mourning is a natural reaction to loss andallows time for reﬂection and reestablish-ment of emotional equilibrium. As indi-viduals begin to appraise their conditionrealistically, examine the limitations thatit imposes, and adjust to the associatedlosses, they may gradually seek alterna-tives and adaptations to become integrat-ed into a broader world.
The ultimate goal of adjustment is
acceptance of the condition and its asso-ciated limitations, along with a realisticappraisal and implementation ofstrengths. As individuals accept their con-dition, they attain their maximal func-tional capacity. The amount of time thatindividuals need to reach acceptance isdependent on personality, the reaction offamily and signiﬁcant others, life circum-stances, available resources, and the typesof challenges that confront them. Someindividuals never reach acceptance.Maladjustment and nonacceptance arecharacterized by immobility, markeddependency, continued anger and hostil-ity, prolonged mourning, or participationin detrimental or self-destructive activities.

Just as coping mechanisms are vital partsof human nature, serving to protectagainst stress, reduce anxiety, and facili-tate adjustment, overuse or maladaptiveuse of coping mechanisms can postponeor inhibit adjustment.
FUNCTIONAL ASPECTS OF CHRONICILLNESS AND DISABILITY
The functional effects of chronic illness
or disability are many and varied. Eachindividual has different needs, abilities,and circumstances that determine howchronic illness or disability affects his orher functional capacity. The extent towhich the condition is handicappingdepends to a great extent on individuals’perception of the condition, the environ-ment, and the reactions of family, friends,and society in general. The severity of thecondition as measured by diagnostic testsdoes not always indicate the severity offunctional impairment. Also, individuals’ability to function is not always directlycorrelated with the severity of the condi-tion itself.
Professionals working with individuals
with chronic illness or disability needto understand the potential limitations orrestrictions associated with a speciﬁc con-dition or treatment in order to help indi-viduals and their families makeappropriate changes. The effects of chron-ic illness and disability are far-reaching;they include psychological, social, andvocational effects, and changes andadjustments in both general lifestyle andactivities of daily living. The medical diag-nosis per se is not as important as thedegree to which function in each area ofan individual’s life is affected. The inter-active nature of function between each ofthe areas determines the extent to whichindividuals reach their maximal potential.A focus on any one area without full con-sideration of the impact of the illness ordisability on all other areas can dilute theeffectiveness of rehabilitative efforts. Thusunderstanding and working effectivelywith individuals who have a chronic ill-ness or disability requires a broad outlookthat goes beyond medical diagnosis. Themost important factor is the individual’sability to function with the conditionwithin his or her environment and allareas of his or her life.
Psychological Issues in Chronic Illnessand Disability
Individuals react both cognitively and
emotionally to events that involve them.These reactions, in turn, affect thefurther course of those events. Psycho-logical factors are ever present in allaspects of chronic illness and disabilityand inﬂuence individuals’ response totheir condition; sometimes these factorsare part of the symptoms of the condition.They affect not only individuals’ adjust-ment and subsequent functional capacity,but also the outcome and prognosis.
Lifestyle Issues in Chronic Illnessand Disability
Lifestyle comprises daily tasks and activ-
ities of daily living within an individual’senvironment. It includes the ability to per-form tasks related to grooming, house-keeping, and preparing meals. It alsoincludes activities related to transporta-tion, daily schedules, rest or activity, recre-ation, sexuality, and privacy. At times,limitations in performing the activities ofdaily living result from environmentalconsiderations that serve as barriers toeffective functioning. Modiﬁcations suchas widening doorways to permit the pas-sage of a wheelchair, placing handrails ina bathroom, or installing more effective20
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

Functional Aspects of Chronic Illness and Disability 21
lighting may be required to increase func-tional capacity. Other lifestyle modiﬁca-tions may be necessary because of theadditional tasks and time commitmentsrelated to medical treatment of a speciﬁccondition. In some instances, restrictionsof diet or activity may require a consider-able lifestyle change. Continued treat-ments, medical appointments, and relatedactivities may require signiﬁcant alterationof the daily schedule.
Social Issues in Chronic Illnessand Disability
The social environment can be deﬁned
as individuals’ perceived involvement inpersonal, family, group, and communityrelationships and activities. Social well-being is based on emotionally satisfyingexperiences in social activities with thosewithin the individual’s social group.Chronic illness and disability often leadto changes in social status. Individualsmay ﬁnd themselves in a socially devaluedrole. As a result, they may experiencechanges in social relationships or inter-actions, or they may have to limit thenumber of social activities, all of whichcan result in social isolation. Even whenindividuals with chronic illness or disa-bility attempt to remain socially active,they may have difﬁculty entering commu-nity facilities because of environmentalbarriers or because of prejudice or stereo-typing.
Many factors contribute to an individ-
ual’s adaptation or adjustment to the so-cial limitations associated with a partic-ular medical condition. Individuals’ per-ception or misperception of the reactionsof others in social groups may determinethe level of acceptance that they receive.The degree to which they are able toadapt, accept, and adjust to their function-al limitations is determined in part bytheir interactions with others in theirenvironment, as well as by their interpre-tation of the reactions of others.
Vocational Issues in Chronic Illnessand Disability
The signiﬁcance of work in the rehabil-
itation of people with chronic illness anddisability has been well documented(Cunningham, Wolbert, & Brockmeier,2000). Work involves more than remuner-ation for services rendered and does notnecessarily include only activity related tofinancial incentives. Work provides asense of contribution, accomplishment,and meaning to life (Ben-Shlomo,Canﬁeld, & Warner, 2002; Bond et al.,2001; Corrigan, Bogner, Mysiw, Clinchot,& Fugate, 2001). Consequently, loss of theability to work extends beyond ﬁnancialconsequences to social and psychologicalwell-being. It also means the loss of asocially valued role. For many individuals,work is not only a major part of their iden-tity, but also a source of social interaction,structure, and purpose in life.
The degree to which chronic illness and
disability affect individuals’ ability andwillingness to work depends on a varietyof factors in addition to the limitationsimposed by the illness or disability itself(Young & Murphy, 2002). Other factorsinclude the nature of the work, the phys-ical environment of the work setting, andthe attitudes of employers and coworkers.Psychosocial variables may also compli-cate functional capacity and, thus, therehabilitation process. At times, individ-uals with chronic illness or disability maycontinue to perform the same work theyperformed before the onset of the condi-tion. At other times, certain work tasks,environmental conditions, or work sched-ules must be modiﬁed to accommodatethe limitations imposed by the chronic ill-

ness or disability. If modiﬁcations cannotbe made in these cases, individuals mustchange employment. Some individualsmust assume disability status becauseappropriate modiﬁcations cannot be madeor because their limitations are severe. Jobstress or the attitudes of employers orcoworkers can signiﬁcantly interfere withindividuals’ ability to return to the workforce. Problems with transportation to andfrom work because of limitations causedby the condition may also make a returnto work more difﬁcult. In other instances,the time required to carry out treatmentrecommendations related to the conditionmay make completing a full day at workvirtually impossible.
Individuals’ capacity to function at a job
can depend on cognitive, psychomotor,and attitudinal factors, as well as on thephysical aspects of the illness or disabili-ty. Accurately assessing individuals’ capac-ity to return to work consists of more thanevaluating physical factors. Individuals’fear of reinjury, vocational dissatisfaction,or legal issues can also hamper return towork. Their ability to relate to and inter-act with others within the work environ-ment must also be considered. Interests,aptitudes, and abilities are always pivotalfactors in determining vocational success,regardless of limitations. Effective rehabil-itation that enables individuals to func-tion effectively in their job often involvesthe interdisciplinary efforts of many typesof medical and nonmedical professionalsto conduct assessment, evaluation, thera-py, and vocational guidance.22
CHAPTER 1 P SYCHOSOCIAL AND FUNCTIONAL ASPECTS OF CHRONIC ILLNESS AND DISABILITY

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NORMAL STRUCTURE AND FUNCTION
OF THE NERVOUS SYSTEM
The nervous system consists of the cen-
tral nervous system and the peripheralnervous system (Table 2–1). The nervoussystem is a complex network that servesas the communication center for the body.It controls and coordinates activities andfunctions throughout the body by send-ing, receiving, and sorting electricalimpulses. Disruption of any part of thenervous system affects body function insome way, either internally
or externally.
Speciﬁcally, functions of the nervous sys-
tem include the following:
1. Organizing and directing motor re-
sponses of the voluntary muscle sys-
tem, enabling the body to move more
effectively as a whole and to achievepurposeful movement. This coordi-nation of voluntary muscle makespossible complex activities such aswalking or playing a piano, as well asactivities as simple as maintainingmuscle tone and posture while at rest.
2. Monitoring and recognizing stimuli
within the environment and inter-preting changes as information to beobserved or acted upon. This func-
tion makes possible reﬂex action suchas pulling away one’s hand from ahot surface as well as perceivingmusic being played in the next room.
3. Monitoring and coordinating inter-
nal body states so that internal or-gans function as a unit, internal bodyconstancy is maintained, and protec-tive action is taken. For example: inresponse to lack of oxygen, breathingbecomes more rapid; in response tocold, the body shivers; when threator danger is encountered, the heartbeats more rapidly.
Table 2–1 The Nervous System
I. Central nervous system
A. BrainB. Spinal cord
II. Peripheral nervous system
A. Afferent (sensory)B. Efferent (motor)
1. Somatic nervous system2. Autonomic nervous system
a. Sympathetic nervous systemb. Parasympathetic nervous system
The nervous system also controls cog-
nitive functions, such as learning andremembering, feeling emotion, reasoning,Conditions of the
Nervous System: Part I
Conditions of the BrainCHAPTER 2
25

generating and relaying thoughts, and dis-playing the general personality traits thatare characteristic of how each individualresponds to stimuli.
Nerve Cells
Specialized cells called neurons are the
functional units of the nervous system.Neurons transmit messages to and fromthe brain. They consist of a cell body andprocesses or nerve ﬁbers that extend be-
yond the cell body. In most cases a singlelong nerve ﬁber, called an axon, conducts
nerve impulses (and information) awayfrom the cell body to other neurons.Smaller, shorter nerve ﬁbers, called den-
drites , conduct nerve impulses toward the
cell body after receiving information fromother neurons. Fibers that carry informa-tion from parts of the body to the brainare called afferent neurons (sensory neu-
rons). Fibers that carry information fromthe brain to other parts of the body arecalled efferent neurons (motor neurons).
Surrounding neurons is a fatty sheath
called myelin , which, much like the cov-
ering of electrical cords, provides insula-tion, thus helping electrical impulses ﬂowsmoothly and reliably. Information ispassed from neuron to neuron by bothelectrical and chemical impulses. The elec-trical impulse, which has been picked upby the dendrites, is passed through the cellbody to the axon. The electrical impulsemoves down the full length of the axontill it reaches its tip. At the tip of the axonare tiny processes that release chemicals
known as neurotransmitters , which, through
chemical means, transfer the impulsefrom one neuron to another across the
space between the two neurons. This space
is called the synapse . The electrical im-
pulse, through the vehicle of neurotrans-mitters, then moves to the next neuron’sdendrites, and the process begins again(Figure 2–1). After neurotransmitters arereleased, they are either taken up again bythe neuron or destroyed.
Longer axons are generally grouped in
bundles. When transmitting impulseswithin the central nervous system, thesebundles are referred to as tracts . Those
bundles located outside of the centralnervous system are referred to as nerves .
The Central Nervous System
The central nervous system is made up of
the brain and spinal cord, which are bothprotected by bony coverings. On the inte-rior of the bony coverings are three mem-branes ( meninges ) that provide additional
protection:
• The dura mater is the outer mem-
brane, lying most closely to the bony
covering of the brain and spinal cord.
• The arachnoid membrane is the middle
membrane, a cobweb-like membrane.
• The pia mater is the inner membrane
that lies next to the brain and spinalcord.
Between each of the membrane layers
are spaces. The space between the duramater and the inner surface of the bonycovering is the epidural space . The space
between the dura mater and arachnoidmembrane is the subdural space , and the
space between the arachnoid membrane
and the pia mater is the subarachnoid space .
The central nervous system is also pro-
tected and cushioned by cerebrospinal
ﬂuid (CSF), which is formed by specialized
capillaries called the choroid plexus in inner
chambers within the brain called ventricles .
The CSF bathes the brain and spinal cord.It circulates from the ventricles into thesubarachnoid space; then it ﬂows to theback of the brain, down around the spinalcord, and then ﬂows back to the brain,where it is reabsorbed into the blood26 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Normal Structure and Function of the Nervous System 27
through the arachnoid membrane. Theamount of CSF produced and absorbed isequally balanced, so that under normalconditions it remains constant within thecentral nervous system.
Another protective device is the blood-
brain barrier , a structural arrangement of
capillaries that selectively determinewhich substances can move from theblood into the brain. Whereas substancessuch as oxygen and glucose are necessaryto brain survival and, consequently, movefreely across the blood-brain barrier, mostpotentially harmful substances, such astoxins, are prevented from crossing intothe brain.
The central nervous system is composed
of white matter and gray matter. The white
matter makes up the inner part of the
brain and the outer portion of the spinalcord and consists of myelin (covered)axons that conduct nerve impulses. It iscalled white matter because of its whitishappearance due to the myelin covering.Gray matter makes up the thin outer lay-
er of the brain and the inner portion ofthe spinal cord. Small segments of graymatter are also embedded deep within cer-
Dendrite
DendritesAxon
AxonSynaptic Knob
SynapticCleftDirection ofNerve Impulse
Figure 2–1 The Neuron. Source: Reprinted with permission from M. J. Miller, Pathophysiology:
Principles of Disease , p. 369. © 1983, W. B. Saunders Company.

tain parts of the white matter of the brain.Gray matter consists of groups of neuroncell bodies. It is called gray matter becauseof its grayish appearance. The gray mat-ter of the brain receives, sorts, and process-es nerve messages, and the gray matter ofthe spinal cord serves as a center for reﬂexaction (automatic response to stimuli).
The Brain
The brain, which is directly connected
to the spinal cord, serves as the primarycenter for the integration, coordination,initiation, and interpretation of mostnerve messages. The brain regulates andmonitors many unconscious body func-tions, such as heart rate and respiration,and also coordinates most voluntarymovements. In addition, it is the site ofconsciousness and intellectual function.
The brain is protected by the bony cov-
ering of the skull ( cranium or cranial bones ).
The largest part of the brain, the cerebrum ,
is covered with a thin outer layer of graymatter called the cortex , which contains
billions of nerve cells. The cortex has threespecialized areas that serve three majorfunctions:1. The motor cortex coordinates voluntary
movements of the body.
2. The sensory cortex is responsible for the
recognition or perception of sensorystimuli, such as touch, pain, smell,taste, vision, and hearing.
3. The associational cortex is involved in
cognitive functions, such as memory,reasoning, abstract thinking, and con-sciousness.
The cerebrum is divided into two
halves,
called hemispheres: the right hemi sphere
and the left hemisphere . The two hemi-
spheres communicate with each other.Dividing the hemispheres and connectingspeciﬁc areas within them are bundles ofnerve ﬁbers called the corpus callosum .
Each hemisphere has centers for receivinginformation and for initiating responses.The left hemisphere mostly receives infor-mation from and sends information to theright side of the body, and the right hemi-sphere mostly receives information fromand sends information to the left side ofthe body. Deep within the cerebral hemi-spheres are groups of gray matter calledbasal ganglia , which are part of the
extrapyramidal system . (Extrapyramidal
denotes nerve ﬁber tracts that lie outsidethe pyramidal tract, a relatively compactgroup of nerve ﬁbers that originate fromcells in the outer layer of the brain.)Extrapyramidal function is concernedwith postural adjustment and gross volun-tary and automatic muscular movements.Basal ganglia help to maintain contractiletone in muscles in the trunk and extrem-ities, enabling individuals to maintain bal-ance and posture and engage in move-ment such as walking. The basal gangliaalso play a role in enabling individuals toreact swiftly, appropriately, and automat-ically to stimuli that demand an immedi-ate response, such as, after tripping,enabling the individual to adjust move-ment in order to avoid a fall.
Each hemisphere of the cerebrum is
divided into lobes that contain areas relat-ed to speciﬁc functions. The frontal lobe is
located in the front of each hemisphereand contains motor areas that initiate vol-untary movement and skilled move-ments, such as those involved in writing.Other areas in the frontal lobe controlhigher intellectual functions such as fore-sight, analytical thinking, and judgment.The parietal lobe is located in the middle
of each hemisphere and is primarily thesensory area, integrating and interpretingsensation such as touch, pressure, pain,and temperature. Some memory functionsare also located in the parietal lobe, espe-28 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Normal Structure and Function of the Nervous System 29
cially those responsible for storage of sen-sory memory. The temporal lobe is located
under the frontal and parietal lobes andis primarily responsible for the interpreta-tion of and distinction between auditorystimuli. The occipital lobe is located at the
back or posterior portion of each hemi-sphere. It is the primary area for receptionand interpretation of visual stimuli.
Several parts of the cerebrum are in-
volved in language function, whichinvolves receiving, interpreting, and inte-grating visual and auditory stimuli as wellas expressing thoughts in a coordinatedway so that others may comprehend it.Language function is located in the lefthemisphere of the cerebrum in most indi-viduals, whether they are right- or left-handed. An area located over the temporaland parietal lobes, called Wernicke’s area ,
is the major area responsible for receptive
function , or the ability to integrate visual
and auditory information in order tounderstand a communication received. Anarea located in front of the temporal lobeand in the frontal cortex is called Broca’s
area, which contributes to expressive
function, or the ability to integrate andcoordinate words so that the meaning canbe comprehended (Figure 2–2).
A structure known as the thalamus lies
within the center of the brain. The thala-
mus acts as a relay station that sorts, inter –
prets, and directs sensory information.Below the thalamus is the hypothalamus ,
which coordinates neural and endocrineactivities. It helps regulate the body’sinternal environment and behaviors that
Brain
StemTemporal
LobeVentriclesBroca’s
AreaFrontal
LobeParietal LobeSubarachnoidSpace
Wernicke’sArea
ArachnoidMembrane
SubduralSpace
EpiduralSpace
DuraMater
OccipitalLobe
CerebellumPia Mater
Figure 2–2 The Brain.

are important to survival, such as eating,drinking, and reproduction. Below thehypothalamus is the pituitary gland , an
endocrine gland that will be
discussed in
more detail in a later chapter.
A group of structures consisting of both
gray and white matter surrounding the thal-
amus is called the limbic system . The limbic
system plays a role in the expression ofinstincts, drives, and emotions as well as
the formation of memories. A band of gray
matter called the hippocampus is involved
in learning and long-term memory, help-ing to determine where important and rel-evant aspects of facts will be stored.
Beneath the occipital lobe of the cere-
brum is a structure called the cerebellum .
The cerebellum is primarily responsible forthe coordination and integration of vol-untary movement and for maintenance ofequilibrium, posture, and balance. Thecerebellum also regulates and coordinatesﬁne movements of the extremities, whichhave been initiated by the frontal lobe.
The brain stem , located beneath the cere-
bellum at the base of the brain just abovethe spinal cord, acts as a relay station,transmitting nerve impulses between thespinal cord and the brain. The brain stemis the primary center of involuntary func-tions. Control of vital organ functionssuch as regulation of heartbeat or respira-tion occurs in the brain stem. Areas in thebrain stem also regulate the diameter ofblood vessels, consequently helping tocontrol blood pressure. Reﬂex actions,such as coughing and swallowing, are con-trolled in the brain stem. The brain stemalso contains scattered groups of cells,called the reticular formation , that are
involved in the initiation and mainte-nance of wakefulness and alertness.
The brain requires both oxygen and
nourishment in the form of glucose to
function and survive. Oxygen and glucoseare transported to the brain by blood car-ried by four major arteries, two carotid
arteries and two vertebral arteries . The ver-
tebral arteries join to form the basilar
artery . The carotid and basilar arteries then
connect at the base of the brain to formthe circle of Willis , from which cerebral
arteries branch out to carry blood to the
rest of the brain.
CONDITIONS AFFECTINGTHE BRAINTraumatic and AtraumaticBrain Damage
The brain, like any other tissue, needs
oxygen in order to function. Anythingthat interferes with the brain’s ability toget oxygen or causes damage to the braindirectly will impact its ability to functioneffectively. The manifestations of braindamage are dependent on:
• the cause of the damage • the area of the brain damaged• the extent of the damage
Generally, brain damage is classiﬁed as
one of two types:
1.
Atraumatic (nontraumatic) brain dam-
agecaused by interference with oxy-
gen reaching the brain (such as withchoking, carbon monoxide poison-ing, or infection) or problems with-in the brain itself (such as stroke, orstructural problems within the brainor blood vessels in the brain)
2. Traumatic brain injury (TBI), caused
by an outside force that impacts thehead hard enough to cause damageto the brain
Both atraumatic (nontraumatic) and trau-
matic brain damage are considered acquired
brain injuries because they occur after birth
and are not the result of genetic disorder,birth trauma, or degenerative disease.30 C HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 31
Atraumatic Brain Damage
Atraumatic brain damage, as just
explained , refers to conditions in which the
brain has sustained damage due to condi-tions other than traumatic injury.Speciﬁcally, atraumatic brain damage is
caused by conditions that cause restrictionor interference with blood and oxygenreaching parts of the brain. When a partof the brain receives no oxygen ( anoxia )
or too little oxygen ( hypoxia ), the tissue
can die. Examples of conditions that cancause atraumatic brain damage are stroke,congenital malformations (such as arteri-
ovenous malformations , in which blood ves-
sels are abnormal at birth), aneurysms (a
weakened area in an artery located in thebrain that then balloons out and can rup-ture), infections or inﬂammation of thebrain or surrounding membranes (such asmeningitis or encephalitis ), or other condi-
tions that deprive the brain of oxygen,such as strangulation, near drowning, orinhalation of noxious gases.
Stroke (Cerebral Vascular Accident)
Stroke, also known as cerebral vascular
accident , is a sudden alteration in brain
function resulting in weakness or paraly-sis in a body part as well as other neuro-logical deﬁcits due to decreased blood ﬂowto a part of the brain. Stroke is usually theculmination of progressive disease thathas occurred over the course of manyyears. Heart disease or ischemic vascular dis-
ease(arteriosclerosis), hypertension (high
blood pressure), and diabetes are oftenassociated with stroke. There are threemain causes of stroke:
1. The most common cause is blocking
of a cerebral artery by a clot ( throm-
bus) that has formed inside the
artery, a condition referred to as cere-bral thrombosis . Formation of the
thrombus blocks blood ﬂow to anarea of the brain. Because brain tissueneeds the oxygen contained in bloodto survive, tissue that cannot obtainneeded nourishment because of theblockage dies within a short period oftime. This tissue death is called aninfarct . The amount of damage de-
pends on how large an area of thebrain has been deprived of blood sup-ply from the clot.
2. Another cause of stroke is embolism .
In this case a clot forms in anotherpart of the body and then breaks off,traveling through the blood to thebrain and lodging in one of the cere-bral arteries. Again, when the clotoccludes blood ﬂow to a part of thebrain, surrounding brain tissue dies.
3. A third cause of stroke is hemorrhage ,
which occurs because of rupture of ablood vessel. A common cause ofcere
bral hemorrhage is hypertension
(high blood pressure). When blood
vessels are weakened because of dis-ease, such as with arteriosclerosis, orbecause of congenital weakness aswith an aneurysm, increased pressuremay cause the blood vessel to burst.Death of brain tissue occurs in thisinstance not only because a certainarea of the brain has been deprivedof oxygen, but also because theescaped blood compresses brain tis-sue against the skull, causing furtherdamage.
The amount and degree of function lost
as the result of stroke depends on:
• the side of the brain affected• the speciﬁc area of the brain that has
been damaged
• the amount of damage that has
occurred

Often after stroke, in addition to the ini-
tial damage to an area of the brain, sur-rounding brain tissue becomes edematous
(swells), causing additional deficits.Although death of brain tissue causes per-manent damage, areas of the brain thathave only experienced swelling mayrecover, and function in these areas maybe restored. Consequently, individualsexperiencing stroke may not know theextent of permanent functional limita-tions until months after the stroke asoccurred.
At times, temporary blocking of the
cerebral arteries causes slight, temporaryneurological deﬁcits. These “ministrokes”are referred to as transient ischemic at-
tacks (TIAs ). Although neurological deﬁ-
cits experienced from TIAs are usuallytemporary, their occurrence forewarns of
the possibility of a larger stroke unless treat-
ment controls the underlying condition.
Infections of the Central Nervous System
Any infection of the brain or the mem-
branes that surround the brain and spinalcord can cause serious neurological effects,some of which may be permanent.
Meningitis . Meningitis refers to an
inﬂammation of the meninges (mem-
branes surrounding the brain and spinalcord). It can be caused by bacteria, virus-es, or other organisms. There are manytypes of meningitis. The speciﬁc name giv-en to the meningitis infection is frequent-ly related to its cause or location. Forinstance, cerebral meningitis refers to
meningitis of the brain, whereas cere-
brospinal meningitis refers to meningitis of
both the brain and spinal cord. Menin-
gococcal meningitis (commonly known as
spinal meningitis ) is caused by a bacterium
that settles in the lining of the throat andis spread easily through respiratory secre-tions. The organism is relatively common.Normally the lining of the throat is sufﬁ-cient to act as a barrier to the bacteria;however, when the barrier is insufﬁcient,the infecting organism invades the blood-stream and reaches the meninges, causingthem to become inﬂamed. The organismalso gains access to the cerebrospinal ﬂu-id and begins to multiply.
The hallmark of meningitis is its rapid
onset. Individuals with meningitis are usu-ally acutely ill, initially with fever and ﬂu-like symptoms. Within a short period oftime they develop severe headache, neckrigidity, and discomfort when exposed tobright lights. If the cause is bacterial in ori-gin, prompt treatment with antibioticsreduces the chance of disease progression.The use of medication and prompt treat-ment has greatly reduced the number offatalities from meningitis; however, if itoccurs in individuals whose physical stateis weakened or if diagnosis and treatmentare delayed, it can still be fatal. Althoughmost individuals with meningitis recovercompletely, some may have residual neu-rological deﬁcits such as deafness, paraly-sis, or cognitive difﬁculties.
Encephalitis . Encephalitis is an inﬂam-
mation of the brain due to direct invasionof an organism. It may be caused by anendemic virus, such as the West Nile virus,a mosquito-borne virus (Huhn, Sejvar,Montgomer, & Dworkin, 2003; Marﬁn &Gubler, 2001), or it may be secondary toanother infection, such as measles orchickenpox. Some individuals with en-cephalitis may experience severe head-ache, stiff neck, and coma. There is noadequate treatment for encephalitis, ex-cept for maintaining comfort and prevent-ing complications. The symptoms cansubside in a few weeks, leaving no perma-nent damage; however, the condition canalso be life-threatening. Some individuals32 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 33
develop irreversible neurological changesas a result of encephalitis.
Although meningitis or encephalitis
and resulting deﬁcits can occur in any agegroup, children and older adults, or thosewith compromised immune systems, areoften the most susceptible to more severemanifestations of these diseases.
Traumatic Brain Injury
Traumatic brain injury is broadly deﬁned
as an injury to the brain from externalforces, such as vehicular accidents, falls,violence, or sports or recreational events(NIH Consensus Development Panel onRehabilitation of Persons with TraumaticBrain Injury, 1999). It is not degenerative,is not the result of a disease, and is notcongenital in origin. Damage to the brainoccurs from a blow to the head that ishard enough to cause the brain to movewithin the skull, or from an impact thatfractures the skull, injuring the braindirectly.
Almost two million people, of all ages,
sustain brain injury each year in theUnited States. TBI can cause myriad ef-fects, including physical, cognitive, emo-tional, and behavioral deﬁcits that impactevery aspect of an individual’s life. Thiswide range of deﬁcits presents uniquechallenges to rehabilitation of individualswith TBI.
Types of Traumatic Brain Injury
There are two types of traumatic brain
injuries:
•Open or penetrating head injury
•Closed head injury
Open (penetrating) injuries refer to injuries
in which the skull is fractured (such aswith a blow to the head in which the skullis broken) or penetrated (such as with agunshot wound). Functional impairmentsexperienced with open or penetratinginjury may be more localized and are usu-ally related to the speciﬁc area of the brainaffected. At times, functional impair-ments in open head injury may be moreextensive if additional damage is sus-tained. For example, in addition to thetrauma to the brain itself, bone fragmentsfrom the injury may also lacerate andinjure the brain, blood vessels, ormeninges (lining surrounding the brain).
In closed head injury (such as a blow to
the head or violent shaking of the head,as in shaken baby syndrome), the skull isnot fractured; rather, the brain is damagedbecause the head has been hit with sufﬁ-cient force that the brain slams against theother side of the skull or twists within theskull, causing shearing of blood vessels ornerve ﬁbers throughout the brain. This iscalled diffuse axonal injury . Injury is caused
to the brain both from the external forceas well as from movement of the brainwithin the skull. The initial impact to thebrain is called the coup, and the impact of
the brain on the opposite side of the skullis called the contre coup . Functional limi-
tations associated with closed head injurydepend on where and how much shear-ing occurred in the brain and may bemore diffuse because of the more exten-sive damage to the brain itself.
Additional injury may occur as an indi-
rect result of edema (swelling) of the
brain, hemorrhage, or the formation of ahematoma (sac ﬁlled with blood) within
the skull as a direct result of the injuryitself. Bleeding within the cranial vault isreferred to as intracranial hemorrhage . Be-
cause the brain is conﬁned within the
skull, there is no space available for expan –
sion if swelling or bleeding should occur.As a result, swelling or bleeding compres-ses the brain, increasing intracranial pres-sure and interfering with brain function.

Unless recognized and treated promptly,these events can cause additional perma-nent brain damage or death.
Bleeding and blood clots compress the
brain, increasing intracranial pressure. An
epidural hematoma is bleeding that occurs
in the space between the outer membraneof the brain (the dura mater) and the skull.Although bleeding generally occurs rapid-ly, it may not be recognized immediately.Individuals who have been injured maycarry on a lucid conversation, only to slip
into drowsiness and unconsciousness hours
later. Epidural hema tomas carry a high
mortality rate because they may not beimmediately recognized and consequent-ly not immediately treated.
A subdural hematoma is a hemorrhage
that occurs in the space beneath the duramater. Although symptoms may be appar-ent immediately, they may also appearmore gradually, becoming evident days oreven weeks after the injury. In bothinstances, immediate action is essential tostop the bleeding and to relieve theintracranial pressure before permanentdamage to the brain occurs.
Measuring the Severity of Traumatic
Brain Injury
A variety of instruments are available to
measure the severity of TBI . These instru-
ments are used to predict the condition ofthe individual after discharge and can beuseful measures for rehabilitation servicesafter hospitalization (Wagner, Hammond,Grigsby, & Norton, 2000).
Brain injuries are classiﬁed as:
• mild• moderate• severe
One basis of classiﬁcation is the length
of time the individual is unconscious afterinjury and the depth of unconsciousnessor coma. The length of unconsciousness
is also used as a predictor of prognostic out –
come. Generally, the longer the period ofunconsciousness, the more severe the in-jury to the brain and the greater subse-
quent residual effects. An instrument called
the Glasgow Coma Scale (Jennet, Snoek,
Bond, & Brooks, 1981) has become widely
accepted as a classiﬁcation system for rat-ing the seriousness of brain injury. Thescale is used to assess the level of con-sciousness on a continuum ranging fromalert to coma state. Scores are assigned ac-cording to the level of response in each ofthree areas: eye opening, motor response,and verbal response (Table 2–2). Scoresranging from 3 to 15 may be obtained.
The lower the score, the deeper the level of
unconsciousness. The Glasgow Coma Scale
provides a means whereby individuals’level of consciousness can be assessed sys-tematically. An initial assessment providesa baseline from which changes in neuro-
logical status can be measured. The scale isusually used in the early postinjury period
in the emergency and critical care units.
Another scale used to measure level of
brain injury is the Rancho Los Amigos Cog-
nitive Scale . The scale basically describes
levels of arousal and cognitive function-ing. It measures increasing levels of con-sciousness, so it often is used to give agross indication of stages of recovery afterbrain injury. The range of the scale is from1 to 8, with higher scores indicating high-er functional level. In the treatment andrehabilitation phase after injury, individ-uals may remain at one level of uncon-sciousness or coma for an extended periodof time or may move from one level ofconsciousness to the next. The RanchosLos Amigos Scale of Cognitive Function,as a measure of cognitive function, is usu-ally used to assess changes in the level ofconsciousness during the postinjury peri-od and as a broad indicator of the extent34 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 35
to which independent functioning is pos-sible. In this way a speciﬁc treatment canbe instituted to promote appropriatebehavior as the individual moves throughdifferent levels (Table 2–3).
Table 2–2 The Glasgow Coma Scale
Category Score
Eyes open
Never 1To pain 2To verbal stimuli 3Spontaneously 4
Best verbal response
None 1Incomprehensible sounds 2Inappropriate words 3Disoriented and converses 4Oriented and converses 5
Best motor response
None 1Extension (decerebrate rigidity) 2Flexion abnormal (decorticate rigidity) 3Flexion withdrawal 4Individual localizes pain 5Individual obeys 6
Source : White & Likavec (1992).
The Disability Rating Scale (Rappaport,
Hall, Hopkins, Belleza, & Cope, 1982) isalso used to estimate functional capacityafter brain injury. The scale evaluates indi-viduals on eight categories of disabilityand their ability to function. The highestpossible score is 30. The lower the individ-ual scores on the Disability Rating Scalethe better. Functional ability is scored onthe following areas:
• Level of arousal, awareness, and
responsiveness
• Cognitive skills needed for self-careTable 2–3 Rancho Los Amigos Scale of
Cognitive Functioning
Level I No response to sounds, light, or
touch.
Level II Generalized response to stimuli,
such as responding to a loud noisebut not turning toward the noise.Movement is not consistent and doesnot appear to have a purpose. Wheneyes are open, they do not appear tobe focusing on anything in particular.
Level III Localized response. The individual
begins to open eyes and look atspeciﬁc objects. The head turns inthe direction of sound. Simplecommands are followed, such as“squeeze my hand.”
Level IV Confusion and agitation. The indi-
vidual becomes very restless andagitated regardless of the circum-stances. Conversation may at timesappear to be coherent. The individ-ual may become verbally abusive.
Level V Confused, with conversation often
not making sense. The individualappears confused although may beable to follow simple instructions.The individual seems less agitatedbut may become frustrated.
Level VI Confused, but verbal responses are
appropriate. Some memory problemsregarding recent events may bepresent. Capable of most self-careactivities. Some judgment and prob-lem-solving difﬁculties, but the indi-vidual is often aware of this deﬁcit.
Level VII Automatic, appropriate response.
Oriented with little or no confusion.Lacks insight and problem-solvingskills.
Level VIII Purposeful and appropriate. Indepen-
dent. Can process new informationand problem-solve.

• Dependence on others• Psychosocial adaptability, including
ﬂexibility and ability to adapt to dif-ferent people and situations
Levels of Traumatic Brain Injury
Mild brain injuries constitute about 70
percent of all TBIs (Busch & Alpern, 1998)and are characterized by a traumaticallyinduced disruption of brain function inwhich there is at least one of the symp-toms as listed in Table 2–4. Individualswith mild brain injuries have a GlasgowRating of 10 or above and may have fewif any outer signs of brain injury or nodetectable anatomic damage to the brain.As a result, the brain injury itself may beundiagnosed and consequently untreated(Clements, 1997). Individuals with mildbrain injury may experience subtle butdisruptive symptoms that persist monthsor even years after the initial injury. Thisgroup of symptoms has come to beknown as postconcussion syndrome and can
consist of symptoms such as headache,vertigo (dizziness), tinnitus (ringing in
the ears), sleep disturbance, depression,irritability, reduced attention span, ormemory impairment. Because with mildbrain injury there often are few, if any,objective signs of brain damage, individ-uals experiencing these symptoms mayhave their credibility questioned, and theymay be labeled as malingerers (Koch,Merz, & Torkelson Lynch, 1995). Cog-nitive deﬁcits associated with mild braininjury may cause individuals considerabledistress and adversely affect both socialand occupational functioning.
Moderate brain injury is deﬁned by a
Glasgow Coma Scale score of 9 to 12. In-dividuals with moderate brain injury mayhave loss of consciousness for a few min-utes or several hours. There may be con-fusion or disorientation that lasts for a fewdays or several weeks. Physical, cognitive,or psychosocial deﬁcits may last for weeksto months or may be permanent.
Table 2–4 Manifestations of Mild Brain Injury
Individual experiences at least one ofthe following:
1. Brief loss of consciousness (30 minutes
or less)
2. Brief period of time after the injury
during which the individual feelsstunned and disoriented
3. Loss of memory for events occurring
immediately before or after the injury,lasting no longer than 24 hours
4. Temporary neurological deﬁcit 5. Initial Glasgow Coma Scale score of
13–15.
Source : Berrol (1992).
Severe brain injury is deﬁned as a Glasgow
Coma Scale score of 8 or less. Individualswith severe brain injury remain in a comafor an extended period of time, rangingfrom days to months. Coma is deﬁned as
prolonged unconsciousness in whichthere is little, if any, meaningful responsefrom the individual and he or she isunable to be awakened. Individuals aresaid to be in a vegetative state when they
react to painful stimuli and may opentheir eyes in response to stimulation buthave no meaningful response with theenvironment (Giacino & Zasler, 1995).The more severe the injury, the moresevere the permanent consequences ordeﬁcits experienced. Thus the potentialconsequences of brain injury vary tremen-dously, depending on the type of injuryand the area of brain damaged as well asfactors that existed before the event.36 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 37
Conditions Associated with Traumatic
Brain Injury
Posttraumatic epilepsy is experienced by
some individuals after TBI. In the earlypostinjury period, seizures may be relatedto increased intracranial pressure or oth-er direct results of injury. Seizures occur-ring later may be due to the formation ofscar tissue in the brain and may occur overa year after the initial injury.
Individuals with TBI may also develop
posttraumatic hydrocephalus , in which there
is interference with reabsorption of CSF.Posttraumatic hydrocephalus can cause in-
creasing neurological or functional deteri-
oration. It may be treated by surgicallyimplanting a shunt in the brain to divertand drain the CSF.
The prognosis for indi-
viduals who develop posttraumatic hydro-
cephalus is variable.
Right-Sided Versus Left-SidedBrain Damage
Although the manifestations of brain
damage vary, the outward signs andsymptoms of closed head injury or ofstroke are frequently related to which sideof the brain has been damaged.
Left-Sided Damage
The most visible sign of left-sided brain
damage, regardless of the underlyingcause, is right-sided motor and sensoryparalysis. For individuals who are right-handed, implications for everyday taskssuch as feeding themselves, dressing,writing, or a number of other activities aresigniﬁcantly affected. For most people,regardless of whether they are right-
or
left-handed, the language center, which
processes verbal symbols, is located in theleft side of the brain. Consequently, indi-viduals with left-sided damage will mostprobably have problems with verbaland/or written communication. (See apha-sia, described below.) Although they maybe able to understand more than they canspeak or write, often they also have difﬁ-culty understanding verbal and/or written
communication. However, even though in-
dividuals may have difﬁculty with speechand language, their ability to learn andcommunicate should not be underesti-mated. By the same token, individuals’ability to understand speech should not beoverestimated. In general, short, concisestatements
are more successfully commu-
nicated than long, complicated ones.
In general, besides having problems
with language, individuals with left-sidedbrain damage tend to be slow, hesitant,anxious, and disorganized when present-ed with a new or unfamiliar situation.Reassurance and frequent reinforcementfor tasks performed correctly help reduceanxiety and enhance the individuals’ability to perform.
Right-Sided Damage
The most visible sign of right-sided
brain damage is left-sided motor and sen-sory paralysis. Invariably, right-sided braindamage is also accompanied by some de-gree of damage to visual perception orvisual-motor integration. Spatial-perceptu-al deﬁcits can include loss of depth per-ception or lack of awareness of stimuli onthe left side of the body, causing difﬁcul-ty with navigation within the environ-ment. For instance, individuals may missthe table with a glass when putting itdown, or bump into a doorway whenattempting to go through it. Since thosewith right-sided brain damage have dif-ﬁculty processing visual cues, an unclut-tered and structured environment pre-vents distraction and enhances their abil-ity to perform. Ability to read may also be

compromised because of the inability tomove down the page without skippinglines.
Memory impairment may also be pres-
ent so that individuals are unable to rec-ognize familiar people or places. In otherinstances memory impairment is manifestas disorientation in familiar environ-ments, so that individuals may requirespeciﬁc instructions about how to getfrom place to place. In other instances,memory impairment results in individu-als misplacing personal items and thenconcluding that someone else must havetaken them.
Because language function is often not
affected, the abilities of individuals withright-sided brain damage may be overes-timated. Individuals themselves may bedisinhibited and unaware of deﬁcits andmay overestimate their own abilities toperform tasks, acting quickly and impul-sively. As a result of diminished self-aware-ness, they may tend to set unrealistic goalsand appear insensitive to the needs of oth-ers. In other instances, individuals mayhave difﬁculty decoding nonverbal cuesfrom others and as a result may be obliv-ious to others’ reactions or feelings.
Functional Consequences ofBrain Damage
Because the brain is responsible for so
many functions, damage to the brain,whether traumatic or atraumatic , can have
a profound impact on all areas of an indi-vidual’s life. Regardless of whether braindamage is caused from an accident, a blowto the head, stroke, infection of thebrain, exposure to toxins, or lack of oxy-gen, manifestations of brain damage mayaffect many functions. The effects experi-enced from brain damage depend onwhich part of the brain was damaged andthe extent of the damage incurred. In gen-eral, potential consequences of braindamage can be broken down into fourcategories (Groswasser & Stern, 1998):
1. Motor control and perception 2. Communication effects3. Cognitive changes4. Personality change and affective
response
Motor and Perceptual Consequences of
Brain Damage
The motor and perceptual consequences
of brain damage depend on whether the
damage was diffuse or local. Functional im –
pact can affect any of the following areas:
1. Movement, coordination, or balance2. Visual-spatial relations3. Perception 4. Vision and hearing 5. Touch, taste, and smell 6. Eating and swallowing7. Endurance 8. Bowel and bladder function
In addition, individuals with brain dam-
age may also experience seizure disordersand, in some instances, persistent pain.
Movement, Coordination, or Balance
Whether brain damage is atraumatic
(such as stroke) or traumatic (such as froma gunshot), damage conﬁned to onehemisphere of the brain will result insymptoms related to the extent of damageand which hemisphere was affected.Because one side of the brain controls theopposite side of the body, damage to onehemisphere of the brain affects functionof the body on the opposite side. Conse-quently, right cerebral damage can causeparalysis or weakness of the left side of thebody ( left hemiplegia ) that affects the left
arm and leg, whereas left cerebral damage38 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 39
can result in paralysis or weakness of theright side of the body ( right hemiplegia )
that affects the right arm and leg. Theresulting paralysis or weakness interfereswith the individual’s ability to walk so thathe or she may need assistive devices suchas a cane, walker, brace, or in some in-stances a wheelchair.
When individuals experience diffuse
axonal injury , such as in closed head injury,
changes of movement affecting bothsides of the body may be present. Indi-viduals may experience problems withmuscle coordination ( ataxia ) that affect
balance, causing them to walk with anunsteady gait or to lurch from side to sideas they walk. They may experience othermotor changes, including dyskinesia
(abnormal movements) or dystonia (ab-
normal muscle tone). Dystonia can
con-
sist of too little tone ( ﬂaccidity or hypo-
tonicity ), which decreases the ability to
move, or too much muscle tone ( spastic-
ityor hypertonicity ), which heightens
reﬂexes or abnormal movements.
Even when the motor function of mus-
cles remains intact and muscle strength,coordination, and sensation are normal,there may be reduced ability to organizeand sequence speciﬁc muscle movementsto perform a task ( apraxia ).
Individuals with apraxia are aware of
what they want to do and how to do it,but they are unable to organize musclemovements to perform the task. Con-sequently, a number of tasks, from dress-ing and eating to performing higher-levelactivities, may be affected.
Visual-Spatial Relations
Visual-spatial deﬁcits cause problems
with depth perception and judgment ofdistance, size, position, rate of movement,form, and the relation of parts to wholes.Visual-spatial changes as a result of braindamage interfere with the ability to inter-pret visual information accurately. Con-sequently, there may be difficultyorienting position and navigating move-ment within the environment, or individ-uals may demonstrate inappropriatejudgment of space or distance or the rela-tionship of the distance between twoobjects. As a result, they may appear care-less or clumsy, frequently bumping intofurniture, having difﬁculty navigatingdoorways, knocking items off tables orcounters, or missing the table whenattempting to put a glass down. Visual-spatial deﬁcits can affect other activities ofdaily living as well. For example, individ-uals may ﬁnd it difﬁcult to read becausethey continue to lose their place on thepage, or they may have difﬁculty dressingbecause they confuse the inside and out-side of clothes as well as left and right.Because of difﬁculty judging distances,individuals with even minor visual-spatialdeﬁcits may have difﬁculty driving a car.
Perception
Perceptual problems affect the ability to
understand or interpret stimuli or objectswithin the environment. Depending onwhat part of the brain is damaged, manydifferent perceptual problems may occur.Although some perceptual problems mayimprove over time, others will be perma-nent.
There may be loss of comprehension of
sensations ( agnosia ) in which individuals
lose the ability to recognize familiarthings such as words, faces, or objects.Some individuals, especially those withbrain damage localized to the right side,
may experience a condition called anosog –
nosia (one-sided or unilateral neglect) in
which body parts or objects on one sideof the body are ignored. For instance, anindividual with anosognosia may shave

only one side of his face, or only put onone shoe. In some instances, anosognosiais visual, so that there is an inability toperceive objects on either the right or leftof the central ﬁeld of vision. In theseinstances, individuals may bump intothings on the ignored or neglected side oftheir body. Sometimes, signals from allsenses on one side of the
body are
involved so that individuals may not rec-
ognize their own arm or leg or are unre-sponsive to verbal stimuli that originatefrom one side of the body. Nonrespon-siveness to verbal stimuli on the impairedside is different from merely losing hear-ing in one ear. All stimuli on the affectedside are ignored, while stimuli on the indi-vidual’s unaffected side evoke a response.
Vision and Hearing
Visual problems may be present even
though the eye itself is not injured.When the part of the brain that receives,perceives, or interprets nerve impulsesfrom the eye has been damaged, visualdeﬁcits may still be present. These caninclude total blindness, diplopia (double
vision), blurred vision, visual ﬁeld losssuch as cuts in the peripheral ﬁeld ofvision ( blind spots ), hemianopia (loss of
vision in half the visual ﬁeld), or colorblindness.
As with vision, even though the ear has
not been damaged directly, hearing deﬁ-cits may be present if the area of the brainresponsible for receiving, perceiving, or in-terpreting sound has been damaged ( sen-
sorineural hearing loss ). Individuals may
experience ringing in the ears ( tinnitus )
as well as partial or total loss of hearing.
Touch, Taste, and Smell
Brain damage that involves the parts of
the brain responsible for sensation canlead to a variety of abnormalities, such asdecreased feeling or absence of feeling invarious body parts. These changes mayresult in numbness ( anesthesia ), the
inability to feel pain ( analgesia) , or the
inability to sense movement of body parts.Individuals may also experience abnormalsensations ( paresthesia ) such as pain, tin-
gling, or burning in various locations intheir body.
If the olfactory nerve or corresponding
area of the brain has been damaged, theremay be no sense of smell ( anosmia ).
Although loss of sense of smell may notappear to be a deﬁcit that signiﬁcantlyaffects the ability to function, it can affectthe ability to detect hazards such assmoke, gas leaks, or other importantwarning signs. Lack of sense of smell alsoaffects the ability to taste. Inability to tastemay affect the individual’s will to eat andconsequently affect his or her nutritionalstatus as well as the ability to detect foodthat is spoiled.
Eating and Swallowing
Swallowing reﬂexes may be affected so
that individuals have difﬁculty swallowing(dysphagia ) and in some instances difﬁ-
culty with chewing. The gag reﬂex mayalso be impaired so that there is increasedsusceptibility to choking. Because of dif-ﬁculty with swallowing or performingchewing movements, food may be pock-eted in one side of the mouth, increasingthe risk of gagging or choking. This canbe dangerous because of the risk of aspi-
ration (food or liquid entering the lungs
rather than the stomach). When individ-uals are unable to swallow food because ofswallowing difﬁculty, a special diet con-sisting of pureed
food may be needed or
tube feedings may be necessary to prevent
aspiration into the lungs. In addition,because of difﬁculty or inability to swal-40 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 41
low, saliva may build up in the mouth,causing the individual to drool.
Endurance
After brain damage individuals may
experience extreme mental and physicalfatigue when completing both physicaland mental tasks, especially when tasksare unfamiliar or require signiﬁcant con-centration. Physical and mental activitiesthat, prior to injury, were easy for the indi-vidual to complete may also be exhaust-ing to complete after the injury. Sleeppatterns may be altered so that quality ofsleep is affected, compounding the prob-lem. Tasks may be performed better earli-er in the day, since performance levels candeteriorate later in the day due to fatigue.
Bowel and Bladder Function
In some instances control of bladder or
bowel function may be lost ( inconti-
nence ) after brain damage. At times prob-
lems are caused by the individual’sinability to recognize the need to urinateor defecate. In other instances, individu-
als are unable to urinate at will or to com –
pletely empty the bladder when uri nat-
ing. There may be need for bladder and/or bowel retraining, or the need for indi-viduals to wear or utilize a catheter (tube
inserted into the bladder to drain urine).
Posttraumatic Seizures
Seizures may be experienced in the peri-
od immediately after the brain damage.They can be mild or severe, temporary orpermanent. In some instances, seizuresoccurring in the immediate postdamagephase resolve after swelling of
the brain
recedes. In many other cases, how ever, indi-
viduals continue to have seizures , a condi-
tion called posttraumatic epilepsy . Communication Consequences
Brain damage can affect all forms of
communication, including the ability tospeak, comprehend, or convey languagethrough either written or verbal means.Speech refers to the physical ability to
produce sounds and/or movement of thelips, tongue, or other structures that areused to produce language. Language re-
fers to how words, as symbols, are put to-gether to convey and understand con-cepts. The ability to use and understand
words (language) is controlled in the brain.
In addition, the ability to use certain mus-cles that enable individuals to form wordsand project speech is controlled withinthe brain. When the area of the brain thatcontrols either speech or language isdamaged, limitations may occur.
Motor difﬁculty in structures related to
speech may affect the individual’s abilityto speak. Coordination and accuracy ofmovement of the muscles, lips, tongue,or other parts of the speech mechanismmay be impaired secondary to weaknessor paralysis of the muscles needed tospeak. This condition is called dysarthria .
Impairment may range from speech thatis slightly slurred to speech that is un-intelligible. Paralysis or weakness of mus-cles may also cause vocal cord dys-function, which in turn can affect voicequality.
Other motor problems can cause artic-
ulation disorders in which there is no sig-
niﬁcant weakness or lack of coordinationfor reﬂexive action but rather the inabil-ity to position and sequence musclemovements. For example, individualsmay be able to scrape a food particle offtheir teeth with their tongue, but theymay be unable to coordinate the musclesthat move the tongue to produce a pho-netic sound. This condition is known asapraxia of speech .

Another communication consequence
of brain damage may be the inability tocomprehend or use language ( aphasia ).
Aphasia can affect either verbal or writtencommunication. It results from dysfunc-tion of the language centers in the brain,rather than impairment in the muscu-lature involved in producing speech.Although there are a number of types ofaphasia, two common categories are:
•nonfluent (expressive ormotor )
aphasia
•ﬂuent (receptive or sensory ) aphasia
Broca’s aphasia is a type of nonﬂuent
aphasia characterized by misarticulation,
laborious speech, hesitancy, and reducedvocabulary and grammar. Individuals maybe able to understand and read simplematerial; however, as the complexity orlength of the message increases, difﬁcul-ty becomes more apparent. Althoughthey are able to comprehend, they mayhave difﬁculty expressing thoughts inspeech and writing because of difﬁcultyputting words and sentences together log-ically. Word-ﬁnding difﬁculties ( dysno-
mia) are also common. Reading ability
may be better than writing ability. Speechmay be labored, slow, and/or difﬁcult tounderstand, and small connecting words,such as prepositions, may be omitted.
Wernicke’s aphasia is a type of ﬂuent
aphasia in which there is effortless speech,
relatively normal grammatical structure,and increased verbal output, but withreduced information content, so thatwhat the individual says makes littlesense. Auditory and reading compre-hension is usually poor. Individuals withWernicke’s aphasia are typically unawareof their communication difﬁculties.
In some instances individuals may ex-
perience global aphasia , in which there
is severe difﬁculty communicating becauseof both the inability to use language (touse words and organize them into coher-ent sentences) and severe difﬁculty under-standing language, either written orspoken.
Language impairment may differ
depending on the area of the brain dam-aged. Because the center of languagefunction is located in the left cerebralhemisphere for most individuals, commu-nication deﬁcits can occur when damageinvolves the left side of the brain.Individuals with right cerebral damageoften have language function left intact.
Cognitive Consequences
Brain damage can alter a variety of cog-
nitive skills:
• Memory• Attention and concentration• Self-awareness• Problem solving and decision making• Information processing and concept
formation
• Judgment
Memory
Memory encompasses the ability to
store and retrieve information. Memoryproblems affect the individual’s ability torecognize and recall people, places, facts,and concepts as well as to problem-solve,form goals, organize, and plan. Memoryfor both new and old information may beaffected.
Several types of memory exist:
1. Immediate memory lasts only sec-
onds or minutes unless converted in-to short-term memory. An exampleof immediate memory is remember-ing a phone number long enough todial the number, but then not com-mitting it to memory for later use.42 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 43
2. Short-term memory lasts from min-
utes to hours, but it is then lost if notconverted to long-term memory. Anexample of short-term memory maybe learning facts for a test but notcommitting the facts to long-termmemory for continued use.
3. Long-term memory is memories that
are stored and are able to be retrievedin the future, whether after weeks oryears.
A variety of memory problems may be
experienced after brain damage. Someindividuals may be able to remember factsbut are unable to remember how to dospeciﬁc tasks. For instance, an individualmay be able to remember the names andbirthdates of family members but be un-able to remember how to operate a wash-ing machine. Some individuals experienceretrograde amnesia in which they are
unable to remember things that occurredprior to the time of brain damage.Individuals with remote memory impair-
ments may have forgotten their own per-
sonal history so they do not recognizefamily members, or they may not be ableto remember what type of work they hadbeen engaged in prior to brain damage.
After brain damage individuals can
have difﬁculty remembering or learningnew information so that they are unableto acquire new memories or recall recentconversations and events. In some in-stances individuals make up answers toquestions, or make up situations or events(confabulation ). This results not from
faulty memory but from the tendency tojuxtapose unrelated memories together. Atother times, in conversation, individualsmay get stuck on one theme, repeating aquestion, phrase, or concept again andagain ( perseveration ). Perseveration can
also pertain to tasks that the individualrepeats over and over, such as continuingto wipe the same spot on a counter untilsomeone intervenes.
Individuals with brain damage may be
unable to remember skills that were oncevery familiar. For instance, they may beunable to complete simple daily tasks,such as dressing, because they are unableto remember the steps involved orbecause, after completing the ﬁrst steps ofthe task, they forget their original goal.
Memory problems can be the most lim-
iting of all of the potential cognitive con-sequences of brain damage because theyaffect the individual’s ability to learn,store, and retrieve information. The abil-ity to proﬁt from experience is oftenlimited as well. Consequently, individualsmay continue to make the same mistakesover and over, since the ability to applywhat was learned from past experience isusually diminished. The ability to gener-alize from one situation to another mayalso be impaired. Therefore, what islearned in one setting may not be able tobe transferred to another. For example, anindividual who has learned a skill in arehabilitation setting may be unableto perform that skill in his or her ownhome.
Attention and Concentration
After brain damage individuals may ﬁnd
it difﬁcult to focus attention and to con-centrate on a speciﬁc activity. Conse-quently, they may be unable to follow atrain of thought or perform multiple stepinstructions. They may have difﬁcultyfocusing on one task, may be easily dis-tracted, or may be unable to “shift gears”from one task to another. Individuals withbrain damage may ﬁnd it difﬁcult to per-form multiple tasks at one time, such aswriting down messages or notes whiletalking on the phone, or carrying on aconversation while polishing furniture.

Self-Awareness
Individuals with brain damage may
have limited ability to recognize or under-stand the limitations they are experienc-ing. They may lack insight into theappropriateness of their behavior and maybe unaware of the impact certain aspectsof their behavior have on people, remain-ing oblivious to subtle reactions or emo-tional cues from others. Because they maybe unaware of their deﬁcits, they may beunable to assess the extent of their disabil-ity and may therefore set unrealisticgoals. There may also be an inability tomonitor and adjust their own actionsaccording to feedback from others. Whenthey do receive feedback, they may dis-count it because they disagree with oth-ers’ observations regarding their behavioror performance.
Problem Solving and Decision Making
Planning and organizing and therefore
problem solving may be difﬁcult afterbrain damage. Sequencing tasks may beproblematic. For example, when preparinga meal, individuals may not recognize thatfood items that take more time to cookshould be prepared ﬁrst. Consequently,they may fully prepare the mashed pota-toes before even starting to make themeatloaf. In other instances individualsmay have difﬁculty following steps inorder. For instance, when dressing theymay put on their slacks before they put onunderwear or put their socks on over theirshoes.
There may be the inability to recognize
problems as they occur, and if a problemis identiﬁed, the inability to generate alter-native solutions or to select a solutionwhen one is presented. Individuals mayconsider only immediate informationrather than looking at the situation as awhole. For example, if they want to visita friend in another city, they may recog-nize that they can take a train to get there,but they may not be able to consider howthey would obtain money for the trainfare, how they would obtain a ticket, orhow they would get to the train station.Because individuals with brain damagesometimes have difﬁculty thinking orplanning for the future, reasoning anddecision making may be more difﬁcult.For instance, they may see no need to goto the grocery store for supplies if they arenot currently hungry.
There may also be lack of ability to ini-
tiate and sustain activity. Performance canbecome inconsistent, so that tasks per-formed well on one day may not beperformed well on subsequent days. Indi-viduals with damage to the left side of thebrain may ﬁnd problem solving especial-ly difﬁcult. When presented with a newproblem, they may respond slowly and ina cautious, disorganized fashion. In mostinstances, it is helpful to divide tasks intosmaller steps to avoid confusion. Indi-viduals may need frequent feedbackthroughout even simple tasks such asdressing to be assured that the task isbeing performed correctly.
Information Processing
Even when hearing and vision are un-
impaired, more time may be needed tosynthesize verbal or visual input. Theremay be delayed response to visual and/orverbal stimuli, so that individuals mayﬁnd it difﬁcult to maintain pace in a socialsetting. In some instances comprehensionof input itself may be severely disrupted.Information-processing information maybe disrupted not only in terms of speed,but also in the ability to sequence and cat-egorize information, so that there is difﬁ-culty understanding concepts. As a result,44 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 45
abstraction may be difﬁcult, and individ-uals may tend to think only in concreteterms and cues and stimuli may be takenliterally. For instance, in money exchange,the phrase “Do you have anything small-er?” may be taken quite literally by indi-viduals with brain damage because they
are unable to distinguish between “smaller”
as referring to denomination and “smal-ler” as referring to size.
Judgment
Judgment may be impaired because of
loss of ability to learn from experience,problem-solve, self-monitor, or interpretcues from other individuals or the envi-ronment. Individuals may demonstrate arigidity of response that precludes alterna-tive responses. For instance, because of theinability to recognize the need to adjustplans or to plan alternative activities, anindividual who had planned to go swim-ming may proceed with his or her plans,even though the weather has become coldand rainy. Lack of judgment can also causeindividuals to endanger themselves orothers when they attempt to perform tasksor engage in situations for which theyhave limited skills. They may behaveimpulsively and act quickly withoutthinking or without anticipating the con-sequences of their behavior.
Personality Change and Affective Response
The psychosocial effects of brain dam-
age pose not only serious limitations forindividuals but can also be the most dif-ﬁcult challenge for family and friends toface. Potential psychosocial effects canconsist of the following:
• Personality changes• Anger or irritability• Nonconformance to social norms• Apathy and depression• Loss of self-esteem
Personality Changes
Personality changes associated with
brain damage may be slight or extreme,but they can be severely disabling. Indi-viduals who were very meticulous andprecise prior to brain damage may becomecareless and sloppy post damage. Indi-viduals who were once jovial and out-going may, after brain damage, becomequiet and withdrawn. Such behavior mayat ﬁrst be misinterpreted by others as lazi-ness, disinterest, or uncooperativenessrather than as a symptom of the conditionitself. In other instances individuals who,prior to damage, had been calm and tol-erant may, post damage, be emotionallyexplosive, demonstrating outbursts ofanger or episodes of severe anxiety. Socialinteractions are often affected by thesepersonality changes, so that individualsare unable to main
tain relationships. Once
personality changes are recognized as symp-
toms associated with brain damage, com-pensa
tory behaviors can be learned to
overcome them.
Anger or Irritability
Aggressive behavior displayed after
brain damage may be the result of frustra-tion, but it can also be a direct physiolog-ic consequence of damage to the brainitself. Aggression can be expressed active-ly or passively, verbally or physically.There may be decreased patience or over-reaction to stresses in the environment, orindividuals may be more sensitive to envi-ronmental stimuli and may become dis-tracted or react to stimuli with irritation.Because individuals with brain damage
may have low frustration tolerance, aggres –
sive behaviors and emotional outbursts

can be common. Individuals may havesudden mood swings, turning from hap-py to sad or complacent to volatile withlittle or no provocation.
Nonconformance to Social Norms
Disinhibition can also be a conse-
quence of brain damage, so that there areinadequate social skills to function effec-tively within the environment. As a result,individuals may make rude or embar-rassing remarks to others, exhibit inappro-priate sexual behavior in public, or makeinappropriate sexual remarks. They maymisinterpret gestures of others, such as ahug, as an indication that the individualdesires a more passionate encounter. Insome instances individuals with brain
damage may have heightened sexual drive
and become overdemanding sexually.
Substance abuse is frequently a contrib-
utor to accidents that result in TBI. If sub-stance abuse or dependence was a prob-lem prior to injury, it may also contributeto problems post injury. Because of thestress of adjusting to changes associatedwith brain damage, lack of self-awarenessand insight, and inability to recognizecues from the environment, substanceabuse may become problematic post in-jury. Since individuals with brain damageare more sensitive to the effects of alcoholor drugs, use often further impairs cogni-tive, psychomotor, and psychosocial skills,making it more difﬁcult for them to inte-grate into the community or into theworkplace. In addition, drugs and alcoholmay interact with other prescribed med-ications, causing serious effects. Individ-uals with brain damage are also moreprone to seizures. Alcohol and drugs canlower the seizure threshold, increasing therisk of seizures for these individuals. Asindividuals with brain damage achievegreater levels of independence, the likeli-hood of substance abuse increases. Assess-ment of alcohol and/or drug use shouldbe ongoing throughout the rehabilitationprocess.
Apathy and Depression
Depression is a natural reaction to the
loss experienced with many disabilities,whether loss is related to cognitive, motor,sensory, social, or vocational functions. Attimes it may be difﬁcult to discern theextent to which depression is the directconsequence of physiologic damage to thebrain or a personal reaction to losses asso-ciated with the disability. As individualsbecome increasingly aware of losses,restrictions, and alterations in lifestyle,they may go through a grieving processthat leads to depression. As a conse-quence, they may become increasinglywithdrawn and have difﬁculty taking ini-tiative to interact socially with others.
Loss of Self-Esteem
After brain damage some individuals
have no memory of what they were likeprior to injury. Others may develop anincreasing awareness of their disability oran awareness that they are unable to per-form the tasks they performed previous-ly. They may recognize the role changesthey are experiencing and may sense thattheir status has changed within the fam-ily, social, and work setting. This loss ofstatus may diminish their self-image, sothat they become preoccupied with feel-ings of worthlessness and grief.
Treatment and Management ofBrain Damage
Comprehensive, individualized interdis-
ciplinary treatment and rehabilitationprovided by a diverse team of profession-46 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 47
als are necessary to achieve both short-term goals and global outcomes. Inter-ventions are directed at preventative,restorative, and compensatory strategies.The course of recovery and rehabilitationof individuals with brain damage is vari-able, but it is almost always lengthy, last-ing from months to years. The rate ofrecovery may vary over time. Physiciansinvolved in the care of individuals withbrain damage usually include a primaryphysician such as an internist or family
physician , as well as specialists such as a
neurologist , neurosurgeon , and physiatrist .
Other health professionals involved in theindividual’s care, treatment, and/or reha-bilitation may include nurses , respiratory
therapists , physical therapists , dietitians or
nutrition specialists , speech/language pathol-
ogists , audiologists , pharmacists , occupa-
tional therapists , recreational therapists ,
clinical or counseling psychol
ogists , neuro-
psychologists , cognitive retrainers ,social work-
ers, and rehabilitation counselors .
Initial Treatment
The initial treatment for individuals
experiencing brain damage, whether fromtraumatic or atraumatic causes, is intend-ed to stabilize the condition and enhancethe recovery process by preventing com-plications from occurring. Damage to thebrain can cause increased muscle tone,paralysis, or weakness. If these changes are
left untreated, permanent deformities such
as contractures (deformity and immobil-
ity of a joint due to permanent contrac-tion of a muscle) can occur, which couldinterfere with the individual’s future func-tion. Because individuals are kept immo-bile in the initial stages after braindamage, they are also susceptible to oth-er complications, such as pneumonia,pressure sores, urinary tract infection, andblood clots. Should these complicationsarise, potential for recovery could be com-promised. Consequently, in the initialstages of treatment special attention is giv-en to maintaining nutritional status and
preventing complications from occurring.
Along with traumatic brain damage,
there is often damage to at least one oth-er major organ system. These injuries mayinclude spinal cord injury, musculoskele-tal injury, or injury to internal organs.Treatment of any complicating associatedinjury is necessary to prevent deteriora-tion, which could jeopardize recovery,rehabilitation, and in some instances sur-vival. In the case of atraumatic damage,direct treatment of any underlying condi-tions (such as hypertension, infection inthe case of meningitis, or diabetes in thecase of stroke) is also important to stabi-lize the condition and prevent furtherdamage from occurring.
Neurosurgical procedures are sometimes
indicated in the immediate treatmentphase of brain damage. Careful observa-tion is essential to detect early signs ofincreased intracranial pressure due toswelling of the brain or intracranial bleed-ing, which, unless relieved, could causeadditional damage or death. Treatment ofincreased intracranial pressure can be sur-gical or nonsurgical. Surgical interventionmay involve placing a shunt that allowsexcess CSF to drain into the general bodycirculation. If individuals with traumaticbrain damage have an open skull fracture,surgery may be necessary to remove frag-ments of bone or other foreign materialsand to repair the skull. If increased intra-cranial pressure is caused by a blood clot(e.g., a subdural or epidural hematoma) orhemorrhage, two small holes may beplaced into the skull ( burr holes ) and the
blood clot removed or the bleeding con-trolled. In some instances individuals mayundergo a craniotomy , a surgical proce-
dure in which the skull is surgically

opened and the clot or foreign objectremoved or bleeding controlled throughthe surgical incision. Nonsurgical inter-ventions for increased intracranial pres-sure consist of giving medications toremove ﬂuid and to decrease swelling ofthe brain or prevent further clot forma-tion. If individuals have an aneurysm ormalformed arteries or veins, surgery toremove the aneurysm or correct the mal-formation may be performed.
Postacute Treatment and Rehabilitation
After the condition has stabilized,
appropriate postacute treatment requiresearly and active intervention by the inter-disciplinary team. In the early treatmentphases after brain damage, physical thera-
pymay focus on activities to prevent joint
and muscular complications. Physical ther-
apists work with individuals early after the
initial phase of brain damage to providerange-of-motion exercise to extremities,thus preventing deformity, as well as lat-er in the recovery period to assist withambulation. Later, physical therapy maybe directed toward helping individualsimprove balance, muscle control, andambulation as well as other physicalmovements. Individuals who experiencehemiplegia (paralysis on one side of the
body) may need special instruction inambulation techniques ( gait training ).
Depending on the extent of permanentdamage to the brain, individuals may useassistive devices to perform a variety offunctions and activities. Braces or splintsmay be necessary to help them increasefunctional capacity and become inde-pendent. Individuals with paralysis of anarm may be taught to use special toolssuch as a plate guard to keep food fromsliding off the plate, or special eating uten-sils or other tools designed to help in dai-ly living activities. If there is paralysis ofan upper extremity, the weight of the par-alyzed arm can cause separation of thearm from the shoulder joint ( subluxa-
tion). To prevent this from occurring,
individuals with this condition may weara sling to support the arm.
Individuals with brain damage may
need assistance to increase their awarenessor orientation to time, place, and persons.Occupational therapy can help individuals
with brain damage integrate available sen-sory information so that they can use itas a basis for motor activity and increasetheir ability to perform the activities ofdaily living. For example, helping themlearn skills and use assistive devices forsuch daily activities as maintaining per-sonal hygiene, dressing, and eating maybe a focus of therapy.
Speech and language therapies may focus
on the mechanical difﬁculties of speech,the formation and execution of language,or the development of alternative commu-nication systems. Speech and language
therapists may help individuals with both
verbal and nonverbal communication.They may focus on speech or languageacquisition or on conversational skillstraining. The speech therapist can also help
individuals with brain damage developsocial skills that relate to communication,such as techniques to structure the en-vironment so that communication effec-tiveness is maximized. In some instances,alternative methods of communication,such as writing or using a picture board,may be used. If individuals have impairedswallowing capabilities, speech pathologists
may also be involved in helping themlearn how to swallow again. In someinstances, speech pathologists may also beinvolved in cognitive remediation.
Clinical or counseling psychologists may
conduct psychotherapy or counselingwith the individual with brain damageand/or family members in order to facili-48 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 49
tate the adjustment process. Neuropsycho-
logists may be involved in neurological
assessment. Some neuropsychologists mayalso be involved in cognitive retraining orremediation, helping individuals withbrain damage learn ways to compensatefor areas of lost cognitive function.
Often cognitive changes, rather than
physical changes, hamper effective dailyfunctioning. In these instances, cognitive
remediation strategies designed to amelio-
rate sensory/perceptual, language-related,and problem-solving deﬁcits may be amajor focus of the rehabilitation effort.The goal of therapy is to return individu-als with brain injury to as much independ-
ent functioning as possible in as many areas
as possible. Cognitive strengths and weak-nesses are identiﬁed through observationand neuropsychological assessment . How
cognitive abilities and limitations in areassuch as memory, organizational ability,reasoning, or judgment affect individuals’ability to function in the environment isevaluated, and cognitive strategies aredevised to help them compensate for theircondition or improve it. Individuals arethen helped to transfer these strategiesfrom the clinical setting to their own envi-ronment. In some instances, dependingon the individuals’ life circumstances
and the extent of the brain damage, long-
term supportive care may be needed.
Most individuals who have experienced
brain damage should abstain from alcoholor drugs that have not been medically pre-scribed. The use of alcohol and other sub-stances can increase the potential forseizures after brain damage. In addition,the combination of alcohol or drugswhen taken with prescribed medicationscan have dangerous effects. Furthermore,alcohol and other substances may accen-tuate any existing residual from braindamage, increasing the chances of addi-tional accident or injury as well as pre-venting individuals from functioning totheir maximal capacity.
Several approaches are utilized for indi-
viduals with brain damage after they aremedically stable, including home-based pro-
grams , outpatient rehabilitation programs ,
community reentry programs , day treatment ,
residential community reentry or transition-al living programs , or neurobehavioral pro-
grams . These programs offer individuals
therapies designed to improve functioningor to help them develop social skills, orthey may provide care and supervision forindividuals who require some assistance inmeeting basic needs, as in a supported liv-
ing program or independent living center .
Functional Implications ofBrain Damage
Psychological Issues in Brain Damage
The emotional reactions experienced by
individuals after brain damage can range
from depression to mood swings or psycho-
sis (Busch & Alpern, 1998). Although theextent of personality change or other psy-chological symptoms varies from individ-ual to individual, it is safe to assume thatwhether damage is mild or severe, somepsychological symptoms will be exper-ienced. Symptoms may differ at different
phases of recovery. Those in the early stages
of recovery may deny the extent of theirlimitations. Later they may experiencefeelings of frustration because of difﬁcul-ty with memory or because they are un-able to perform tasks they were once ableto perform. Later feelings of anger, grief,
anxiety, or helplessness may occur, or there
may be feelings of worthlessness or guilt.
Because of the damage sustained, some
individuals are no longer able to compre-hend the world around them or respondto it in the same way they did before. Theymay show loss of emotional control in the

form of emotional lability, suddenlyswitching from laughing to crying or cry-ing to laughing when there is no appar-ent cause. At times emotional lability isexpressed as prolonged crying that, ratherthan being caused by depression or sad-ness, is instead a direct result of damageto the brain. If emotional lability exists,the family may need support and guid-ance in dealing with the individual’s out-bursts. The emotional reaction can oftenbe diverted if the individual’s attentioncan be directed to another activity.
Individuals may demonstrate impulsiv-
ity with regard to money, sex, drugs, orinteractions with others. In some in-stances there may be outbursts of verbalor physical aggression. They may lose sen-sitivity to the impact of their behavior onothers. In instances in which individualsare aware of their behavior, they maybecome self-conscious and anxious,
avoid-
ing contact with others, or they may
become overcautious and hypervigilant.
Personality traits that were present pri-
or to brain damage may become exagger-ated after the damage has occurred, orthere may be dramatic personality change,so that an individual who was quiet andpassive prior to brain damage may becomeboisterous and aggressive after his or herinjury. A person who was once self-directed and took initiative may becomeapathetic and unable to complete tasksindependently.
Counseling and/or psychotherapy is an
important part of total rehabilitation inmost disabling conditions, and it can beused to treat depression, reduce denial,increase self-esteem, or help individualsform realistic goals. However, the workmay be challenging if the individual haslost the capacity for insight or is unableto participate in abstract reasoning. Coun-seling may be directed toward providingemotional support for both individualsand their family and toward helping allinvolved adjust and relate to each otherin the context of the changes broughtabout by brain damage.
In the case of TBI, substance abuse is
often a contributor to the original acci-dent that caused the injury. After the in-jury has occurred, individuals maycontinue in the same pattern of substanceabuse behavior they were involved withprior to the injury. Consequently, sub-stance abuse evaluation should be con-ducted routinely and treatment institutedas needed. Substance abuse may also be amaladaptive means of coping with thestress and depression individuals experi-ence following brain damage. In eithercase, the effects of alcohol or other drugsfurther impair individuals’ functioningability and also have the potential to inter-act with other medications an individualmay be taking. In addition, substanceabuse may precipitate a seizure, which theindividual is already more prone to expe-rience. Abstinence is, therefore, the bestpolicy for individuals with TBI. Some indi-viduals may believe that substance use hasbeen a signiﬁcant part of their social rela-tionships. In these instances, they mayneed to learn other circumstances underwhich to engage in social activity andshould be encouraged to participate insocial and recreational activities that donot involve alcohol or other drugs.
Role changes as a result of injury im-
pact not only the individual but also on
his or her family. Often because of changes
in temperament, behavior, and personal-ity, there is a disruption in family cohe-sion and feelings of entrapment by familymembers. Depending on the extent of rolechange, individuals may feel diminishedsocial status, social isolation, and conse-quently loss of self-esteem. Often thephysical ramiﬁcations and residuals of theinjury are less troublesome for family and50 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 51
friends than the associated behavioral andpersonality changes caused by the braindamage. Personality changes may put astrain on family relationships.
Social Issues in Brain Damage
After brain injury, social relationships
are often drastically altered. No relation-ship is more signiﬁcantly altered than thatof the family. Brain damage dramaticallyand permanently impacts not only theindividual experiencing it but also thewhole family system. Severe brain damageproduces prolonged stress in the family.Not only must the family cope with pro-found physical, cognitive, and emotionalchanges in the individual, but the stressof caregiving and the ﬁnancial burden can
be extreme. Because brain damage, whether
traumatic or atraumatic, occurs suddenly,neither the family nor the individual hasthe opportunity to prepare for the emo-tional and economic impact. Normalfamily development is disrupted and anyprior family stress exacerbated.
The family also has signiﬁcant inﬂuence
on how the individual reacts to the dam-age and its residual effects. Depending onthe circumstances of the injury, familymembers may place blame on the individ-ual or others, may be angry, or may ex-press other negative emotions that canhave a negative impact on the individualand his or her rehabilitation potential.Family members may misinterpret person-ality change or speciﬁc behaviors as delib-erate or spiteful and coming from deep-seated anger toward the family, or theymay assume the individual could controlbehavior if he or she wanted to. Individualfamily members may feel trapped andmay be resentful of the caregiving rolethey now assume, reminding the individ-ual with brain damage of their depend-ence, or they may belittle the individual.In other instances, glad to have the indi-vidual home again, the family mayencourage or enforce dependence.
Family structure may be altered because
the individual’s condition may alter theroles and functions of other family mem-bers. In many cases, individuals who wereonce self-sufﬁcient and living independ-ently may, post injury, need support andcare from a spouse or other family mem-ber. Depending on the severity of thebrain damage, the personality and copingability of the caregiver, and the previousrelationship between the individual andthe family, the situation can breed resent-ment and stress, which in turn can havea negative impact on rehabilitation poten-tial. Even when the spouse or other fam-ily members willingly assume responsibil-ity and do not consider their responsibil-ity a burden, most still undergo tremen-dous emotional turmoil as they adjust tochanges in the individual with brain dam-age. The primary caregiver may neglect hisor her own physical and emotional needs,as well as the needs of other family mem-bers. In some instances the behavior of theindividual with brain damage may makeeven simple social interactions embarrass-ing, so that the family eventually feel it iseasier to stay within their home environ-ment and they become increasingly social-ly isolated. Marital relationships can beginto deteriorate. Determining premorbidfamily function can be helpful in identi-fying problems and working toward solu-tions. If there was signiﬁcant marital strainprior to brain damage, the stress afterdamage will only be increased.
Support, counseling, education about
the nature of the disability and how tocope with the individual’s behavior, andidentiﬁcation of support resources can beof considerable help in restoring familyfunctioning. Members should be given theopportunity to work through their feelings

and be assured that their feelings are nat-ural. Emphasis should be placed on main-taining the well-being of self and othersin the family unit as well as attending tothe needs of the individual with braindamage. Overall, the individual and fam-ily should be assisted in attaining realis-tic expectations and directed to pursuingreasonable goals.
Lifestyle Issues in Brain Damage
The complexity of brain damage is
extensive and impacts general activities ofdaily living. The degree to which homemodiﬁcations or assistance in independ-ent living is needed will depend on theaffected individual’s physical, cognitive,and perceptual limitations. Although thegoal of rehabilitation is to assist individ-uals to achieve as much independence inas many areas as possible, because of issuesof problem solving, judgment, and im-pulse control with brain damage, safetycan also be an issue.
The nature of the accommodations,
modiﬁcations, and assistive devices usedin the home depends on the physical lim-itations caused by brain damage. Forexample, if the individual experiencesparalysis of an upper extremity, items keptin cabinets and cupboards should bemoved for ease of reach, or special adap-tive devices may be needed for eating orto assist in dressing. In the case of limita-tion in lower extremities, bathroom mod-iﬁcations such as a raised toilet seat, grabbars, and a bench in the shower or tubmay be needed, or doorways may need tobe modiﬁed to accommodate a wheel-chair. In other instances, adaptive
devices
such as a leg brace may be needed.
The capability of individuals after brain
damage to operate a motor vehicle isdependent not only on their physical abil-ity but also on their cognitive and emo-tional limitations, which could harm notonly the individual but also the generalpublic. Driving is a complex task, requir-ing organizational ability, problem solv-
ing, decision-making ability, reﬂex actions,
visual-motor skills, coordination, and phys-
ical manipulation. Limitation in any ofthese areas could affect individuals’ abil-ity to drive. If a seizure disorder is present,
the problem is compounded. Thus a com-
prehensive assessment may be needed toevaluate the individual’s capability todrive. Since the facilities and profession-als qualiﬁed to provide this type of assess-ment may be limited except in urbanareas, such an evaluation may not beavailable to all who need it (Handler &Boland Patterson, 1995).
Because in some instances eating behav-
ior is also affected, eating habits, weightgain or loss, and nutrition may need to bemonitored. In some cases, individuals mayrefuse to eat; in other instances there maybe a constant urge to eat without feelingfull. Speciﬁc strategies to ensure adequatenutrition and weight stabilization mayneed to be implemented. For instance,there may be a need to institute a regularschedule for individuals so that they takemeals at the same time each day. If thereare problems with eating or swallowing orif tube feedings
are necessary, privacy
should be provided.
Memory problems can interfere with
individuals’ ability to perform even smalltasks of daily living. Encouraging individ-uals to keep a note pad of scheduledevents, appointments, and importantinformation can help them remember spe-ciﬁc events. Strategically placing notes inthe home or at work can help individualsremember speciﬁc tasks that may other-wise be overlooked, such as turning off thelights or closing the door.
Almost inevitably, brain damage will
cause cognitive, psychological, and some-52 C HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 53
times physical changes that in some wayaffect sexuality (Dombrowski, Petrick, &
Strauss, 2000). Sensory-motor changes can
cause erectile dysfunction in males, andmotor changes can cause spasticity orataxia that can affect sexual behavior.Cognitive changes that inhibit or regulateemotional responses can result in disinhi-bition, impaired judgment, or impairedimpulse control, which also can affect sex-ual activity. Psychological factors such asdepression or decreased self-esteem candecrease sex drive. The
anxiety or emo-
tional reactions of the indi vidual’s sexual
partner may also adversely affect sexualfunction and drive.
A primary issue impacting sexual activ-
ity may be social isolation and limitedsocial contacts. Individuals with braindamage may have lost friends and con-tacts, or they may not have had a sexualpartner at the time of the injury. Whenopportunities to increase social interactiondo occur, they may, in their desire to beaccepted, be anxious to be accepted andthus become vulnerable and are takenadvantage of. Individuals with brain dam-age who do not have a sexual partner mayneed to learn acceptable outlets throughwhich they can express sexual needs.
Some individuals with brain damage
may experience disinhibition, impairment
in judgment, or inability to control sexu-al impulses. In these instances, they mayengage in socially inappropriate behav
iors,
such as inappropriate sexual advances or
responses, or masturbation in public.Individuals may need help in learning tointerpret social and environmental cuesand in learning more socially appropriateways of expressing sexual need.
Individuals or family members may be
reluctant to bring up sexual problems; insome instances the individual with braindamage may be unaware that a problemexists. Talking openly about sexuality andassessing speciﬁc sexuality issues in thecontext of their speciﬁc values enables in-dividuals to discuss speciﬁc concerns andto identify ways to adapt to changes insexuality. In addition, identifying specif-ic sexuality issues can lessen the effects ofproblem areas (Hibbard Bufﬁngton, 1996).
Vocational Issues in Brain Damage
Return to work for individuals with
brain damage involves many factors. Be-cause of the wide variations in disability
related to brain damage, no one model can
be applied to all individuals. The changesthat may occur present unique chal-lenges. The degree to which individualswith brain damage are able to maintainemployment depends on the extent of thedamage and associated functional limita-tions as well as on their prior background,age at the time brain damage occurred,preinjury education, occupation, andwork history (Keyser-Marcus et al., 2002;Kreutzer et al., 2003; Wagner, Hammond,Sasser, & Wiercisiewski, 2002). Personalmotivation and support from family arealso important factors in determining theindividual’s rehabilitation potential.
Factors that seem most related to the
ability to return to work and to maintainemployment after brain damage are theseverity of the damage, age, and work his-tory prior to brain damage (Felmingham,
Baguley, & Crooks, 2001; Wagner, Hammond,
Sasser, Wiercisiewski, & Norton, 2000). Asthe severity of brain damage increases, therate of return to work decreases. Personswith greater disability have been shown torequire more extended time and moreextensive rehabilitation services beforeplacement (Malec, Bufﬁngton, Moessner,& Degiorgio, 2000). Although the major-ity of individuals with mild brain damagemay be able to return to work, individu-als with moderate and severe brain dam-

age have poorer outcomes (Fabiano &Daugherty, 1998). Even when job place-ment is accomplished, for individuals withmoderate to severe disability, job retentionmay be difﬁcult. The type of occupationthey were in before the injury also appearsto inﬂuence return to work outcome, withthe highest rate of return to work beingamong persons with higher decision-making jobs (Orr, Walker, Marwitz, &Kreutzer, 2003). Age also appears to be asigniﬁcant determinant of return to work.Generally, the older the individual is at thetime of injury, the less likely he or she isto return to work (Rothweiler, Temkin, &Dikmen, 1998). Lastly, work history priorto brain damage appears to be related toability to return to work post damage.Those individuals with poor work histo-ry prior to experiencing brain damage aremore likely to have more problems return-ing to work after brain damage hasoccurred (Rubin & Roessler, 2001).
Cognitive deﬁcits and psychosocial dif-
ﬁculties may have greater impact onreturn to work than physical limitations.Individuals who retain average to above-average intellectual abilities and interper-sonal skills after brain damage occurs areoften better able to compensate for otherlimitations and maintain or gain em-ployment. For others, however, the levelsof interpersonal functioning and cognitiveself-awareness are often limited. Braindamage may result in drastic changes inpersonality and personal ability as well asin impaired self-awareness, which is alsoa frequent contributor to employmentproblems. Individuals who experienceemotional lability may have more difﬁcul-ty in the workplace and with coworkers.
Memory impairment may be a debilitat-
ing effect of brain damage. Individualswith memory problems may forget whatthey have learned and thus may not beable to beneﬁt from experience. Helpingthem ﬁnd alternative ways to performtasks and to develop strategies to reduce,organize, and retrieve information canreduce the disabling effects of memory im-pairment. Since individuals may have dif-ﬁculty organizing their day, implementingstructured routines, using written notes orlists, or using audiotaped reminders mayhelp improve performance. Usually notesor lists will be most effective if informa-tion is kept simple with no extraneousdetails. Too much information may causethe individual to become overwhelmedand confused.
Individuals with brain damage may not
be aware of their deﬁcits and may overes-timate their abilities. Judgment may alsobe affected. Poor self-awareness or inaccu-rate self-perception can be a contributorto employment problems. Individualsmay be unable to recognize job errors andmay consistently rate their performancemore highly than it is rated by theiremployer. Consequently, there may belimited beneﬁt from feedback.
The ability to communicate verbally or
in writing or to comprehend words andconcepts inﬂuences all aspects of job selec-tion, training, and performance. Specialconsiderations need to be given to limita-tions in the ability to communicate.Visual-perceptual skills are integral tomany jobs, both skilled and unskilled. Theability to perceive detail and to scan,match, or accurately perceive patternsmay affect a number of daily life activities,including reading, driving, and navigatingthe environment.
Motor skills limitations affecting ﬁnger
dexterity or eye-hand or eye-foot coordi-nation may also be present. When theselimitations exist, work involving precisionor operation of various tools or equipmentmay be difﬁcult. Individuals who haveexperienced paralysis of one of the upperextremities may be limited in their abili-54 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 55
ty to lift, carry, or pull or push. If one ofthe lower extremities has been affected,they may require assistive devices, such asa cane, walker, braces, or in some in
stances
a wheelchair. Consequently, ambu lation
may be restricted to short distances. Am-bulation on uneven surfaces should beavoided; if a wheelchair is used, environ-mental modiﬁcations may be required.One should also consider that they per-form tasks less quickly and have less phys-
ical stamina and endurance. Often individ-
uals may be able to perform a task well ifthey are allowed to take their time, ratherthan feeling pressured to rush. Thus com-petitive environments in which speed isa priority may not be the best environ-ments for individuals with brain damageand may actually decrease their quality ofwork.
Brain damage is not a progressive con-
dition, and unless there is accompanyingchronic illness that produces additionalsymptoms or that increases the chance ofanother stroke occurring, the individual’soverall life expectancy is not affected.Brain damage is, however, a lifelong dis-ability. Just as the rehabilitation needs ofindividuals vary, depending on the age atwhich the damage occurred, so will reha-bilitation needs change over the individ-ual’s lifetime. Consequently, ongoingmonitoring and contact with individualsmay be necessary to help them maintainmaximum potential in the workplace.
In some instances, supported em-
ployment or job coaching may be appro-priate. In other instances, performance-based feedback or prompts may be sufﬁ-cient to help the individual maintainemployment. Returning to gainful em-ployment represents a series of complexchallenges because of the number, com-plexity, and interaction of problems, all ofwhich may contribute to difﬁculty in
maintaining long-term employment. Mon-itoring performance as well as maintain-
ing communication with the employercan contribute to job retention. Helpingindividuals learn compensatory strategies,providing appropriate workplace accom-modations, and educating employersabout the nature of brain damage and itsconsequences can greatly increase thechances of successful job placement.
Cerebral Palsy
Damage to the brain before, during, or
shortly following birth results in a condi-tion known as cerebral palsy . Cerebral
palsy is not a disease, but rather a complexof symptoms covering a wide number offunctional impairments. It is characterizedby chronic disorders of movement or pos-ture. It may be accompanied by seizuredisorders, sensory impairment, and cogni-tive limitation (Nelson, 2003).
No two peo-
ple with cerebral palsy are alike. Cerebral
palsy is not progressive, com municable, or
inherited. The condition is also not cur-
able, since once damage to the brain is sus-tained, damage is permanent. However,different therapies and training programscan help individ
uals manage symptoms
and increase their functional capacity.
Consequently, therapies are designed toenhance individuals’ abilities rather thanto reverse the condition.
Causes of Cerebral Palsy
The cause of cerebral palsy varies. It can
be caused by:
• a birth injury in which the infant
experiences direct damage to thebrain, such as from ruptured bloodvessels or compression of the brain
• exposure of the mother to toxic
chemicals while pregnant or infec-tious disease experienced by her dur-ing pregnancy

• other causes, such as Rh or A-B-O
blood type incompatibility betweenparents of the infant
• lack of oxygen to the brain of the
fetus before birth or shortly after birthdue to conditions such as umbilicalcord strangulation, prolonged labor(which stresses the fetus), or prema-ture separation of the placenta fromthe uterus
Characteristics of Cerebral Palsy
The word cerebral refers to the brain. The
word palsy refers to movement or posture.
One characteristic of cerebral palsy is theinability to totally control and coordinatemovement. The type of cerebral palsy andsymptoms experienced depends on thelocation and extent of the damage to thebrain. Some individuals have minor, bare-
ly detectable symptoms, while others have
severe functional disability. Depending onthe type of cerebral palsy, individuals mayexperience a number of symptoms affect-ing movement. One symptom may bespasticity , in which there is abnormality
of muscle tone resulting in muscle stiff-ness and exaggerated muscle contraction.Spasticity interferes with dexterity and theability to perform various muscle move-ments. Some individuals with cerebral pal-sy experience ataxia (disorder in
the
accuracy of muscle movement), which af-
fects balance and coordination of gait. Stillothers have dyskinesia (unwanted, invol-
untary muscle movements), which inter-feres with the ability to conduct purpose-ful movements or causes movement whennone is desired. Some individuals have acombination of spasticity, ataxia, anddyskinesia.
Abnormal movements may include pur-
poseless, jerky, or abrupt movements ( athe-
tosis ), especially of the upper extremities;
or slow, continuous writhing movements(choreoathetosis ). In rare instances, ato-
nia, in which there is lack of muscle tone,
and muscle ﬂaccidity may be present.
Although cerebral palsy primarily affects
muscle control, the brain is responsible formany other activities as well. Consequent-ly, additional manifestations resultingfrom cerebral palsy may include visual or
hearing impairments, perceptual disorders,seizures, communication dif
ﬁculties, men-
tal retardation, learning difﬁculties, orbehavioral disorders, depending on theparts of the brain affected.
Classiﬁcation of Cerebral Palsy
Clinically, cerebral palsy is classiﬁed ac-
cording to the type, location, and degreeof movement manifestations and to thetone of muscles at rest. Clinical types ofcerebral palsy based on classiﬁcation ofmovement are as follows:
1.Spastic cerebral palsy , in which indi-
viduals experience high muscle tone(hypertonia ) so that muscles and
joints are tight and stiff, limitingmovements in areas of the body thatare affected. Individuals often expe-rience an increase in hypertonia withactivity and interference with resid-ual motor function.
2.Ataxic cerebral palsy , in which individ-
uals have difﬁculty with balance andcoordination.
3.Athetoid cerebral palsy , in which indi-
viduals exhibit uncoordinated, jerky,or twisting movements in affectedbody parts, particularly in ﬁngers andwrists.
4.Mixed-type cerebral palsy , in which
individuals experience manifesta-tions of more than one clinical typeof cerebral palsy.
Cerebral palsy can also be classiﬁed ac-
cording to the location of manifestations:56 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Conditions Affecting the Brain 57
1.Hemiplegia indicates that manifesta-
tions are found on only one side ofthe body, such as an arm and a leg onthe right side.
2.Quadriplegia indicates that manifes-
tations affect all four extremities.
3.Paraplegia indicates that manifesta-
tions only affect the legs.
4. Other classiﬁcations, such as monople-
gia,in which only one limb is involved,
and triplegia ,in which three limbs are
involved, are rare.
Another way of classifying cerebral pal-
syis by degree:
1.Mild cerebral palsy describes mani-
festations that affect only ﬁne motormovement.
2.Moderate cerebral palsy describes man-
ifestations that affect general musclemovement, ﬁne motor movement,and clarity of speech so that activitiesof daily living and communicationmay be affected but the individual isstill able to function.
3.Severe cerebral palsy describes manifes-
tations that signiﬁcantly affect theability to walk, use the hands, or tocommunicate so that ability to func-tion in activities of daily livingand/or communicate is extensivelycompromised.
Lastly, cerebral palsy may be classiﬁed
according to muscle tone at rest:
1.Isotonic , in which muscle tone is
normal
2.Hypertonic , in which there is in-
creased muscle tone
3.Hypotonic , in which there is de-
creased muscle tone
Management of Cerebral Palsy
Management of cerebral palsy is direct-
ed toward providing functional supports toindividuals based on the speciﬁc symp-toms exhibited so that functional capac-ity can be enhanced and additional disa-bility prevented. Although cerebral palsyitself is not progressive, altered tone and/or activity of muscles may cause condi-tions that result in additional limitationsand disability, such as contractures (loss
of range of motion or ﬁxation of a joint)or scoliosis (lateral curvature of the
spine). Consequently, in addition to help-ing individuals reach their maximumfunctional capacity, management of cere-bral palsy is also directed toward prevent-ing conditions that could impedefunction.
All aspects of symptom management
should be directed toward giving individ-uals the opportunity to control and man-age their own situation as much as pos-sible. Major goals of management ofteninclude maintaining joint range of motionto prevent contractures and other defor-mities, and increasing muscle control andcoordination to help individuals counter-act abnormal postures.
Interventions begin at an early age and
include a number of services, dependingon the speciﬁc needs and symptoms of theindividual. Physical therapy may be insti-
tuted to increase and enhance motor skilland balance. Occupational therapy may be
utilized to help individuals learn how tomanage activities of daily living as well asother daily functions. Orthotics in the form
of prescribed braces or splints may be usedto help prevent or correct deformity.Braces can help individuals improve func-tional mobility as well as appearance. Thetype of brace depends on the type of dis-ability experienced.
Medical interventions may also be in-
volved in the management of cerebral pal-sy. In some instances medications may beprescribed to promote muscle relaxationwhen excessive muscle spasticity or exces-

sive muscle tone is present. Anticonvul-sant medications may be prescribed ifindividuals also have a seizure disorderassociated with cerebral palsy. Orthopedic
surgery may be needed to correct jointdeformities or to lengthen muscles or ten-dons in order to decrease muscle spasm.
The best management of complica-
tions is through prevention. Contracturescan be prevented through regular passiveexercise or surgical intervention to length-en the muscles that are contracted. Boweland bladder incontinence can be managedthrough training programs that help indi-viduals establish dietary control and a reg-ular evacuation schedule, as well asprograms to increase awareness of the sen-sory stimuli that indicate a need for evac-uation. Individuals may decrease theirdental problems through training in oralhygiene and regular dental care. A specif-ic program of weight bearing and muscleactivity as well as a diet adequate in cal-cium can help to prevent osteoporosis.Training that helps individuals increaseposture control and the use of braces andsplints can retard the development ofdegenerative joint disease and scoliosis.Training to help individuals developimproved breathing patterns, coughing,and lung expansion can decrease thechances of aspiration and consequentlyrespiratory infection.
Adequate rest at night as well as estab-
lishment of rest periods throughout theday can decrease fatigue. Evaluating indi-viduals’ total energy output and adjustingtheir tasks and schedules to ﬁt their needscan help preserve energy and preventexcessive fatigue.
Complications of Cerebral Palsy
Because of the manifestations of cere-
bral palsy, a variety of complications thatare secondary to the condition itself canalter the individual’s functioning ability orwell-being. Contractures can limit bothpassive and active joint movement andcan interfere with self-care, walking, andsitting. Some individuals with cerebral pal-sy also experience bowel and bladder in-continence because of their inability toattend or respond to sensory stimulus in-dicating the need to urinate or defecate.Other individuals may experience dentalproblems that are exacerbated by their in-ability to brush their teeth adequately. Be-cause of insufﬁcient muscle activity, someindividuals may be more prone to osteo-porosis, which in turn can cause pain andincrease susceptibility to fractures. Poorlyaligned joints may predispose them todegenerative joint disease, resulting inpain and increased immobility. In someinstances, if individuals have poorly sup-ported sitting posture, scoliosis (lateral
curvature of the spine) may occur, com-promising breathing as well as the func-tion of internal organs. If coughing andswallowing ability is insufﬁcient, aspira-tion of food or ﬂuids may place individ-uals at risk of respiratory infections orpneumonia.
Although not necessarily a complica-
tion, fatigue secondary to manifestationsof cerebral palsy can also interfere withindividuals’ ability to function efﬁciently.
Because they may experience difﬁculty with
motor control and coordination, moreenergy may be expended to carry out evenroutine motor activities. Involuntarymovement or spasticity may also increasethe amount of energy expended. As aresult, individuals with cerebral palsymay become fatigued more easily.
Psychosocial Issues in Cerebral Palsy
Although data regarding the psychoso-
cial adjustment of adults with cerebral pal-sy are limited, cerebral palsy as a develop-58 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

mental disability poses many of the sameproblems as other developmental disabil-ities. Misunderstanding of the conditionby parents, teachers, or others with whomindividuals with cerebral palsy come incontact can perpetuate a sick and depend-ency status rather than one of empower-ment. How individuals with cerebral palsywere treated in childhood can inﬂuencetheir self-perception and functioning inadulthood. With any type of developmen-tal disability there is the risk of overpro-tectiveness by parents and others, whichcan impede the individual’s emotionaldevelopment by restricting access to expe-riences that are vital to the developmentof adequate coping strategies. As a result,children may learn, at an early age, to usemaladaptive behavior to achieve goals. Ifthis behavior is continued into adulthood,it may impede the individual’s ability tointegrate effectively into the larger socialmilieu. When children have been keptoverly dependent on parents, have beengiven little responsibility for home chores,have not been confronted with the typi-cal consequences of behavior, or have notlearned acceptable means of expressingemotions, lack of experience and maturesocial development can serve as a handi-capping factor in adulthood.
In other instances, children who have
often been the focus of a wide variety ofservices and activities from an early agemay continue these expectations intoadulthood, demonstrating a sense of ego-centricity that may limit positive socialinteractions and lead to further social iso-lation. If these behaviors persist into adult-hood, they may become more of animpediment to social integration than themanifestations of the condition itself. Attimes brain damage associated with cere-bral palsy may also create behavior deﬁcitsthat can interfere with the developmentand maintenance of social relationships. For adolescents with cerebral palsy,
opportunities to participate in social activ-ities, information related to sexuality, andopportunities to participate in sexualexploration and relationships may havebeen limited. In addition, adolescentswith cerebral palsy may have a distortedbody image and a low self-concept, whichcan affect their social competence, dating,and sexual behavior. Although individu-als with cerebral palsy, as adolescents aswell as adults, experience normal desires,they may lack the skills necessary to ful-ﬁll those needs. In addition to barriers ofinadequate information, skill, or opportu-nity for appropriate sexual expression,they may also experience physical barri-ers because of their disability that makesexual expression more difﬁcult.
Communication issues for individuals
with cerebral palsy can include hearing orauditory comprehension problems, visu-al disorders, or speech deﬁcits. Individu-als with communication problems as theresult of cerebral palsy may have grown upin an environment in which family,friends, and others became accustomed totheir adaptive communication methods.In adulthood, however, when relation-ships change and higher standards of per-formance are expected, communicationmay become increasingly difﬁcult. Forinstance, those unfamiliar with the indi-vidual or with cerebral palsy itself maymisinterpret problems with hearing orunintelligible speech as lack of cognitiveability. In other instances, because theindividual may be difﬁcult to understand,acquaintances may begin to avoid inter-actions with the individual who thenbecomes socially isolated. Depending onthe severity and type of cerebral palsy,decreased mobility, problems with eating,or problems with personal hygiene mayfurther restrict the individual’s socialinteractions.Conditions Affecting the Brain 59

Vocational Issues in Cerebral Palsy
Cerebral palsy is not a progressive con-
dition, and there is no progressive deteri-oration as a direct result of the cerebralpalsy itself. However, it is a lifelong con-dition. Consequently, follow-up through-out the individual’s life may be necessary.As individuals age with their disability,additional limitations may develop. Forinstance, fatigue is a consideration forindividuals with cerebral palsy, regardlessof age. However, as individuals becomeolder, endurance for the same activitiesonce performed may be decreased.
Long-term goals are appropriate and
desirable. The degree to which individu-als are able to achieve their goals in a spec-iﬁed occupation will be dependent ontheir physical, psychosocial, and lan-guage abilities, as well as on their motiva-tion and social support. Speciﬁc skills andabilities may be enhanced with compen-satory measures and/or with practice.Since the functional limitations associatedwith cerebral palsy are individual, specif-ic vocational limitations will be depend-ent on the symptoms each individualexperiences. In some instances, verbalcommunication is severely impaired, andin other cases it may be totally unaffect-ed. Some individuals may have limitedmobility or ambulation problems; othersmay have signiﬁcant difﬁculty with mobil-ity or ambulation. Whereas some individ-uals will be ambulatory, others mayrequire a wheelchair. In some instancesindividuals may have difﬁculty concen-trating or remembering, and in otherinstances individuals’ cognitive abilitiesare unaffected.
Since most jobs require some degree of
social skill, when individuals have difﬁcul-ty in this area, social skills training maybe beneﬁcial (Salkever, 2000). Matchingthe work setting to the individual’s specif-ic needs, interests, and abilities is impor-tant for anyone with a disability; howev-er, in the case of cerebral palsy, attentionto these factors may be even more impor-tant to increase the potential for vocation-al success.
Epilepsy
Epilepsy is a chronic disorder of the
nervous system. It is not a disease, butrather a symptom of an underlying neuro-logical condition in which neurons in thebrain create abnormal electrical dischargesthat cause seizures (temporary loss of con-
trol over certain body functions). There isno single cause of epilepsy, and it canaffect anyone at any age. It can be causedby a number of conditions in which func-tion of the brain has been affected, suchas head injury or stroke. Sometimes,however, no clear-cut cause can be iden-tiﬁed. In this case, epilepsy is consideredas idiopathic .
Although the essential feature of epilep-
sy is recurrent seizures, not all seizures aredue to epilepsy. Seizures can occur as aresult of a temporary dysfunction of thebrain brought on by certain conditions,even though there are no permanentchanges in brain function. Acute condi-tions such as meningitis (infection of the
covering of the central nervous system),diabetic coma, hypoxia (too little oxygen
to the brain), and drug intoxication orwithdrawal can all cause temporary dis-turbed brain function resulting in aseizure. If the underlying cause of braindysfunction is reversible so that no perma-nent alteration of brain function existsand seizures do not recur, individuals arenot considered to have epilepsy. The termepilepsy is reserved for individuals with
recurring seizures and with a chronicabnormality of the brain that results inseizures.60 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Classiﬁcation of Seizures
Individuals with epilepsy can exhibit a
variety of seizures with varying symptoms,ranging from muscle spasms or confusionto total loss of consciousness. Seizures canbe mild or severe, can occur frequently orrarely, and can change their pattern ofoccurrence over time. Depending on thetype, seizures usually last only seconds tominutes. Between seizures most individ-uals are able to function normally.
Seizures are classiﬁed according to the
part of the brain that demonstrates ab-normal electrical activity and the type ofseizure experienced. Classiﬁcation of seiz-
ures is important so that appropriate man-
agement and treatment can be determined.
Seizures are classiﬁed as generalized , in
which nerve cells discharge abnormallythroughout the brain, or partial , in which
the abnormal nerve cell discharge is lim-ited to one speciﬁc part of the brain
(Browne & Holmes, 2001). Although there
are many classiﬁcations of seizures relat-ed to epilepsy, common types of seizuresare listed in Table 2–5. What follows is abrief description of common types ofseizures.
Generalized Tonic-Clonic Seizure
(Grand Mal)
An abnormal discharge of nerve cells
throughout the brain results in a general-
ized tonic-clonic seizure, sometimes called
a grand mal seizure. Some individuals ex-perience an aura (warning sign) immedi-
ately before the seizure begins. They maysee a ﬂash of light, have an unusual tastein the mouth, or have other unusualsensations. As the seizure develops, indi-viduals lose consciousness and fall down,entering a tonic state in which there is gen-
eralized body rigidity. Muscles then entera clonic state so that the whole body un-dergoes rapid, jerky movements. Theteeth are clenched tightly together and
control of the bladder or the bowel may be
lost. The seizure generally lasts less than
a few minutes. When the seizure ends, con-
sciousness is gradually regained, but indi-viduals may experience confusion,difﬁculty in speaking, and headache. Al-though postseizure symptoms usually dis-appear within several hours, the fatigueexperienced may be overwhelming, oftennecessitating an extended period of rest orsleep.
Table 2–5 Common Types of Seizures
Associated with Epilepsy
Generalized seizures
1. Tonic-clonic (grand mal)2. Absence (petit mal)
Partial seizures
1. Simple-partial (focal) 2. Complex-partial (psychomotor)
Although a tonic-clonic seizure may be
frightening to those who witness it, indi-viduals experiencing the seizure are usu-ally in no imminent danger unless thereare hard, sharp, or hot hazards within theimmediate environment. No attemptshould be made to move individuals ex-periencing a seizure except when neces-sary to protect them from such hazards.To avoid injury, there should be no at-
tempt to restrain individuals during a ton-ic-
clonic seizure, to pry open clenched
teeth, or to place hard objects in the indi-vidual’s mouth. Individuals should beplaced on their side during a seizure sothat secretions can drain from the mouthand do not compromise the airway.Conditions Affecting the Brain 61

Absence Seizure (Petit Mal)
Like tonic-clonic seizures, absence
seizures are classified as generalized,
because nerve cells discharge throughoutthe brain. Children most commonly ex-perience this type of seizure. Absenceseizures are characterized by brief blankspells or staring spells and a loss of aware-ness of the surroundings. The seizuregenerally lasts for only seconds. The indi-vidual does not fall, and there are usual-ly no outward motor manifestations ofabsence seizures, although abnormalblinking or slight twitching may occuroccasionally. Because of the limited visi-ble symptoms of the seizure, those aroundthe individual may misinterpret absenceseizure as daydreaming or inattentiveness.
When children experience frequent
absence seizures, school performance maybe disrupted. Because there may be nosigniﬁcant signs that are easily observedduring the seizure, the seizure disordermay not be diagnosed, and poor schoolperformance may be attributed to othercauses. Recognition of symptoms andappropriate diagnosis are crucial to enablechildren to achieve maximum schoolpotential. Absence seizures may disappearspontaneously with age, although someindividuals who have had absence seizures
later go on to develop tonic-clonic seizures.
Partial Seizures
When nerve cells discharge in an isolat-
ed part of the brain, partial seizures occur.One type of partial seizure is a focal seizure ,
in which there is no loss of consciousnessand symptoms are very localized, depend-ing on the part of the brain affected. Onetype of focal seizure, a Jacksonian (simple-
partial) seizure , begins with convulsive
symptoms in one part of the body, suchas a hand or foot. The convulsive musclemovement then progresses in an orderlymanner up the extremity. Jacksonianseizures can remain limited to one part ofthe body or can go on to develop into full-blown tonic-clonic seizures.
Other types of partial seizures may have
more complex symptoms. Complex-partial
(psychomotor ) seizures are characterized by
a loss of awareness of the surroundings.Individuals may pace, wander aimlessly,make purposeless movements, and utterunintelligible sounds. The seizure can lastup to 20 minutes, with mental confusionlasting for a few minutes after the seizureis over. Observers may misinterpret symp-toms of complex-partial seizures, oftenattributing the symptoms to alcohol,drug abuse, or mental illness.
Status Epilepticus
Status epilepticus is a term used to de-
scribe seizures that are prolonged or thatcome in rapid succession without full re-
covery of consciousness between seizures.
It is a medical emergency that can belife–threatening and consequently re-quires immediate medical attention and
treatment (Lowenstein & Alldredge, 1998).
Diagnosis of Epilepsy
Individuals who are having a seizure for
the ﬁrst time usually undergo medicalevaluation by a neurologist to determine
whether the seizure is a symptom of anacute medical or neurological illness that
can be treated and resolved or a symptom of
a chronic neurological problem that will re-quire ongoing treatment for control. Exten-
sive physical examination and blood testsare usually part of initial screening, as wellas a detailed history of the precipitatingfactors that appeared to trigger the seizure.
When individuals have more than one
seizure, or when other symptoms or his-62 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

tory indicate that epilepsy may be thecause of seizure activity, a more extensivemedical evaluation is conducted. A pri-mary diagnostic tool for evaluating indi-viduals after seizures is electroencephalo-
graphy (EEG), a noninvasive procedure in
which the electrical activity of the brainis recorded on a graph. Magnetic resonance
imaging (MRI), a noninvasive procedure in
which rapid detailed pictures of bodystructures are produced, may also be usedto identify structural anomalies in thebrain that may be related to seizures.
Treatment and Management of Epilepsy
Treatment of epilepsy is dependent on the
cause of the seizure activity and the typesof seizures experienced. Generally indi-
viduals who have had only a single seizure
are not considered to have epilepsy. Al-though they may be thoroughly evaluat-ed in an attempt to determine the causeof the seizure, they usually are only mon-itored and not given medication (Will-more, 1998). If the seizures are caused bya tumor, scar tissue, or another abnormal-ity that can be corrected, surgical interven-tion to remove or repair the abnormalitymay be indicated. In most instances, how-ever, when epilepsy is diagnosed, the stan-dard treatment of most types of seizuresis the regular use of one or more anticon-
vulsant or antiepileptic medications.
Although medications do not cure epi-
lepsy, they can effectively control seizuresand enable many individuals to carry onfull and productive lives. Successful con-trol of seizures, however, requires the indi-vidual’s strict, long-term compliance withmedication instructions. The anticonvul-sant medications used to treat epilepsy arealso not without side effects, and toxiceffects are common during long-termtreatment. Depending on the medication,side effects can include gum overgrowth,nausea, dizziness, clumsiness, visual difﬁ-culty, or fatigue.
Once medication for treatment of
seizures has begun, it is generally main-tained for at least two years, regardless ofwhether the individual has remainedseizure free (Browne & Holmes, 2001). Ifthere have been no recurrent seizures afterthis time, the physician may considerwithdrawing the medication. Individualswho have had no additional seizures afterbeginning the medication, or who haveexperienced side effects, may be temptedto alter or discontinue their medication.The consequences could be dangerous andat times life-threatening. Consequently,individuals should never attempt to alteror discontinue their medication withoutconsulting their physician.
The medication prescribed is based on
the type of seizure and on whether morethan one type of seizure is experienced.The general goal of treatment with med-ication is to maximize control of seizureswithout causing toxic side effects, such asliver damage or bone marrow suppression.The physician periodically monitors lev-els of the medication in the blood. Basedon medication blood level and its effec-tiveness in controlling seizure activity, thephysician may alter medication dosagesaccordingly. Measuring the blood levels ofthe anticonvulsant also helps the physi-cian monitor individuals’ compliancewith the medication regime as well asidentify any toxic effects.
In some cases, even when individuals are
compliant with taking anticonvulsant med-i
cations, seizures remain uncontrolled.
Many of these individuals experienceseveral seizures a month or, at times, sev-eral seizures a day, despite following astrict treatment regimen of medication.When seizures are severely disabling andcannot be controlled by medication, sur-gery may be recommended to treat epilep-Conditions Affecting the Brain 63

sy. Under these circumstances, surgery
may involve removing a portion of the
brain structure, resecting a portion of thebrain, or disconnecting the affected por-tion from the rest of the brain. Surgery it-self may leave residual effects. The amountof disability experienced, if any, after thistype of surgery depends on indi
vidual cir-
cumstances. Anticonvulsant med ications
may still be needed even after surgery.
Alcohol can lower the seizure threshold
and therefore precipitate seizures. Alcoholand antiepileptic medications may alsointeract and cause untoward effects. Con-
sequently, individuals with epilepsy should
consult their physician about alcohol use.
Individuals with epilepsy should be
helped to identify factors that may trig ger
a seizure. They should also avoid activ ity
that would be hazardous if a seizureshould occur, such as swimming alone oroperating heavy equipment. A medicalidentiﬁcation bracelet should be worn byindividuals with epilepsy at all times.
The general prognosis for individuals
with epilepsy depends on the type ofseizure, the underlying cause, the admin-istration of appropriate treatment, and theindividual’s willingness and ability to fol-low the prescribed treatment regimen. Iftheir condition is accurately diagnosedand appropriately treated, most individu-als with epilepsy can live active, produc-tive lives. Prompt detection and earlymedical intervention can greatly improvethe ability to control seizures and enhancethe general quality of life for the individ-ual with epilepsy.
Psychosocial Issues in Epilepsy
Individuals with epilepsy may face
many psychological and psychosocialchallenges. They must learn to deal withthe uncertainty of whether and whenanother seizure will occur. No matter howwell controlled seizures are, individualslive with the possibility, even if remote,that another seizure will occur. The time,place, and social circumstances underwhich a seizure may occur are unknown.If individuals experience a seizure in pub-lic, they risk feelings of embarrassmentand onlookers’ potential misperception ofthe seizure. Individuals may feel they haveno control over their lives and behavior.At times, even when seizures are adequate-ly controlled, anxiety over the possibilityof having a seizure or other psychosocialdysfunction may be the most disablingfactor associated with the condition. As aresult, individuals may have difﬁcultyestablishing interpersonal relationships,building self-esteem, and obtaining ormaintaining employment.
The family is crucial in the adjustment
of individuals with any disability. Adjust-ment and emotional development dependon when the diagnosis of epilepsy is made
and the reaction of the individual’s family
to it. When epilepsy is diagnosed in child-hood, parental feelings of fear, anxiety,
guilt, overprotectiveness, or mourningcan af
fect not only the child’s ability to
accept his or her disability but also his orher self-concept and social adjustment.
Overly protective parents may foster depen –
dency in their child. Children may learn
to use their condition as an excuse for in-
activity or avoidance of responsibility. When
they are teenagers, concerns related to
whether they will drive a car, participate insports, or engage in dating may cause addi-
tional stress and lessening of self-esteem.
Diagnosis of epilepsy in adulthood can
also disrupt interpersonal and family rela-tionships. The impact on individuals’social identity may be threatened, so thatthey go to great lengths to conceal theirdisability in order to avoid potentialrejection. Partners of individuals with epi-lepsy may be fearful of observing a seizure64 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

or may be concerned that the disorder ishereditary. Because of anxiety and misin-formation, they may be unwilling to learnmore about the condition or to providethe support that the individual withepilepsy needs.
Sexual activity, in most cases, need not
be affected by epilepsy (Frazer & Gumnit,1990). Although some medications usedto treat seizures may have some effect onlibido, most do not. Psychological issuesof low self-esteem or poorly developedsocial skills may produce greater limita-tions. Individuals may be reluctant toform intimate relationships because of thefear of having a seizure. Counseling maybe necessary to help them overcome thisfear so that appropriate intimate relation-ships can be established.
Between seizures, epilepsy is an invisi-
ble disability. Unless individuals are hav-ing a seizure, there are no outward signsof disability. Although considerable efforthas been devoted to educate the publicabout the condition, misinformation andlack of acceptance still exist, and epilep-sy continues to carry a stigma for manyindividuals. In some cultures, historicalmisconceptions about epilepsy havelinked it to demonic possession and insan-ity. In other instances people with epilep-sy were not permitted to participate invarious events because of their diagnosis.Attitudes have changed in recent years asthe result of public education programs,improved placement of individuals withepilepsy into the world of work, and in-creased ability to control seizures. In manyinstances, however, individuals withepilepsy may still experience unjust re-strictions that deny them access to partic-ipation in routine activities. The stigmaand shame associated with epilepsy maycause individuals and/or their family todeny or minimize the condition. Indi-viduals may try to pass as someone with-out a disability because of anticipatedrejection due to real or perceived publicattitudes.
Operating a motor vehicle has a direct
impact on independence and social well-being. Inability to drive can limit socialinteraction, educational experiences, andemployment opportunities. In most in-stances, individuals with epilepsy candrive without signiﬁcant risk of accidentif their seizures are controlled with med-ication.
In the past, individuals with epilepsy
were restricted from driving motor vehi-cles because of concerns for public safetyas well as personal safety. Although todaymost states permit individuals with epilep-sy to obtain driver’s licenses, the length oftime they must be seizure free in order toobtain the license varies from state tostate. State laws do not always take intoaccount individual differences; some makeblanket rules that apply to all individualswith epilepsy regardless of their personalcircumstances.
Alcohol consumption is frequently an
activity at social occasions. Individualswith epilepsy should always consult theirphysician about alcohol consumption, es-pecially in regard to taking alcohol whenthey are also taking anticonvulsant med-ication; however, each individual situationmust be considered.
Sports activities are also an important
means of socializing as well as helping in-dividuals build self-conﬁdence and self-esteem. In some situations, restrictions on
participation in various activities are placed
on individuals with epilepsy even thoughno basis for limiting the activities exists.Although some individuals’ seizures maybe precipitated by fatigue or other sports-related circumstances, others may have areduced incidence of
seizures with exercise.
Consequently, authorities should consider
the individual’s speciﬁc circumstancesConditions Affecting the Brain 65

rather than issuing blanket restrictions;each case should be considered individu-ally. Restrictions may be necessary for spe-ciﬁc activities that present a hazard should
a seizure that involves loss of consciousness
take place. For instance, individuals withepilepsy should not swim alone. Activitiessuch as ﬂying an airplane, rock climbing,or other activities in which a seizure couldcause severe and possibly fatal conse-quences should also be avoided. In mostinstances applying common sense enablesindividuals to participate in activitieswhile also avoiding potential hazards.
Even when seizures are relatively well
controlled, individuals may still fear hav-ing the “occasional” seizure and the phy-sical and social consequences it maybring. In addition to the embarrassmentof having a seizure in public, individualsmay also fear injury. Injury during aseizure while performing routine tasks,such as setting clothes on ﬁre from gasstoves or falling in the bathtub, can occur.Consequently, individuals may need helpin establishing commonsense safety pre-cautions for activities of daily living.Family, friends, and coworkers should alsobe informed about appropriate proceduresif a seizure occurs.
Despite control of seizures, individuals
may still feel the weight of their restric-tions on the freedoms, activities, andevents that others take for granted. Forinstance, in most states individuals arerequired to report that they have epilep-sy when applying for a driver’s license.They may be required to obtain a writtenstatement from the physician to verifythat they can return to regular activitiesafter a seizure occurs. If ﬂickering lightsprecipitate seizures, they may need to
avoid certain theaters, bars, or other places
that use strobe lights for decoration oreffect. Taking medication regularly asprescribed, obtaining proper rest, andreducing stress are other self-managementissues individuals with epilepsy mustconsider. The stress, uncertainty, restric-tions, isolation, and difﬁculty with em-ployment all require adjustment andcoping skills if individuals are to achievetheir full potential.
Vocational Issues in Epilepsy
Most individuals with epilepsy have the
same range of IQ as the general popu-lation, unless other conditions that affectintellectual function are involved. How-
ever, individuals with epilepsy experience
both unemployment and underemploy-ment (Bishop, 2004; Fisher, 2000). Manyproblems in the workplace for individualswith epilepsy continue to be related tomisperceptions and stigma rather thanphysical limitations. Although some in-dividuals with epilepsy, especially epilep-sy associated with head trauma, may haveneuropsychological limitations that canaffect employment, many do not.
Con-
sequently, special consideration must be
given to each individual situation.
Epilepsy is a chronic condition that
requires a continuous relationship withthe medical community. Medication is, ofcourse, a major part of treatment andrequires close medical supervision.Multiple medications may be needed tocontrol seizures, and the medicationsthemselves may have associated sideeffects. Individuals must be diligent in tak-ing medication because missed doses mayprecipitate a seizure. When individuals arein denial of their condition, their denialmay be manifest as poor compliance withmedication, which in turn causes poorseizure control and may consequentlyaffect employment potential.
Because of the chance of an unpre-
dictable loss of consciousness that mayplace individuals with epilepsy or others66 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

at risk of injury, some occupations, suchas airplane pilot or interstate truck driver,may be unrealistic for the individual topursue. Understanding how seizures affectjob function is critical. It is important toassess the type and number of seizuresindividuals have, the degree to whichseizures are controlled with medication,and how compliant individuals are in fol-lowing the treatment regimen. Determin-ing the situational patterns to seizures(such as a regular time when seizures oc-cur) or the factors that precipitate seizures(such as fatigue, stress, or ﬂickering lights)is important to help in
dividuals avoid or
alter situations in which seizures may
occur. If fatigue tends to precipitate aseizure, care should be taken so the indi-vidual does not become overly fatigued.Likewise, if seizures are related to the indi-vidual’s sleep pattern, he or she may beunable to work on a rotating shift. It isalso helpful to know if individuals expe-rience an aura and if they consequentlywould be able to remove themselves fromdangerous situations before the seizurebegins. Finally, individuals who experi-ence seizures should probably not workalone in an isolated environment, espe-cially if the environment imposes somethreat of danger if a seizure should occur.
Employers who fear risk of lawsuits aris-
ing from workplace injuries may be overly
conservative with restrictions for employ-
ees with epilepsy. However, many jobs once
thought to be inappropriate for individu-als with epilepsy may not be contraindi-cated if proper safety equip
ment is used.
In addition, many states have speciﬁc reg-
ulations protecting employers from exces-sive liability if injury occurs even thoughadequate safety precautions were main-tained. Work potential can be maximizedwith continued education of employers,adequate safety precautions, and consid-eration of individual needs. DIAGNOSTIC PROCEDURES USEDFOR CONDITIONS OF THE NERVOUSSYSTEMSkull Roentgenography (X-ray)
Roentgenograms (radiographic studies or X-
rays) of the skull provide visualization of
the bones making up the skull as well asstructures such as the sinuses. They arehelpful in identifying fractures or otherabnormalities of surrounding structures.X-ray ﬁlms are usually taken by a radiolo-
gy technician . The ﬁlms are then read and
interpreted by a radiologist (physician
who specializes in radiology).
Computed Tomography(CT Scan, CAT Scan)
A noninvasive radiographic technique
called computed tomography is a test that
applies computer technology and digitalimaging techniques to X-ray studies toproduce images of cross-sections of thebody. Unlike conventional roentgenogra-phy, CT shows one “slice” of the structurebeing studied at a time, in sequence. Onregular X-ray ﬁlms, bone (e.g., the skull)can block the view of parts lying behindit (e.g., the brain). CT scans show both thebone and the underlying tissue and areable to detect abnormalities that could notbe visualized on plain X-rays.
A scan of the head by CT can detect
tumors and blood clots inside the brain. It
can also reveal an enlargement of the ventri-
cles of the brain due to inadequate drain-age of CSF ( hydrocephalus ), as well as
other types of abnormalities in the brainand skull. It can also be used to identifytumors or other sources of pressure on thespinal cord.
Individuals who are undergoing a scan
by CT are placed within a large cylinderthat contains an X-ray tube and a recep-tor. Usually a special substance ( contrastDiagnostic Procedures Used for Conditions of the Nervous System 67

medium ) is administered to the individual
intravenously to highlight certain struc-tures and make the results more readable.The tube is rotated around the individual.X-rays are sent from the tube to the recep-tor, which measures the amount of radi-ation that each body tissue or organabsorbs during each rotation. A comput-er converts this
information to a visual
image on a screen. Images are monitored
on a video screen and later photographedfor more careful study by the radiologist ,
a physician who has been specially trainedin the ﬁeld of radiology and who readsand interprets results from radiologicaltesting.
Magnetic Resonance Imaging (MRI)
Magnetic resonance imaging (MRI) is a
noninvasive procedure that may be usedto obtain detailed information aboutbody organs, especially soft tissue. It isused in disorders of the brain to diagnoseor evaluate conditions such as braintumors, aneurysms or malformations ofblood vessels, strokes, multiple sclerosis,hydrocephalus, or abscesses. It may also beuseful in determining the extent of TBI. Inspinal cord injury, MRI may be used inconjunction with regular X-ray ﬁlms,myelograms , and scans obtained by CT to
identify spinal cord compression, swelling,or bleeding. In addition, MRI is very sen-sitive in detecting conditions such as her-niated disk or other destructive conditionsof the spine.
During the test individuals are placed in
a narrow cylinder. When in the cylin
der,
a strong magnetic ﬁeld of radio waves
causes biological substances in the body(protons ) to change their alignment and
become aligned in a certain direction.When the radio waves are discontinued,the protons return to their normal posi-tion. The change is recorded electronical-ly, and a computer translates the degree ofchange into highly detailed images thathelp physicians distinguish between nor-mal and abnormal body tissues.
Although the procedure is relatively
safe, it may be contraindicated for someindividuals. Because of the conﬁningnature of the cylinder used for the test,individuals with claustrophobia, thosewho are confused or agitated, or thosewith severe mental retardation may beunable to be tested. Likewise, individualswho are extremely obese may not be ableto be placed within the cylinder. OpenMRI machines that do not require individ-uals to be placed into a conﬁning cylin-der are available at some medical facilities.Because of the use of magnetic force inconducting an MRI, the test may be con-traindicated for individuals with cardiacpacemakers, metal implants, or othermetal fragments such as shrapnel becauseof potential injury.
Brain Scan (Brain Nuclear Scan)
Brain scan, also sometimes called brain
nuclear scan, uses radionuclides (radioiso-
topes ) to identify changes in brain tissue,
including tumors, infarction (death of tis-
sue), infection, or blockage of blood ves-sels in the brain. A small amount of theradioactive material (radionuclide) isinjected intravenously. The radioactivematerial localizes in areas of the brain thatare abnormal. A small camera records theconcentration of radioactive material thathas accumulated in various parts of thebrain. These data are then transcribed bya computer to form images on ﬁlm. Thescan is usually performed by a radiologist
or nuclear medicine technician .
A reﬁnement of nuclear scanning is the
single photon emission computed tomography(SPECT). This test uses computer methodssimilar to that of a CT scan (described68 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Diagnostic Procedures Used for Conditions of the Nervous System 69
above), and a scanning camera rotatesaround the body recording images of col-lections of radionuclides in areas of abnor-mality. SPECT scans are used to examineblood ﬂow to the brain.
The radiation hazard in nuclear scan-
ning is very slight because the dosage ofthe radionuclide is very small and theduration of the exposure brief. In manyinstances nuclear scanning can provideuseful information so that more danger-ous, invasive tests can be avoided.
Positron Emission TransaxialTomography (PET Scan)
In order to study the biochemical or
metabolic activities in cells of body tissue,positron emission transaxial tomographymay be used. Individuals are injected withor inhale a biochemical substance taggedwith a radionuclide . When the particles
from the radionuclide combine with par-ticles normally found in the cells of cer-tain tissues, they emit special rays ( gamma
rays) that a scanner can detect. The scan-
ner then translates these emissions intocolor-coded images. PET scans are able toassess chemical activities in body tissue,especially those related to blood ﬂow andmetabolism. Consequently, informationfrom the PET scan not only shows thestructure of an area of the body, but it alsoprovides information about how body tis-sues function. In the brain, this type ofscan can be used to evaluate tumors or dis-orders that may alter cerebral metabolism,such as Parkinson’s disease, multiple scle-rosis, or epilepsy.
Cerebral Angiography
Abnormalities of circulation in the brain
can be visualized radiographically throughcerebral angiography . Because blood vessels
cannot be readily observed on regular X-rays, a contrast dye must be injected inorder to view vessels on X-ray ﬁlms. Cere-bral angiography is considered an invasiveprocedure because a catheter is insertedinto an artery and radiopaque dye isinjected. A series of X-ray ﬁlms are thentaken. The test enables physicians to iden-tify blockages that may be interfering withblood ﬂow to certain parts of the brain.
Lumbar Puncture (Cerebrospinal FluidAnalysis, Spinal Tap)
When a laboratory analysis of an indi-
vidual’s CSF is needed, a lumbar puncture
is done. To remove the ﬂuid, a physicianinserts a needle into the subarachnoidspace of the spinal column at the lumbararea. The test may be done to determinewhether there is blockage of the ﬂow ofspinal ﬂuid, to detect any bleeding, todetect infection, such as encephalitis or
meningitis , or to identify other central
nervous system disorders. Although a lum-bar puncture is often performed for diag-nostic purposes, it can also be done fortherapeutic reasons, such as to reduceincreased pressure or to instill medica-tions. It is often performed in an outpa-tient setting under local anesthesia.
Electroencephalography (EEG)
A graphic recording of electrical activ-
ity of the brain ( brain waves ) can be
obtained through electroencephalography
(EEG). The procedure is helpful in identi-fying tumors, seizure disorders, and oth-er types of brain dysfunction, such as drugintoxication, or determining brain func-tion after brain injury. It may also be usedto evaluate sleep disorders.
EEG is a noninvasive procedure in which
electrodes are placed in various areas of the
scalp and connected to a machine thatrecords brain waves graphically. It may be

performed by a physician or by a special-ly trained technician. The test is usuallyevaluated by a neurologist (physician who
is trained to diagnose and treat conditionsof the nervous system).
Neuropsychological Tests
Neuropsychological tests are procedures
that are used to assess major functionalareas of the brain and to describe theimpact of brain dysfunction on manyareas of an individual’s life, includingemotional, social, educational, and voca-tional areas, to name a few. The tests areperformed by a clinical neuropsychologist ,
an individual with advanced graduatetraining in the ﬁeld of neuropsychology.Information gained from these tests maybe used for diagnosis, monitoring ofchanges, or treatment planning. In addi-tion to assessing cognitive processes, mostneuropsychological tests also assess per-ceptual and motor skills. Neuropsycho-logical tests can be used to assess memory,abstract reasoning, problem solving, spa-tial abilities, and emotional and personal-ity consequences of brain damage ordysfunction.
Examples of commonly used neuropsy-
chological tests are the Wechsler Intelli-gence Scales, the Wechsler Memory Scales,the Halstead-Reitan NeuropsychologicalTest Battery, and the Luria NebraskaNeuropsychological Battery.
PSYCHOSOCIAL ISSUES IN NERVOUSSYSTEM CONDITIONS INVOLVINGTHE BRAIN
Individuals with conditions involving
the brain experience many of the sameissues as individuals with other types ofdisability. Conditions involving the brainare often compounded, however, by emo-tional and cognitive changes that
caninterfere with individuals’ capabilities.
Unlike other chronic illnesses or disabili-ties, conditions involving the brain oftenaffect individuals’ social and behavioralresponses, and their impact can be greatfor both the individual and the family.Fuller discussion of psychosocial issues inconditions affecting the nervous systemcan be found in Chapter 3.
CASE STUDIESCase I
Mr. T. is a 35-year-old auto parts sales-
man who was injured in an automobileaccident when his car was hit head-on bya driver who was intoxicated. The driverof the other car was only slightly injured;however, Mr. T. sustained a closed headinjury. When admitted to the trauma unit,his Glasgow Coma Scale was recorded as9. He remained in a coma for eight days.
Mr. T. stayed at the trauma center for
four weeks before being transferred to arehabilitation center. While at the rehabil-itation center, he worked on learning howto walk, talk, and regain physical strengthand endurance. Residuals from his headinjury include mild dysarthria. In addi-tion, he has developed a seizure disorder,having experienced several grand malseizures for which he is currently receiv-ing medication, which appears to controlthe seizures.
Questions
1. What other potential residual effects
might you expect resulting from Mr.T.’s brain injury, in addition to thosementioned?
2. How might Mr. T.’s seizure activity
affect his rehabilitation potential?
3. Given Mr. T.’s residuals from his head
injury, what aspects of his former oc-70 C
HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

Case Studies 71
cupation as an auto parts salesmanwould you expect to be affected byhis injuries?
4.
What speciﬁc accommodations would
you expect Mr. T. to need because ofhis injury?
Case II
When Mr. G., a night watchman at a gas
plant, had his annual physical, he wasfound to have severe hypertension (highblood pressure). After ongoing monitoringand evaluation, he received a diagnosis ofhypertension and was placed on antihy-pertensive medication. Because Mr. G.experienced no symptoms, however, heoften failed to take his medication as pre-scribed, and he ﬁnally stopped taking italtogether. Several weeks later he collapsedat work, was taken to the emergencydepartment by ambulance, and was diag-nosed with left cerebral damage from astroke. After several weeks of hospitaliza-tion and rehabilitation, he returned home,but it was unclear whether or not hewould be able to return to work.
Questions
1. What residual effects of left-sided
cerebral damage might you expectMr. G. to have?
2. Although obviously you would need
more detailed information about Mr.G.’s functional capacity to deter-mine whether or not he might beable to return to work, how mightmanifestations from left-sided braindamage affect Mr. G.’s ability to be anight watchman at a gas plant?
3. What psychosocial issues might you
consider in Mr. G.’s case?
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4), S34–S38.72 C HAPTER 2 CONDITIONS OF THE NERVOUS SYSTEM : PARTI

NORMAL STRUCTURE ANDFUNCTION OF THE SPINAL CORDAND PERIPHERAL NERVOUS SYSTEMThe Spinal Cord
The spinal cord is part of the central
nervous system (see Chapter 1) andextends from the brain stem to the lowerpart of the back. Bony coverings calledvertebrae surround the spinal cord and
protect it. This bony covering as a wholeforms the vertebral column . The vertebral
column consists of 7 cervical vertebrae
located in the neck area; 12 thoracic verte-
brae located in the upper and middle
back; and 5 lumbar vertebrae located in the
lower back. The sacrum located below the
lumbar vertebrae consists of fused (joined)
bone. At the tip of the sacrum is the coc-
cyx, or tailbone.
The spinal cord conducts impulses to
and from the brain. The outer white mat-ter of the spinal cord, which consists ofbundles or tracts of myelinated ﬁbers ofsensory ( afferent ) and motor ( efferent )
neurons, conveys electrical impulses upand down the spinal cord between theperipheral nervous system (those nerveslying outside the central nervous system)and the brain. In most instances, sensoryinformation traveling up the right side ofthe spinal cord crosses over to the left sideof the brain, so, for example, the lefthemisphere of the brain would interpretpain in the right hand. Conversely, motorimpulses originating in the left brain crossto the right side of the spinal cord and ini-tiate a response to the right side of thebody. Because of this crossover effect,damage on one side of the brain typical-ly causes symptoms on the opposite sideof the body.
The inner gray matter of the spinal
cord, which is composed of cell bodiesand unmyelinated neurons, acts as a coor-dinating center for reﬂex and other activ-ities, such as voluntary movements andcontrol of internal functions. A reﬂex cen-ter in the gray matter of the spinal cordis where sensory and motor neurons con-nect. This part of the spinal cord serves asa center for spinal reﬂexes. A reﬂex can be
deﬁned as an automatic response to a giv-en stimulus. Spinal reﬂexes control notonly muscle reﬂexes but also the reﬂexesof internal organs.
The gray matter within the spinal cord
resembles an H. The projections of the Hare named according to the direction towhich they project. The posterior horns
extend toward the back, and the anteriorConditions of the
Nervous System: Part II
Conditions of the Spinal Cord and
Peripheral Nervous System and
Neuromuscular ConditionsCHAPTER 3
73

horns project toward the front. Cere-
brospinal ﬂuid, which nourishes and pro-tects the spinal cord, ﬁlls both the central
canal , located within the center of the
gray matter, and the subarachnoid spacesurrounding the outer portion of thespinal cord.
Motor ( efferent ) impulses originate in
the motor cortex of the brain, extenddown the spinal cord through descending
tracts , and exit through motor spinal
nerve roots that extend through openingsbetween the vertebrae that surround thespinal cord. Sensory ( afferent ) impulses
from the body enter the spinal cordthrough spinal nerve roots that alsoextend through openings between verte-brae and then travel up ascending tracts in
the spinal cord to the brain.
Spinal nerve roots are named for the
vertebral level from which they exit. Forexample, the nerve roots that leave thespinal cord at the cervical level are labeledC-1 through C-8, and the nerve roots thatleave at the thoracic level are labeled T-1through T-12. The sensory ( afferent )
nerves carry body sensations
into the sen-
sory nerve roots ( posterior roots )at the back
of the spinal cord, where they are thencarried up the spinal cord to the brain.Motor ( efferent ) impulses travel from the
brain down the spinal cord and exit frommotor nerve roots ( anterior roots ) at the
front of the spinal cord. Motor nerveﬁbers then carry impulses to the volun-tary muscles in the body.
Many types of neurons work together
to transmit impulses through the spinalcord. Sensory impulses entering the spinalcord at the lumbar region are relayed ver-tically to the brain through a number ofconnecting sensory neurons. Motorimpulses from the brain to the peripher-al nerves, however, are conductedthrough two separate categories of motorneurons. Upper motor neurons originate inthe brain and are contained entirelywithin the central nervous system. Lower
motor neurons , although originating in the
central nervous system, have fibersextending to the peripheral nerves in vol-
untary muscles. Dysfunction of eitherupper or lower motor neurons can gener-ally affect the voluntary muscles. Thelocation of the dysfunction determinesthe nature of the disorder.
The Peripheral Nervous System
A nerve is a bundle of ﬁbers outside the
central nervous system that transmitsinformation between the central nervoussystem and various parts of the body. Theperipheral nervous system consists of all
nerves that extend from the brain andspinal cord. In order to function effective-ly, the peripheral nerves must be connect-ed to the central nervous system. Someperipheral nerves connect directly to thebrain ( cranial nerves ), and others connect
directly to the spinal cord ( spinal nerves ).
Cranial and spinal nerves are essentiallinks between the rest of the body and thecentral nervous system.
The 12 pairs of peripheral nerves that
connect and transmit messages directly tothe brain are called cranial nerves . Some
cranial nerves contain only sensory ﬁbers,whereas others contain both sensory andmotor fibers. Cranial nerves mediatemany aspects of sensation and muscularactivity in and around the head and neck.Cranial nerves and their related functionsare illustrated in Table 3–1.
Peripheral nerves that connect and
transmit messages directly to the spinalcord are called spinal nerves . There are 31
pairs of spinal nerves. Each nerve dividesand then subdivides into a number ofbranches. Nerves at each level travel tospeciﬁc parts of the body, conveyinginformation between those areas and the74 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

Normal Structure and Function of the Spinal Cord and Peripheral Nervous System 75
central nervous system. Spinal nerves andtheir related functions are illustrated inTable 3–2.
Nerves control both voluntary and in-
voluntary functions in the body. Nervesthat control voluntary functions (such as
movement of the muscles in the extrem-ities) are called somatic nerves. Nerves that
are concerned with the control of invol-untary functions are part of a subcatego-ry of the peripheral nervous system calledthe a utonomic nervous system .
The autonomic nervous system inte-
grates the work of vital organs, such as theheart and lungs. Its primary function is tocoordinate the activity of internal organsso that they can make adaptive respons-es to changing external situations inorder to maintain internal equilibrium.Nerve ﬁbers monitor the activities ofinternal organs as well as changes in theexternal environment. When changes arenecessary to maintain internal homeosta-
sis(equilibrium), or to protect the body, the autonomic nervous systemstimulates immediate, involuntary re-sponses. For example, in response to aspeck of dust in the eye, tears are pro-duced. In response to a fearful situation,the heart beats faster.
Table 3–2 Spinal Nerves andRelated Functions
Spinal Nerve Area of Function
Cervical (C1-C8) Back of head, neck,
shoulders, arms, hands,diaphragm
Thoracic (T1-T12) Chest, back, regions of
abdomen
Lumbar (L1-L5) Lower back, parts of
thighs and legs
Sacral (S1-S5) Regions of thighs,
buttocks, legs, bowel,bladder, genital functionTable 3–1 Cranial Nerves and Related Functions
Cranial Nerve Area of Function
I. Olfactory SmellII. Optic VisionIII. Oculomotor Movement of eye musclesIV Trochlear EyelidsV. Trigeminal Sensation in head, face, and teeth, motor activity of chewingVI. Abducens Pupil dilation, focusing of lensVII. Facial Taste, sensation of external ear, control of salivary glands, tears,
muscles in facial expression
VIII. Vestibulocochlear Sensation of sound, balance, orientation of headIX. Glossopharyngeal Swallowing, sensation of pain, taste, touch from tongue and throatX. Vagus Heartbeat, digestion, speech, swallowing, respiratory function,
gland functions
XI. Accessory Movement of head and shoulders, muscles of pharynx and larynx
in throat, production of voice sounds
XII. Hypoglossal Tongue movement, speech, swallowing

The autonomic nervous system is divid-
ed into two subsystems:
1. The sympathetic nervous system2. The parasympathetic nervous systemThese two systems work together and in
opposition to control internal organs andregulate their function. Hormones andemotions can affect both systems.
The sympathetic nervous system be-
comes active during stress and emergen-cies. It prepares the body for action, deep-ening respirations, making the heart beatfaster, dilating the pupils, stimulating pro-duction of stress hormones, and increas-ing blood supply to the large muscles ofthe body. The parasympathetic nervoussystem dominates when the body is atrest. It activates mechanisms that focuson body conservation, such as decreasingthe heart rate and constricting the pupilsof the eye. The parasympathetic nervoussystem is also an important component ofsexual arousal in both males and females.
CONDITIONS AFFECTING THESPINAL CORDSpinal Cord Injuries
Spinal cord dysfunction can result from
a number of causes. Most often, it is dueto injuries from motor vehicle accidents,sports injuries, falls, or violence such asgunshot wounds. Other causes of spinalcord dysfunction are compression of thecord from conditions such as herniateddisc, spinal tumors, infectious disorderssuch as polio or tetanus, degenerative dis-orders such as multiple sclerosis, or con-genital disorders such as spina biﬁda.
Ultimately spinal cord dysfunction
results in some combination of sensory,motor, or reﬂex deﬁcit. When the spinalcord is injured, transmission of impulsesbetween the brain and other parts of thebody below the level of injury are inter-rupted. Consequently, some damage tomotor, sensory, or reﬂex function belowthe injury occurs. There may be numb-ness, complete paralysis, or exaggerated,absent, or diminished reﬂexes below thelevel of the injury. The extent of function-al impairment or loss depends on the partof the spinal cord injured and whetherthe cord is bruised, compressed, or sev-ered. For instance, in some cases, swelling,bleeding, or a tumor may compress thecord without severing it. In theseinstances, removal of the source of com-pression can, at times, restore function.When the spinal cord is severed, howev-er, transmission of nerve impulses may bepermanently lost or impaired.
When the spinal cord is completely sev-
ered ( complete spinal cord injury ), there is
no nerve function below the level of theinjury and hence no voluntary motor orsensory function below that level. If sev-erance is not complete
(incomplete spinal
cord injury ), some motor or sensory func-
tion below the level of the injury may beintact. In these instances, one portion ofthe spinal cord may be nonfunctionalwhile another portion maintains somefunction; or certain nerve tracts may stillbe functioning, but in an abnormal way.The term paraparesis refers to partial
paralysis and indicates that some functionremains below the level of the injury.Sometimes even when the spinal cord isnot completely severed, all motor andsensory function below the level of theinjury may be lost if the remaining nervesare destroyed from lack of blood supply,degeneration, or compression. In general,the higher the level of spinal cord injury,the greater the functional impairment.
Manifestations of Spinal Cord Injury
Symptoms of spinal cord injury specif-
ically reﬂect the level of the injury and76 C HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

the function of the neurons involved (Fig-ure 3–1). If spinal nerves are unable totransmit messages between the centralnervous system and the peripheral nerv-ous system, function below the level ofinjury will be disrupted. The degree offunctional loss is dependent on the degreeto which the spinal cord is injured. Forexample, if the afferent nerve roots in the
ascending tracts (sensory tracts ) are injured,
some sensory loss below the level of in-jury will exist. If efferent nerve roots in the
descending (motor tracts ) are injured, some
motor loss below the level of injury isexpected. When both sensory and motortracts are injured, as with complete sever-ance of the spinal cord, both motor lossand sensory loss occur below that level.
Ambulation is affected to some degree
regardless of the level of injury, with theexception of injury at the sacral level (S-2
through S-4), in which ambulation mayreturn to normal. Individuals with spinalcord injuries above T-12 usually require awheelchair for ambulation. At lower lev-els of injury, ambulation for short dis-tances may be possible with braces orcrutches.Conditions Affecting the Spinal Cord 77
C1C2C3C4C6C7C8T1T2T3T4T5T6T7T8T9T10T11T12L1L2L3L4L5S1S2S3S4S5C1
CoccygealNerveSacralNervesLumbarNervesThoracicNervesCervicalNerves
Figure 3–1 Spinal Nerves. Source: Reprinted with permission from M. J. Miller, Pathophysiology:
Principles of Disease , p. 380. © 1983, W. B. Saunders Company.

Most individuals lose voluntary control
of bladder and bowel function after spinalcord injury. Bladder evacuation may beaccomplished through external collectiondevices such as condom catheters , which
may be used for men; however, there is noequivalent device for women. Indwelling
catheters are still used in some instances
for both men and women, although be-cause of the risk of urinary tract infection,this practice is becoming less common.When feasible, intermittent catheterization
several times a day is recommended. Inlower-level injuries, such as at the lumbar
level, bladder evacuation may be accom-
plished by applying external pressure tothe lower abdomen. Bowel managementmay be accomplished through the use ofsuppositories or rectal stimulation; how-ever, the individual may still be con-cerned about involuntary bowel move-ments in public.
Spinal Cord Injuries at the Cervical Level
(C-1 through C-8)
Diving accidents, motor vehicle acci-
dents, or a blow to the head with a heavyobject may cause fractures of the cervicalvertebrae. An injury to the spinal cord atthe cervical level (C-1 through C-8) results
in quadriplegia (paralysis of both upper
and lower extremities). Injuries at C-1 orC-2 are often fatal because the function-ing of all muscles, including the musclesof respiration, is lost. Individuals who sur-vive these injuries require ventilatorysupport in order to breathe and are total-ly dependent on others for self-care. Indi-viduals with injuries at C-3 or C-4 alsohave compromised ventilatory capacityand will also require special respiratoryequipment, as well as be dependent onothers for self-care; however, at this levelof injury individuals may be able tomanipulate a wheelchair through the useof a mouth stick. Most individuals withspinal cord injuries at C-4 or above musthave an attendant for personal care,dressing, and transfers.
At C-5, some gross movement of the
upper extremities is possible, such as bend –
ing the arm at the elbow. Individuals maybe able to hold a light object between thethumb and ﬁnger, or they may be able tomaneuver small objects with the assis-tance of hand splints. Assistance will stillbe required for most activities; however,individuals may be capable of transfer ontheir own with the assistance of specialequipment. Total independent living isprobably not feasible, but independentelectrical wheelchair ambulation may bepossible. Individuals with injury at C-6also have gross motor movement of upperextremities and may be able to retainsome independence in self-care, such asby feeding and dressing with the aid ofspecial orthotic equipment. Propelling awheelchair manually may be possiblewith a modiﬁed hand rim, althoughmany individuals continue to operate amotorized chair. With the use of handsplints, individuals may also be able towrite. Independent transfer from bed tochair or to a car may also be possible, aswell as driving with the use of specialadaptive devices.
Individuals with C-7 injuries are capa-
ble of straightening their arm and are ableto sit up in bed, dress themselves, and
transfer, so almost total independence with
some adaptations in the environmentmay be achieved. Fine motor movementsof the hands are impaired, but writingmay be possible with the use of a specialdevice that can be strapped to the hand.Driving is possible with hand controls.With C-8 injuries individuals have somesensation in their hands and may becometotally independent with modiﬁed envi-ronment and some adaptive devices.78 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

Spinal Cord Injuries at the Thoracic Level
(T-1 through T-12)
Spinal cord injuries occurring at T-1 or
below result in paraplegia (paralysis of
the lower extremities). Upper extremities,
for the most part, are unimpaired, withthe exception of T-1 injuries, in whichthere may be slight weakness and someloss of ﬂexibility in the hands. Individualswith injury between T-1 and T-3 mayneed a brace or other support to maintainposture in an upright position becauseeven though the upper extremities arefunctional, the muscles of the trunk areparalyzed. In most cases, individuals withinjuries at T-1 through T-12 are able toattain total independence in self-care,wheelchair ambulation, and transfer. In-dividuals with injury at T-7 to T-12 maybe able to walk with the use of long legbraces; however, because of the strenuousnature of the activity, ambulation mayonly be possible for short distances.
Spinal Cord Injuries at the Lumbar Level
(L-1 through L-5)
Many of the muscles of mobility are in-
tact with L-1 through L-5 injuries. All up-per body muscles and many of the legmuscles are functional. Ambulation withbraces and/or use of cane or crutches maybe possible, especially for short distances.Individuals are able to gain total inde-pendence in care, although hand controlsstill may be necessary for operating amotor vehicle. Although bowel and blad-
der function are still impaired, reﬂex empty –
ing of bowel and bladder may be possible.
Spinal Cord Injuries at the Sacral Level
(S1 through S-4)
Ambulation is usually possible with lit-
tle or no equipment. Bowel and bladderfunction may still be impaired to somedegree.
Treatment and Management of
Spinal Cord Injury
Initial Treatment of Spinal Cord Injury
The initial treatment of spinal cord in-
juries focuses on preventing further injury,
stabilizing individuals’ physical condi-tion, and in some instances performingsurgery to realign the spinal column orachieve decompression of the spinal cord.Many individuals with spinal cordinjuries, especially those who received theinjury as the result of an accident, willhave other injuries, such as fractures,injury to internal organs, or brain injuries,that further complicate their care.
Individuals with injuries to the cervical
spine are usually placed in skeletal traction
to immobilize the spine. In some in-stances, individuals may have cervicalorthoses, such as a halo brace (Figure 3–2),
in which metal pins are inserted into theskull and attached to a metal “halo” that
surrounds the head. The “halo” is attached
with two metal rods to a “vest” worn onthe torso of the individual. The halo braceis used to allow mobility while keepingthe head and neck in proper position.Traction is usually not used to stabilizeand immobilize thoracic or lumbar frac-tures because there is no effective way toprovide it.
Postacute Treatment and Rehabilitation
After the condition has been stabilized
and acute medical needs met, individualsare usually transferred to a rehabilitationunit, where they learn skills or learn touse adaptive devices that will help themto achieve the maximum level of inde-Conditions Affecting the Spinal Cord 79

pendence. A wide variety of health profes-sionals are usually involved in this phaseof rehabilitation, including physiatrists,
physical therapists, rehabilitation nurses,occupational therapists, orthotists, psycholo-gists, social workers , and rehabilitation coun-
selors .
Physical therapy begins as soon as pos-
sible to prevent deformities such as con-
tractures (permanent contractions of a
muscle so that a joint becomes ﬁxed orimmobile) or footdrop, as well as to buildstrength. An immediate treatment goal isto have individuals with either paraple-
gia(involvement of lower extremities) or
quadriplegia (involvement of all fourextremities) placed into an upright posi-tion as soon as possible to prevent com-plications such as respiratory problemsfrom occurring. A tilt board or circular bed
is used to accomplish this goal. Individ-
uals are strapped securely to the tilt board
or circular bed while in a prone position.The board is then gradually raised or thecircular bed rotated until the individual isupright.
As the individual’s condition stabilizes,
treatment is directed toward teach
ing self-
care. Most individuals with spinal cord
injury become mobile with a wheelchair.Many types of wheelchairs are availablewith a variety of options, including de-80 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII
Figure 3–2 Halo Brace. Source: Copyright © 1999. Rachel Clarke.

tachable armrests and footrests, remov-able back panel, a lapboard,
and a carryall
bag. Power-operated wheel chairs are avail-
able for individuals who have little or nouse of their upper extremities. Thesechairs are battery operated and can becontrolled with a switch adapted to theparticular individual’s ability. Because ofthe size of the battery-operated wheel-chairs, they are heavy and therefore difﬁ-cult to transport.
Individuals are taught a variety of self-
care skills, including dressing, hygiene,and grooming. It may be necessary toinstall speciﬁc adaptive devices, such asgrab bars and a raised toilet seat, in thehome. Because most spinal cord injuriesaffect bladder and bowel function, in-structions in catheter care and bowel re-training are usually necessary.
Potential Complications Associated with
Spinal Cord Injury
Individuals with spinal cord injury are
at risk of developing additional healthproblems that could result in a secondarydisability and consequently additionalfunctional limitations. The extent of therisk is related to the level of injury. In gen-eral, the higher the level of injury, thegreater the risk of developing secondarydisabling conditions. It is therefore imper-ative that individuals with spinal cordinjuries, family members, and profes-sionals working with them are aware ofthe risk and use prevention strategies tolessen the risk.
Altered Symptoms of Illness
Because of the lack of sensation that
accompanies most spinal cord injuries, aswell as the interruption to nerve path-ways, symptoms of various conditions un-related to the spinal cord injury itself maybe not be recognized, and consequentlymay not receive prompt treatment. Forexample, because pain is not felt, appen-dicitis may not be discovered until theappendix ruptures. In some instancessymptoms may be expressed differently inindividuals with spinal cord injury thanin individuals without spinal cord injury.For example, those without spinal cordinjury may experience severe ﬂank painin response to a kidney infection; howev-er, individuals with spinal cord injurymay, instead, experience an abrupt in-crease in spasticity. As a result, the symp-tom may not be recognized as beingrelated to kidney infection and the kidneyinfection may not be immediately diag-nosed and treated. Individuals with spinalcord injuries, caregivers, and profession-als should be made aware of this alter-ation of symptom presentation andshould be alerted to report or investigatenew symptoms or accentuated old symp-toms as soon as they are noted.
Pressure Sores (Decubitus Ulcers)
One of the most common complica-
tions associated with spinal cord injury ispressure sores ( decubitus ulcers ), which
result from lack of blood supply to a bodypressure point, such as the buttocks, sac-rum, heel, or back. Pressure sores developwhen continuous pressure is exerted to abody part over time (Pires & Adkins, 1996;Woolsey & McGarry, 1991). Pressure on abody part interferes with blood supply,eventually resulting in breakdown andulceration of the skin. Because individu-als with spinal cord injury are oftenimmobile, areas of pressure on certainbony prominences are more likely todevelop. Since these individuals usuallyhave no sensation below the level ofinjury, they are unable to feel pressure,Conditions Affecting the Spinal Cord 81

and because of the paralysis they areunable to easily shift their weight torelieve the pressure. Inadequate skin care,irritation, and nutritional deﬁciency canfurther contribute to the development ofthe problem. Pressure sores may appear tobe small on the surface, but the depth ofthe ulcer may be more extensive. Un-treated pressure sores can progress fromredness to breakdown of the skin, infec-tion, and eventually death ( necrosis ) of
skin tissue, which could extend throughthe tissue all the way to the bone. Thuspressure sores are not only debilitatingbut can be potentially life-threatening.
Individuals with spinal cord injury
must be aware of the risk of pressure soresand the importance of monitoring theirskin. Education about the importance ofdecreasing the amount of pressure onbony prominences by regularly changingposition, maintaining good nutrition,and following good skin care is an impor-tant part of the rehabilitation process. Anumber of prescribed wheelchair cush-ions are available that can help distributepressure to prevent skin breakdown andendurance in a wheelchair. In addition tohaving the correct cushion prescribed,individuals with spinal cord injury shouldalso be helped to learn how to positionthem properly.
Urinary Tract and Bowel Complications
Urinary tract infections are the second
highest reported complication of spinalcord injury. Because the bladder is emp-tied in abnormal ways, it may not emptyoften enough or may not empty com-pletely. When this happens, the urine leftin the bladder acts as a reservoir for infec-tion. Because individuals with spinal cordinjury are generally unable to controltheir bladder, they may need to have acatheter inserted into the bladder to drainurine and prevent incontinence. Thebladder and its contents normally containno pathologic organisms, but there isalways the potential for the introductionof infectious organisms when a catheter isinserted. For individuals with spinal cordinjury, urinary tract infections can be aserious, debilitating, and, at times, life-threatening problem. Untreated urinarytract infection can lead to pyelonephri-
tis(infection of the kidney) and, in
severe cases, septicemia (infection in the
blood).
Because of the inactivity after paralysis,
the amount of calcium in the blood in-creases. As a consequence, the risk of de-veloping kidney stones ( renal calculi ) is
increased. The stone may form in the kid-ney itself or may lodge in the ureters
(tubes leading from the kidney to thebladder) so that they obstruct urine ﬂow,causing urine to back up into the kidneys(urinary reﬂux ), and eventually causing
damage to the kidney itself.
Education of individuals with spinal
cord injury about the urinary tract andthe risk of infection or stone formationand how to decrease this risk is crucial topreventing secondary urinary tract com-plications. In addition, individuals withspinal cord injury should be made awareof the importance of self-monitoring andpromptly reporting symptoms they maybe experiencing so that prompt diagnosisand treatment may be instituted.
Secondary conditions related to bowel
elimination may also be problematic. In-continence of fecal material may not onlycontribute to skin breakdown and urinarytract infection, but may result in socialisolation if individuals become concernedabout the probability that incontinencewill occur. Other problems may relate toimpaction (fecal matter that becomes
hardened and is unable to be evacuated)or in some instances to a condition called82 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

paralytic ileus , in which the intestine
ceases to function.
Individuals can decrease the risk of
these conditions by establishing a patternof regular elimination, monitoring dietand ﬂuid intake, and learning speciﬁctechniques for enhancing optimal bowelfunction.
Contractures
Contractures (loss of range of motion,
or ﬁxed deformity of a joint) may occur in
paralyzed limbs if the joints are not moved
through their regular range of motion.
Contractures of the upper extremities in in-
dividuals with quadriplegia can interferewith the use of assistive devices. If indi-viduals with paraplegia or quadriplegiadevelop contractures of the hip or knee,
it may be difﬁcult to assume adequate posi-
tioning in a wheelchair. Regularly movingthe joints through the full range of mo-tion through passive exercises conductedby another person, or by using special
equipment, can prevent contractures from
occurring. In addition, proper wheelchairseating as well as positioning of joints canhelp reduce risk of contractures.
Spasticity
Spasticity refers to the exaggerated
involuntary movement of paralyzed mus-cles. Although spasticity may be absentimmediately after injury, it can occur longafter individuals leave the rehabilitationfacility. Because communication betweenthe peripheral nervous system and the brain
is interrupted by spinal cord injury, sig-nals received by the peripheral nerves are
“short–circuited.” Rather than traveling tothe brain to be interpreted and appropri-ately adapted, they
instead return from
the spinal cord direct ly to the muscle. The
resulting muscle con traction can some-times be violent and can occur with evenslight stimulation. Spasticity can be debil-itating, not only because it is disruptiveand can potentially cause embarrassmentto the individual, but also because insome instances it can be so strong that itcauses individuals to fall from theirwheelchairs. Spasticity can also contributeto the for
mation of contractures. Although
in some instances spasticity can be useful
to help individuals perform certain func-tions such as shifting position or stand-ing, more often it is a source of discom-fort. When spasticity is a cause of con-cern, individuals may be helped to learna program of stretching that may helpdiminish occurrences. In other instancesmedications may be used to reduce spas-ticity; however, generalized weakness, theside effects of sedation, or other sideeffects may make this treatment lessdesirable. In severe cases when othertreatments are ineffective in controllingspasticity, individuals may resort to sur-gery, such as rhizotomy (surgical resec-
tion of a nerve root) to relieve spasticity.
Osteoporosis
Bone is a dynamic substance that is
continually depositing and reabsorbingcalcium. The combined stress of weightbearing and muscle pull that occurs withnormal activity helps bones maintaintheir calcium content. Inactivity can con-tribute to softening and weakening ofbones ( osteoporosis ). Individuals with
spinal cord injury have an increased rateof calcium removal from the bone and areconsequently more susceptible to frac-tures, which could be caused from fallsbut also from a simple activity such as awheelchair transfer. Calcium that isexcreted through the urinary system canalso contribute to urinary tract stones, asnoted above. In some instances calcium isConditions Affecting the Spinal Cord 83

deposited in soft tissues so that functionof the joint or muscle is disrupted.
Adequate diet, passive exercise and
strength-building exercises, and electricalstimulation to the muscles can be used to
help reduce risk. In addition, proper train –
ing in safety procedures when operatinga wheelchair and transferring can helpprevent falls and potentially broken bonesif osteoporosis is present.
Cardiac and Respiratory Problems
In the initial stages after injury, individ-
uals are susceptible to thrombophlebitis
(formation of blood clots in the legs) orpulmonary embolism (a blood clot that
travels to the lungs), a serious and poten-tially life-threatening disorder. As theindividual’s condition stabilizes, this con-dition becomes less of a treat. In the ini-tial period after injury, individuals mayalso experience a condition called
ortho-
static hypotension , in which blood pres-
sure becomes signiﬁcantly lower whenthe individual is moved from a ﬂat toupright position.
Although these conditions become less
prominent after the ﬁrst month, individ-uals with spinal cord injury continue tobe more prone to respiratory disorders, es-pecially conditions such as pneumoniathat can be debilitating as well as life-
threatening. Individuals with high cervical
or high thoracic injuries, because of weak-ened chest muscles, have more difﬁcultyexpanding the lungs and clearing
respira-
tory secretions. Consequently, they are
more susceptible to infection of the lungs.
Individuals with spinal cord injury also
have a more sedentary lifestyle, whichcan affect the cardiovascular system. Be-cause of the increased susceptibility tocardiovascular conditions, they shouldrefrain from smoking or using tobaccoproducts or drinking alcohol excessively.Good nutrition, an exercise program, andweight control are also important in pre-venting cardiovascular disease. Because ofthe increased risk of cardiovas
cular condi-
tions, individuals should have regular
medical examinations and comprehen-sive health care programs that accommo-date their needs.
Autonomic Dysreﬂexia
Autonomic dysreﬂexia is an abnormal
reﬂex condition characterized by a sud-den rise in blood pressure, profuse sweat-ing, and headache as the result of exces-sive neural discharge from the autonom-ic nervous system. It may be triggered byevents as simple as overdistension of thebowel or bladder. Unless the individualreceives immediate treatment to decreasethe blood pressure, there is risk of stroke.Autonomic dysreﬂexia commonly occursin individuals who have experienced aninjury to the upper spinal cord. Identify-ing and preventing the situations or con-ditions that trigger autonomic dysreﬂexiaare important to prevention.
Other Neurological Complications
Diaphoresis (profuse sweating) and
paresthesia (abnormal painful sensations
below the level of injury) are other poten-tial sequelae of spinal cord injury.
Sexual Dysfunction
Nerves to the genital region are almost
always affected to some degree by spinalcord injury. This does not mean, howev-er, that other aspects of sexuality, such assexual attraction to others, sexual desire,and the need to express oneself as a sex-ual being, are changed. Many men andwomen remain sexually active after spinalcord injury; however, in most instances,84 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

modiﬁcations of sexual behavior andfunction may need to be made.
Genital function is controlled by the
parasympathetic and sympathetic nerv-ous systems as well as by motor nervesand will be dependent on the level of in-jury and on whether the injury is com-plete or incomplete. Consequently, thehigher the level of injury, the more signif-icantly will genital function be affected.Most individuals with spinal cord injury,both males and females, will have littlesensation directly in the genital area.Because mobility is affected, they will alsoneed to alter their technique in sexualperformance.
Many males, regardless of level of in-
jury, continue to have reﬂex erections, and
in many instances erection can be pro-duced through manual stimulation.
The
ability to produce an erection through psy-
chological arousal is absent in most menwith spinal cord injuries; however, theymay have a weakened sexual re
sponse due
to psychological stimuli. Some individuals
have used techniques such as penileimplants to achieve intercourse. Ejacula-tion is absent for most men with spinalcord injury, or, if it does occur, individu-als experience retrograde ejaculation, sothat semen is deposited into the bladderrather than externally. As a result, fertili-ty in males is signiﬁcantly affected, espe-cially males with complete severance ofthe spinal cord. Some techniques, such aselectro ejaculation , in which ejaculation is
stimulated through electrical means, havebeen used so that sperm can be obtainedfor artiﬁcial insemination.
Females with spinal cord injury are still
able to engage in sexual intercourse, butlubrication in response to psychologicalarousal is usually absent. Menses typical-ly are absent the ﬁrst months after injury;however, with the return of menstruation,in most instances female fertility is unal-tered. Consequently, women who do not
wish to become pregnant need to use someform of contraception. When women with
spinal cord injury become pregnant, theyare able to carry the pregnancy to term,although because of altered sensation, itmay be more difﬁcult for them to deter-mine when labor begins.
Psychosocial Issues in Spinal Cord Injury
Spinal cord injury interrupts and alters
not only physical functioning, but psy-chosocial functioning as well. Individualswith spinal cord injury, in addition toexperiencing changes in movement andsensation, decreased mobility and inde-pendence, and changes in bowel andbladder functioning and sexual function-ing, also experience altered self-conceptand in many instances loss of self-
esteem.
Frustrated goals, loss of self-regard, or loss
of the illusion of omnipotence and con-trol can result in internalized anger, anx-iety, and guilt. To a great degree, howindividuals adjust will be related to howthey conceptualize the losses they experi-ence, to their individual coping style andto the amount and type of social supportavailable.
Although depression is common after
spinal cord injury, it is not universal andis not necessary for adjustment to occur(Cushman & Dijkers, 1991). Some indi-viduals are more likely to exhibit depres-sive symptoms after spinal cord injurythan others. Those who had difﬁcultycoping with stress prior to the injury orwho had a history of substance abuse orrelationship problems demonstrate in-creased difﬁculty adjusting after injury,whereas those who have greater personalresources and demonstrated optimal ad-justment prior to injury are more likely todisplay adequate adjustment after injury(Elliot & Frank, 1996).Conditions Affecting the Spinal Cord 85

Lack of social support is strongly asso-
ciated with depressive symptoms afterspinal cord injury (Rinala, Young, Hart,Clearman, & Fuhrer, 1992). Outward dis-plays of depressive symptoms may resultin avoidance by others. Thus the individ-ual becomes isolated, which in turn caus-es more depression. In general, individ-uals who demonstrate a greater internallocus of control also demonstrate less psy-chosocial distress and better adaptation(Livneh, 2000).
Not only must individuals with spinal
cord injury incorporate new behaviorsand
mobility techniques in order to func-
tion, but they must also continuallyadapt to their changing environment.Although spinal cord injury causes radicalchanges in mobility and independence,most individuals are able to return totheir community, and many to their ownhome with environmental modiﬁcations.The degree of successful reentry into thecommunity is largely dependent on theindividual’s social support, access to ade-quate housing and transportation, andthe availability of quality attendant careif needed.
In some instances, establishing new
relationships and/or reestablishing ormaintaining old ones may be difﬁcult.Relationships may need to be reexaminedand redeﬁned. Signiﬁcant others may ex-perience many of the same or parallelreactions and losses as the individual withspinal cord injury. The sudden incapaci-ty of a family member or signiﬁcant oth-er due to spinal cord injury may result inshock, denial, anger, or depression in oth-er family members. If they think the causewas avoidable or blame the individual orothers for the injury, they may exhibithostility, pessimism, anxiety, and higherlevels of social distress. Family relation-ships may be strained if family caregiversexperience stress related to ﬁnances or tohelping individuals with activities of dai-ly living and self-care. Family membersand signiﬁcant others may need the samedegree of help and support as those indi-viduals with spinal cord injury so thatthey can cope with the condition and inturn offer support to the individual. Roleobligations may need to be shifted, nego-tiated, and shared.
The focus of many rehabilitation pro-
grams for spinal cord injury is on helpingindividuals attain maximum functionali-ty related to self-care activities or em
ploy-
ment, with less attention being given to
recreational activities that could enableindividuals to become active in a largersocial sphere. The lack of structured peerrecreation activities and peer support canlead to social isolation and prevent indi-viduals from living effectively with theirdisability (McAweeney, Forchheimer, &Tate, 1996). After spinal cord injury, thelevel of participation in social and recre-ational activities will be dependent on theattitude and interests of the individual,the number of recreational opportunitiesand resources available, and the degree ofaccess to appropriate adaptative devicesand adequate sources for equipment re-pair. Adaptive devices that enable individ-uals with spinal cord injury to participatein many sports and other recreationalactivities are available; however, adequatetransportation, quality attendant care ifneeded, and other environmental restric-tions must also be considered.
Increased access to public buildings,
businesses, and services enables individu-als with spinal cord injury to participatemore fully in a broader range of commu-nity activities and to explore and pursuea number of social roles; however, archi-tectural and attitudinal barriers still exist.Consequently, in order to live most effec-tively with their disability, individualswith spinal cord injury must also learn to86 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

assert the importance of their own needsand advocate for themselves.
Because of the physical needs resulting
from spinal cord injury, individuals mustmodify their daily routines to functioneffectively in personal, social, or occupa-tional spheres. For example, extra timemay be needed for daily activities related
to personal hygiene. Individuals may need
to conform daily routines to a structuredbowel and bladder program. Transporta-tion, or moving from one place to
anoth-
er, may require extra time and plan ning.
Awareness of the demands involved inroutine self-care will help individualsestablish and adjust daily routines so thatthey can participate in social and occupa-tional activities.
Individuals with spinal cord injury
continue to be sexual beings. Sexualadjustment is an integral and necessarypart of total psychological adjustment.Changes in sexual functioning as a resultof spinal cord injury may be a source ofextreme frustration for adults. Those whoexperience spinal cord injury in adoles-cence or preteen years may have addedchallenges with acceptance, self-worth,and self- esteem. Discussion and accom-modation of sexual needs as well as reas-surance that sexual expression is stillpossible in their life are an important partof rehabilitation.
Individuals should be provided with
opportunities to obtain accurate and com-plete information about sexual activity inspinal cord injury, and information
should be provided in the context of their
personal values.
Vocational Issues in Spinal Cord Injury
Unless individuals with spinal cord in-
jury have an associated brain injury, theyshould experience no cognitive deﬁcits.The level of injury determines, to a greatextent, the amount and kind of activity inwhich they are able to engage and whatassistive devices or special accommoda-tions may be needed. Individuals withvery low spinal cord injuries (sacral orlumbar level) may be able to walk shortdistances with the assistance of braces andcrutches, whereas those with higher lev-els, such as at the thoracic level, will prob-ably require a wheelchair for mobility;individuals with cervical injuries willrequire a powered chair. When the injuryis in the cervical area, they will have lim-ited ability to use the upper extremities,and at the highest levels of injury theywill be unable to use the upper extremityat all. Consequently, special adaptiveequipment will be required to use a tele-phone, computer, or other equipmentthat requires hand use.
Environmental barriers such as steps,
table heights, and width of doorways willneed to be considered in the work envi-ronment. Since many individuals withspinal cord injury also have difﬁculty
with
temperature regulation, the work en-
vironment should be climate controlled.
The individual’s preinjury education
and vocational interests and skills, as wellas his or her functional capacity after in-
jury, are important considerations in voca –
tional placement. Age or the presence of ﬁ-
nancial disincentives are also factors that
inﬂuence the individual’s employment status.
Spinal cord injury is a lifetime disabili-
ty. Consequently, periodic checkups should
be instituted to identify those who areexperiencing difﬁculty or encounteringnew barriers to access in the workplace, sothat appropriate accommodations can beinstituted (Roessler, 2001).
Spina Biﬁda
Spina biﬁda is one of several different
congenital conditions known as neural tubeConditions Affecting the Spinal Cord 87

defects . These defects involve incomplete
development of the brain, spinal cord, and/
or coverings of these structures. Otherneural tube defects besides spina biﬁda areanencephaly , in which infants are born
with underdeveloped brains and incom-plete skulls, and encephalocele , in which
infants are born with a hole in the skullthrough which brain tissue protrudes. Inmost cases infants with either of theseconditions do not survive, or if they do,
they experience severe mental retardation.
Spina biﬁda, however, does not involve
the brain but rather the spinal columnand is a condition in which one or morevertebrae are left open so that the spinalcord is exposed.
Types of Spina Biﬁda
There are three types of spina biﬁda:•Spina biﬁda occulta refers to an
opening in one or more vertebrae ofthe spinal column. This is the mildestform of spina biﬁda, which does notinvolve any damage to the spinalcord. Many individuals with thisform of spina biﬁda may be unawarethat their condition even exists.
• Spinal meningocele refers to a more
serious type of spina biﬁda. In thisform of the condition the meninges
(protective coverings around thespinal cord) protrude through theopening in the spinal column. Theprotruding part is called a meningo-cele and contains only the meninges,not portions of the spinal cord. Insome cases surgery can correct thisproblem so that there is little or nodamage to the nerves of the spinalcord. In other instances, however,individuals with a spinal meningo-cele may have residual effects result-ing from spinal cord damage. •Myelomeningocele , the most com-
mon and most severe form of spinabiﬁda, is a condition in which nervesof the spinal cord as well as the
meninges protrude through the open-
ing of the vertebrae to the outer partof the body. Because there is no pro-tective covering for the cord andmeninges, spinal ﬂuid may leak fromthe protrusion, and the risk of infec-tion is great. When this defect occurs,it usually results in paraplegia (paral-
ysis of the lower extremities) as well
as poor bladder and bowel control. Al-
though surgery is usually performedimmediately to correct the defect,symptoms of paralysis in the lowerextremities usually persist.
Manifestations of Spina Biﬁda
Manifestations of spina biﬁda depend
on the type, the part of the spinal cordaffected, and the severity of the condi-tion. They can range from mild, in whichthere are few if any symptoms, to severe,in which there is muscle paralysis, loss ofsensation, and loss of bowel and bladdercontrol. Many children with the severe
type of spina biﬁda also experience hydro-
cephalus , a condition in which ﬂuid
builds up in the brain. In cases of hydro-cephalus, surgical implantation of a shuntis necessary so that the ﬂuid can bedrained to prevent excessive pressure onthe brain. If hydrocephalus is not correct-ed, mental retardation can result.
Because spina biﬁda is congenital, more
severe forms of the condition impinge onnormal motor development. Dependingon the social, economic, and psychologi-cal circumstances of the individual andhis or her available resources, cognitivedevelopment could also be affected. Al-though the condition itself is not progres-sive, problems associated with the condi-88 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

tion may increase over time. For example,in more severe cases when paralysis ispresent, uneven posture compounded byvertebral abnormalities may lead to scol-
iosis (lateral S-shaped curvature of the
spine). Scoliosis can lead to respiratoryproblems, impede effective functioning ofother internal organs, and decrease en-durance. Paralysis and associated boweland bladder problems can also predisposeindividuals to develop decubitus ulcers
(pressure sores). In addition, bowel andbladder problems increase the potentialfor chronic urinary tract infections.
Adult males with more severe forms of
spina biﬁda may have difﬁculty maintain-ing erection and may have difﬁcultieswith fertility. Conversely, females
may be
capable of engaging in sexual rela tions and
have normal fertility, but sensation to thegenital area may be absent.
Treatment and Management of
Spina Biﬁda
Since the condition is obvious at birth,
treatment is usually instigated within 24hours of birth. Treatment depends on theextent of the neurologic problems, thelevel of the lesions, and any complica-
tions, such as hydrocephalus or infection ,
that may be present. The early surgical in-terventions that are available today havesigniﬁcantly increased the survival rate ofpeople with spina biﬁda, as well as theirquality of life. In addition, greater aware-
ness of the potential for complications has
enabled health care personnel to treat the
symptoms of complications as soon as they
occur and also to take active measures toprevent complications from occurring.
Psychosocial Issues in Spina Biﬁda
Spina biﬁda, as a congenital condition,
produces important variances in life ex-periences that have a potential impact onthe psychological and social develop-ment of the child. The degree to whichthe child achieves maturity and indepen-dence in later life is largely shaped by thebiological, psychological, and social ex-periences of childhood.
When a child is born with a congenital
disability, common parental reactions in-
clude denial, guilt, anxiety, rejection, anger,
or overprotectiveness. If, during this vul-nerable time, parents are not providedwith necessary support, parent-infantattachment and bonding may be altered,leading to disorders of parenting. Child-rearing style has a profound effect on thechild’s personality development. Parentswho do not establish norms and expecta-tions for a child’s behavior may create apsychosocial disability that has a greaterimpact than the physical disability asso-ciated with the congenital condition. Inaddition, a secondary disability of socialisolation may result from the amount oftime needed for medical care and hospi-talization. Early intervention, active stepsto promote socialization, and familycounseling may help to overcome manyof these problems.
A normal part of development is grad-
ual separation of parent and child emo-tionally. When this does not occur be-cause of parental overprotectiveness oroverinvolvement, the child may experi-ence prolonged dependence and the in-ability to take control, which, in turn,adversely affects normal developmentand delays or impedes his or her ability toform an independent identity. As a result,the individual may develop emotionaldependence and remain in the parents’home past maturity rather than establish-ing his or her own living environment.
Children with spina biﬁda and the
resulting physical limitations may not beprovided the same opportunities to testConditions Affecting the Spinal Cord 89

their physical and intellectual capabilitiesas are provided to able-bodied individualsof the same age group. Doubting thechild’s capabilities or setting expectationsthat are too high or too low can alsocontribute to low self-esteem and in-creased dependence. At times, in anattempt to boost the child’s self-concept,parents, teachers, and others may show-er a child with attention, emphasizing orhumoring unrealistic expectations. In sodoing, they may foster an egocentric per-sonality that may be more of a handicapthan any physical limitation the childexperiences.
During adolescence, when a normal
part of development is body image andthe quest for identity, the child with spinabiﬁda may experience anxiety over ap-pearance, acceptance by peers, and sexu-ality. Difficulties with interpersonalrelationships may rise from limited expe-rience in learning and practicing socialskills. Helping the individual develop ap-propriate social skills throughout his orher development can help him or herform relationships during adolescenceand adulthood.
Sexual education is important regard-
less of the disability or the age of its occur-rence. In the case of congenital dis-ability,
issues of sexuality may be ignored as the in-
dividual reaches adolescence. As a result,
individuals may have limited op portuni-
ty to explore or express sexual desires.
Anticipatory guidance provided to par-
ents from the time they are ﬁrst told about
their child’s diagnosis can be extremelyhelpful in preventing many of the prob-lems that can affect the child’s psychoso-cial development and can help the childgain full affective and personality growthand maturity. As the child goes througheach stage of development, new needsand new demands arise. Social encountersoutside the home should be encouragedas well as participation in sports, camp-
ing, and other adaptive recreational events
designed to promote physical independ-ence and social maturity.
Vocational Issues in Spina Biﬁda
Unless the individual has associated
mental retardation because of other com-plications associated with spina biﬁda,intellectual ability should not be altered.Limitations associated with spina biﬁda
are dependent on the severity of the condi –
tion. Those individuals with paraplegiahave the same functional limitations asindividuals with paraplegia from othercauses. Vocational success and ability to
function in society are determined primar –
ily by the emotional and personality devel-
opment achieved throughout childhood.
Poliomyelitis and Post-Polio Syndrome
Poliomyelitis, also known as infantile
paralysis , or polio, is an acute infectious
viral disease that was prevalent in theUnited States in the ﬁrst half of the twen-tieth century. The virus enters the bodywhen contaminated water or food is in-gested or when hands that have been con-
taminated with the virus touch the mouth.
Poliomyelitis affects the nerve cells
that control muscles. The brain stem,spinal cord, and neuromuscular systemmay be affected (Salcido, 2000). Thenerve cells or motor neurons affected by the
poliovirus are located in the anterior horn
of the spinal cord and extend to the mus-cles. As neurons are affected, muscle cellslose the ability to contract, resulting inparalysis. If motor cells are able to over-come the virus, paralysis may be tempo-rary. Motor cells unable to overcome thevirus, however, die, resulting in perma-nent paralysis or in some instances weak-ness of affected muscles. The extent of90 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

paralysis is unpredictable. Although thedisease primarily affects children, thedevastating epidemics of polio that spreadacross North America and Europe fromthe 1930s to the mid-1950s severely dis-abled adults as well.
In 1955 Dr. Jonas Salk developed the in-
activated poliovirus vaccine which wasfollowed in 1960 with the development ofa live, attenuated oral poliovirus vaccineby Dr. Albert Sabin. As a result of the vac-cines, widespread immunization againstpolio was begun, and polio is now nearlyabolished in the United States and othercountries in which an immunization pro-gram is
widely available. But although
poliomyel itis has been nearly eradicated
in the industrialized world, the residualsof the
condition experienced by those
who con tacted the disease prior to immu-
nization are still present. In addition,small outbreaks continue to occur indeveloping countries, and a few cases con-tinue to
appear in the industrialized world
as well.
Manifestations of Poliomyelitis
Individuals in the initial stages of polio
are acutely ill. Initial symptoms are usu-ally nonspeciﬁc, such as gastrointestinalor upper respiratory symptoms accompa-nied by fever. Symptoms later progress toheadache, stiff neck, and muscle pains.Affected muscles become paralyzed or
weakened. In some individuals only a small
group of muscles are affected; in others,paralysis is widespread and may includethe whole body. Extremity involvement isoften asymmetrical, so that one extremi-ty may have major paralysis while theopposite limb has only slight weakness ormay not be affected at all. Although mus-cles are paralyzed, functions of sensation,bowel and bladder control, and sexualresponse are left intact.When an extremity is involved in child –
hood during a time of continued growth,the rate of growth of the affected extrem-ity is delayed, resulting in a smaller ex-tremity when full growth is reached. Thelegs are most frequently affected; how-ever, sometimes all four extremities areaffected, sometimes only one extremity isaffected, or sometimes paralysis extendsto only the lateral half of the body ( hemi-
plegia ), with one arm and one leg being
affected. This type of polio is called para-
lytic polio . When the poliovirus affects the
brain stem, the muscles that controlbreathing and swallowing are also affect-ed. This is called bulbar polio . When res-
piration is affected, individuals requiremechanical respiratory support such asthe “iron lung.”
Functional limitations resulting from
polio depend on the nerves affected andthe degree of damage. Individuals withaffected lower extremities have difﬁcultywith ambulation and may require awheelchair, cane, or braces. When upperextremities are involved, self-care skillsmay also be affected. If the trunk musclesare affected, a muscle imbalance mayresult, lead to scoliosis (lateral curvature
of the spine), which can interfere withbreathing as well as the functioning ofinternal organs.
After the initial acute episode of polio-
myelitis, some degree of function mayreturn, but some of the residuals are per-manent. The degree of residual disabilityis dependent on the extent of the perma-nent damage to nerves that has occurred.
Manifestations of Post-Polio Syndrome
Poliomyelitis itself is not a progressive
condition. Consequently, many individu-als who contracted the disease 30 or moreyears ago adapted to residual paralysis,muscle weakness, or other symptoms andConditions Affecting the Spinal Cord 91

went on to live productive lives with lit-tle medical intervention needed. In the1980s, however, individuals who had
previously been diagnosed as having polio-
myelitis began to seek medical advicebecause of new symptoms that rangedfrom mildly to severely debilitating. Atﬁrst they were not taken seriously. Manywere classiﬁed as having “emotional dis-turbances,” or symptoms were merelyattributed to “aging.” As more and moreindividuals who had had polio soughthelp for new symptoms, however, theirsymptoms were taken more seriously andthe term post-polio syndrome was coined to
describe this phenomenon.
Post-polio syndrome is a noncontagious
neurological disorder that produces a vari-
ety of symptoms in individuals who hadrecovered from poliomyelitis many yearsearlier. Common symptoms of post-poliosyndrome appearing 30 to 40 years afterthe acute bout of poliomyelitis include:
• abnormal muscle fatigue as well as
generalized fatigue
• new muscle weakness in muscles not
previously affected
• muscle pain ( myalgia ) and/or joint
pain
• respiratory difﬁculty
The cause of post-polio syndrome is un-
known (Burk & Agre, 2000). It appearsthat most of the motor neurons original-ly damaged in the initial bout of polio areinvolved in post-polio syndrome andthat most individuals who had polio areat risk to develop the syndrome. Individ-uals who initially had experienced severepolio seem to be at greatest risk for devel-
oping post-polio syndrome; however, indi-
viduals less severely affected initially also
can experience a decline in function. Those
who had been able to walk without assis-tive devices may require them because of
post-polio symptoms. Those who had usedassistive devices for ambulation may ﬁnd
it necessary to begin using a wheelchair.
Post-polio syndrome is progressive, so
deterioration will continue. Despite in-
creasing decline, however, individuals will
not return to the level of disability theyexperienced when polio was in its acutestate. With appropriate exercise, strengthand function can be improved and dete-rioration slowed, if not halted.
Diagnosis of Polio and
Post-Polio Syndrome
The diagnosis of poliomyelitis in its
acute state is based primarily on the med-ical history of the individual and on thesymptoms. Spinal tap or fecal sample canbe used to conﬁrm the diagnosis. Thediagnosis of post-polio syndrome is, attimes, more difﬁcult.
One step in diagnosing post-polio syn-
drome is to identify or eliminate otherconditions that may be responsible forthe symptoms. Symptoms of post-poliosyndrome may be difﬁcult to distinguishfrom other degenerative disorders of mus-cles and joints, such as osteoarthritis orosteoporosis. General medical evaluation,routine laboratory tests, electromyographic
studies (graphic record of the contraction
of a muscle as the result of electrical stim-ulation), and nerve conduction studies may
help to identify and exclude other dis-eases. Magnetic resonance imaging may be
used to exclude other conditions of thespine that could cause similar symptoms(Burk & Agre, 2000).
Treatment and Management of
Post-Polio Syndrome
No speciﬁc treatment is available to
alter the course of post-polio syndrome.Individuals with symptoms of increasing
muscle weakness, fatigue, and pain should92 C HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

ﬁrst have a thorough physical exam-ination by a physician to rule out otherpotential causes of symptoms. Treatmentis largely directed toward managingsymptoms and helping individuals main-tain functional status and independenceas long as possible. Good health practices,including proper nutrition and adequaterest, are important.
Generalized fatigue is treated with
lifestyle changes consisting of energyconservation measures. Physical activitiesshould be paced to prevent excessivefatigue. Individuals may require frequentrest periods throughout the day. Usingadditional assistive devices, such as awheelchair rather than crutches, mayhelp to conserve energy.
Mild to moderate weakness may be
treated by increasing muscle strengththrough nonfatiguing exercise. Exercisesthat are tolerable and that do not con-tribute to more weakness and fatigue maybe prescribed. Physical therapists general-ly instruct individuals about proper exer-cise protocols so that overuse and exces-sive fatigue can be avoided. Individualsare instructed to exercise for short inter-vals, to rest between bouts of exercise, andto exercise only every other day to pre-vent excessive muscle fatigue.
Individuals with respiratory difﬁculty
may require noninvasive positive-pressureventilation at night. Because individualswith post-polio syndrome are more sus-ceptible to infectious diseases, pneumoniaand inﬂuenza vaccines are usually recom-mended. Tobacco use should be avoided.
The use of braces to decrease mechani-
cal stress on joints may be necessary tohelp muscle and joint pain. Changes inorthotics or in the mode of ambulationmay be required. Moving from braces orcrutches to a wheelchair can also reducestress on joints. If the individual with
post-polio syndrome is overweight, weightreduction may be recommended toreduce fatigue and stress on muscles andjoints. For those whose respiratory mus-cles were also affected by the initial infec-tion, weight control can also help toprevent respiratory difﬁculty.
Psychosocial Issues in
Post-Polio Syndrome
Since poliomyelitis is not a progressive
disease, many individuals believed theirrecovery to be permanent and adaptedand adjusted to the functional limitationsand residual effects associated with thecondition, going on to lead full and pro-ductive lives. Individuals with residualdisability from polio have worked foryears to minimize their disability andmaximize their assets. Now, however, theunexpected symptoms associated withpost-polio syndrome threaten their func-tion and independence and can be psy-chologically devastating.
The symptoms of this new “secondary
disability” can be frightening as well asfrustrating for the individual, who again
must adjust and adapt to continuing func –
tional limitations, the potential use ofnew assistive devices, and an alteration inlifestyle. After regaining function previ-ously through much physical and emo-tional effort, being forced to deal againwith disability symptoms that are muchlike the initial symptoms can be discour-aging. Individuals may reject new assis-tive devices because they symbolize theloss of a physical ability that they feelthey earned through great effort.
Vocational Issues in Post-Polio Syndrome
Many individuals with poliomyelitis have
achieved gainful employment and livedproductive lives with residuals of polio.The onset of symptoms related to post-Conditions Affecting the Spinal Cord 93

polio syndrome, however, may make anumber of alterations necessary in the
work setting. In some instances, depending
on performance requirements, the indi vid-
ual may be unable to perform all of the
job duties. Thus altering job duties or retrain-
ing for other job duties may be necessary.
Even when remaining in the current
job is possible, individuals may experi-ence increased fatigue, so that frequentrest periods may be needed, or they mayneed a more sedentary job structure. Theability to lift, reach, walk, or climb maybe altered.
The symptoms of post-polio syndrome ,
whether pain, weakness, or fatigue, maynecessitate additional assistive devices.Individuals who once ambulated withoutassistive devices may require a cane,crutches, or braces. Individuals who onceused crutches or braces may require awheelchair for ambulation. Adapting theworkplace to accommodate these devicesmay be necessary. If, because of increasedsymptoms and disability, the individuals’current mode of transportation is nolonger accessible, transportation to andfrom work may be a barrier. In addition,because of increased disability, individu-als may require additional time to getready for work.
In some instances the onset of new
symptoms and increasing limitations mayresult in depression, which can interferewith the individual’s ability to work effec-tively. Supportive counseling may be nec-essary to enable the individual to copewith increasing disability.
NEUROMUSCULAR CONDITIONSParkinson’s Disease
Parkinson’s disease is a slowly progres-
sive disorder of the central nervous sys-tem, leading to progressive impairment ofmotor function. Although its cause
re-
mains unknown, evidence suggests that
both genetic and environmental factorsmay play a role (Janson, Leone, & Freese,2002; Nussbaum & Ellis, 2003). Parkin-son’s disease involves extensive degener-ative changes in the basal ganglia (the gray
matter imbedded in the white matter ofthe brain, which has a role in complexmovements) and the loss of or de-crease in levels of dopamine (a neurotrans-
mitter) in the basal ganglia. Most of thedisabling symptoms associated withParkinson’s disease are due predominant-ly to drastic reductions of dopamine lev-els in the brain. Although Parkinson’sdisease occurs most commonly after theage of 50 (Litvan, 1998), greater awarenessand improved methods of detection haveincreased the number of diagnosed casesof Parkinson’s disease among youngerindividuals.
Secondary parkinsonism is a term used to
describe a parkinsonian syndrome inwhich individuals experience Parkinson-like symptoms that are due to other caus-es. Secondary parkinsonism can be asso-ciated with the ingestion of certain drugs(prescription or illicit) or exposure to tox-ic substances, such as carbon monoxide orother chemicals. Secondary parkinsonismgained attention in the early 1980s whenthe “designer drug” MPTP, which mim-icked the action of heroin, entered thestreet market. A number of young adults,after taking the drug, suddenly developedpermanent signs and symptoms of severeParkinson’s disease. Some medicationsused to treat mental illness may also pro-duce Parkinsonlike side effects if notclosely monitored.
A variety of other conditions mimic
Parkinson’s disease, causing similar symp –
toms. These symptoms are collectivelycalled parkinsonism and should be distin-guished from Parkinson’s disease. 94 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

Neuromuscular Conditions 95
Manifestations of Parkinson’s Disease
The four most common symptoms of
Parkinson’s disease include:
• tremor• muscle rigidity•akinesia (complete or partial absence
of movement, or difﬁculty with vol-untary movement, especially of theextremities)
• postural instability
(Janson et al., 2002)
In early stages of the condition, indi-
viduals may exhibit extreme slowness ininitiating or maintaining movements(bradykinesia ). Individuals who have
Parkinson’s disease may walk with small,shufﬂing steps and may have difﬁculty ris-ing from a chair or bed. They may ﬁnd itdifﬁcult to initiate or to stop voluntarymovements. While walking, for example,they may experience gait hesitation and
suddenly “freeze,” taking seconds to re-gain motion; in other instances, they maycontinue ﬁve or six more steps beyondwhere they want to stop. The impairmentexperienced with bradykinesia can inter-fere with activities such as shaving, but-toning clothes, or cutting food,
all of
which take longer and become more difﬁ-
cult to perform as the disease pro gresses.
Because Parkinson’s disease affects both
the central and autonomic nervous sys-tems, some individuals may also experi-ence urinary or bowel problems.
Individuals with Parkinson’s disease are
sometimes said to have a poverty of spon-taneous movement. They may blink lessfrequently and may develop a masklike,expressionless face. They may develop dif-ﬁculty swallowing ( dysphagia ), which
results in saliva accumulation and drool-ing. Because the individual is unable toswallow quickly, the rate of swallowingdecreases and eating becomes slower andmore deliberate as the condition progress-es. As food collects in the mouth and theback of the throat, individuals may beprone to coughing and choking episodes.
Motor changes related to Parkinson’s dis-
ease may cause speech changes related toincoordination and reduced movement ofthe muscles that control breathing, voice,pronunciation, and rate of speaking. Vol-ume of speech may be decreased ( hypo-
phonia ), and there may be no verbal inﬂec-
tions. Individuals’ ability to write mayalso be affected. Reduction in amplitudeof movement may affect individuals’ abil-ity to write so that their handwritinggradually becomes smaller and smaller
(micrographia ) until it is no longer legible.
Tremor of a limb, usually most notice-
able in one hand, is the most frequent ear-
ly symptom of Parkinson’s disease. The
tremor intensiﬁes when the hand rests inthe lap ( resting tremor ) and diminishes
with voluntary movement. The tremor isnot present during sleep, however.
The posture of individuals with Parkin-
son’s disease becomes stooped, and theirarms fail to swing with their stride whenthey are walking. The loss of postural re-ﬂexes makes it difﬁcult for these individ-
uals to maintain an upright position if they
are suddenly bumped, increasing the riskof falls. To keep from falling, they mayinadvertently quicken their steps as if to“catch up” with their own center of grav-
ity. Muscle tone is increased, creating mus-cle
rigidity, which also interferes with
movement and causes severe im-mo bility.
Because greater effort is necessary to
engage in voluntary movement, fatigue isalso increased.
Mental and behavioral changes do not
always occur as a result of Parkinson’s dis-ease, but cognitive changes, as well aschanges in emotions and behavior, can bepart of the symptoms. Dementia can alsooccur in some individuals later in the

course of the condition. Apathy, passivi-ty, depression, and loss of initiative maybe noted. Some studies indicate thatdepression is present in a large number ofindividuals with Parkinson’s disease andcan have more impact on quality of lifethan the symptoms of the condition orthe side effects of treatment (Phillips,1999a). As the individual becomes aware
of his or her decreasing cognitive abilities,
depression related to losses may result.The degree to which depression reﬂectsphysiologic changes rather than a reac-tion to the disease itself is not known.
There is no cure for Parkinson’s disease.
The disease is characterized by progressive de –
bilitation, although the progression oc-
curs slowly over years. Treatment, usually in
the form of medication, physical therapy,and exercise, along with maintenance ofgeneral health, can reduce the effects ofthe symptoms so that the individual withthis condition may remain active longer.
Diagnosis of Parkinson’s Disease
There is no single test that can be used
to diagnose Parkinson’s disease. Individ-uals with initial symptoms are usually re-ferred to a neurologist (physician who spe-
cializes in the evaluation and treatment ofnervous system disorders) for evaluation.Physicians usually base their diagnosis onthe presence of tremor, stiffness, and slowmovement. Because many other condi-tions may have similar symptoms in ear-ly stages of development, and becauseinitially symptoms may be attributed toaging, misdiagnosis can frequently occur(Aminoff, Burns, & Silverstein, 1997).
Treatment and Management of
Parkinson’s Disease
Currently, there is no cure for Parkin-
son’s disease; however, the administrationof a medication called levodopa (L-dopa)
frequently decreases its symptoms. Levo-dopa works by helping to increase the lev-el of the neurotransmitter dopamine in the
brain. At ﬁrst, small amounts are usuallyprescribed, and the dosage is then gradu-ally increased.
Although helpful, levodopa also can
have serious side effects and limited long-term efﬁcacy (Jankovic, 1999; Janson,Leone, & Freese, 2003). Some individualsmay experience side effects such as nau-sea or abnormal involuntary movementscalled dyskinesia . These effects are gener-
ally related to the dosage of the medica-
tion, occurring more frequently with high-
er dosages. In some individuals, levodopacauses mental confusion or decreasesalertness.
Exercise and activity are especially im-
portant for individuals with Parkinson’sdisease because of the tendency of themuscles to be stiff and rigid. Muscles canatrophy (become smaller, or shrink) with-
out the stimulation that exercise provides,decreasing the individual’s ability for self-care. Individuals are usually encouragedto engage in a daily exercise routine, suchas walking a prescribed distance, doingsimple calisthenics, or doing active range-of-motion exercises. Other aspects oftreatment are directed toward preventingcomplications. Individualized physicaltherapy directed toward joint mobility,correction and prevention of postural ab-normalities of trunk and limbs, and main-tenance of normal gait are important to
help individuals maintain function as long
as possible. Passive stretching of extremi-ties, muscle massage, resistive exercises,and training are techniques used by phys-
ical therapists to achieve this goal.
Neurosurgical intervention as a treat-
ment for Parkinson’s continues to be ex-plored. One technique, deep brain stimu-
lation , has been used with some success at96 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

specialized clinics in Europe and NorthAmerica. The procedure involves implant-ing an electrode into a target area of thebrain. The electrode is then tunneledunder the skin to an external stimulatorthat can be switched on or off by the per-son with Parkinson’s disease (Phillips,1999b). Another surgical procedure, palli-
dotomy , consists of identifying and des-
troying the part of the basal ganglia thatsecretes the substances that are believedto destroy portions of the basal ganglia.Surgical procedures appear to be mosthelpful for those individuals who are un-able to control symptoms satisfactorilywith medication.
Parkinson’s disease is a chronic, lifelong
condition with progressive deterioration.
Because of the gradual onset and slow pro-
gression of the condition, however, withappropriate assistive devices and othertherapies most individuals have many
years to remain productive and functional.
Psychosocial Issues in Parkinson’s Disease
Parkinson’s disease has a profound im-
pact on the individual’s life, as well as onthe family. Parkinson’s disease is a visibleneurological disorder. Not only is it difﬁ-cult for the individual to move, but thereis also stooped posture, ﬂexed arms withlack of swinging when walking, and oftentremor of an extremity. One aspect of themovement disorder is lack of facial ex-pression and spontaneous movementswhen talking. Because they are unable touse these sources of body language, indi-viduals have reduced capability for non-verbal communication. They may alsoexperience speech disturbances as a resultof motor difﬁculties; thus both verbal andnonverbal communication may be difﬁ-cult. Acquaintances or strangers as well asfamily members may attribute lack ofexpression to disinterest, dementia, or lowintellectual ability. Individuals may be-come stressed or frustrated by the atti-tudes of others and consequently
begin to
withdraw from social interactions or be
reluctant to participate in them.
Anxiety and depression may occur from
the time of initial diagnosis and may con-tinue as symptoms progress. For individ-uals who had prided themselves in efﬁ-ciency, communication, or manual skills,deterioration of these skills can be partic-ularly stressful. Stress can make symptomsof Parkinson’s disease worse, further con-tributing to the individual’s social isola-tion. Because the disease is progressivelydebilitating, the individual and his or herfamily must continually readjust to in-creasing loss of functional capacity, whichcan also contribute to
anxiety and depres-
sion. If mental deteriora tion, confusion, or
personality changes occur, family mem-bers may have increased difﬁculty coping.In some instances individuals with Park-inson’s disease may also demonstrate de-creased initiative and impaired judgment,which can be the source of additionalstress for the family. As the individualbecomes more dependent on others forself-care, the social support system maybecome further eroded.
Information regarding sexual function
in Parkinson’s disease is scarce; however,sexual problems are frequently present inneurological disorders. Depression is con-sidered a contributor to sexual problemsin the general population. Consequently,since individuals with Parkinson’s disease
frequently experience depression, it stands
to reason that some sexual problems mayexist. The medications taken to treatsymptoms of Parkinson’s disease may alsocontribute to sexual dysfunction. Factorsassociated with many types of chronic ill-ness can cause lack of interest in sexualactivities. As the time needed for treat-ment and for activities of daily living,Neuromuscular Conditions 97

such as dressing, eating, and cleaningoneself, increases, the interest in sexualactivity and the energy for it may bedecreased.
Activities of daily living such as dress-
ing or bathing can be very tiring and timeconsuming. Individuals should allowenough time so they don’t feel rushed.Since balance is sometimes a problem,special safety precautions, such as grabbars or a tub bench or shower chair,should be used when bathing. Clearingthe environment of potential hazards thatcould cause the individual to fall can pre-vent the complications that could resultfrom the fall. Walking aids such as crutch-es or walkers may be useful in helpingindividuals avoid falls.
Vocational Issues in Parkinson’s Disease
Whether persons with Parkinson’s dis-
ease can continue working is an individ-ual decision and based on the speciﬁccircumstances. In most instances, workthat is more sedentary and that does notrequire signiﬁcant verbal communicationmay be continued longer than work thatrequires more strenuous activity. Sinceindividuals have difﬁculty with balanceand gait, jobs that require considerablewalking, stooping, or bending should beavoided. Transportation to and from workmay be the largest obstacle to maintain-ing employment.
Highly stressful occupations should be
avoided because stress tends to increasethe severity of symptoms. For some indi-viduals, work becomes increasingly difﬁ-
cult and the effort to continue working may
produce a tremendous strain. For these
individuals, not working may bring a sense
of relief from the physical and mentalstress of attempting to carry out varioustasks and responsibilities. For others, theinability to work may have a detrimentaleffect. In most cases, individuals will beable to continue working if the work is
not extremely demanding physically or does
not require manual dexterity. Schedulingfrequent rest periods and restructuring theworkload may help to increase the totalamount of work that can be done duringthe workday. Because stiffness and musclerigidity are common symptoms of thecondition, working in a cold environmentshould be avoided because of the possibleincrease in muscle stiffness.
Huntington’s Disease(Huntington’s Chorea)
Huntington’s disease is a progressive,
genetic condition of the central nervoussystem in which neurons in the basilganglia of the brain degenerate. Most in-dividuals develop symptoms between theages of 30 and 50 years. The condition ad-vances slowly and progressively. Althoughthe rate of deterioration varies from per-son to person, as does the rate at whichsymptoms appear, Huntington’s diseaseleads to total disability and death after15 to 20 years (Cattaneo, Rigamonti, &Zuccato, 2002).
Manifestations of Huntington’s Disease
Huntington’s disease is characterized by:
• cognitive deﬁcits• motor impairments• behavioral changes
Cognitive changes usually occur in the
early stages, with the individual at ﬁrstbecoming increasingly absent-mindedand having difﬁculty with concentration.As the condition progresses, mental dete-rioration ( dementia ) occurs.
Early signs of motor impairments in-
volve movements of the ﬁngers, whichgive the impression that the individual is98 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

ﬁdgeting. As the condition progresses,movement and coordination continue todeteriorate, with bradykinesia (slowness
of movement) and rigidity interferingwith the individual’s ability to walk.Jerky, involuntary movements ( chorea )
are also present. Motor difﬁculty alsoaffects the individual’s ability to speakand to swallow.
The individual also experiences person-
ality change. Behavioral changes associat-ed with the condition range from de-lusions to impulse-control problems.
Diagnosis of Huntington’s Disease
Diagnosis is usually based on the indi-
vidual’s symptoms and family history. In
most instances extensive neurological test –
ing is not necessary. Evaluation is usuallydone by a neurologist (physician who eval-
uates and treats neurological disorders).
Treatment and Management of
Huntington’s Disease
There is no known treatment to slow
progression or to cure Huntington’s dis-ease. Treatment is usually directed towardpreventing complications and treatingsymptoms. Physical therapy , a major com-
ponent of the treatment program, canhelp the individual improve or stabilizemotor ability, prevent contractures, oradapt the environment to promote safe-ty and maximum independence. Occupa-
tional therapists may help individuals
improve coordination abilities and activ-ities of daily living skills. Speech therapists
may help individuals maximize theirspeech capability as well as their ability toswallow. In some instances cognitiveretraining and memory training may beuseful. Avoiding exposure to upper respi-ratory infections as well as other commu-nicable diseases is also advised.It is sometimes difﬁcult to distinguish
which behavioral symptoms are related tothe condition itself and which are relatedto the individual’s anxiety about havingthe condition. Psychotropic medicationsmay be used to help alleviate or controlbehavioral symptoms, regardless of theircause. Medication may be prescribed foranxiety or depression, irritability, or moodswings. In some instances psychotropicmedication may also be used to controlsome of the involuntary, jerky move-ments individuals may be experiencing.
A major portion of treatment is direct-
ed to assisting individuals and their fam-
ilies manage self-care as the condition pro-
gresses. Individual counseling, family coun-
seling, and genetic counseling of family
members may be important interventions.
Psychosocial Issues in
Huntington’s Disease
Individuals and their families must
cope with continuing losses, both physical
and mental, as the condition progresses aswell as with the knowledge that Hunting-ton’s disease is a progressive condition inwhich continued deterioration can be ex-pected. The cognitive and behavioral
changes for affected individuals may make
it more difﬁcult for them to cope. Individ-uals as well as family members may feelhelpless and hopeless. As a result, theymay be reluctant to participate in activi-ties designed to maintain
or improve their
current level of function.
Faulty judgment and impulsivity relat-
ed to behavioral changes may result inunsafe situations for the individual. Thosewho are in denial about their conditionand their limitations may also be exposedto situations that could result in unsafepractices or injury.
As the condition progresses, individu
als
become less able to care for themselves andNeuromuscular Conditions 99

thus become more dependent on others.
As communication becomes more difﬁ-
cult, social interactions also become moredifﬁcult, resulting in increasing social iso-lation. Personality changes that may pro-duce violent or hostile behaviors furtherstress support systems.
Because Huntington’s disease has a
genetic component, family members maybe under the additional stress of knowingthat they may themselves be at risk fordeveloping Huntington’s disease. Coun-seling, education, and support can help toreduce the stress that family membersmay be experiencing.
Vocational Issues in Huntington’s Disease
Huntington’s disease is a progressive,
degenerative disease; however, in the ear-ly stages before mental deterioration andphysical incapacitation are present, short-term training may be appropriate. As thecondition progresses and individuals haveincreasing difﬁculty with memory, com-munication skills, and physical ability,sheltered employment may be the mostfeasible alternative.
Amyotrophic Lateral Sclerosis(ALS; Lou Gehrig’s Disease)
Amyotrophic lateral sclerosis (ALS), also
sometimes referred to as Lou Gehrig’s dis-
ease in memory of the baseball player who
died of ALS in 1941, is a progressive, de-generative condition in which there isdestruction of the motor neurons (nervecells that convey impulses to initiate mus-cular contraction). Damaged portions ofthe nerve tracts are replaced by scar tissueor plaques.
The cause of ALS is unknown, although
current medical theory suggests a multi-factorial etiology that may include genet-ic, viral, autoimmune, and neurotoxic fac-tors (Rowland & Shneider, 2001; Walling,1999). Individuals are usually affected inmiddle or later life, with males affectedmore frequently than women.
Manifestations of ALS
Symptoms of ALS depend on the area of
the nervous system affected; both upperand lower extremities are affected. Thereare two primary forms of ALS:
• Spinal form• Bulbar form
The spinal form of ALS is characterized
by muscular weakness, muscle atrophy
(decrease in size), spasticity, and hyperac-tive reﬂexes. Individuals may ﬁrst com-plain of tripping, stumbling, or awk-wardness when walking or running.Others may complain of difﬁculty withsimple activities such as buttoning a shirtor picking up small objects. In the bulbarform individuals may ﬁrst notice difﬁcul-ty in breathing, slurring of speech or low-ered volume when speaking, or difﬁcultywith swallowing.
As the condition progresses, symptoms
become worse, spreading to other parts ofthe body so that eventually, whether theindividual ﬁrst experienced the bulbar orspinal form of ALS, he or she eventuallyexperiences all the symptoms. Individualsbecome increasingly weak and immobile.Progressive paralysis leads to increasingloss of function so that ﬁnally individu-als are completely dependent on othersfor help with all activities of daily living.Excessive production of saliva and difﬁ-culty in swallowing can cause drooling.Some individuals may also experiencemuscle pain as a result of muscle spastic-ity. They may experience respiratory mus-cle weakness leading to breathingproblems, and in later stages of the con-dition they may require ventilatory assis-100 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

tance in order to breathe. Cog nitive func-
tion, sensation, vision, hearing, and bow-
el and bladder function are usually notaffected.
Diagnosis of ALS
There is no reliable laboratory test to
detect the presence of ALS. Diagnosis isusually based on the symptoms the indi-vidual exhibits and their progression andthe individual’s medical history, and by
ruling out other causes for the symptoms.
Treatment and Management of ALS
There is no cure for ALS, and no effec-
tive treatment is currently available. Treat-ment goals are generally directed towardhelping individuals and their family copewith the disability and to help the indi-vidual with ALS to remain independent aslong as possible, be comfortable, andavoid complications. Treatment of symp-toms is used to maintain muscle function,relieve discomfort, and forestall complica-tions such as respiratory infections anddecubitus ulcers. Medications to reducespasticity may be used; however, thesecan also increase muscle weakness andcause sedation. Physical therapy may be
helpful to maintain function and toreduce the painful symptoms brought onby muscle spasm.
Occupational therapists can provide sup-
port and help individuals to adapt theirenvironment in order to maximize func-tioning. Individuals with speech difﬁcul-ties may utilize speech therapists to help
them learn communication techniques.Speech pathologists may be utilized to help
individuals who have difﬁculty with swal-lowing. If individuals have breathing dif-ﬁculty, respiratory therapists may be used to
help them learn techniques in respirato-ry management.Psychosocial Issues in ALS
The social, economic, and psychologi-
cal impact of the condition is substantial.It is common for individuals with ALS toexperience fear, anxiety, and depression,especially as the condition progressesand the individual recognizes rapid pro-gressive deterioration of physical func-tion. Because of loss of mobility and in-creased dependency, feelings of helpless-ness and powerlessness are also common.Some individuals may experience discour-agement and become angry as their phys-ical limitations increase. They may
ex-
perience grief with each subsequent loss of
function. There may be loss in social rela-tionships leading to social isolation.
Changes in physical appearance and
physical ability may cause individuals toquestion their self-worth. They may feelguilty because of their increased depend-ence on others and may express concernand frustration over the burden they feelis being placed on family members.
Family members are also affected.
Family members’ roles are often modiﬁed.Since individuals with ALS need sub-stantial help with most activities of dailyliving, family members most often ﬁndthemselves in a caregiving role even inthe early stages of the individual’s condi-tion. Expenditures for medical care andequipment can be sizeable. If the individ-ual with ALS is also the major breadwin-ner, ﬁnancial issues may become a majorconcern. Family members may quit workto assume the caregiving role, which fur-ther contributes to financial distress.Family members may also have feelings ofpowerlessness, anger, or anxiety about thefuture. They may vacillate betweenresentment and guilt.
Despite the signiﬁcant disability indi-
viduals with ALS experience, they retaintheir cognitive and intellectual ability andNeuromuscular Conditions 101

still have needs for recreation, entertain-ment, and companionship. Using specialtechniques and equipment that enhancetheir functional capacity and independ-ence in self-care can help them exert per-sonal control over their life and thusmaintain self-esteem. Since communica-tion is usually significantly affected,equipment such as voice ampliﬁers, ortechniques such as eye blinking if individ-uals’ condition has progressed so thatthey can communicate no other way, aremeans to help them maintain meaning-ful relationships.
Vocational Issues in ALS
As ALS progresses, activity becomes
more and more difﬁcult. The conditiongenerally progresses fairly rapidly over acourse of 3 to 5 years, although someindividuals survive for up to 10 years.Many individuals live productive livesafter their diagnosis is made, continuingto work despite advanced symptoms. Thedegree to which they are able to con-tinue working depends on the require-ments of the job and how symptomsaffect their ability to perform. Older age,female gender, short time from symptomonset to diagnosis, and disease severity arekey prognostic factors (del Aguila,Longstreth, McGuire, Koepsell, & vanBelle, 2003).
The physical demands of work should
be light and sedentary. Even if the indi-vidual is still ambulatory, a wheelchair-accessible work environment should beconsidered, since individuals will requirea wheelchair for ambulation as the con-dition progresses. Transfer may becomemore difﬁcult in later stages of the condi-tion. Since communication can also be aproblem, occupations in which the abili-ty to speak makes up an important part ofthe job should be avoided.Guillain-Barré Syndrome
Guillain-Barré syndrome is an inﬂam-
matory condition of the peripheral nerves
(nerves lying outside the central nervoussystem). The exact cause of Guillain-Barrésyndrome is unknown, but symptoms arealmost always preceded by an infectiousillness that has occurred about 10 days be-fore. A particular bacterium appears to becommonly involved. Because of immun-
ological differences, not everyone who isin
fected with the bacterium develops
Guillain-Barré syndrome. However, someindividuals develop antibodies in re-sponse to the virus that attack not onlythe bacteria but also peripheral nerves. Asa result, these individuals developGuillain-Barré syndrome.
Manifestations of Guillain-Barré Syndrome
The severity of Guillain-Barré syndrome
varies greatly. Some individuals may haveonly mild muscle weakness, whereas oth-ers may become totally paralyzed anddevelop complications such as inabilityto breathe, abnormal blood pressure orheartbeat, or other conditions that can belife-threatening. An acute and progressivecondition, Guillain-Barré syndrome ischaracterized by muscular weakness thatusually begins in the lower extremitiesand spreads upward ( ascending paralysis ).
Paralysis of both upper and lower ex-tremities can occur, and chest and facialmuscles can be affected. Breathing canbe affected so that ventilatory support isneeded. The amount of paralysis varies:some individuals experience only mildfootdrop, whereas others develop com-plete paralysis. Abnormal sensations, suchas numbness, tingling, or the sense ofsomething crawling under the skinin the feet, hands, or face, may also bepresent. Individuals may also experience102 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

muscle aches or back pain as early symp-toms.
Symptoms develop rapidly, over hours
to days, or sometimes over a few weeks.
Generally, they reach their maximum
intensity within 3 to 8 weeks. For the most
part, the symptoms are reversible, and re-covery occurs after progression of thesymptoms cease. Although most individ-uals recover completely, the rate of re-covery is variable and can take up to 2years. In some individuals, permanentdisability or even death can result. Someindividuals, even though they appear to
have recovered, may develop fatigue with
sustained activity. Poor endurance withregard to walking or other activities of dai-ly living can be an ongoing problem.
Diagnosis of Guillain-Barré Syndrome
Diagnosis is usually based on symptoms
and physical examination. Electromyography
may be used to differentiate symptoms
from other causes of generalized weakness.
Treatment and Management of
Guillain-Barré Syndrome
Early treatment usually requires hospi-
talization. Treatment is primarily sympto-
matic and used to treat complications that
may accompany Guillain-Barré syn-drome. General physical rehabilitation isstarted early, usually under the guidanceof a physiatrist (physician who specializes
in rehabilitation and physical medicine).Physical therapists are usually involved in
the early stages of the condition to helpindividuals prevent muscle atrophy
(shrinkage), contractures, or pressure sores.
Occupational therapists help individuals
learn how to strengthen muscles, learnenergy conservation techniques, and per-form activities of daily living. If speech orswallowing is affected, a speech therapistmay be needed to help individuals improve
speech patterns or facilitate swallowing.
Psychosocial Issues in
Guillain-Barré Syndrome
The initial stages of the condition can
be extremely frightening. Individuals whowere healthy suddenly ﬁnd themselvesparalyzed and unable to care for them-selves. If respiration is affected and indi-viduals are placed on mechanical ven-tilation, the inability to breathe in itselfis frightening. In addition, individuals ona respirator are unable to communicate,further adding to apprehension and feel-ings of helplessness.
Even though most individuals regain
function, the unpredictability of the con-dition and progression of symptoms leadto fear, frustration, and concern for thefuture. Depending on the individuals’ sit-uation and the extent of time needed torecover, ﬁnancial concerns, fear of perma-nent disability, and fear of dependencecan be extremely stressful and can havelong-standing psychological effects evenafter the individual has recovered.
Vocational Issues in
Guillain-Barré Syndrome
Because individuals with Guillain-Barré
syndrome have, in many instances, beenincapacitated for a lengthy period of
time,
most will require an extensive period of
rehabilitation that may include driver
retraining, learning to pace activities, and
in some instances reemployment training.
Individuals may, after a certain amount ofactivity, continue to experience muscleaches or other sensations that interferewith normal activity. Initially they mayconsider returning to work on a part-timebasis and anticipate the need for period-ic rest periods during the day. In the caseNeuromuscular Conditions 103

of those who require a wheelchair for aperiod of time after hospital discharge,architectural barriers at their employ-ment site should be considered.
Myasthenia Gravis
Myasthenia gravis is a neuromuscular
condition characterized by muscle weak-ness and fatigue. It is an autoimmune dis-
ease in which symptoms are caused by adecrease in the neurotransmitter acetyl-
choline at the point at which nerves initi-
ate contraction of a muscle. The eyelids,muscles of the throat, and often musclesof the extremities are affected. A commonsymptom is ptosis (drooping) of the eye-
lid. Speech, chewing, and swallowingmay also be affected.
Diagnosis is usually based on symptoms
and physical examination. Treatmentwith medications in most instances en-ables individuals to live productive liveswith no signiﬁcant disability.
Muscular Dystrophy
Muscular dystrophy refers to a group of
hereditary conditions that are character-ized by progressive muscle weakness,muscle wasting, contractures of the joints,and deformity. Some forms of the condi-tion are rapidly progressive.
Symptoms lead to impairment in
ambulation and mobility and often armfunction. If facial muscles or muscles ofthe gastrointestinal tract are affected,feeding and speech difﬁculties may alsobe present. In some forms of the condi-tion, mental retardation may occur.
There is no speciﬁc treatment for mus-
cular dystrophy; however, physical thera-
py is essential to help individuals prevent
contractures of the joints and maintainmuscle strength and maximum function-al capacity.OTHER CONDITIONS OF THENERVOUS SYSTEMMultiple Sclerosis
Multiple sclerosis is a multifaceted, pro-
gressive condition of the central nervoussystem with myriad physical and psycho-logical consequences. It is one of the mostcommon disabling neurological diseasesin young adults (Confavreux, Vukusic,Moreau, & Adeleine, 2000; McDonald,
2000; Noseworthy, Lucchinetti, Rodriguez,
& Weinshenker, 2000). Most experts nowbelieve that multiple sclerosis is anautoimmune condition in which the body’s
immune system attacks segments ofmyelin , the protective sheath that sur-
rounds and insulates message-carryingnerve ﬁbers ( axons ) in the brain and
spinal cord (Dyment & Ebers, 2002;McDonald, 2000; Wekerle & Hohlfeld,2003). The term multiple sclerosis comes
from the multiple areas of scarring or scle-
rosis that occur when myelin surround-
ing nerve ﬁbers in the brain and spinalcord is destroyed. The scar tissue thatreplaces the areas of myelin that havebeen destroyed interferes with the trans-mission of nerve impulses, causing neuro-logical deﬁcits.
What triggers the autoimmune re-
sponse, causing the body to attack mye-lin, is unknown. Genetic predispositionand geographic factors appear to havesome role in determining susceptibility tomultiple sclerosis. Individuals with north-ern European heritage and those living inmore temperate climates appear to bemore susceptible (Lutton, Winston, &Rodman, 2004). There is speculation thatgenetic factors alone do not increase sus-ceptibility, but rather that the interactionof genetic predisposition with environ-mental factors or exposure to a virus pre-disposes individuals to develop thecondition (Dyment et al., 2002). Multiple104 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

sclerosis most often affects young adultsbetween the ages of 20 and 40, with about70 percent of cases being women(Johnson & Baringer, 2001).
Manifestations of Multiple Sclerosis
The symptoms and the extent of disa-
bility experienced with multiple sclerosisvary from individual to individual,depending on the location and extent ofmyelin destruction. Symptoms are diverseand unpredictable, appearing in varyingcombinations and patterns. Consequent-ly, not all persons with a diagnosis of mul-tiple sclerosis experience the same symp-toms or progression of the condition.
The most common initial symptoms of
multiple sclerosis are dizziness; sensorydisturbances, including numbness, weak-ness, and spasticity, especially of the low-er extremities; unsteadiness; visualproblems; or poor bowel and bladder con-trol. Weakness and fatigue frequentlyaccompany other symptoms. Symptomsﬂuctuate, becoming worse at times ( exac-
erbation ) and better at other times ( re-
mission ) (Antel & Bar-Or, 2003).
Loss of motor, sensory, intellectual, or
emotional function may be associatedwith
multiple sclerosis, depending on the
part of the central nervous system affected.
Symptoms vary greatly not only from per-son to person, but also from time to timein the same person. Because of the vari-ability and ﬂuctuation of symptoms, diag-nosis of the condition is often a chal-lenge. Before being diagnosed with mul-tiple sclerosis, many individuals havegone from one health provider to anoth-er with a host of vague complaints andclinical symptoms that health profession-als may attribute to fatigue, stress, or evenlaziness and withdrawal. Often, the con-dition is not recognized or diagnosedimmediately because the symptoms haveresolved by the time the individual sees aphysician. Before the condition is diag-nosed, individuals may experience con-siderable anxiety, self-doubt, and de-pression because of the continuing vaguesymptoms that cannot be explained.
As symptoms become more pro-
nounced, permanent dysfunction in avariety of areas becomes more apparent.Symptoms may include paresthesia (a
sensation of numbness or tingling insome part of the body); weakness of anextremity; visual disturbances, such asdiplopia (double vision) and dimness of
vision; and vertigo (dizziness or false sen-
sation of circular movement). Individualswith multiple sclerosis may develop difﬁ-culty with coordination and balance(ataxia ), a symptom that may be misin-
terpreted by the casual observer as indi-cating intoxication. There may be partialor complete paralysis of any part of thebody or spasticity of muscles, especially inthe lower extremities. A particular tremorof the hands, called intention tremor , may
be present. The tremor is called intention
tremor because it occurs only when the
individual tries to engage in a purposefulactivity, such as reaching for a glass.
Speech may be slurred, or there may be
scanning speech, in which the individualenunciates slowly with frequent hesita-tions at the beginning of a word or sylla-ble. Individuals with multiple sclerosismay also have difﬁculty in swallowing(dysphagia ), which can contribute to
choking.
Multiple sclerosis may also affect the
genitourinary tract, causing incontinence
(loss of control of the bladder or bowel).Some individuals may experience urinary
retention (the inability to empty the blad-
der of urine). Sexual function can also beaffected. Men may experience erectile dys-function and women may experience
decrease in sexual desire, lubrication prob -Other Conditions of the Nervous System 105

lems, or inorgasmia (McCabe, McDonald,Deeks, Vowels, & Cobain, 1996). How-ever, women with multiple sclerosis areable to become pregnant and carry preg-nancy to term. There is no evidence thatpregnancy causes an increase in symp-toms or exacerbations of the condition(Hansell, 1995).
Some individuals experience cognitive
changes as a result of multiple sclerosis,but intellectual function remains intactfor many. Some individuals, however,may experience impairment in perform-ing tasks that require conceptualization,memory, or new learning, as well as difﬁ-culty with tasks that require either rapidor precise motor responses. Some mayhave difﬁculty with abstract reasoningand problem solving. Depression is com-mon, although the degree to which it is areaction to the disease or a manifestationof neurological dysfunction is not known.Other individuals, rather than experienc-ing depression, experience an inappropri-ate euphoria.
Treatment and Management of
Multiple Sclerosis
The diagnosis of multiple sclerosis is
based on the results of a full neurologicalexamination and tests such as magnetic
resonance imaging . There is no speciﬁc
treatment for multiple sclerosis, nor isthere a cure. Treatment is usually direct-ed toward controlling individual symp-toms and preventing exacerbations andcomplications. Treatment may improvesymptoms somewhat or help to preventor delay future exacerbations of symp-toms, but it can have risks that must becarefully weighed by each individual.Consequently, the potential side effects ofeach treatment must be weighed againstthe potential beneﬁt for functional capac-ity or delay of decline.A variety of medications may be used to
treat speciﬁc symptoms. Some medica-tions may exacerbate symptoms or ad-versely affect remaining function, andsome have the potential to impair mem-ory and learning. Consequently, the typeof medication prescribed varies with theindividual. Medications are commonlyused for bladder management, control ofspasticity, or emotional symptoms.
Although some individuals with multi-
ple sclerosis never experience urinaryproblems, for those who do, anticholiner-
gic medications , which inhibit the actions
of the parasympathetic nervous system,are sometimes helpful in relieving bladdersymptoms such as frequency and urgency.Conversely, cholinergic medications , which
stimulate the actions of the parasympa-thetic nervous system, may be helpful inrelieving urinary retention. When urinaryproblems are present, individuals may bereferred to a urologist (physician who spe-
cializes in evaluation and treatment of thegenitourinary tract). Bladder training maybe helpful in reducing bladder problemsand in helping the individual to managebladder control. Use of a catheter or san-itary pads may also decrease the embar-rassment of possible leakage of urine.Other ways of managing bladder controlare monitoring the time of day that ﬂu-ids are ingested and ensuring a readyavailability of restrooms to minimize thechance of accidents. If individuals haveproblems with urinary retention, theymay be taught to insert a catheter intotheir bladder to drain accumulated urine.
Most patients to some degree experi-
ence spasticity. Relaxants or antispasmod-
ics may be prescribed for muscle spasm or
spasticity; however, at doses high enoughto control spasticity, weakness may beexacerbated.
Steroids are at times prescribed, especial-
ly in acute phases of exacerbations to sup-106 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

press the symptoms, but they do notaffect the progression of the disease.Because of the potential harmful effects ofsteroids taken over extended periods oftime, they are usually prescribed only ona temporary basis to decrease exacerba-tions, and not as ongoing therapy.
Individuals who experience depression
or anxiety may have antianxiety agents or
antidepressants prescribed. Since suicide
rates are relatively high among individu-als with multiple sclerosis (Livneh &Antonak, 1997), psychiatric consultationmay also be indicated.
In general, individuals with multiple
sclerosis should remain as active as they
can without developing excessive fatigue.
Physical therapy may be prescribed to help
with problems of mobility or with the useof assistive devices, such as walkers, ifneeded. Speciﬁc exercises that help to de-crease calcium loss from bones, strength-en weak muscles, and maintain musclestrength and joint mobility may also beprescribed. Physical therapy that includesmassage and passive range-of-motionexercises may also be beneﬁcial. Indi-viduals with multiple sclerosis who expe-rience speech problems may be referred toa speech therapist or may use assistive
devices , such as a communication board
and voice ampliﬁer.
For many individuals with multiple
sclerosis, exposure to heat can have a tem-porary adverse effect on symptoms. En-vironments in which body temperature isincreased, such as during hot or humidweather, illness with fever, or even a hotbath, can make the individuals feel worse,although heat does not necessarily causea worsening of the condition itself. Indi-viduals should therefore avoid hot andhumid environments.
Since there is no cure for multiple scle-
rosis, it is a lifetime condition. Multiplesclerosis is usually not fatal, and mostindividuals with the condition can expectto live a normal life span, although withvarying degrees of disability. There is noformula for estimating the general out-come for all individuals. Symptoms oftenﬂuctuate with periods of remission (when
symptoms get better) and periods of exac-
erbation (when symptoms get worse). Al-
though the symptoms may partly resolve
when the disease is in remission, exacerba –
tions can leave permanent residual defects.
The condition and deterioration of
function show no predictable pattern.The general prognosis for individualswith multiple sclerosis is unpredictable,with varying rates of progression andvarying rates of disability. For some indi-viduals (about 20 percent) the conditionremains relatively stable with only mildsymptoms, such as slight weakness,unsteadiness, or vision problems, and nolong-term disability. The majority of indi-viduals (about 65 percent) develop arelapsing-remitting pattern in which there
are continuing exacerbations when symp –
toms become worse with periods of com-plete or partial remission in which thereis no significant overall disability orrestriction of general activity. Many ofthese individuals retain their mobility 20or more years after diagnosis, with limit-ed disability (Schapiro, Scheinberg,Weiner, & Wolinsky, 1997). For some in-dividuals the disease progresses slowlywith no remissions and gradually increas-ing disability. Still others experi
ence rap-
id progression with total disability.
Psychosocial Issues in
Multiple Sclerosis
Most individuals with multiple sclero-
sis are young adults who have livedthrough formative years of childhood andadolescence as relatively healthy individ-uals and are at a stage of their life inOther Conditions of the Nervous System 107

which they are beginning to assumemany social and economic responsibili-ties, such as choosing a career, establish-ing intimate personal relationships, andperhaps starting a family. When the diag-nosis of multiple sclerosis has been estab-lished, the limitations and unpredict-ability of the condition can severely affectthe individual’s self-concept. Restrictionson abilities, activities, and social relation-ships call for signiﬁcant initial psychoso-cial adjustment and alteration of self-concept as well as continual readjustmentas exacerbations, remissions, and new dis-abling features of the condition occur.Consequently, the long-term experienceof living with multiple sclerosis throughyoung adulthood, middle age, and olderadulthood requires not only initialacceptance of the condition but also con-tinued ﬂexibility and adjustment as thecondition changes.
The ambiguity of the condition and the
erratic nature of the symptoms producesigniﬁcant stress. When the diagnosis ofmultiple sclerosis is ﬁnally established, in-dividuals may react in a number of ways.Those who have been newly diagnosedmay be unwilling to accept the diagnosisand continue to search for someone whowill provide an alternative diagnosis. In
other cases, individuals who have searched
for years for an explanation for theirvague and elusive neurological symptomsmay feel a sense of relief at ﬁnally beinggiven a diagnosis to explain what theyhave been experiencing. Others may reactwith shock and disbelief; still others mayreact with fear and anxiety. As with oth-er chronic conditions, the individual’sreaction to the diagnosis of multiple scle-rosis is dependent on a number of indi-vidual factors.
As the diagnosis and implications of the
condition are accepted, individuals mayattempt to gain some control over thecondition and its symptoms. Although
they may adapt to the realization that mul-
tiple sclerosis is a lifelong condition, theunpredictability of the condition is still asource of stress. Despite planning, unfore-seen exacerbations may occur, interferingwith plans and activities without warn-ing. Exacerbations can renew a sense ofvulnerability, undermining optimism orenthusiasm for long-range planning andcausing anxiety about the future.
Even when individuals have mild cases
of multiple sclerosis, manifestations of thecondition can be stressful. In mild cases,individuals may have few visible symp-toms or may experience only vague symp-toms of weakness or fatigue. Family,friends, or colleagues, unable to observevisible signs of disability, may be unableto understand why they cannot keep pacewith others or continue to perform all thetasks in the same time frame as they wereonce able to do. They may be accused ofbeing lazy or attempting to get out ofactivity when actually their level of ener-gy is reduced because of the condition. Asa result, they may attempt to push them-selves beyond their capability or beyondwhat is in their own best interest withregard to management of their condition.
Bladder problems resulting from multi-
ple sclerosis may cause embarrassment,causing the individual to withdraw fromsocial and work activities. A variety ofsteps can be taken to minimize the sociallimitations that such problems may pres-ent, as described previously.
Although alcohol is not contraindicat-
ed for most people with multiple sclero-sis, if balance problems are experienced asa result of the condition, alcohol willcompound the problem. Likewise, alcoholcan be dangerous when taken in combi-nation with some medications. Conse-quently, individuals with multiple sclero-sis should always consult their physician108 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

before deciding whether alcohol may beconsumed on social occasions. Because ofthe wide variation of disease progressionand its associated functional limitations,comprehensive evaluation of the individ-ual’s environment must be established sothat adequate modiﬁcations and compen-sations can be made. Increasing the func-tional capabilities of each person has thepotential to reduce the social and psycho-logical impact of multiple sclerosis.
Vocational Issues in Multiple Sclerosis
Many individuals with multiple sclero-
sis experience problems with unemploy-ment and underemployment (Bishop,Tschopp, & Mulvihill, 2000). Educationalattainment, symptom severity, and thepresence of cognitive limitations appearto be signiﬁcant predictors for employ-ment status for a number of individuals(Roessler, Rumrill, & Fitzgerald, 2004).Since multiple sclerosis varies greatlyamong different persons, each individ-ual’s vocational potential must be consid-ered separately. Those with mild symp-toms, slowly progressive multiple sclero-sis, or those who have extended periodsof remission are capable of being gainful-ly employed for many years. Others, withmore serious manifestations of the condi-tion, can, with appropriate accommoda-tions and assistive devices, often remainemployed despite exacerbations or pro-gression of the condition.
Mobility, communication, vision, and
cognitive function are common areasthat need to be addressed. The speciﬁcaccommodations and needs of each indi-vidual must be evaluated. For example,those who experience symptoms requir-ing the use of a wheelchair will needwheelchair accommodations. Those withcommunication difficulties, such asslurred speech, may need other types ofaccommodations or considerations regard –
ing job placement. Individuals with bal-ance problems may need to avoid situa-tions in which falling could be hazardousor may need a walking aid, such as a caneor crutch, that could be helpful in pre-venting a catastrophic fall. If vision isaffected, speciﬁc accommodations relatedto visual needs may be warranted. If cog-nitive function is affected, individualsmay beneﬁt from cognitive retraining or
memory enhancement programs (Roessler,
Fitzgerald, Rumrill, & Koch, 2001).
Emotional stress as well as physical
stress can cause a temporary worsening ofsymptoms. The degree of emotional stressthe individual experiences on the job, aswell as the level of physical activityrequired, should be considered. Excessivefatigue, particularly to the point ofoverexhaustion, should be avoided. Al-though individuals do not need to curtailphysical activity, they should attempt toavoid pushing themselves to exhaustion.They may minimize the effects of fatigueon job productivity by learning to pacethemselves so that activities are plannedwhen energy levels are higher, such as atthe beginning of the day. Individualsshould also learn to moderate their paceof activities and ﬁnd levels conducive tooptimize energy. Frequent rest periodsmay be needed throughout the day. Itmay be important to break tasks intosmaller steps, resting at intervals in be-tween. Adapting work hours to individualneeds, involving individuals in moresedentary work, or using energy-savingtechnology may be advisable to increasework capacity.
Because heat also affects symptoms,
individuals should avoid hot and humidenvironments. They should avoid pro-longed exposure to the sun and duringhot days stay in an air-conditioned envi-ronment as much as possible.Other Conditions of the Nervous System 109

Individuals with multiple sclerosis have
increased susceptibility to complicationsfrom infectious disease. Therefore, envi-ronments in which there is signiﬁcantexposure to people with colds, ﬂu, or oth-er infectious diseases should be avoided.Although there are limitations associatedwith the condition, many people withmultiple sclerosis are able to continue towork with only minor adjustments. Lossof time at work during exacerbationsshould be expected; however, generallythese episodes are not excessive. Assistivedevices, new equipment, ready access torestrooms, and job restructuring canenhance the individual’s ability to contin-ue work. Speciﬁcally, environmental fac-tors, accommodations that allow for moresedentary work, ﬂexible schedules, anduse of technology can all be instrumentalin helping individuals with multiple scle-rosis maintain employment.
Central Sleep Apnea
Sleep apnea is one of the most chronic
disorders of adults and the second mostcommon breathing disorder during sleep(Drazen, 2002). Sleep apnea is character-ized by frequent episodes of apnea (ces-
sation of breathing) during sleep(Gottlieb, 2002). The result is daytimesleepiness, which is a signiﬁcant problembecause it can increase the incidence oftrafﬁc accidents (Yamamoto, Akashiba,Kosaka, Ito, & Horie, 2000). Lack of sleepalso causes stress, so that affected peoplebecome irritable, undergo changes in per-sonality, or have difﬁculty with memory,causing social and family disruption(Findley, Smith, Hooper, Dineen, &Suratt, 2000). It can also lead to disabili-ty because of the increased risk of hyper-
tension (high blood pressure) and heart
disease (Nieto, Young, Lind et al., 2000;Roux, D’Ambrosio, & Mohsenin, 2000;Shahar, Whitney, Redline et al., 2001) andin some instances death (Drazen, 2002;Veale, Chaileux, Hoorelbeke-Ramon et al.,2000).
There are two types of sleep apnea. The
most common type, obstructive sleep apnea ,
is discussed in Chapter 12. The secondtype of sleep apnea, central sleep apnea ,
occurs when the brain fails to send appro-priate messages to the muscles needed toinitiate breathing. It can be caused fromstroke, infections affecting the brain stem,or neuromuscular diseases that involverespiratory muscles.
Narcolepsy
Narcolepsy is a complex neurological
sleep disorder involving the central nerv-ous system that is linked to a disruptionof the sleep control mechanism (Siegel,2000). It is characterized by episodes ofexcessive sleepiness and uncontrollablesleep during the day (Stansberry, 2001). Itcan occur at any time and during anyactivity, such as while engaging in conver-sation, while driving, eating, or evenwhen reading (Siegel, 2000).
Diagnosis is usually based on a persist-
ent history of excessive daytime sleepi-ness not due to other causes and is con-ﬁrmed through tests conducted at a sleep-disorders clinic. Treatment usually con-sists of planned short nap periods duringthe day, and in some instances prescrip-tion of central nervous system stimulants.
The physical, psychosocial, and voca-
tional implications of narcolepsy can bedevastating. Individuals who are not ade-quately diagnosed and treated have ahigh risk of motor vehicle accidents andmay have difﬁculty reaching their fullpotential either in school or in employ-ment (Siegel, 2000). Even with treatment,symptoms of narcolepsy may not be ade-quately managed. The fear of the embar-110 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

rassment that may result from an attackcan cause individuals to limit their socialinteractions. Safety concerns regardingoperation of potentially dangerous equip-ment may be an issue if the individual’ssymptoms are not adequately controlled.Employers, teachers, and others comingin contact with the individual should behelped to understand the individual’scondition so that if an attack does occur,symptoms will not be misinterpreted.
Lyme Disease
Lyme disease is a multisystem inﬂam-
matory disease that affects the nervoussystem as well as the joints and muscles.It is the result of an infection caused by atype of organism called a spirochete and is
transmitted by an infected tick. In the ear-ly stages it is characterized by a reddenedarea around the site of the tick bite.
Lyme disease is rarely, if ever, fatal and
is not contagious. Although most people,if treated early, have no permanent dis-ability, some individuals can go on todevelop other abnormalities of the centralnervous system, including gait spasticity,facial palsy, memory loss, mild confusion,joint pain, or meningitis (Hayes &Piesman, 2003).
Diagnosis is usually based on symptoms
and blood tests. Early treatment withantibiotics can signiﬁcantly improve out-come and prevent chronic affects.
Bell’s Palsy
Sudden partial or complete paralysis of
one side of the face is characteristic ofBell’s palsy (Salinas, 2002). Individualsmay experience a sagging eyebrow, inabil-ity to close their eye, and drooping of oneside of the mouth. Bell’s palsy occurswhen a nerve running from the brain tothe face becomes inﬂamed. As the inﬂam-mation progresses, the nerve swells,becomes compressed, and is no longerable to transmit signals; consequently,paralysis results. A growing body of evi-dence links reactivation of herpes viruseswith the development of a large numberof cases of Bell’s palsy (Gilbert, 2002).
Although most individuals recover from
Bell’s palsy within a month, during theacute phase, if they are unable to closetheir eye, the eye may need to be protect-ed with an eye patch, or artiﬁcial tearsmay need to be used. Individuals may alsohave anti-inflammatory steroids pre-scribed soon after the symptoms appear.
DIAGNOSTIC PROCEDURES INCONDITIONS OF THE SPINAL CORDOR NEUROMUSCULAR ORPERIPHERAL NERVOUS SYSTEM
In addition to the diagnostic procedures
discussed in Chapter 2, X-ray and elec-tromyography may be used to diagnoseneuromuscular conditions or conditionsinvolving the peripheral nerves.
Spine Roentgenography (X-ray)
X-ray ﬁlms of the spine are called spinal
X-rays and are used to identify fractures or
other abnormalities of the vertebrae andto evaluate the spaces between vertebraldiscs of the spinal column. X-ray ﬁlms areusually taken by a radiology technician . The
ﬁlms are then read and interpreted by aradiologist (physician who specializes in
radiology).
Electromyography (EMG) and NerveConduction Velocity Studies
Electromyography is a procedure used to
evaluate the electrical activity of certainmuscles and is helpful in the diagnosis ofcertain muscle diseases. It may be per-Diagnostic Procedures: Spinal Cord or Neuromuscular or Peripheral Nervous System 111

formed by a physician, physical therapist,or specially trained technician. A smallneedle that is attached to an electrode isinserted into the muscle being examined,and the electrical activity of the selectedmuscle is recorded both at rest and dur-ing exercise. Nerve conduction studies are
often performed in conjunction withEMG and are helpful in diagnosing con-ditions affecting peripheral nerves . For the
nerve conduction portion of the proce-dure, a stimulating electrode that deliversa mild electrical charge is placed on theskin over a nerve. An electrode placedover a muscle records the activity of thenerve distally at the nerve-
muscle junc-
tion. EMG makes it possible to identify
defects in the transmission of impulsesfrom nerves to muscles. It may be used inthe diagnosis of a number of neuromus-cular disorders and peripheral nerveinjuries.
GENERAL ISSUES IN NERVOUSSYSTEM CONDITIONS
Conditions of the nervous system have
widespread effects. Physical deﬁcits canprevent individuals with such disordersfrom performing even routine self-care.Problems with speech can alter the way
individuals communicate. Emotional abil –
ity may cause difﬁculty in social relation-ships. Cognitive deﬁcits may interferewith work as well as everyday activitiessuch as managing ﬁnances, performinghousehold tasks, or carrying out self-careactivities.
Symptoms of some conditions of the
nervous system, such as epilepsy, can becontrolled with medication. Other condi-tions, such as multiple sclerosis andParkinson’s disease, involve progressivedeterioration, and treatment focuses oncontrolling symptoms, preventing com-plications, and promoting function andindependence as long as possible. Whenthere has been permanent damage to thenervous system, such as that caused byhead injury or spinal cord injury, treat-ment is directed toward rehabilitationand prevention of complications.
In many instances, treatment of nerv-
ous system disorders involves helpingindividuals to compensate for neurologi-cal deﬁcits or to learn alternative methodsof performing routine tasks. Assistivedevices such as canes, braces, and wheel-chairs may be indicated for individualswith special motor needs. Other means ofassistance for individuals with neurolog-ical disorders that interfere with motorfunction are “help animals,” such as dogsor monkeys that have been especiallytrained to retrieve various items or to per-form other tasks that are difﬁcult orimpossible for an individual with a neu-rological disorder.
Psychosocial Issues in Conditions ofthe Nervous System
Psychological Issues
Adjustment to any chronic condition or
disability can be difﬁcult. Individualswith neurological disorders may face par-ticular challenges because their disordersaffect many different functional areas. Aswith all disabling conditions, psychologi-
cal reactions are individually determined,
based in part on the way the individualhas dealt with life problems in the past.Individuals with traumatic brain injury,stroke, or multiple sclerosis may have cog-nitive and emotional impairments as wellas impairment of motor function as adirect consequence of their injury or con-dition. Because they need to learn com-pensatory strategies for a number ofactivities and social interactions, psycho-logical adaptation to the disorder be-112 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

comes multifaceted. It is often difﬁcult todetermine the degree to which behavioraland affective changes are physiologic andthe degree to which they are situational-ly induced.
For many neurological conditions,
available treatment is limited and direct-ed mainly toward controlling symptomsor preventing complications. As a result,individuals with these conditions mayfeel they have little control over the con-dition or their future. When conditionsare progressively debilitating, as in multi-ple sclerosis or Parkinson’s disease, indi-viduals must continually readjust asadditional functional capacity is lost.Under these circumstances, they mayexperience a helpless rage or bitternessbecause of a condition over which theyhave no control.
The rate of progression and degree of
loss of functional capacity in many neu-rological conditions are often unpre-dictable. The uncertainty about whetherdisability will be minimal or will progressto severe disability can produce stress andhardship. Although wheelchair use canprovide mobility and an added sense offreedom and independence, some indi-viduals may have a negative emotionalreaction to using a wheelchair, viewingit instead as a symbol of the inability towalk. In other instances, even thoughthe disability experienced is minimal,they may grieve over the lost ability tofunction.
Often conditions of the nervous system
not only impose permanent loss of func-tion, but also involve complex self-con-cept and body image changes. Traumaticbrain injury, stroke, and spinal cordinjury provide no time for gradual adjust-ment. Individuals who had been previ-ously active are suddenly faced withadjusting to loss of functional capacityand alteration in physical appearance.Reactions may include hostility, anger, orwithdrawal.
Individuals injured because of acci-
dents may feel remorse or self-recrimina-tion for failure to prevent the accidentfrom occurring. If the accident was thefault of a third party, they may feel chron-ic anger toward the offender or may turnanger inward and become depressed. Insome instances, the quest for retributionbecomes a negative force, eroding theindividual’s life as he or she continuallyseeks some sort of justice.
By their very nature, symptoms of
many neurological conditions necessitateassistance in care and function. Individ-uals may harbor resentment over thedependency imposed by the condition.Those who experience paralysis may feelan increased sense of vulnerability, fearingthat escape from a dangerous
situation or
defending themselves against threat
would be difﬁcult or impossible. Theirreaction may vary from overdependenceto overcompensation, in which they takeunnecessary risks to test or prove theirindependence and strength.
When there is loss of the capacity to
care for basic physical needs, this loss isdifﬁcult to accept. Learning to accept nec-essary assistance from others for basicneeds, such as feeding, personal hygiene,and bowel and bladder care, requiresreconstituting views of privacy and self-reliance. Impairment of bladder and bow-el control may be an especially difﬁcultarea of adjustment. Not only are suchactivities private and mishaps a potentialsource of embarrassment, but both mayalso be associated with the shame andhumiliation experienced in early child-hood when control of these most basicbodily functions was a central issue ofdevelopment.
Most individuals with neurological dis-
orders adjust and learn to be self-reliant,General Issues in Nervous System Conditions 113

despite the fact that their ability to carefor their basic physical needs is decreased.Those who have experienced disability asa result of an accident may turn theirexperience into a positive force directedtoward broader social issues, such as seatbelts in automobiles, helmets for motor-cyclists, or laws against drunk driving.Other individuals use their experience tocreate public awareness of the needs ofindividuals with a disability and to edu-cate others about disability issues. Just asneurological conditions have a spectrumof functional consequences, so also theadjustment of individuals with neurolog-ical conditions is highly individualized,and no two individuals with the same dis-ability will have a reaction that is quitethe same.
Lifestyle Issues
The effects of neurological disorders on
an individual’s lifestyle are varied andcomplex. Activities of daily living are of-ten altered so that help from family mem-bers or others is necessary. Subsequentloss of privacy for most intimate details ofdaily life, such as bathing or other aspectsof self-care, may be part of the generalcondition. Even when individuals are ableto manage their own personal care, theadditional time required to carry out mostactivities may be considered a liability.
Special adaptations may have to be
made within the environment, such aswidening doorways for wheelchairs, low-ering countertops in kitchens, raising toi-let seats, and adding ramps and railings.Although not all neurological conditionsrequire the use of a wheelchair, most re-quire some consideration of environmen-tal factors. For example, individuals withepilepsy may need to avoid environmen-tal conditions such as ﬂashing lights,which may precipitate seizures. Becausethose with multiple sclerosis may beespecially sensitive to hot, humid condi-tions, they may need to remain in a
cool
environment with decreased humid ity.
Individuals with balance or coordinationproblems caused by brain injury mayneed to avoid uneven terrain or other sit-uations in which they may fall or bethrown off balance.
Alterations in daily schedules and rou-
tines may be necessary to allow addition-al time for dressing, bathing, and otherself-care needs. If wheelchairs are used,the environment must be made naviga-ble. Wheelchairs can provide more free-
dom of movement for those with paralysis,
for those who have difﬁculty walkingbecause of problems with coordination,or for those who fatigue easily and use awheelchair to conserve energy. Freedomof movement is limited, however, if thereare stairs but no elevator, if bathrooms aretoo small to accommodate a wheelchair,or public transportation is unequippedwith lifts or mechanisms for transportingindividuals in wheelchairs.
Individuals with neurological disorders
can usually drive, even with paralysis, if the
vehicle is equipped with special controls.If the disability includes cognitive or per-ceptual deﬁcits, however, driving may
not
be possible. Although regulations vary from
state to state, individuals with epilepsymay have to demonstrate that they havebeen seizure free or that medication hasadequately controlled their seizures overa number of months or years before theyare permitted to drive a motor vehicle.
When fatigue exacerbates the symp-
toms of a neurological disorder, as in mul-tiple sclerosis, or when fatigue is part ofthe symptomatology, as in post-poliosyndrome, it may be necessary to spaceout activities or to arrange for frequentrest periods during the day. It is some-times helpful to divide activities that were114 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

once completed in a short amount of timeinto a series of smaller tasks, allowing restperiods in between.
A number of nervous system conditions
can lead to sexual difﬁculties. Generally,physiologic responses require an intactnervous system. Sexual function may bemost disrupted by conditions involvingthe spinal cord. Although women with aspinal cord injury are still capable of inter-course, sensory loss in the genitals is com-mon in both men and women. Men maystill experience reﬂex erections but areusually incapable of psychologically stim-ulated erections. Reproductive functionmay also be a concern after spinal cordinjury. Women with such an injury gen-erally remain fertile and are capable ofconceiving and delivering a child.However, men with spinal cord injurymay be infertile because of the inability toejaculate, retrograde ejaculation, ordecreased sperm formation.
Sexual intercourse may be more difﬁ-
cult in conditions involving spasticity,such as cerebral palsy or multiple sclero-sis. In some instances, the stimulationand arousal experienced as part of sexualexcitement may make the spasms worse.In other instances, special arrangementsfor positioning or other technical assis-tance may be necessary for sexual inter-course to occur.
Sexuality is more than genital acts of sex.
Some form of sexual expression is possiblefor almost all individuals with a disabili-ty. Individuals may need to learn newforms of sexual expression to meet theirneeds, or in other instances they mayneed to learn to control sexual expression.For instance, individuals with quadriple-gia may be helped to learn alternatemeans of sexual expression, or individu-als with brain damage who exhibit sexu-al disinhibition may be helped to learnhow to use appropriate social behaviors.Although loss or alteration of sexual
function is initially a severe blow to self-esteem and sense of attractiveness, indi-viduals can express sexual feelings and
needs through a variety of alternatemeans.
Those with neurological disorders
can develop long-term intimate relation-ships that include love, respect, and mu-tually satisfying expression of sexualfeelings.
The ﬁnancial impact of many neurolog-
ical conditions can be devastating. Thecost of medical care, rehabilitation, assis-tive devices, and environmental restruc-turing can be signiﬁcant. The ﬁnancialadjustments that must be made have asigniﬁcant impact on the individual’s gen-eral lifestyle. Assistance by various socialagencies can help reduce the ﬁnancialburden and reduce the stress caused bythese concerns.
Social Issues
Many factors associated with disorders
of the nervous system can affect socialfunction. A supportive environment, in-cluding the family, plays an instrumentalrole in individuals’ response to disability.Misinterpretation or misperception of theindividuals’ disability and associatedfunctional limitations, however, can actas a barrier to effective social interactionand personal adjustment.
Although some functional limitations
associated with neurological conditionsare visible, such as the mobility restric-tions indicated by use of a wheelchair,others are not so readily recognizable. Forexample, the fatigue experienced in mul-tiple sclerosis, the visual or perceptualproblems experienced because of braindamage, or the difﬁculty with bladdercontrol in spinal biﬁda may create a con-ﬂict of expectations when others do notunderstand the reason behind certainGeneral Issues in Nervous System Conditions 115

behaviors. Those who do not understandthe consequences of multiple sclerosismay interpret fatigue as laziness or at-tempts to avoid work. Visual and percep-tual problems associated with brain injurymay be interpreted as clumsiness. Indi-viduals with spina biﬁda who need readyaccess to a restroom may be viewed ashaving a neurotic preoccupation with thelocation of restroom facilities. The conse-quences of stroke, which may involveemotional lability or memory, attention,or judgment problems, may be perceivedby others as rudeness, insensi
tivity, or irre-
sponsibility rather than man ifestations of
the condition itself. The gait disturbanceor slurred speech associ
ated with multiple
sclerosis may be viewed by others as a sign
of intoxication.
In addition to the stigma often attached
to disability, some conditions have asso-ciated myths or misinformation attached.Individuals with epilepsy, for example,may encounter social stigma because ofoutdated and erroneous beliefs about thecause or meaning of the seizures they ex-perience. Seizures can be frightening forthose who observe them. Lack of under-standing may cause people to avoid socialcontact with individuals with epilepsy soas to avoid the possibility of witnessing aseizure. Misunderstanding may also causepeople to avoid individuals with cerebralpalsy with communication difﬁcultiesbecause they want to avoid the discom-fort of attempting to understand what theindividual is saying.
All chronic illness and disability affect
family members and social interactions.Family members may become overly pro-tective, shielding individuals from re-sponsibility. Individuals may be excludedfrom family problems or decision making.In other instances family members mayﬁnd it difﬁcult to express anger towardtheir family member with a disability,instead coddling the individual or hidingtheir own displeasure over the individ-ual’s actions.
Some of the symptoms or behaviors
manifested in the neurological conditionmay be more troublesome to the individ-ual exhibiting the symptom than to thosearound them. Individuals may fear be-coming a burden on family members andconsequently withdraw from close per-sonal interactions, while family members,willing and anxious to provide help andsupport, are hurt at what they view as theindividual’s rejection of their attempts tohelp. In other instances, individuals mayassume that others would not want tointeract with them because of their dis-ability, when actually they are greatlyadmired by others because of their abili-ty to cope.
Wheelchair use may also affect social
function. Because not all social events orsituations are accessible to individualsusing a wheelchair, they may either avoidan activity or make special arrangementsto attend it. Although most public placeshave made provisions for accessibility,some are more desirable than others. Forexample, a multilevel historic site mayonly be accessible to individuals in wheel-chairs through a back entrance or freightelevator. In order to reach a stage for pres-entation, individuals may need to be“pushed” up a ramp rather than negoti-
ate the ramp themselves. In addition, differ-
ent angles of eye contact can create multi-
ple emotional impacts for individuals inwheelchairs, who must continually lookupward at their peers. This can producean impression of differing social stature,both in the individuals and in those withwhom they engage in conversation.
Social interaction difﬁculties associated
with neurological disorders may be man-ifest in poor social performance, socialanxiety, and low self-esteem. Individuals116 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

with such a disorder may experience con-
siderable frustration, as well as lowered self-
esteem and self-assurance, in the struggleto cope with social demands. Some neu-rological conditions result in impairedcapacity for social perceptiveness, dis-tractibility, an absence of social initiation,or behavioral problems (e.g., disinhibitionor impulsivity). These symptoms can sig-niﬁcantly affect individuals’ ability tointeract effectively in social settings. Inthese instances, social skills training orcontinuing supervision or prompting inthe social setting can help individuals tointegrate more fully into social situations.
Vocational Issues in Conditions of theNervous System
The capabilities of individuals with
nervous system conditions vary widely,depending on the nature of the condi-tion. For progressive conditions or condi-tions characterized by remissions andexacerbations, such as multiple sclerosis,ongoing evaluation of limitations andremaining function is necessary. For oth-er conditions, such as spinal cord injuryor traumatic brain injury, in which thedamage is permanent but not progressive,initial evaluation of capabilities andremaining function may sufﬁce.
Brain damage affects a number of func-
tions, all of which should be assessed. Notonly should cognitive functions, such asmemory, problem-solving ability, andspatial and temporal orientation, beassessed, but also motor abilities, such ascoordination, balance, speed of perform-ance, and muscle dexterity.
The degree of job stress is a factor to be
considered for those with a number ofneurological conditions. In some in-stances, stress in the workplace may addto fatigue that is already a consequence ofthe condition itself. In other instances,stress may precipitate symptoms, as itdoes in epilepsy or multiple sclerosis.Moreover, when individuals who experi-ence poor motor speed or decreased pro-cessing ability as a result of disability arerushed or feel stressed, the quality of theirwork may suffer.
When communication skills are affect-
ed by a neurological condition, alternatemeans of communicating in the work-place or job modiﬁcations may be need-ed. In many instances, even thoughindividuals’ communication may be dif-ﬁcult to understand, patience and practiceallow coworkers to establish basic patternsof communication that make interchangein the workplace possible.
Some conditions have speciﬁc charac-
teristics that must be considered in anassessment of the workplace. For example,for individuals with epilepsy it is impor-tant to assess the degree to which seizuresare controlled and to identify whetherany stimuli in the work environmentcould precipitate seizures. Hot, humidenvironments should be avoided by indi-viduals with multiple sclerosis. Indi-viduals with high thoracic spinal cordinjuries or cervical injuries often experi-ence difﬁculty with heat regulation andconsequently should avoid extremes oftemperature. In addition, because of theloss of sensation in the extremities, situ-ations in which there is a possibility ofburns or frostbite should be avoided.Individuals with spinal cord injuries,multiple sclerosis, or Parkinson’s diseasemay be especially susceptible to upper res-piratory problems. Consequently, theextent to which there is exposure to pol-lutants or upper respiratory infections,which could threaten respiratory func-tion, should be considered.
Accessibility of the workplace should be
evaluated if individuals use a wheelchairor other assistive devices and environ-General Issues in Nervous System Conditions 117

mental factors could interfere with mobil-ity. Availability of elevators as opposed tostairs, desk or workbench height, width ofdoorways, and size of bathrooms are allimportant environmental considerations.
In addition to the disability itself, a vari-
ety of other factors that could interferewith reaching full vocational poten
tial
should be considered. The availability of
transportation, the additional time re-quired for various activities, and the atti-tudes of coworkers can determineindividuals’ success or failure in the work-place. In all instances, a realistic appre-ciation of the individual disability iscrucial. Because of the complexity andmultifactorial aspects of nervous systemconditions, each person and his or herspeciﬁc needs and abilities should be
con-
sidered. Expectations for performance
may be too high or too low and may notmatch the individual’s abilities. Con-sequently, viewing the individual as anindividual with speciﬁc needs and abili-ties rather than putting him or her in acategory is crucial for vocational success.
CASE STUDIESCase I
Mr. G., a 27-year-old male, experienced
a spinal cord injury (T-10) when he fell offa roof while working as a self-employedcarpenter. He had been interested inbeing a carpenter since he could remem-ber, and he wanted to follow in the foot-steps of both his father and
grandfather.
He had attended high school, where he
enrolled in building trades courses andmechanical drawing classes. He marriedhis high school sweetheart several yearsafter high school, and they now have twochildren. Mrs. G., who has been workingas a legal secretary since their marriage,was devastated by the accident; however,with the help of family who live nearbyshe has begun to adjust to the perma-nence of her husband’s injury. Mr. andMrs. G. live in a rural community of2,000, and it is 170 miles from the near-est city. After the acute phase of theinjury, Mr. G. was transferred to a rehabil-itation center in the city where his voca-tional potential will be established.
Questions
1. What types of limitations would you
expect Mr. G. to experience given hislevel of injury?
2. What types of adaptive devices will
Mr. G. most likely need to achieve hisgreatest level of independence?
3. What types of alternative vocational
choices might Mr. G. have, given hisoccupation and training?
4. What lifestyle issues may need to be
considered?
5. How does Mr. G.’s family status
inﬂuence his rehabilitation goals?
6. What specific issues would you
address when working with Mr. G. toestablish his rehabilitation plan?
Case II
Ms. L. is a 32-year-old female who has
recently been diagnosed with multiplesclerosis. Ms. L. had sought medical atten-tion for several years before the diagnosiswas made because she experienced doublevision, weakness in her extremities, anddizziness. Because she was also goingthrough a separation and divorce at thesame time, her symptoms were attributedto “stress and depression,” and antide-pressant medications were prescribed.When her symptoms became worse andadditional testing was conducted, thediagnosis was made. The weakness in Ms.L.’s legs has continued, so that she has118 C
HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII

some difﬁculty walking and has collapsedseveral times. She has signiﬁcant blurringof vision, and she has had some numb-ness and weakness in her right hand,which is her dominant hand. Ms. L. livesalone. She has a master of science degreein education and has continued to workas a sixth-grade teacher, although shewonders how much longer she will beable to continue. She is currently in remis-sion; however, she has had exacerbationsabout every 3 months. She currently livesin a suburb of a large metropolitan citybut questions whether she should consid-er moving to her hometown, a small mid-western city, where she can be closer toher parents.Questions
1. What issues speciﬁcally related to Ms.
L.’s age and life situation should beconsidered in working with her todevelop a rehabilitation plan?
2. What speciﬁc issues in the diagnosis
of multiple sclerosis should be con-sidered?
3. Are there accommodations that Ms.
L. would need either at home or inher job to enable her to maintain hermaximum level of independence?
4. Will symptoms of her multiple scle-
rosis prevent her from continuingemployment? What issues wouldyou discuss?Case Studies 119

120 C HAPTER 3 CONDITIONS OF THE NERVOUS SYSTEM : PARTII
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NORMAL STRUCTURE ANDFUNCTION OF THE EYE
The eyeballs are spherical organs
encased in the orbital cavities of the skull.Muscles located on the top, bottom, andside of each eye enable it to rotate in dif-ferent directions (Figure 4–1). The eyelidserves a protective function. Through fre-quent blinking, the eyelid helps keep theeye moist, preventing irritation. Thelacrimal glands , which lie in the upper out-
er side of the eye behind the eyelid, secretetears to keep the eyeball moist and helprid the eye of foreign material.
In front of the eye lies a transparent
curved structure called the cornea , which
admits light and protects the inner eyefrom foreign particles and organisms.Although the cornea contains no bloodvessels, it is richly supplied with nervecells. Connected to the cornea and com-pletely covering the eyeball except for thepart covered by the cornea is a ﬁbrousmembrane called the sclera . The sclera
forms the white part of the eye and hasthe primary function of supporting andprotecting the eye and maintaining eyeshape. Lining the exposed area of the scle-ra and inner eyelid is a sensitive mem-brane called the conjunctiva . Lying
underneath the sclera and also surround-ing the eyeball is the choroid coat , which
contains most of the blood vessels thatnourish the eye.
The colored part of the eye is called the
iris. At the center of the iris is a round
opening called the pupil, which admitslight to the inner part of the eye. Thecornea covers both the iris and the pupil.Smooth muscle ﬁbers on either side of thepupil cause it to contract or dilate, there-by automatically regulating the amount oflight that enters the eye. In bright lightthe pupil contracts to reduce the amountof light admitted. In the dark, the pupildilates to admit as much light as possible.
Directly behind the iris is a space called
the posterior chamber . Contained in the
posterior chamber is a structure called the
ciliary process , which produces a transpar-
ent ﬂuid called the aqueous humor . The
aqueous humor escapes from the posteri-or chamber through the pupil into a spacelying between the iris and cornea calledthe anterior chamber , which lies between
the iris and the cornea. The aqueoushumor then drains from the eye intolymph channels and into the venous sys-tem through a sievelike structure calledthe canal of Schlemm (trabecular network ),
which is located at the junction of the irisand the sclera. The balance between theamount of aqueous humor produced andConditions of the Eye
and BlindnessCHAPTER 4
123

the amount drained helps to maintainnormal intraocular pressure (pressure with-
in the eyeball).
The aqueous humor nourishes both the
cornea and a structure located directlybehind the iris called the lens. The lens is
a small transparent disk enclosed in atransparent capsule. Attachments aroundthe circumference of the lens, called cil
iary
muscles , automatically contract or ex-
pand, changing the shape of the lens fromfat to thin or vice versa in response to theproximity or distance of an object beingvisualized. The changing shape of the lenspermits the eye to focus for near or farvision, a process called accommodation .
To focus on objects in the distance, the cil-iary muscles relax, thinning and ﬂatten-ing the lens. To focus on objects close by,the ciliary muscles contract so that thelenses becomes more rounded. Behind thelens is a larger cavity known as the vitre-
ous space . This space is ﬁlled with a jelly-
like, translucent substance called thevitreous humor , which helps to maintain
the form and shape of the eyeball.
At the very back of the eye is the inner-
most coat of the eye, the retina . The reti-
na contains two layers, a pigmented layerthat is ﬁxed to the choroid and an innerlayer that contains special light-sensitivecells called rodsand cones .
Rods are involved with detecting light
and dark as well as shape and movementand are primarily necessary for nightvision and peripheral vision. Rods containa derivative of vitamin A, rhodopsin , a
highly light-sensitive substance that breaks124 CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS
Muscle
Conjunctiva
Iris
Cornea
Pupil
Lens
Anterior chamber
Posterior chamber
Canal ofSchlemm
ScleraVitreoushumorCiliary muscle
Fovea(Macular area)
Optic discOptic nerveRetinaChorid
Aqueous humor
Figure 4–1 The Eye.

Measuring Vision 125
down rapidly when exposed to light. Thischemical process causes a reaction thatactivates the rods so that the eye adjusts,enabling individuals to see in
the dark.
This process is called adaptation.
Cones are involved primarily in daylight
and color vision as well as in the percep-tion of sharp visual detail. Most of thecones are located in a spot on the retinacalled the macula . The macula is the area
of clearest central vision. The center of themacula, the fovea , contains no rods and is
the area where vision is clearest in goodlight.
The optic nerve enters the back of the eye
through an area called the optic disk . This
area is sometimes called the blind spot
because it does not contain light-sensitivecells. Light rays pass through the cornea,enter the pupil, pass through the lens, andregister on the retina. Sensory cells of theretina receive light stimuli and convertthem into electrical impulses. These elec-trical impulses are then transmitted to theoptic nerve, which carries them to theoccipital lobe of the brain, where they are
interpreted.
After exiting the optic disk, the optic
nerves from each eye combine at the baseof the brain just in front of the brain stemto form the optic chiasm . At this point half
of the nerve tracts from each eye crossover to the opposite side of the brain. Botheyes receive information from a combina-tion of both visual ﬁelds. Depth perception
requires that the brain receive input fromboth eyes. The portion of the visual ﬁelddetected by both eyes is called the binoc-
ular visual ﬁeld and is necessary for depth
perception.
MEASURING VISION
Visual acuity is deﬁned as the sharp-
ness of the visual image perceived. Visualacuity tests are used to measure the levelof best vision and to measure the need forcorrective lenses. A standard test of visu-al acuity is the Snellen test . The chart used
for the test contains a series of letters onnine lines of decreasing size. Lines areidentiﬁed according to the distance fromwhich they can be read by individualswith unimpaired vision. For example,individuals with normal visual acuity canread the top line of the chart at 200 feetand the last line at 20 feet. When takingthe Snellen test, individuals view theSnellen chart at the equivalent of 20 feetand read the lines on the chart from thelargest to the smallest. Results of the testare expressed as a fraction, the numeratordenoting the equivalent distance from thechart at which the individual being test-ed views the chart (20 feet) and thedenominator denoting the distance fromthe chart at which a person with normalvision would be able to read the same line.Consequently, a visual acuity of 20/100means that the individual being tested cansee at 20 feet what a person with normalvisual acuity could see at 100 feet, indicat-ing that the individual has a visual im-pairment. On the other hand, a result of20/10 would indicate that the individualbeing tested has better-than-normal visu-al acuity, since he or she can see at 20 feetwhat individuals with normal visual acu-ity can only see at 10 feet.
Visual ﬁeld is deﬁned as the size of the
area that individuals can see without turn-ing the head or moving the eyes.Peripheral vision (side vision) is meas-
ured by a curved device called a perimeter .
Individuals look into the perimeter, anda test object is systematically moved fromoutside the peripheral ﬁeld of visiontoward the center until the individualindicates visualization of the object.Central vision (vision in the center of the
visual ﬁeld) is tested with the individuallooking at a tangent screen on which a test

object is systematically moved across thescreen. Individuals’ ability to see theobject at certain points is then mapped,outlining their central ﬁeld of vision.
TYPES OF VISUAL IMPAIRMENTS
When any deviation from normal
vision exists, individuals are considered tohave a visual impairment . Visual impair-
ments range from mild impairment tototal loss of vision. In general, conditionsinvolving the eye that result in visualimpairment can be categorized as follows:
• Refractive errors•
Difﬁculty with coordination of the eyes
• Opacities of the eye• Injuries to the eye •
Damage secondary to other conditions
• Degenerative changes of the eye
Visual impairments may be temporary,
reversible, progressive, or permanent andmay involve the following:
• Central ﬁeld of vision: Individuals are
able to see images in the periphery ofthe visual ﬁeld but not images in thecenter.
• Peripheral ﬁeld of vision: Individuals
are able to see images in the center ofthe ﬁeld of vision but not in theperiphery ( tunnel vision ).
• Night vision: Individuals have difﬁ-
culty seeing at night ( night blindness ).
• Color vision: Individuals have difﬁcul-
ty distinguishing colors, especially redand green. Rarely, individuals havecomplete lack of color vision withassociated low visual acuity ( achro-
matopsia ).
• Binocular vision: Individuals have dif-
ﬁculty with the coordinated use ofboth eyes to produce a single image.As a result, they may have doublevision ( diplopia ). Severe visual impairment can be deﬁned
as the inability to read ordinary newsprinteven with the aid of glasses (Lighthouse,1990). When deviations of vision are greatenough to cause total loss of light percep-tion, the term blindness is used. Many
individuals, however, have some usablevision if they use special aids or devices toperform most tasks. When ordinary glass-
es, contact lenses, medical treatment, and/
or surgery is unable to correct sight to thenormal range, individuals are said to havelow vision or to be partially sighted (Butler,
1997). The term legal blindness describes
both those who have total loss of visionand those who have some remaining visu-al function but are severely handicapped
by visual impairment. In the United States,
legal blindness has been deﬁned as follows:
1. Visual acuity not exceeding 20/200 or
worse in the better eye with correct-ing lenses
2. Central ﬁeld of vision limited to an
angle of 20 degrees or less
CONDITIONS CAUSING VISUALIMPAIRMENT OR BLINDNESSRefractive Errors
Refractive errors make up the most com-
mon type of eye disorder and occur whenchanges in the cornea, aqueous humor,lens, or vitreous humor prevent properbending of light rays to converge on theretina. One type of refractive error,myopia (nearsightedness), results from
elongation of the eyeball so that light raysfocus on a point in front of the retina.Individuals with this condition have goodvisual acuity for close objects but difﬁcul-ty seeing objects in the distance. The op-posite type of refractive error is hyperopia
(farsightedness), in which the eyeball isshorter than normal so that light rays126
CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS

focus on a point beyond the retina. Indi-viduals with hyperopia have good visualacuity for objects in the distance but havedifﬁculty focusing on things at closerange. Another type of refractive error,astigmatism , results from irregularity of
the shape of the cornea or at times thelens so that vision is distorted. Myopia,hyperopia, and astigmatism can occur atany age.
Presbyopia is a condition usually asso-
ciated with aging in which there is grad-ual loss of accommodation due to loss ofelasticity of the lens and weakening of theciliary muscle. Individuals with presby-opia must hold small objects and printedmaterial farther and farther away to seeclearly because the eye can no longeradjust the shape of the lens to allow clearvision of close objects.
Refractive errors are usually remedied
with corrective lenses. A surgical proce-dure called radial keratotomy (discussed lat-
er in the chapter) is now often performedto correct myopia. The procedure attemptsto correct nearsightedness by altering theshape of the cornea, causing it to ﬂatten.
Difﬁculty with Coordination of the Eyes
To achieve good vision, both eyes must
work together so that images from eacheye can fuse into one single image. Whenthis does not occur, vision may be
imped-
ed to some degree. Uncoordination of the
eyes can be the result of heredity, disease,or damage to the brain.
Nystagmus is a condition in which the
eyes move involuntarily even though thegaze is ﬁxed in one direction. The move-ment may be in any direction, but most
often movement is horizontal. Nystagmus
may be congenital or may develop late asa result of a neurological disease or otherdisorder. Although the condition maycause little visual disturbance and beunnoticeable to the individual, it may bedistracting and noticeable to others.
Strabismus is a condition in which the
eyes cannot be directed to the same objector in which the eyes are crossed, turninginward. It may result from un
equal ocu-
lar muscle tone or from a neuro logical con-
dition. It ca n often be cor rected by surgery,
corrective lenses, medications, or a com-bination of the three.
Suppression amblyopia (lazy eye) is a
condition in which one eye does not de-velop good vision, usually because of stra-bismus. The condition is usually treated atan early age by placing a patch over theeye. The eye usually responds to earlytreatment and the condition is corrected.If, however, the condition is not treatedearly, the condition can persist for life.
Opacities of the Eye
Opacities of the Cornea
Any condition, including injuries, in-
ﬂammation, or disease of the cornea, thatcauses scarring or clouding of the corneacan cause permanent partial or total lossof vision. Because of its rich nerve supply,inﬂammation or injury to the cornea cancause severe pain. Prompt treatment of acorneal inﬂammation can prevent subse-quent formation of scar tissue, which caninterfere with vision. When clouding orscarring of the cornea causes permanentvisual loss, a corneal transplant (discussed
later in the chapter) may be performed.When a corneal transplant is successful,vision may be restored with few, if any,restrictions.
Cataract
A cataract is a clouding or opacity of
the lens of the eye. Although cataracts area common cause of visual impairment inConditions Causing Visual Impairment or Blindness 127

older adults, they may also be congenital,hereditary, the result of ocular trauma orinﬂammation, or associated with a varietyof other conditions, such as diabetes. Theycan also be drug induced, such as from theuse of high levels of certain types ofsteroids. Individuals with cataracts oftendescribe their vision as looking through acloudy pane of glass, or through a fog.
Although cataracts are generally bilater-
al, they may form at different rates in eacheye. As the lenses become more opaque,vision gradually diminishes. If cataractsare the result of injury, such as from radi-ation or a foreign object striking the lens,loss of vision occurs more rapidly. Cata-racts associated with aging progress moreslowly over time.
Because there is no way to return the
lens to its normal transparency, treatmentof cataracts involves removing the lensand then replacing it with an implant,with glasses, or with both. There are twomajor methods of cataract removal. Onemethod, extracapsular cataract extraction , is
a surgical procedure in which the lens isremoved but the posterior portion of itscapsule is left in position. An intraocularprosthetic lens is generally inserted intothe eye at the time of surgery. The secondmethod, intracapsular cataract extraction , is
a surgical procedure in which the lens andits capsule are completely removed. Bothtypes of surgeries are usually performed onan outpatient basis. Any implanted lenshas a ﬁxed focal length, so that vision isclear at only one distance. Thus, individ-uals who have undergone cataract surgerymay continue to need corrective lenses,such as bifocals.
Injuries to the Eyes
Eye injuries are common and also pre-
ventable. The most common type of eye in –
jury is an injury to the cornea caused bya foreign body in the eye. Although oftenconsidered minor injuries, these types ofinjuries can become serious if there is ascratch or abrasion to the cornea thatbecomes infected or causes scarring, whichcan impede vision. When the corneabecomes so scarred that vision is severelycompromised, a surgical procedure, corneal
transplant (keratoplasty ), may be per-
formed. Corneal transplantation may alsobe performed when the shape of thecornea is distorted. Donor eyes for cornealtransplantation come from individualswho have recently died. During the surgi-cal procedure, the opaque area of corneafrom the recipient’s eye is replaced withthe clear donor cornea, which is suturedinto place. Because the cornea has noblood vessels, the healing process is slow.Although a corneal transplant can restorevision, there is also the chance of graft re-jection or the need for a second operation.
More serious injuries are chemical burns,
corneal laceration, or bleeding into theanterior chamber ( hyphema ), all of which
can threaten vision. Chemical agentswith an alkaline base , such as cleansing
agents, fertilizers, plaster, or refrigerants,can penetrate the eye rapidly, leading tocell disruption and tissue death. Chemicalagents with an acid base cause protein
coagulation in the eye so that penetrationis not as rapid but scarring of tissue canstill result in visual loss. In both instancesimmediate irrigation of the eye candecrease the amount of damage; howev-er, emergency medical treatment shouldalso be sought immediately.
Some injuries involve puncture or lac-
eration of the eye. Common causes arework-related injuries, such as from chop-
ping or sawing wood, or chiseling or ham-
mering metal on metal, in which a strayfragment of material causes the laceration.In some instances, the puncture is so smallthat it may not be immediately
detected.128 CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS

Puncture or laceration of the eye warrants
immediate medical attention.
Deep penetration or laceration of the
eye can cause bleeding into the anteriorchamber ( hyphema ). Blows to the head
or the eye can also cause damage to theinternal structures of the eye, causinghemorrhage, retinal damage, or otherinjury. When bleeding causes increasedintraocular pressure, surgical interventionmay be needed to relieve the pressure andprevent further damage.
Any injury to the eye necessitates a con –
sultation with an ophthalmologist (a
physician who specializes in diseases andtreatment of the eye). The degree of visu-al loss that results from an eye injury is afunction not only of the extent and typeof injury but also frequently of the delayor promptness of emergency treatment.
Most eye injuries are preventable. Using
appropriate eye protection for work,home, and sports activities that carry a riskof eye injury is one of the major forms ofprevention.
Inﬂammation and Infections of the Eye
The most common eye disease is con-
junctivitis (inﬂammation of the mem-
brane that lines the eye, the conjunctiva .
It may be caused by infectious organisms,allergy, or chemicals. In most instances,conjunctivitis is easily treated, is self-lim-iting, and has no permanent effects.Some types of infectious conjunctivitis,such as gonococcal conjunctivitis , or tra-
choma , however, can cause ulceration of
the cornea and subsequent blindness.
Uveitis is an inﬂammation of the uveal
tract (iris, ciliary body, choroid ). It may be
associated with an autoimmune diseasesuch as ankylosing spondylitis (see Chapter
14), inﬂammatory bowel disease (see Chap-
ter 10), or local or systemic infection, suchas
human immuno deﬁciency virus (HIV).Symptoms may include decreased visionand sensitivity to light ( photophobia ). It
is usually treated with topical medication.
Keratitis is an inﬂammation of the
cornea that can be associated with a num-
ber of infectious conditions, including her-
pes simplex ( herpetic keratitis) ; it can also
be a complication of HIV. Another com mon
cause of keratitis is use of contacts. Microb –
ial keratitis is associated with the use of
contact lenses and is usually caused by im-
proper handling of contact lens equipment
and solutions (Bienfang, Kelly, Nicholson,& Nussenblatt, 1990). One decreases therisk of infection by washing hands andusing the appropriate technique for clean-ing and applying contact lenses.
Glaucoma
Glaucoma is a condition involving in-
creased intraocular pressure. If left untreat-ed, permanent damage to the optic nervecan result, causing blindness. Glaucomacan occur as a primary condition or sec-ondary to other conditions, such as dia-betes, trauma, infection, or prolonged useof medications such as steroids. Glaucomaoccurs when the amount of aqueoushumor produced exceeds the amountbeing drained from the eye, much like asink into which water continues to ﬂoweven though the drainage pipe is blocked,resulting in overaccumulation of water inthe sink.
Types of Glaucoma
There are several types of glaucoma.
Broad categories are based on the reasonfor the problem with the aqueous ﬂow.
Chronic Open-Angle Glaucoma
The most common type of glaucoma,
chronic open-angle (simple ) glaucoma , occursConditions Causing Visual Impairment or Blindness 129

when the outﬂow of aqueous humor fromthe eye is reduced. Because the outﬂow nolonger equals the inﬂow, the amount ofaqueous humor builds and pressure in theeye increases. Open-angle glaucoma gen-erally progresses slowly over many years,producing no symptoms until the opticnerve is sufﬁciently damaged to reducevisual acuity and visual ﬁeld. At this point,the damage is irreversible. Vision loss gen-erally begins with the loss of peripheral
(side) vision so that individuals can seeonly straight ahead, as if looking througha tunnel ( tunnel vision ). Because loss of
peripheral vision is often gradual, individ-uals may be unaware of the problem untiladvanced stages of the condition. If un-treated, the ﬁeld of vision continues tonarrow until all vision is lost. There is nocure for chronic open-angle glaucoma.However, if the condition is detected ear-ly, appropriate medical treatment can con-trol it for many years. Consequently, earlydetection is important.
Acute Closed-Angle Glaucoma
Acute closed-angle glaucoma develops
much more rapidly than chronic open-angle glaucoma and is a medical emer-gency. Symptoms include sudden severepain, sharply decreased vision, nausea andvomiting, and rapid damage to the opticnerve with associated vision loss. Acuteclosed-angle glaucoma results from anabrupt blockage and obstruction of thecanal of Schlemm so that aqueous humor
rapidly accumulates in the anterior
chamber of the eye. Although acute closed-
angle glaucoma is much less commonthan chronic open-angle glaucoma, it isa medical emergency and must be treat-ed immediately to prevent blindness.Initially it may be treated with medica-tions, but surgical intervention is alsooften necessary.Treatment of Glaucoma
Treatment of glaucoma is directed to-
ward reducing the intraocular pressure bydecreasing the amount of aqueous humorproduced or by increasing its outﬂow. Thiscan be accomplished with medication orthrough surgically creating a new pathwayfor drainage.
Medication for the treatment of glauco-
ma, whether eye drops or oral medication,must be used daily throughout life to con-trol eye pressure and prevent further dam-age to vision. In either type of glaucoma,early detection and treatment are criticalto prevent irreversible damage to the opticnerve and subsequent blindness. Regard-less of the type of glaucoma, lifetime med-ical supervision is required. Most peoplewith glaucoma can lead normal, unre-stricted lives without blindness if the con-dition is identiﬁed early and the medicalregimen is followed as prescribed.
Treatment of Chronic
Open-Angle Glaucoma
Chronic open-angle glaucoma may be
controlled with medication in the form ofeye drops alone to decrease production ofaqueous humor or in combination withoral medication that reduces pressure inthe eye, thus halting progression of thedisease. Because eye drops are absorbedinto the bloodstream, they may affect oth-er body functions and cause systemic sideeffects ranging from generalized weaknessto central nervous system, cardiovascular,or gastrointestinal symptoms. Oral med-ications for the treatment of glaucomawork by decreasing the production ofaqueous humor. Like eye drops, oral med-ications can also affect other body func-tions and cause systemic side effects.Consequently, individuals who use eyedrops or oral medication for treatment of130
CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS

glaucoma should be under continuingmedical supervision, not only to monitorthe condition itself but also to identify anyside effects of the medication.
When intraocular pressure cannot be
successfully controlled with medication,individuals with chronic open-angle glau-coma may have a surgical procedurecalled trabeculectomy that relieves pressure
by creating a passageway for the aqueoushumor to drain. Individuals may also needto continue using eye drops or oral med-ication after surgery to control pressure;however, in some instances surgery mayeliminate the need for medication.
Treatment of Acute Closed-Angle Glaucoma
Acute closed-angle glaucoma results
from the forward displacement of the iris,which narrows or obstructs the path foraqueous humor outﬂow. Eye drops calledmiotics constrict the pupil, thus enlarging
the drainage passageway and facilitatingthe outﬂow of aqueous humor. Because ofthe emergency nature of acute closed-angle glaucoma, oral or intravenous med-ication is given immediately to relievepressure on the optic nerve temporarily.Once the pressure level has dropped to asafe level, a surgical procedure called iri-
dotomy may be performed. Iridotomy
involves the removal of a small section ofthe iris so that the aqueous humor canﬂow freely from the posterior to the ante-rior chamber of the eye, thus preventingfurther eye damage by relieving built-uppressure. This procedure is often per-formed with a laser. At times iridotomymay also be performed prophylactically inthe unaffected eye after an acute attack.
Retinopathy
Any disease or disorder of the retina is
a retinopathy . Retinopathies are often namedfor their cause. For example, arteriosclerot-
ic retinopathy is due to changes that occur
in blood vessels in the retina because ofarteriosclerosis. Hypertensive retinopathy is
due to changes that occur in blood vesselsin the retina because of high blood pres-sure. In both instances, treatment of theprimary underlying condition can controlthe progress of retinopathy.
The most common type of retinopathy,
and the most common cause of blindness,is diabetic retinopathy . Diabetic retinopathy
is the result of damage to the retina and is
a complication of diabetes mellitus (see
Chap ter 9). Usually there are no symptoms
in early stages of diabetic retinopathy.Consequently, regular comprehensive eyeexaminations by a physician are impor-tant in helping to prevent visual loss.
There are two categories of diabetic
retinopathy:
1.Nonproliferative diabetic retinopathy
2.Proliferative diabetic retinopathy
Nonproliferative diabetic retinopathy is
caused by changes in blood vessel wallsthat allow ﬂuids to leak into retinal tissue.At the same time, small blood vessels inthe retina may become occluded, disturb-ing circulation in the retina so that someretinal tissue receives too little oxygen anddies ( necrosis ).
Proliferative diabetic retinopathy results
from extensive areas of closure of thesmall blood vessels in the retina. As aresult, retinal tissues receive too little oxy-gen ( ischemia ) and growth of new vessels
is stimulated. These new blood vessels areabnormally fragile and prone to bleed,causing hemorrhage into the vitreous
humor . The degree of vision loss depends
on the amount of hemorrhage. Vesselsmay burst, ﬁlling the back of the eye withblood and resulting in signiﬁcant visualloss. In some instances, scar tissue associ-ated with new vessels can pull on the reti-Conditions Causing Visual Impairment or Blindness 131

na so that it detaches from underlying tis-sue. (See “Retinal Detachment” below.)
Surgery may be performed to remove
vitreous gel and hemorrhage ( vitrectomy ),
or laser treatment may be performed tostop the bleeding. Laser photocoagulation is
a procedure in which an intense beam oflight from a laser is used to seal leakingblood vessels of the retina. The laser beampasses through the lens of the eye and vit-reous ﬂuid without harming the struc-tures. It then is directed to a very preciselydeﬁned area to destroy fragile vesselsprone to hemorrhage or diseased areas ofthe retina in which there may be addition-al proliferative vessel changes. Laser pho-tocoagulation may help reduce the risk ofvisual loss, but it does not stop the pro-gression of diabetic retinopathy. Lasertreatment is usually performed on an out-patient basis.
Retinal Detachment
With detached retina, the sensory lay-
er of the retina becomes separated fromthe pigmented (choroid) layer, deprivingthe sensory layer of blood supply. De-tached retina may result from a suddenblow to the head, a tumor in the choroidlayer, retinal degeneration caused fromconditions such as arteriosclerosis, orhemorrhage with conditions such as dia-betic retinopathy.
Symptoms may develop suddenly or
slowly over time. Individuals may noticeﬂashes of light or a loss of vision in dif-ferent areas of the visual ﬁeld, or they mayexperience a complete loss of vision in theaffected eye. Usually there is no painaccompanying symptoms. Retinal detach-ment in one eye may indicate an in-creased risk of detachment in the othereye. Prompt diagnosis and surgical treat-ment are essential to prevent permanentvision loss. A surgical procedure calledscleral buckling is sometimes used to treat
retinal detachment. Scleral bucklingmechanically restores contact of the reti-na with the choroid. The area of the scle-ra that lies over the retinal defect isdepressed with an implant so that thechoroid and retina are pressed together.
Retinitis Pigmentosa
A hereditary condition, retinitis pig-
mentosa , involves the slowly progressive
loss of peripheral vision. Although thereis progressive restriction of the visual ﬁelddue to loss of peripheral vision, the re-maining central visual acuity is oftengood. Frequently the ﬁrst symptom ofretinitis pigmentosa is difﬁculty withnight vision ( night blindness ), which usu-
ally begins in late youth or early adult-hood. Total bilateral loss of vision canoccur in later stages of the disease. Thereis no cure or treatment for the condition;however, a number of assistive devicesmay be utilized to enhance function. (See“Assistive Devices and Low-Vision Aids”later in the chapter.)
Macular Degeneration
Degenerative changes in the macula,
the part of the eye needed for seeing ﬁnedetail and central vision, results in a con-dition known as macular degeneration .
Macular degeneration usually occurs
after the age of 50, from no apparentcause. Painless loss of central visual acu-ity is usually slow, with visual distortionor blurring of vision being the ﬁrst symp-tom. Eventually individuals may developa blind spot in the center of their ﬁeld ofvision that gradually increases in size asthe condition progresses. Since warningsigns of macular degeneration are absentuntil central vision is affected, regular eye
examinations are important for early iden -132 CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS

tiﬁcation of the condition and so treat-ment to its progress can be implemented.
Macular degeneration does not result in
complete blindness, but it destroys someor all of the sight in the center of the ﬁeldof vision. There are two types of maculardegeneration: dry form and wet form.Most cases of macular degeneration are dry
form, in which there is atrophy (shrink-
age) and thinning of the macula, causingmild to moderate vision loss. The type ofmacular degeneration called wet form is
characterized by signiﬁcant loss of visiondue to abnormal blood vessel formationand hemorrhage.
Both types of macular degeneration are
characterized by loss of central visionwhile peripheral vision remains intact.There is no treatment or cure for maculardegeneration, but the use of assistivedevices may increase visual function (see“Assistive Devices and Low-Vision Aids”below). Activities such as reading may be-come difﬁcult because of distortion of let-ters or parts of words or sentences in thecenter of the reading material that appearto be missing. Large print with black typeand white background may make readingeasier, as well as assistive devices such asa magnifying glass.
DIAGNOSTIC PROCEDURES FORCONDITIONS OF THE EYEComprehensive Eye Exam
Eye examinations usually include an
external eye exam that measures eyemovements and the size of the pupils andtheir ability to react to light. It alsoincludes testing visual acuity and visualﬁeld as described earlier in the chapter.This part of the exam may be performedby an optometrist (nonphysician who
specializes in correcting refractive errors).An ophthalmologist (physician who spe-cializes in evaluation and treatment ofconditions of the eye) may also conducttests of visual acuity, but he or she alsochecks ocular movement, the function ofthe optic nerve, and light reﬂexes of thepupils, as well as identifying any opticnerve pathology.
Individuals may also be asked to view a
chart through an instrument called a re-fractor. The physician then shines a lightthrough the refractor onto the retina toestimate the eye’s ability to focus on dis-tant objects. Also included in a compre-hensive eye exam is a tonometry (described
below) and a slit-lamp exam , which eval-
uates the eye’s structures, including thecornea and iris, and screens for cataracts.A retinal examination to check for retinal
disease may also be included. This requiresthat the pupil of the eye be dilated.
Tonometry
Tonometry is used to measure pressure in
the eye in order to detect glaucoma. Aninstrument called a tonometer is placed
directly on the cornea after the cornea hasbeen anesthetized with drops of a localanesthetic. The tonometer measures theamount of pressure within the eye, thusmaking it possible to detect glaucoma.
Gonioscopy
For gonioscopy , a special contact lens
that contains a mirror is gently placed onthe eye. The ophthalmologist uses the lenslike the periscope of a submarine to exam-ine structures inside the eye. The test isespecially helpful in detecting glaucoma.
Ophthalmoscopic Examination
A direct ophthalmoscopic examination is
a procedure used to examine the internalstructures of the eye. It is performed withDiagnostic Procedures for Conditions of the Eye 133

an instrument called an ophthalmoscope
that is placed close to the eye. The oph-thalmoscope contains a light that shinesinto the eye and magniﬁes internal struc-tures so that the physician can note anypathologic changes.
The internal structures of the eye may
also be observed with a slit lamp , a type
of microscope that is placed in front of theeye. The physician shines a ﬁnely focusedslit of brilliant light onto the eye to mag-nify details of the cornea, iris, and lens. Aslit lamp is especially useful in identifyingforeign bodies in the eye, evaluatingcorneal ulcers, and diagnosing cataracts.
Fluorescein Angiography
The purpose of ﬂuorescein angiography is
to detect changes in the blood vessels of
the retina. A ﬂuorescein dye is either taken
orally or injected into the bloodstream. When
the dye reaches the blood vessels of the eye,
special ultraviolet lights enable the phys-ician to photograph the vessels for lat
er
study. Any swelling or leakage of the vessels
of the retina is apparent on the photograph.
TREATMENT AND MANAGEMENT OFCONDITIONS OF THE EYE ANDBLINDNESSEyeglasses and Contact Lenses
Corrective lenses may be in the form of
eyeglasses or contact lenses. Because there
are so many different types of eye disorders
that interfere with visual acuity corrective
lenses must be prescribed individually. They
are prescribed by an ophthalmologist (a
physician who specializes in the diagno-sis and treatment of disorders of the eye,
including surgery and prescription of med-
ications and optical corrections) or anoptometrist (an individual who does not
have a medical degree but is trained tomeasure refractive errors and perceptualdysfunctions of the eye, as well as to diag-nose visual conditions and prescribe opti-cal corrections). Optometrists do not pre-scribe medications or perform surgery.Lenses for glasses are made by opticians ,
technicians who have been trained to ﬁll
optical prescriptions. They grind and con-
struct the lens according to the prescribedspeciﬁcations.
When visual acuity at several different dis –
tances must be corrected, bifocal or trifocal
lenses may be prescribed. Individuals with
bifocal lenses use the lower portion of the
lens for near vision and the upper portion
for far vision. Trifocal lenses have three differ –
ent divisions: one for near vision, one for in –
termediate vision, and one for far vision.
There are several different types of con-
tact lenses; however, the most common are
hard and soft corneal lenses. Hard lenses
cover the central area of the cornea and are
generally more durable. Soft lenses coverthe entire cornea and are generally morefragile. Regardless of the type, contactlenses must be individually prescribed andconstructed. They are helpful for a varietyof visual conditions, but they do not cor-rect astigmatism.
Generally, there are no complications
associated with wearing eyeglasses. Con-tact lenses, however, can damage the eyeif they are not worn and cared for proper-ly. Not all people can or should wear con-tact lenses. Overwearing of hard lenses cancause corneal abrasions and associatedcomplications. Individuals who do not usegood hygienic practices when insertingthe contact lens may develop an infectionof the eye, in which case contact lensesshould not be worn.
Refractive Eye Surgery
Growing numbers of individuals, hop-
ing to discard corrective lenses, have refrac-134 CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS

tive surgery to correct their vision. Refrac-tive surgery may be used to treat
myopia
(nearsightedness), hyperopia (farsighted-
ness), or astigmatism (irregularity in the
shape of the cornea or lens resulting in dis –
tortion of the visual image). Three primarysurgical techniques are used in refractoryeye surgery: radial keratotomy , photorefrac-
tive keratectomy , and laser-assisted in situ
keratomileusis . Although refrac
tive eye sur-
gery is often effective in correct ing the
visual problem, there is also the potentialfor complications (Buckingham, 2000).
Prosthetic Devices andEye Replacement
When injury or disease necessitates re-
moval of the eyeball, or when there is a con-
genital absence of the eye ( anophthal-
mia), a prosthetic eye may be constructed
and worn by the individual. The prosthetic
eye, while not contributing to vision, serves
a cosmetic purpose and can enhance theindividual’s body image and self-concept.
Visual impairment due to conditions in
which there is interruption of electricalstimulation somewhere along the visualpathway or to the retina, where photore-ceptors are located, has spurred researchinto the use of electrical stimulation toovercome visual loss. Optical prostheses ,
which emulate functions of photorecep-tors located in the retina, are an exampleof devices that have the potential forrestoring rudimentary vision (Scarlatis,2000). Although still in experimentalstages, the development of optical pros-theses is an important advance that maymake it possible for individuals with reti-nal damage to have partial vision restored.
Assistive Devices and Low-Vision Aids
Assistive devices and low-vision aids
should be part of an overall program to en-hance the life of the individual, not just toenhance the visual system. The types of de-
vices used should be based on individual
needs as well as the willingness and abili-ty
to use them. The overall goal is to recap-
ture, strengthen, and maintain individ-uals’ self-conﬁdence for safe, independentfunctioning. These aids enhance remain-ing visual abilities through the use of indi-
vidually prescribed adaptive equipment ap-
propriate to individuals’ speciﬁc lifestyle.
Adaptive equipment for activities of
daily living may include optical devices,such as high-powered lenses and telescop-ic spectacles, or nonoptical devices thatare readily available and require no specialtraining, such as large-print reading mate-rial or large-button telephones. Low-tech-nology devices such as talking watches,raised dot markings for oven dials, ortemplates for check signing require littletraining and may require only simpleadaptations. Additional devices, such astalking clocks and timers, writing guides,talking books, and audiocassettes, alsohelp to meet the communication needs ofindividuals with visual impairments.
High-technology devices are more soph-
isticated electronically and may requirespecialized training. Examples are videomagniﬁers and computer systems. Videomagniﬁers use closed-circuit television andcan magnify a printed page on a televisionscreen for reading. Numerous computersoftware programs and adaptive devicescan be used to enlarge printed materialsor to convert print into synthetic speechoutput. These devices include large-printcomputer monitors, programs that enlargeprint size on the screen, printers that mod-ify font size, synthetic speech softwareprograms with external audio units, andtypewriters equipped with syntheticspeech output that interface with person-al computer units. Speech packages allowfor adjustments in the rate of speech andTreatment and Management of Conditions of the Eye and Blindness 135

the tone of voice to meet the needs of theindividual user.
One of the best-known tactile aids is
Braille . Hard-copy Braille uses the familiar
raised dot method, whereas soft-copy Braille
is stored on electromagnetic tape and pre-sented as patterns by a set of pins that rep-resent a Braille dot. Individuals place theirﬁngers on display units through which thepins protrude. Another type of tactile aidis an electromechanical vibratory system . A
small camera is passed over a line of print,and each printed letter is then displayedas a pattern of vibration that the individ-ual can feel with the ﬁnger.
A number of professionals recommend
and provide training in the use of optical,nonoptical, low-technology, and high-technology aids. These include occupation-
al therapists , low-vision specialists , orienta-
tion and mobility instructors , rehabilitation
teachers , and adaptive technology specialists .
The key to the successful use of any
assistive or adaptive device is making surethat the individual using the device isinvolved in selecting it so that the devicemeets his or her requirements, capabilities,and needs. This customization helps indi-viduals view the device positively and useit to best enhance their own functionalcapacity (Lacey & MacNamara, 2000).
Orientation and Mobility Training
The goal of orientation and mobility train-
ingis to enable individuals with a visual
impairment to achieve as much mobilityas possible according to their capabilitiesand desires and to recapture, strengthen,and maintain self-reliance for safe and in-dependent function. Orientation and mobil-
ity (O&M ) specialists provide training that
helps individuals know where they are inrelation to their surroundings and how tosafely navigate within their environment(Turnbull, Turnbull, Shank, Smith, & Leal,2002). O&M specialists help individualsmove independently indoors and out-doors and in familiar or unfamiliar envi-ronments and also provide training in theuse of public transportation, use of thecane, and use of mobility lights or elec-tronic travel aids. Through individualizedtraining, individuals with visual impair-ments learn to orient themselves to theirenvironment by using compensatorystrategies, including illumination tech-niques and the use of contrast, magniﬁ-cation, memorization of location, andauditory and tactile feedback. Compen-satory strategies may involve such thingsas listening for the direction of trafﬁc, armand hand positioning for guidance alongwalls and railings, and systematic searchtechniques for dropped or lost objects.
Mobility Aids
Various types of mobility aids, such as
sighted guides, guide dogs, canes, andelectronic devices, are available to help in-dividuals with a visual impairment moveabout the environment more freely (Cox& Dykes, 2001). Orientation and mobili-ty specialists can help the individual ﬁndthe best system.
Guide dogs not only increase the mobil-
ity of the individual with a visual impair-ment but can also provide protection andcompanionship. Guide dogs undergo in-tensive training before being matchedwith the individual to whom they areassigned. They are taught how to respondto various commands as well as how torespond to curbs, trafﬁc, and other poten-tial hazards in the environment. The indi-vidual and dog train together for anumber of weeks to become an effectiveteam. Not all individuals are able to use aguide dog, and some individuals prefer touse other forms of mobility aids, such asa long cane.136
CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS

The most common mobility aid is the
prescription or long cane, which is usual-ly made of aluminum or ﬁberglass. Anorientation and mobility specialist pre-scribes the cane according to the individ-ual’s height, length of stride, and comfort.The cane is used by those with visualimpairments in a systematic way. Theindividual moves the cane rhythmically inan arc in front of the body to ensure a safespace for the next step. Although this pro-vides some protection, it does not accountfor objects above the waist that are in theindividual’s path. In an attempt to com-pensate for this type of obstacle, somecanes have tone-emitting radar units thatgive a differential pitch for the directionand height of obstacles in front of theindividual. Some individuals prefer col-lapsible, folding, or tele
scopic canes,
which are less obtrusive and can be col-
lapsed and slipped into a purse or undera chair when not in use.
Electronic travel aids may also be used.
These devices emit light beams or ultrasound
waves. When the light beam or ultrasoundwave hits an object in the individual’spath, the device vibrates or emits a sound.
PSYCHOSOCIAL ISSUES INCONDITIONS OF THE EYE ANDBLINDNESSSpecial Issues for Individuals Who ArePartially Sighted
Individuals with low vision or who are
partially sighted do not quite ﬁt into the
category of either the blind or sighted pop-
ulation. Consequently, they often havespecial needs that are overlooked. Thesocial community often lacks understand-ing of the true nature of vision impair-ment, so that individuals with low visionare ridiculed in public for appearing to seemore than would be expected by a personwith visual impairment (Vance, 2000).Individuals with partial sight may beviewed as malingerers by their familiesand acquaintances because they can seesome things but not others. Even whenthey attempt to function with appropri-ate assistive devices, they may be suspect-ed of denying their condition by thosewho expect individuals with visual impair-ments to be dependent and isolated.
Adjustment to vision loss is not necessar-
ily correlated with the degree of remain-ing vision. Individuals with partial sightdo not have fewer adjustment issues thanthose who are totally blind, and in factmay have more adaptation difﬁculties be-cause their partial sight presents an am-biguous situation for others. In addition,individuals with partial sight may exhib-it high levels of anxiety because they maybe unsure about whether or when theywill lose more of their residual vision.
Even when individuals with severe
visual impairments function independent-ly for the most part, there may be someactivities for which they are more depend-ent on assistance from others. The greaterdependence associated with many severevisual impairments may be a source ofconﬂict and may have a negative impacton formerly close relationships, especial-ly if others have misperceptions or misun-derstandings about the nature of theimpairment. In other instances individu-als, in an attempt to demonstrate self-reliance and independence, may rejecthelp from family and friends, causingalienation and social isolation. Counselingindividuals to understand sighted people’sreactions may facilitate social interactionsand enhance the development of con-structive and realistic interactions. Attimes, individuals with visual impair-ment may ﬁnd it helpful to share theirexperiences and problems with otherswho also have low vision.Psychosocial Issues in Conditions of the Eye and Blindness 137

Because partially sighted individuals
have some remaining sight, they mayattempt to “pass” as a sighted person toavoid potential rejection or avoidance byothers. They may deny their disabilityaltogether and associate only with sight-ed persons in an attempt to be acceptedby the mainstream of society. They maymake excuses for awkward behavior orattempt in other ways to conceal the factthat they have low vision. They mayrefuse to use low-vision aids, such as acane, for mobility or reject suitable orien-tation and mobility training. In extremecases they may engage in dangerousactivities such as illegal driving. Peopleoften view the ability to drive as veryimportant to the maintenance of inde-pendence. This makes it extremely difﬁ-cult for individuals who are losing theirvision to give up this activity. Further-more, by its very nature, the gradual lossof vision creates a time period in whichthe decision to stop driving is particular-ly difﬁcult. The emphasis on self-care andindependence for individuals with partialvision must be tempered with judgmentand concern for the welfare of the individ-ual as well as for that of others.
Psychological Issues in Conditions ofthe Eye and Blindness
Vision loss often precipitates a sense of
fear and reduced personal competence,which may result in isolation and socialwithdrawal. Visual impairments may bepresent at birth, or they may develop sud-denly or slowly at any time in an individ-ual’s life. Often visual loss follows anunpredictable and uncontrollable progres-sion. Adjustment to loss of vision dependson many factors, including the degree ofloss and the age at which the individualbecomes visually impaired. Those who arecongenitally blind, for example, have nothad the opportunity to learn conceptssuch as distance, depth, proportion, andcolor. Because of their lack of visual expe-riences in their environment, such as theobservation of others’ tasks or behaviors,concepts that sighted individuals oftentake for granted must be learned by oth-er means. This adaptive learning of tasksthen becomes a natural part of their devel-opment, so that adjustment to visual lim-itations is incorporated into their self-perception and daily activities as a normalpart of growing up.
Individuals with loss of vision later in
life have the advantage of being able todraw on visual experiences in the environ-ment as a frame of reference for physicalconcepts, but they may ﬁnd it more dif-ﬁcult to accept blindness than those whohave never had vision. Individuals wholose vision later in life must modify theirself-perception as a result of physicalchanges and the subsequent need to re-structure daily activities. Individuals whoare newly blind may experience grief anddespair over their loss of visual function.They may become dependent, feel inse-cure in new situations, and perceive amarked loss of autonomy. Some maybecome reluctant to interact in social sit-uations because they want to avoid theawkwardness of initial attempts at socialinteractions. Loss of control over standardmethods of initiating conversations (e.g.,eye contact and other nonverbal cues), thenoticeable discomfort or overhelpfulnessof sighted persons, and often prolongedgaps in conversations may lead newlyblind individuals to believe that they arebeing watched or ignored.
Accommodation to visual loss or blind-
ness is multifaceted. Individuals with avisual loss must adjust their self-conceptand personal goals to take into accountthe realistic limits imposed by vision loss.They must develop adaptive skills and138
CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS

new abilities, and they must draw on per-sonal resources to adjust. Individuals whohave lost vision because of traumaticblindness (conditions in which there hasbeen a sudden loss of partial or total visionbecause of an internal or external event,such as a head injury, direct injury to theeye, or chemical burn) may have to copenot only with sudden loss of vision butalso with insurance company representa-tives, attorneys, and other legal andbureaucratic aspects surrounding the cir-cumstance of their
disability. In these sit-
uations, family mem bers may also react to
the situation, show ing anger, revenge, or
overprotectiveness.
Lifestyle Issues in Conditions of theEye and Blindness
Vision is crucial for many activities of
daily living. Individuals with little or novision must learn new techniques for car-rying out routine activities of self-care andmobility. They must orient themselves tothe home environment so that they maymove freely from room to room withoutrisk of injury. Family members can con-tribute to their sense of mobility by nev-er moving furniture within a roomwithout informing them and by leavingdoors either completely open or complete-ly closed after informing them of the planso that they do not bump into a partiallyopen door.
At ﬁrst, tasks such as pouring water into
a glass without spilling it, buttering bread,or cutting meat may seem insurmountablefor the individual with visual impairment.However, most people learn to preparetheir meals and dine independently oncethey have been oriented to the location offood, tableware, and cooking utensils.Cooking can be learned through tech-niques such as the systematic placementof cooking equipment and utensils andspecial labeling on cans, frozen foods,oven dials, and other items.
Through training, individuals with visu-
al impairments are gradually able to as-sume personal responsibility for self-care.Rehabilitation teachers provide in-home
training in skills of daily living. Activitiessuch as bathing, combing the hair, shav-ing, applying makeup, and dressing in acoordinated fashion can all be performedindependently through skills training andsystematic organization and labeling ofpersonal items.
Although individuals with a moderate
to mild visual impairment may carry onmuch of their personal business with low-vision aids, those with a severe visualimpairment or blindness may need some-one else’s help to read a bill, a check, aninvoice, or a personal letter. Some individ-uals have difﬁculty adjusting to this lossof privacy. In other instances, documentsor forms must be translated into Braille orread to the individual, perhaps reducingthe efﬁciency of action or response to thedocument.
Outside the home, individuals with
severe visual impairments can learntechniques of mobility in new environ-ments with the use of a cane or guide dog.Through these techniques, even thosewho are severely impaired or blind areable to travel to work or other destinationsof their choice. They can also learn meth-ods of carrying money so as to dis-criminate between bills and betweendifferent coins. Individuals can continueto enjoy a number of leisure activities,including outdoor activities such as swim-ming, hiking, and ﬁshing. With specialadaptive procedures, even bicycling is stillpossible.
Although visual loss does not affect sex-
ual activity directly, the impact of loss ofvision on self-esteem may be substantial.In addition, individuals with severe visu-Psychosocial Issues in Conditions of the Eye and Blindness 139

al impairment are unable to see the facialexpressions and other nonverbal forms ofcommunication that are an integral partof sexual relationships. Information aboutrelationships that is normally developedthrough visual modeling may not be avail-able if they have been without sight sincean early age. Consequently, visually im-
paired teenagers or young adults may need
to be taught appropriate social behaviorsthat others generally learn by observation.
Social Issues for Individuals withVisual Conditions or Blindness
Major obstacles to the effective func-
tioning of individuals with visual impair-ment in social environments are socialstereotyping and the attitudes of sightedpeople toward those individuals. Manysighted people view individuals who havesevere visual impairments or are blind ashelpless and dependent. Others believethe myth that people with severe visualimpairments or blindness develop extraor-dinary powers of hearing and touch tocompensate for the loss of vision, ratherthan recognizing that they learn to makemore effective use of other senses in theireffort to interpret their
environment.
Negative attitudes or stereo typical views
held by friends, employers, and casualacquaintances can have a major impact onindividuals with visual impairment.Unfortunately, many people with visualimpairment tend to conform to socialexpectations, thus limiting their ownpotential. Understanding and acceptingthat others may be uncomfortable withthem because of lack of previous interac-tions with people with visual impairmentsor because of misinformation provide thebasis for formulating proactive strategiesfor solving problems, understanding per-spectives, and making social inferencesthat can result in fuller social integration.The inability of individuals with visual
impairment to see the social behavior ofothers affects their social interactions.
Much social interaction and communica-tion
are mediated by watching the nonver-
bal actions and reactions of others, such
as posture, facial expression, or move ment.
Absence of these visual cues can placeindividuals with visual impairment at adisadvantage in a social setting, unless allconcerned have developed increasedawareness and sensitivity to the individ-ual’s inability to observe nonverbal cues.
Although the attitudes of family mem-
bers are important factors in adjustmentto most disabilities, they appear to havean especially powerful inﬂuence on theadjustment of those with visual impair-ment. How families react depends on theirpatterns of belief, feelings, and resources.When family members believe that thedemands being placed on them exceedtheir available resources, stress and strainmay develop. If, on the other hand, fam-ilies are able to meet the major needs oftheir members so that they can pursuerealistic goals, they will be better able tocope successfully.
Family attitudes during rehabilitation
may determine individuals’ motivation tolearn and accept major changes in
lifestyle. Overprotective or overly anxious
families who encourage dependency mayprevent or impede rehabilitation. On theother hand, families that foster positiveattitudes and demonstrate respect andrecognition of the individual with visualimpairment can contribute greatly torehabilitation.
VOCATIONAL ISSUES FORINDIVIDUALS WITH CONDITIONS OFTHE EYE OR BLINDNESS
Person who are blind or who have low
vision are underrepresented in the com-140 CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS

petitive labor market (Crudden &McBroom, 1999). The degree of the voca-tional impact of a visual disorder dependson the nature of the employment, thetype of visual impairment, and the lifestage at which the visual impairmentoccurs. Many individuals with partialvision are able to continue in their ﬁeldof employment with special adaptive orlow-vision aids. Others must learn new jobskills. When visual loss is progressive,ongoing evaluation and planning fordecreasing visual acuity should be part ofthe rehabilitation plan.
Barriers to employment may include
deﬁcits in skills or education, lack of work
experience, lack of job preparation skills,or lack of motivation or information. Paidreader support may be necessary to gaininformation about job openings or train-ing opportunities. In other instances,lack of direction by family and friends orlack of expectation that individuals withvisual impairments can be competitive inthe workplace may make it more difﬁcultto continue looking for work or preparingfor employment.
Not only on-the-job activity, but also
the ability to get to and from work maybe a barrier to employment. If individu-als are no longer able to drive and no pub-lic transportation is available, suitable al-ternatives for transportation to and fromwork must be devised. Unreliable trans-portation can be a major barrier to obtain-ing and maintaining employment.
For individuals with low vision, the lev-
el of visual acuity, and thus the individ-ual’s ability to resolve visual detail, mustbe considered in any position in whichreading or seeing ﬁne visual detail isrequired. Accommodations may involvemaking the image larger through someform of magniﬁcation or using an opticaldevice to make the object appear larger.Individuals with low vision may needadditional lighting to enhance vision;however, lighting that produces glare canbe detrimental to their visual acuity andcomfort. Those with visual ﬁeld deﬁcitsmay have difﬁculty with peripheral or cen-tral vision. Those with peripheral visionproblems have difﬁculty detecting objectsaround them. In addition, peripheralﬁeld deﬁciency can interfere with mobil-ity and with performing near tasks suchas reading or writing. Central vision lossaffects individuals’ straight-ahead visionand probably also reduces visual acuity.Consequently, reading and tasks requiringvisualization of detail will be affected. Thedegree of functional impairment is de-pendent on the size and location of theloss of vision.
CASE STUDIESCase I
Ms. A. is a 32-year-old female who
recently got married. Her husband is alandscaper. They have no children. Short-ly after their marriage Ms. A. became total-ly blind from proliferative diabeticretinopathy. She has no light perception.Ms. A. has a bachelor of science degree inretail management and has been workingfor the past 10 years as a buyer for a majorretail ﬁrm. Her job involves determiningwhich products to buy for distributionbased on her projection of their ability tosell, the sales activities of other retail ﬁrms,and the general economic conditionsand buyer trends. She uses a computer formany of her activities.
Questions
1. With regard to Ms. A.’s blindness, are
there special adaptive devices or spe-cial accommodations that mightassist her at her current job?Case Studies 141

2. What adaptive devices or accommo-
dations might Ms. A. need in otheraspects of her life because of herblindness?
3. Are there issues in Ms. A.’s personal
life that could affect her rehabilita-tion potential?
4. What additional types of services
would help Ms. A. reach her highestlevel of independence?
Case II
Mr. J. is a 25-year-old man with progres-
sive vision loss from retinitis pigmentosa.He currently has moderate visual loss butrefuses to use adaptive devices or specialaids. He did not complete high school, buthe did obtain a GED. He has a scatteredwork history, working for a time in his lateteens as a worker on a river barge, as a per-sonal attendant for an individual with adisability, and most recently as a freightmover at a local factory. He states he haslost contact with his family and appearsto have no close friends or relatives in thearea.
Questions
1. What additional information about
the personal and work characteristicsof Mr. J. would you need to help himto develop a rehabilitation plan?
2.
What speciﬁc characteristics about his
visual impairment would you consid-er?
3. If Mr. J. would use adaptive devices,
which types would be most helpful tohim given his speciﬁc type of visualimpairment?142
CHAPTER 4 C ONDITIONS OF THE EYE AND BLINDNESS
REFERENCESBienfang, D. C., Kelly, L. D., Nicholson, D. H., &
Nussenblatt, R. B. (1990). Ophthalmology. New
England Journal of Medicine, 325 (14), 956–967.
Buckingham, B. K. (2000). Refractive eye surgery:
Focusing on risks. Trial, 35 (5), 18–27.
Butler, R. N. (1997). Keeping an eye on vision: New
tools to preserve sight and quality of life.Geriatrics, 52 (9), 48–55.
Cox, P. R., & Dykes, M. K. (2001). Effective classroom
adaptations for students with visual impair-ments. Teaching Exceptional Children, 33 (6), 68–74.
Crudden, A., & McBroom, L. W. (1999, June). Barriers
to employment: A survey of employed personswho are visually impaired. Journal of Visual
Impairment and Blindness , 341–350.Lacey, G., & MacNamara, S. (2000). User involve
ment in the design and evaluation of a smartmobility aid. Journal of Rehabilitation Research and
Development, 37 (6), 709–723.
Lighthouse. (1990). Statistics on blindness and vision
impairment: A resource manual . New York:
Lighthouse.
Scarlatis, G. (2000). Optical prostheses: Visions of
the future. Journal of the American Medical
Association, 283 (17), 2297.
Turnbull, A., Turnbull, R., Shank, M., Smith, S., &
Leal, D. (2002). Exceptional lives: Special education
in today’s schools ( 3rd ed.). Upper Saddle River, NJ:
Merrill.
Vance, J. C. (2000). A degree of vision. Lancet, 356
(i9240), 1517–1519.

NORMAL STRUCTURE ANDFUNCTION OF THE EAR
The ear is made up of two systems, each
with a speciﬁc function:
• The auditory system is involved
with the detection of sound waves,and consequently hearing.
• The vestibular system is involved
with body equilibrium, orientation,and balance.
The ear consists of three divisions: the
outer, middle, and inner ear (Figure 5–1).
The Outer Ear
The outer ear includes the pinna (auri-
cle) and the external ear canal (external audi-
tory meatus ). The pinna , the visible por tion
of the ear, is made up of elastic cartilagecovered with skin. The external ear
canal is
a little longer than one inch and ex tends
from the opening of the ear to the ear-drum. It contains special glands that pro-duce cerumen (earwax), which protects
the ear against the entry of foreign mate-rial. The function of the pinna is to col-lect sound waves and conduct them to theeardrum ( tympanic membrane ). The Middle Ear
The tympanic membrane separates the
outer ear from the middle ear ( tympanic
cavity ). The tympanic cavity is an air-ﬁlled
cavity connected to the throat by theeustachian tube , which helps equalize air
pressure on both sides of the tympanicmembrane. Changes in atmospheric pres-sure, such as with change in altitude,necessitate equalization of air pressure in
the middle ear. When the ears “pop” dur –
ing altitude changes, the eustachian tubehas allowed air in the middle ear to equal-ize with the pressure outside the head.When this does not occur, considerable
discomfort can result. The eustachian tube,
which is normally collapsed in adults,
opens with yawning or swallow ing, allow-
ing air pressure on both sides of the tym-panic membrane to equalize. Anotherpathway connects the tympanic cavity tothe mastoidal air cells within the mastoid
process of the temporal bone.
The middle ear lies between the ear-
drum and the inner ear. It contains threesmall movable bones called ossicles that
transfer sound vibrations from the ear-drum to the inner ear. These small bones,the malleus , the incus , and the stapes , areHearing Loss and DeafnessCHAPTER 5
143

connected by small ligaments and areattached to the tympanic membrane bythe handle of the malleus. The footplateof the stapes connects to a thin membranecalled the oval window , which connects to
the inner ear. Also connecting the middleear with the inner ear is an opening calledthe round window .
The Inner Ear
The inner ear ( labyrinth ) is a ﬂuid-ﬁlled
cavity that lies deep within the temporal
bone of the skull. The inner ear is impor-
tant not only for hearing (as part of theauditory system ), but also for maintaining
body balance and equilibrium (as part ofthe vestibular system ). Contained within
the inner ear is the cochlea , which is part
of the auditory system , and the semicircu-
lar canals , which are part of the vestibular
system . The cochlea has a snail-like ap-pearance and contains tiny hair cells with-in a structure called the organ of Corti , the
end organ of hearing. Movement of ﬂuidwithin the inner ear stimulates nerve end-ings in both the auditory and vestibularsystems. Impulses from both systems areconverted into nerve impulses and trans-mitted from the inner ear to the brain bythe eighth cranial nerve , sometimes called
the acoustic or auditory nerve , which con-
tains two branches: the cochlear nerve
branch , which conducts sensory informa-
tion about sound, and the vestibular nerve
branch , which conducts impulses regard-
ing body balance and movement.
Sound waves enter the external ear and
move through the external ear canal, strik-ing the eardrum and causing it to vibrate.The chained movement of the malleus,incus, and stapes conducts sound wavesfrom the eardrum to a thin membranecalled the oval window , which connects to144
CHAPTER 5 H EARING LOSS AND DEAFNESS
Semicircular Canals
Incus
MalleusVestibular
Nerve8th Cranial
Nerve
Oval
Window
StapesTympanic
MembraneExternal
Ear CanalAuricle
Cochlear Nerve
Cochlea
Figure 5–1 The Outer, Middle, and Inner Ear.

Hearing Loss and Deafness 145
the inner ear. The vibration of theeardrum ﬁrst moves the malleus, whichtransmits the vibration to the incus; inturn, the incus transmits the vibration tothe stapes. The stapes vibration in the ovalwindow stimulates the ﬂuid in the innerear, which stimulates the tiny hair cells inthe organ of Corti. The movement of thehair cells stimulates the nerve endings lo-cated around their bases to transmit im-pulses to the cochlear nerve , which carries
the impulses to the auditory center of the
brain, where they are interpreted as sound.
The vestibular structures in the ear, con-
cerned with maintaining equilibrium,include the semicircular canals and a small
rounded chamber at their base called thevestibule . The semicircular
canals contain
the nerve endings through which balance
is controlled. Like the organ of Corti, theycontain numerous hair cells that projectinto the ﬂuid of the inner ear. The move-ment of the head sets the ﬂuid in motionand moves the hair cells, stimulating thenerve endings, which then transmit theimpulses to the vestibular nerve . These
impulses are carried to the portion of thebrain involved with maintaining equilib-rium and coordinating movement.
HEARING LOSS AND DEAFNESSFrequency and Intensity of Sound
The sense of hearing is the neural per-
ception of sound energy and is based on
transmission of external sound to thestructure of the outer, middle, and innerear, the nerves to the brain, and portionsof the brain involved in processing acous-tic information. Sound is characterized bypitch (tone) and by intensity (loudness ).
Traveling vibrations known as soundwaves produces sound. Pitch or tone is de-termined by the frequency of vibrations of
sound waves. The faster the vibrations orfrequency, the higher the pitch. Frequen-cies are measured in Hertz or cycles per sec-
ond. Intensity, or loudness, depends onthe amplitude of sound waves. The greater
the amplitude, the louder the sound.Loudness is measured in decibels .
Individuals with hearing loss can have
diminished hearing with regard to fre-quency, intensity, or both. Hearing lossrelated to frequency results in a distortion
of sound so that individuals may be unable
to differentiate between many of
the
sounds of speech. For example, words with
similar sounds such as catand ratmay be
easily confused. Increasing the volumedoes not improve the quality of sound ormake the words more clear if this type ofhearing loss exists. Hearing loss related tointensity or loudness involves more difﬁ-culty in hearing because of reduction insound volume. With this type of hearingloss, ampliﬁcation of sound may improvehearing. In instances where individualshave hearing loss related to both frequen-cy and intensity, increasing volume alonewill not improve overall hearing.
Deﬁnition and Classiﬁcation ofHearing Loss
Disruption of any part of the hearing
system can result in hearing loss. Anydegree or type of hearing loss is classiﬁedas a hearing impairment . The greater the
degree of hearing impairment, the moredifﬁculty individuals have hearing in anumber of situations.
Hearing loss may be partial or total,
temporary or permanent, mild to pro-found. Hearing loss can affect both thevolume and clarity of sound. Some indi-viduals have hearing loss that results inreduced hearing sensitivity . Individuals
with sensitivity loss may be unable to hearsoft speech or may have difﬁculty hearingspeech when there is background noise.

Other individuals have difﬁculty withspeech discrimination . Individuals with dis-
crimination deﬁcit may, even though vol-ume is adequate, be unable to clearly hearcertain phonetic elements of speech (forexample, consonants such as fand s)
because they occur at frequencies wherehearing loss is present. Individuals canhave both sensitivity loss and discrimina-tion deﬁcit at different degrees.
Hearing loss is classiﬁed through a num-
ber of different criteria:
• Cause and location of hearing loss• Duration of loss or age of onset• Degree of hearing loss
Cause and Location
Cause and location of the loss are clas-
siﬁed as:
• conductive • sensorineural• mixed
Conductive hearing loss occurs when
there is damage, obstruction, or malforma-tion in the external or middle ear that pre-
vents sound waves from reaching thecochlea in the inner ear. In many cases,correction of the underlying problem canrestore diminished hearing. Conductiveloss may result from buildup of earwax inthe ear canal, a foreign object in the ear,infection of the middle ear ( otitis media ),
or hardening of the small bones in the ear(otosclerosis ) that are important for con-
ducting sound. Conductive hearing loss isa mechanical problem that alters the loud-ness of sound but does not reduce its clar-ity because the inner ear, where sound is“processed,” is not compromised. Some-times conductive hearing impairments
cannot be corrected and hearing aids may
be used to amplify sound and restore nor-
mal loudness. Conductive hearing losses aregenerally of mild to moderate degree.Individuals with mild to moderate con-ductive hearing loss usually have goodsuccess with hearing aids if hearing aidsare necessary.
Sensorineural hearing loss results from
damage or malformation of the inner ear
and/or the auditory nerve and can lead to
total deafness. Sensorineural loss involves
damage to the hair cells of the inner ear,which in turn interferes with the recep-tion of nerve impulses. In some cases, thehair cell function of the cochlea is unaf-fected; rather, the cause of hearing loss isrelated to the nerve transmission path
way
to the brain. With sensorineural loss, hear-
ing is affected in terms of not only loud-ness but also pitch or clarity. Individualsmay perceive no sound, or sound whenperceived may be distorted. Sensorineur-al hearing loss can be caused by a num-ber of factors, including heredity, acuteinfection such as meningitis or mumps,ototoxic drugs (drugs that damage nerves of
the central nervous system that are asso-ciated with hearing), tumors, or exposureto loud noise. The problem may also bedue to a disorder of the auditory centers of
the brain , such as from head injury or
stroke. When this type of hearing lossoccurs, the individual is said to have cen-
tral deafness . In this type of deafness,
there is a disruption of the ability of thesound stimulus to reach the hearing cen-ters of the brain, or the hearing center inthe brain incorrectly receives and process-es signals. Sensorineural hearing impair-ments are almost exclusively irreversible.Because sensorineural hearing loss in-volves damage to the hair cells of thecochlea and/or nerve cell damage, and be-cause the nerve function cannot berestored, associated hearing loss is usual-ly permanent. Although the cause of sen-sorineural loss should be evaluatedmedically, individuals with sensorineural146
CHAPTER 5 H EARING LOSS AND DEAFNESS

hearing loss should also consult with alicensed audiologist for evaluation andpotential ﬁtting of hearing aids as well asfor training in communication strategies.
When individuals have mixed hearing
loss, there is a combination of conductive
hearing loss and sensorineural hearing loss .
The conductive component may lenditself to medical treatment while the sen-sorineural component remains. Individ-uals in some instances may beneﬁt fromampliﬁcation. The extent of the hearingloss experienced with mixed hearing im-pairments and the success of interventiondepend on the degree and type of sen-sorineural damage.
Duration of Loss or Age of Onset
Another classiﬁcation of hearing loss is
based on when the hearing loss occurred:
•Prelingual hearing loss occurs before
the individual acquires language, usu-ally before the age of 3.
•Postlingual hearing loss occurs after
verbal language is obtained.
•Prevocational hearing loss occurs
after an individual acquires languagebut before entering the work force,usually before the age of 19.
•Postvocational hearing loss refers to
hearing loss that has occurred af
ter
the individual has started to work.
Degree of Hearing Loss
Hearing loss can also be classified
according to the degree of hearing loss:
•Hard of hearing refers to individuals
with mild to moderate hearing loss.
Individuals who are hard of hearingusually have difﬁculty understandingconversational speech through the earwith or without a hearing aid.
•Deaf refers to individuals with severe
to profound hearing loss. Deafness isthe most severe type of hearing loss.Individuals with severe or profound
hearing loss have an extreme inability
to understand conversational speechthrough the ear that precludes theirability to use hearing for communica-tion.
The degree of hearing loss is common-
ly deﬁned on the basis of the audiogram,described later in the chapter. The audio-gram is a method of recording the softestsounds individuals can hear. Normal hear-ing sensitivity ranges from –10 to +20decibels. A general guide for describing thedegrees of hearing loss associated withdecibel losses is found in Table 5–1.
Table 5–1 Functional Implications of Degrees
of Decibel Loss
26–40 Mild hearing loss. In ideal listening
conditions, hearing is minimallyaffected; there may be difﬁcultyhearing faint, distant speech even inideal conditions; background noisemay interfere with hearing.
41–55 Moderate hearing loss. Hears
conversational speech but only atclose distances; understandingspeech is more difﬁcult with back-ground noises.
56–70 Moderately severe hearing loss.
Hears loud conversational speechthat is close by. Has difﬁcultyhearing in group situations.
71–90 Severe hearing loss. Conversational
speech severely affected. Perceptionof sound is usually distorted.
Greater Profound. May hear (or feel fromthan 90 vibrations) only very loud sounds.
Hearing is not the primary communi-cation channel.
Source : Moore, D. F., 2001.Hearing Loss and Deafness 147

Causes of Hearing Loss
Hearing loss may be congenital or
acquired . Congenital hearing loss is present
at birth. The major causes of congenitalhearing loss are genetic transmission (inheri-
ted hearing loss ), caused by the mother’s
prenatal ingestion of drugs that are harm-ful to the developing auditory system ofthe fetus or prenatal exposure to in-fections, such as measles ( rubella ).
Inherited hearing loss may be part of spe-
ciﬁc genetically linked conditions thatinvolve a variety of other abnormalities,or it may occur by itself. The degree, pro-gression, and age of onset of inheritedhearing loss vary widely, depending onthe speciﬁc condition or syndrome. Insome instances, genetics may not causedeafness per se but rather predispose in-dividuals to hearing loss induced bynoise, drugs, or infection (Steel, 2000). Inmany instances hearing loss is multifacto-rial, caused by both genetic and environ-mental factors (Williams, 2000).
Acquired hearing loss occurs after birth or
later in life. There are a number of causesof acquired hearing loss. Premature birthcan be a cause of very early hearing loss,and recurrent ear infections (e.g., otitismedia) with complications often causeconductive hearing loss in young chil-dren. Noise-induced hearing loss is a com-
mon but preventable type of acquiredhearing loss. Avoiding loud noises or wear-ing ear protectors when exposed to loudnoise could drastically reduce the inci-dence of noise-induced hearing loss.
Acquired hearing loss can also result from
injury or disease, such as from traumaticbrain injury or from multiple sclerosisaffecting the auditory pathway. Presby-
cusis (hearing loss associated with aging)
is an acquired hearing loss. The extent to
which degeneration of portions of the au –
ditory system is due to the aging processor to cumulative noise trauma throughoutlife may be difﬁcult to determine.
The type and degree of hearing loss ex-
perienced by individuals, regardless of thecause, are varied. Hearing loss usuallyinvolves more than a reduction in theloudness of sound. Some hearing lossesalso result in a distortion of sound so thatwords may be heard but are difﬁcult tounderstand or are garbled. In this case,increasing the loudness is unlikely toenhance individuals’ ability to understandwhat is being said.
Some individuals with hearing loss
may develop recruitment , a symptom
characterized by an abnormally rapid in-crease in the perception of loudness withsmall changes in signal energy. Individ
uals
with recruitment have a narrow range
between a level of sound loud enough tobe understood and a level of sound thatcauses discomfort or pain. Unexpectedsounds may startle individuals with re-cruitment and distract them from inter-pretating the sound’s meaning. There-fore, increasing the loudness of sounddoes not correct the hearing problem andcan actually increase the discomfort.
Conditions of the Ear Contributing toHearing Loss
Conditions of the Outer Ear
Conditions of the outer ear can con-
tribute to hearing loss when there is anobstruction that disrupts the mechanicaltransmission of auditory stimuli, decreas-ing hearing acuity. Although conditions ofthe outer ear may not have a major impacton hearing or may be correctable, theymay also be disﬁguring, causing cos
metic
concerns. Deformities or abnormal ities of
the outer ear can result from congenitalconditions or from trauma. Other condi-tions of the outer ear that may impede148
CHAPTER 5 H EARING LOSS AND DEAFNESS

hearing are buildup of ear wax ( cerumen ),
foreign bodies in the ears, or growths (e.g.,
polyps) that cause obstruction.
For the most part, partial occlusion of
the external ear canal has no inﬂuence onthe efﬁciency of sound transmission andcauses no signiﬁcant hearing loss. Com-plete occlusion, however, generally results
in a low to moderate conductive loss. Con-
ditions of the outer ear that cause tempo-rary conductive hearing impairments canusually be corrected or alleviated by sur-gical or mechanical intervention.
Conditions of the Middle Ear
Conditions of the middle ear may cause
temporary or permanent hearing loss.
Perforated Tympanic Membrane
A thickened or perforated tympanic mem –
brane (ruptured eardrum) may or may not
impair hearing. Rupture of the eardrummay result from an injury (e.g., a blow tothe ear or head, or an explosion) or infec-tion or inﬂammation.
Otitis Media
Otitis media (inﬂammation and ﬂuid
buildup in the middle ear) can cause con-ductive hearing losses because of collec-tion of ﬂuid in the middle ear or becauseof damage to the tympanic membrane (ear-
drum) as a result of infection or rupture.Usually, with appropriate treatment, per-manent hearing loss will not result. If nottreated promptly, however, otitis mediacan lead to mastoiditis (Hendley, 2002).
Mastoiditis
Mastoiditis is an infection of the mas-
toid cells within the mastoid process locat-
ed in the temporal bone of the skull.Because of the proximity of the mastoidcells to other important structures in thehead, mastoiditis may lead to a number ofcomplications, including paralysis of thefacial muscles and infection or abscess ofthe brain. Mastoiditis is not as prevalentas it once was because of the earlier detec-tion of otitis media and treatment withantibiotics; however, chronic mastoiditisand associated complications can result ifprevious ear infections are left untreated.
Otosclerosis
Otosclerosis is a hardening of the ossi-
cles ( incus , stapes , and malleus of the mid-
dle ear), which transmit sound impulsesto the inner ear. Early symptoms may in-clude trouble hearing on the telephonebut not in crowds. The condition appearsto be partly hereditary. It causes conduc-tive hearing loss because hardening of theossicles reduces the efﬁciency of the trans-
fer of sound impulses to the inner ear.Otosclerosis produces progressive hearingloss accompanied by tinnitus (ringing or
noise in the ear). Some individuals mayalso have vestibular symptoms such as ver-
tigo (dizziness) or impaired equilibrium.
Individuals with otosclerosis often hear
ampliﬁed speech well and without distor-tions; consequently, they are usually goodcandidates for hearing aids. Hearing canalso often be restored or improved withsurgical intervention; however, surgerydoes not fully remedy the loss. When de-termining if surgery is appropriate, indi-viduals’ lifestyle and occupation are con-sidered. Since surgery may affect vestibu-lar function, individuals who require ﬁnebalance for employment may ﬁnd ampli-ﬁcation through hearing aids a betterchoice than surgery. If individuals’ hob-bies or occupations expose them to largeand rapid changes in barometric pressure,or if heavy lifting is required, hearing aidsHearing Loss and Deafness 149

may also be a better choice because of thechance of postoperative complications orvestibular disturbances.
Conditions of the Inner Ear
Many conditions of the inner ear cause
permanent hearing loss.
Labyrinthitis
Labyrinthitis (inflammation of the
labyrinth of the inner ear) may be acutewithout resulting in permanent hearingloss. Labyrinthitis may occur as a compli-cation of otitis media, inﬂuenza, or upperrespiratory infections. Because the innerear is involved, symptoms of vertigo (diz-
ziness), nausea, and vomiting frequentlyaccompany the condition.
Tinnitus
Tinnitus (ringing or noise in the ears)
may or may not be accompanied by hear-
ing loss. It can result from overexposure to
loud noise or can be a symptom of a moreserious condition, such as tumor, highblood pressure, or head injury. It can alsoresult from side effects or toxic effects ofsome medications. In some instances, tin-nitus may be a separate entity, not asso-ciated with a speciﬁc disorder and with noidentiﬁable cause. Treatment of tinnitus is
dependent on the cause. If the cause can be
identiﬁed and treated, the ringing cansometimes be eliminated. In other in-stances, even though the cause is identi-ﬁed, damage may be permanent andtinnitus cannot be cured.
Most people with tinnitus adjust to its
presence and experience no severe resid-ual effects; however, some individualshave difﬁculty adjusting and experiencedisabling effects, including severe emo-tional distress. Some individuals complainof problems sleeping because of the
con-
stant noise and may need backgroundnoise, as from a radio, to mask the sound.
A number of therapies, including group
cognitive therapy, have been used to help
people cope with tinnitus. Adjustment totinnitus does not appear to be related tothe severity of the disorder, but rather tothe coping styles of those affected.
Meniere’s Disease
Meniere’s disease is a disorder of the
inner ear that encompasses the triad ofrecurrent severe vertigo , sensorineural hear-
ing loss , and tinnitus (noise or ringing in
the ears). The cause of Meniere’s diseaseis unknown. One or both ears may beaffected. Meniere’s attacks are episodic,and the disease is characterized by remis-
sions and relapses. When attacks do occur,
they are dramatic, often debilitating indi-viduals during the episode.
Vertigo usually appears suddenly and is
often accompanied by nausea and vomit-
ing. Tinnitus may be intermittent or may be
constant between attacks, becoming worseduring an attack. The hearing loss associa-ted with Meniere’s disease is variable. Typi-cally, hearing loss becomes progressivelygreater with repeated active attacks. Lowertones may be affected ﬁrst, but all tonesare affected as the disease progresses.
Trauma or Disease
Hearing loss may also result from dam-
age to the inner ear or to the acoustic nerve .
Among causes of sensorineural deafness are
traumatic head injury or stroke; hyperten-sion and arteriosclerosis, which produce
vascular changes in the central nervous sys –
tem; exposure to high levels of noise, which
can damage the hair cells in the inner ear;the ingestion of ototoxic agents (drugs or
other chemicals that destroy the hair cells150
CHAPTER 5 H EARING LOSS AND DEAFNESS

of the inner ear or damage the eighth cra-nial nerve); and infections, such as menin-gitis. Growths or tumors inside the headmay cause hearing loss by mechanicallyimpinging on the acoustic nerve or byinvolving it directly. Other neurologicalconditions such as multiple sclerosis mayproduce changes in the auditory pathwaythat contribute to hearing loss.
Presbycusis
Presbycusis is caused by degenerative
changes in the inner ear, neural pathways,or both; however, the reason that presby-cusis occurs is unknown. It has become acatchall term to include many types of
auditory deterioration, but it is commonly
thought to accompany structural changesin the ear due to aging. Most often pres-bycusis occurs in both ears equally. Onsetis slow, and hearing loss can vary in degreefrom mild to severe. Ability to hear high-er tonal frequencies is usually affectedﬁrst, but the ability to hear lower frequen-cies is gradually affected as well. Hearingloss experienced as a result of presbycusismost often is accompanied by word dis-crimination difﬁculties, especially if thehearing loss has greatly affected individ-uals’ per
ception of the higher-pitched
consonants.
CONDITIONS OF THEVESTIBULAR SYSTEM
The vestibular system in the ear con-
tributes to the sense of balance and equi-
librium. Although conditions of the vesti-
bular system can be associated with con-ditions that affect hearing, such asMeniere’s disease , symptoms related to the
vestibular system can also exist with oth-er conditions such as stroke or can resultfrom the side effects of certain medica-tions. Regardless of the cause, conditionsof the vestibular system can cause symp-toms that interfere with individuals’ func-tional capacity.
One of the classic symptoms associated
with vestibular dysfunction is vertigo.Vertigo is an illusory sense of motion, usu-
ally described as a spinning sensation, andoften thought of in terms of dizziness .
Although vertigo can be associated withmany conditions, it is always related tothe body’s vestibular system.
Episodes of vertigo can last from a few
seconds to days. Vestibular neuronitis
(inﬂammation of the vestibular nerve), adisorder of the inner ear, can cause verti-go that lasts for days or weeks and is ex-tremely disabling during that time. Duringthe episode, individuals become nau-seous with vomiting and feel a violentspinning of their surroundings. The causeof vestibular neuronitis is unknown.
Vertigo is also associated with Meniere’s
disease, as discussed above. It can also beassociated with head injury or other neu-rological conditions, such as brain tumoror multiple sclerosis.
DIAGNOSTIC PROCEDURESIdentiﬁcation of Hearing Loss
Before hearing loss can be evaluated or
treated, it must be identiﬁed. Individualswith hearing impairments may not beaware of the degree of loss, or they maydeny that hearing loss exists. An impor-tant tool in the diagnosis of hearing lossmay be simple observation of behaviorsthat may indicate such an impairment.
Indications of possible hearing loss in
infants and small children include unre-sponsiveness to sound, delayed develop-ment of speech, and behavior problems(e.g., tantrums, inattention, and hyperac-tivity). School-age children with undiag-nosed hearing loss may have speechDiagnostic Procedures 151

impairments, may demonstrate atten tion
disorders, or may demonstrate below-
average ability in school.
Adults with undiagnosed hearing loss
may be irritable, hostile, or hypersensitive.They may deny their inability to under-stand or respond appropriately by blam-ing others for not enunciating distinctly.They often avoid situations in which hear-ing is more difﬁcult, such as large crowdsor large groups. Individuals
with undiag-
nosed hearing loss may speak too loudly
and may require increased volume to hearthe television and radio.
Heightened sensitivity and patience are
often necessary when encouraging indi-viduals with suspected hearing loss toobtain evaluation and treatment. Initialresistance to these recommendations isnot unusual.
Physical Examination
Physicians may be the ﬁrst to recognize
or be consulted about a potential hearingloss. Although often omitted, screeningfor hearing loss should be part of everyphysical exam. Various check sheets andquestionnaires may be used initially toelicit the signs and symptoms associatedwith hearing loss. During physical exam-ination physicians may also examine theear canal for obstruction and visualize thetympanic membrane with an instrumentcalled an otoscope . Rudimentary auditory
screening may also be performed in thephysician’s ofﬁce.
Physicians sometimes use tuning forks in
routine physical examinations in theirofﬁces as an initial screening method forhearing impairments. This method canhelp differentiate between conductive orsensorineural hearing loss; however, itdoes not quantify the degree of impair-ment, if one exists. Because of the grossnature of this screening method, it hasbeen widely replaced by other methods.
Tuning forks can be used as a screening
method to detect problems in both airconduction and bone conduction ofsound. The ability to hear by air conduc-tion is tested by placing a vibrating tun-ing fork in the air near the ear but out ofthe individual’s sight. The inability to hearthe sound is an indication of hearing lossthat requires further evaluation. Hearingby bone conduction is evaluated by plac-ing a vibrating tuning fork in differentpositions on the individual’s skull, whichcauses vibration throughout the skull,including the inner ear. Both conductiveand sensorineural impairments can be de-tected in this manner. Abnormal test re-
sults warrant further testing and evaluation.
When problems with hearing are iden-
tiﬁed, referral for additional testing may be
made. For further testing and evaluation,individuals may be referred to an audiol-
ogist , a person with a master’s or doctoral
degree in audiology who specializes in the
evaluation and rehabilitation of individ-uals with hearing disorders. If testing bythe audiologist reveals that
medical inter-
vention is necessary, referral is made to an
otolaryngologist (physician who special-
izes in diagnosis and treatment of condi-tions of the ear and related structures).
Audiometric Testing
Audiometric testing measures the degree
of hearing loss with an electronic devicecalled an audiometer . An audiologist usual-
ly performs the test. Tests routinely usedby audiologists attempt to deﬁne threemajor aspects of hearing:
• The degree of hearing• The type of hearing loss• The ability to understand speech
under various conditions152
CHAPTER 5 H EARING LOSS AND DEAFNESS

Diagnostic Procedures 153
PURE TONE AUDIOGRAM
Frequency in Hertz (Hz)
Right EarHEARING-THRESHOLD LEVEL IN DECIBELS (dB)
American National Standard10125 250 500 1000 2000 4000 8000
NormalHearingSensitivityL R Weber L R L R L R L R
0
102030405060708090
100110
Left Ear
Figure 5–2 Pure-Tone Audiogram.
A complete audiometric evaluation usu-
ally includes pure-tone air audiometry , bone
conduction audiometry , speech audiometry ,
and acoustic immittance measurement . From
audiometric results the type and degree ofhearing loss can be determined as well asthe degree of speech understanding.
Pure-Tone Air Audiometry
The accurate measurement of sound is
an important component of a hearing test.Changes in sound intensity are measured
in decibels and heard as changes in loudness.
Changes in sound frequency are measuredin Hertz and are heard as changes in pitch.
Individuals’ ability to detect sound andpitch in each ear is plotted on the audio-
gram (see Figure 5–2). A pure-tone audio-
gram is a
graph on which an individual’sresponses to calibrated tones are plotted as
thresholds . Numbers across the top of the
audiogram represent pure-tone frequen-cies ranging form 125 to 8,000 Hertz.Along the side of the audiogram are num-bers ranging from –10 to 110. These num-bers represent measurement of decibels.
The audiogram illustrates the degree of
hearing loss. The audometric exam takes
place in a sound chamber (booth) toeliminate distracting sounds. An audiome-
teremits sounds ( pure tones ) or words
through earphones worn by the individ-ual being tested ( air conduction audiometry ).
As the tones are transmitted through theearphones, the individual indicates whensound is ﬁrst heard. Results of the test arethen plotted on the audiogram. The hear-ing level scale is constructed so thataverage normal hearing equals 0 decibel;

normal hearing sensitivity ranges from–10 to +25 decibels. The higher the num-ber on the decibel scale, the greater thedegree of hearing loss. The audiogramtests speech frequencies that range from250 to 8,000 Hertz. The inability to dis-criminate frequencies within this rangemay interfere with everyday communi-cation.
Bone Conduction Audiometry
When audiometric testing reveals a
hearing loss, the audiologist conducts fur-ther testing to determine whether hearing
loss is sensorineural, conductive, or mixed.
Tests used for this purpose include bone
conduction tests . The procedure for bone
conduction audiometry consists of placing
a vibrator on the individual’s mastoidprocess or on the forehead. Calibratedtones from the vibrator go directly intothe inner ear, bypassing the external andmiddle ear systems. The individual’sresponses to the thresholds are plotted onthe audiogram and contrasted with the airconduction test results.
To determine the type of hearing loss,
thresholds for air and bone conduction arecompared. Depending on the differencesbetween the two thresholds, hearing lossis classiﬁed as sensorineural, conductive , or
mixed .
Speech Audiometry
Whereas pure-tone audiometry is used to
determine individuals’ ability to hearspeciﬁc tones, speech audiometry may indi-
cate individuals’ ability to understandspeech in everyday situations. Two meas-ures are speech reception threshold and
speech discrimination threshold . In both tests
individuals wear headphones and listen towords being transmitted through theheadphones without any visual cues. Tests of speech reception threshold help
identify the lowest intensity, or softestsound level ( decibels ), at which an
individ-
ual ﬁrst understands speech. Words from a
standardized list with two syllables, suchas baseball , ice cream , or cowboy , are pre-
sented to the individual through the ear-phones. Individuals demonstrate acuity byrepeating the words they have heard. Thespeech reception threshold should corre-
spond closely to average pure-tone air con-
duction thresholds and provides a checkfor the accuracy of the pure-tone measure-ments. The higher the decibel level re-quired for either threshold, the greater thehearing loss. A speech reception thresholdor average pure-tone threshold of 25 deci-bels (dBHL) (HL = hearing level), for in-stance, is considered borderline normal
hearing for adults. Limits for children are
reduced to 15 to 20 decibels (dBHL) . A
threshold of 26 to 40 decibels is considered
a mild hearing loss.
In addition to measuring how loud
speech has to be to be heard, testing alsodetermines how well individuals under-stand speech once it is loud enough tohear. Speech discrimination tests (sometimes
called word recognition tests ) help to
pro-
vide this information. During the test,
words from standardized lists of phon-etically balanced one-syllable words arepresented to the individual through ear-phones without visual cues. Words arepresented at a suprathreshold level, andthe individual must identify and repeatwords back to the examiner. The test isscored as the percentage of correctlyrepeated words. The lower the percentage,the greater the problem in understanding.Individuals with speech discriminationhearing loss may be able to recognizespeech but be unable to understand it. Thespeech discrimination score
provides a
measure of the ability to under stand words
at a comfortable volume. It assesses the154 CHAPTER 5 H EARING LOSS AND DEAFNESS

ability to judge acoustic information andto distinguish between similar speechsounds, such as the letters pand bor the
letters tand d.
Acoustic Immittance or Impedance
Audiometry
Acoustic immittance measurement in-
cludes a battery of tests that evaluate mid-dle ear status. Tympanometry is a test of
acoustic immittance in which the mobility
or ﬂexibility of the tympanic membraneis assessed by measuring how much soundenergy is admitted into the ear as air pres-sure is varied in the external auditorycanal. The status of the eardrum is assessedby altering the air pressure in the ear canaland measuring the response of the ear-drum to sound transmissions under thesevarying conditions and different stimuli.As sound energy strikes the eardrum, someis transmitted to the middle and inner ear,but some is reﬂected back into the earcanal. If the tympanic membrane is stiff,much of the sound energy is reﬂected backinto the external ear canal. The less im-pedance, the more sound energy is admit-ted to the middle and inner ear. Anincreased level of resistance is diagnosticof middle ear pathology. The results areplotted on a graph called a tympanogram .
The ear’s response is plotted on the verti-cal dimension of the graph, and air pres-sures are plotted on the horizontaldimension. Acoustic immittance testingmay also be used to measure the acoustic
reflex , the movement of the muscles
attached to the malleus and stapes as a
response to intense sound. It shouldoccur in both ears in response to a loudsound, even if only one ear is stimulated.Acoustic reﬂex testing may be helpful indiagnosing condi
tions or problems that
involve the cochlea or auditory nervous
system.Acoustic immittance testing requires no
voluntary responses from the individual.Consequently, tympanometry is frequent-ly used to detect or rule out conductivehearing loss in children or in adults whoare unable to cooperate fully during pure-tone testing.
Electrocochleography
Electrocochleography is a procedure in
which stimulus-related electrical activitygenerated in the cochlea and auditorynerve is recorded. For the test, the individ-ual reclines with electrodes placed in theexternal auditory canal. Sound stim
ulus is
then delivered through earphones. The
test is useful in evaluating inner ear ﬂuiddisorders such as Meniere’s syndrome .
Auditory Brain Stem Response
(ABR) Testing
The ABR records electrical activity gen-
erated as sound travels from the auditorynerve through the auditory brain stempathway. The individual reclines withelectrodes placed on the mastoid or on theear lobe. A stimulus is then presentedthrough earphones, and electroencephalo-gram activity is evaluated and the audito-ry brain stem response assessed. The ABRis useful in ruling out auditory diseasessuch as diseases of the cochlea; degener-ative or demyelinating diseases of theauditory system, such as multiple sclero-sis; or tumors of the auditory system.
Otoacoustic Emissions Testing
Otoacoustic emissions are measured
reﬂections in the outer ear of mechanicalactivity in the cochlea. Otoacoustic emis-sions testing allows one to measure hear-
ing in infants, young children, and difﬁcult-
to-test persons such as those with demen-Diagnostic Procedures 155

tia or mental retardation. The technology
has enhanced the ability to detect hearingimpairment early in life.
Evaluation of the Vestibular System(Disorders of Balance)
Individuals who experience vertigo
(dizziness) or who have problems withbalance are frequently tested for inner earand sensorineural disorders related tovestibular function. These tests are per-formed either by an audiologist or physi-cian. In one test of vestibular nervefunction, the caloric test , either cold or hot
water is introduced into the external audi-tory canal. The water stimulates the ﬂu-ids within the inner ear, thus stimulatingthe vestibular nerve. The introduction ofthe water into the ear creates a reﬂexresponse of the eye called nystagmus
(involuntary horizontal eye movement).By monitoring the direction of eye move-ments, the audiologist or physician candetermine the origin of the dizziness andwhether there may be nerve damage. Eyemovement may be monitored visually orwith electronystagmography , a procedure in
which electrodes are placed near the eyeto record eye muscle activity.
TREATMENT OF HEARING LOSSAND DEAFNESS
Both medical and nonmedical interven-
tions may be used in the treatment ofhearing loss. Medical interventions may in-
volve surgery or medications, and nonmed-
ical interventions may include use of hearing
aids or other assistive listening devices andspecial training programs. Treatment inmost cases involves a variety of profession-als. An otolaryngologist (a physician who
specializes in disorders of the ear and relat-ed structures) provides medical evaluationand treatment of hearing loss. Audio-
logists , in addition to conducting evalu-
ations of hearing function, manage thenonmedical treatment of hearing loss,including selecting and
ﬁtting ampliﬁ-
cation devices. The audiolo gist reviews
hearing test results and consults with indi-viduals about their listening needs beforerecommending which style or type ofhearing aid would be most beneﬁcial.Children with hearing loss may also havespeech production difﬁculties because oflack of auditory feedback. Speech and lan-
guage pathologists often work with individ-
uals to help them with particular aspectsof speech, language, or both in order toincrease intelligibility.
Auditory training is often helpful for in-
dividuals with special problems in commun-
ication. Such training may be included inhearing aid orientation and/or special pro-
grams on listening for the sounds of speech
and other environmental sounds.
Surgical Procedures
Surgical procedures may be performed
to eliminate pathological conditions andto restore or improve hearing.
Myringotomy
When the middle ear is infected, as in
otitis media , or when there is a ﬂuid build-
up in the middle ear, surgical interventionmay be necessary to drain pus or ﬂuid,thus relieving pressure and preventingrupture of the eardrum (t ympanic mem-
brane ). A myringotomy is a procedure in
which an incision is made into theeardrum for this purpose. Because the pro-cedure is performed under controlledconditions, it seldom leaves enough scartissue to have a negative effect on hearing.If ﬂuid has accumulated in the middle ear,156
CHAPTER 5 H EARING LOSS AND DEAFNESS

the physician may perform a needle aspi-ration to remove it. Needle aspiration maynot remove ﬂuid that has invaded themastoid air cell system , however, and addi-
tional intervention may be necessary if the
mastoid system is to be rendered dry. Acommon procedure performed duringmyringotomy is placing ventilation- or pres-
sure-equalizing tubes in the tympanic mem-
brane. The pressure-equalizing tubes act asan artiﬁcial eustachian tube , equalizing
middle ear
pressure. Abnormal eustachian
tube func tion is the most common cause
of middle ear problems. The ventilationtubes usually extrude on their own with-in 3 to 18 months with few complications.When the ventilation tube is in place andoperating properly, conductive hearing loss
due to middle ear disease is usually com-pletely eliminated.
Mastoidectomy
Since the advent of antibiotics for treat-
ment of mastoiditis , mastoidectomy is per-
formed less frequently. Mastoidectomy is
a surgical procedure for removal of infect-ed mastoid air cells , which are located in
the mastoid process. Because the mastoidis a portion of the acoustic system of themiddle ear, there may be permanenthearing loss after surgery, depending onthe nature of the surgery. For example,individuals who have had a radical mas-
toidectomy , in which other structures in
addition to the mastoid cells are removed,may have a greater degree of hearing lossor permanent hearing loss. Individualshaving a simple mastoidectomy , in
which only mastoid cells are removed,may have unaffected hearing.
Tympanoplasty
Surgical procedures involving the mid-
dle ear are referred to generally as tym-panoplasty (repair of the tympanic mem-
brane ). Myringoplasty is a speciﬁc type of
tympanoplasty in which the dam aged
eardrum is repaired. Other types of tym-panoplasty may be performed for the sur-gical repair or reconstruction of the ossicles
of the middle ear. Repairing or recon-structing the conductive mechanisms ofthe middle ear may improve or restore theconductive component of individuals’hearing.
Stapedectomy
The most common surgical treatment
for otosclerosis is stapedectomy , a surgical
replacement of an immobile or ﬁxedstapes with a prosthesis. The surgeryreestablishes a more normal sound path-way between the middle and inner ear; itusually improves hearing but does nottotally restore it.
Devices and Aids for Hearing Loss
Cochlear Implant
Currently, cochlear implants are the
standard treatment for individuals withsevere to profound hearing loss who areunable to have effective oral communica-tion even with the beneﬁt of a hearing aid(Gates & Miyamoto, 2003). A cochlear
implant is an auditory prosthesis . The im-
plant is an inner ear device that helps indi-viduals detect medium to loud environ-mental sounds. Cochlear implants trans-late sounds into digital impulses that arefed directly to the auditory nerve, bypass-ing hair cells of the inner ear. This elec-tronic device consists of a microphone that
picks up sound; a battery-powered processor ,
either at ear level or typically worn on abelt, that converts sound into digitalimpulses; and a receiver implanted into theTreatment of Hearing Loss and Deafness 157

temporal bone that transmits digital
impulses down the electrode that has beensurgically placed in the cochlea and thatstimulates the auditory nerve directly.
The microphone is mounted on the ear-
level processor and picks up sounds in theenvironment. The processor convertssound into digital impulses and sends itto the receiver (the internal component,about the size of a quarter, that is surgi-cally implanted in the temporal bonebehind the ear under the skin). The receiv-er is connected to electrodes in the coch-lea that receive impulses and stimulate theauditory nerve with these digital impuls-es, permitting perception of the digitallyprocessed information as speech.
Cochlear implants can be life-changing
for many individuals with severe to pro-found hearing loss. With the addition ofspeech reading, these individuals may beable to engage in more effective verbal
communication because the implants en-
able them to distinguish the beginningsand endings of words, as well as the into-nation and rhythm patterns being used.Individuals can typically hear moderatesounds, although they may have difﬁcul-ty perceiving speech clearly in noisy en-vironments and may also have difﬁcultyclearly hearing music. Implants can alsohave a positive impact in work environ-ments. In addition to being able to hearverbal communication, individuals withcochlear implants are also better able tohear and identify warning signals (Saxon,Holmes, & Spitznagel, 2001).
Although cochlear implants do not re-
store normal hearing and the sound heard
is not like an acoustic signal, individualswith implants become accustomed tohearing via this electrical stimulation. Notall deaf individuals are candidates forcochlear implants. For adults, the follow-ing criteria should be met before receivinga cochlear implant:• Severe to profound sensorineural
deafness
• Little or no beneﬁt from hearing
instruments
• Postlingually deafened• Motivated and psychologically suit-
able for the implant
The chief predictor of success for a coch-
lear implant is a short duration of deafness
(Fischetti, 2003; Gates and Miyamoto, 2003).
Age does not appear to be relevant, al-
though children who are prelingually deaf-
ened may beneﬁt from early implantation
to facilitate speech development. Indi vid-
uals receiving a cochlear implant should
have realistic expectations for hearingability after the
implantation. Optimal use
of cochlear im plants requires a commit-
ment to rehabilitation, training, and dai-ly practice. Although the cost of a cochlearimplant is typically covered by manyinsurances, reimbursement for auditoryrehabilitation, a key to successful use ofimplants, may be minimal or nonexistent.
Before being treated with cochlear im-
plants, individuals are carefully screenedwith an audiological assessment as well asa thorough hearing history, physical ex-amination, and psychosocial assessment.Individuals who are prelingually deafenedand have strong ties to the Deaf commu-nity may be unprepared for the social ram-iﬁcations of a cochlear implant, sincesome individuals in the Deaf culture (dis-cussed later in the chapter) are strongly
opposed to cochlear implants for cultural
reasons. Consequently, in some instancesa cochlear implant can socially isolateindividuals from other friends who areDeaf (Tucker, 1998).
Hearing Aids
A hearing aid is any mechanical or elec-
tronic device that improves hearing. Hear-158 CHAPTER 5 H EARING LOSS AND DEAFNESS

ing aids come in different shapes and sizesand are prescribed and ﬁtted according toindividual need. Common styles of hear-ing aids are as follows:
• Behind-the-ear style, which curves
around the back side of the ear
• In-the-ear style, which ﬁts in the ear
canal and outer ear bowl
• Canal style, which ﬁts entirely with-
in the ear canal so it is barely visible
All hearing aids, regardless of type or
shape, magnify sound and have:
• a micophone to pick up sound•
an ampliﬁer that makes sound louder
•a receiver that conveys sound to the ear
• a battery that provides power for the
hearing aid to work
Some hearing aids have special features
called telecoil circuitry or tone control .
Hearing aids with telecoil circuitry have
a special switch or push button ( T-switch )
located on the hearing aid case that acti-vates the telecoil. A telecoil is a very small
coil of wire that acts as an antenna, pick-ing up electromagnetic energy that is thendelivered to the receiver of the hearing aidand converted into sound. Also availableis a plug called a boot or shoe that is
designed to ﬁt over the end of a behind-the-ear type of hearing aid and that isequipped with direct auditory
input. This
device enables individu als to be connect-
ed to an external sound source, such as aradio signal or microphone. It improvesthe signal-to-noise ratio, improving soundquality. Telecoil circuitry enables individ-uals to use other assistive listening devicesdiscussed later in the chapter.
Tone control is a feature on convention-
al analog hearing aids (discussed shortly)that allows the audiologist to modify thefrequency sensitivity of the hearing aidampliﬁer to best frequency-shape thehearing aid response to the individual’shearing loss. For instance, individuals withhearing loss at higher frequencies have dif-ﬁculty hearing some higher-pitched tonesof speech. Tone control is an attempt toamplify the high frequencies withoutamplifying the lower frequencies.
The most up-to-date technology in-
cludes digital hearing aids , which change
acoustic signals to a discrete series of dig-ital signals so the audiologist can mostappropriately frequency-shape the hearingaid response to the individual’s hearingloss. As a result, digital hearing aids cantheoretically be programmed for eachindividual hearing loss and provide moreprecision and clarity of sound. Many con-tain special “noise-reduction circuits” thataid in reducing background noise. Digitalhearing aids have become much moreaffordable in recent years, with technolo-gy options from basic digital to premiumdigital with directional microphones.
Conventional ampliﬁcation with older
technology is still available in analog hear –
ing aids . These original ampliﬁcation de-
vices make all sounds louder with the ex-
ception of the soft speech sounds so neces-
sary for good speech intelligibility. Thereare limited adjustments available, andtherefore sound quality is often lacking.
Hearing aid units are dispensed and ﬁt-
ted by audiologists and/or hearing aid dis-
pensers . They may be ﬁtted to one or both
ears. Monaural refers to one hearing aid,
and binaural refers to two. Recent trends
show that binaural ﬁttings are becomingthe norm for a variety of reasons, includ-ing improved localization skills, safety,and ease of hearing in noise. Most indi-viduals are given a written contract thatprovides them with a 30-day trial evalua-tion period, after which, if they are notsatisﬁed, they can receive a refund for thecost of the hearing aid, less any servicecharges. Although the hearing aid indus-try has become more regulated, in orderTreatment of Hearing Loss and Deafness 159

to prevent potential misuse it is highlyrecommended that individuals seekingamplification consult with a licensedaudiologist who specializes in hearing aiddispensing. As always, a referral from afriend, family member, or trusted medicalprofessional is the best way to ensure thatthe individual is working with a skilledand reputable professional.
Although hearing aids may improve
hearing and may be beneﬁcial for manyindividuals, they do not correct hearing
in
the way that glasses can improve vision to
20/20 (Desselle & Proctor, 2000). Hearingaids can improve volume but not alwaysclarity of speech. Because they work asampliﬁers, they make speech louder butnot always more clear. In addition, hear-ing aids amplify not only speech but oth-er sounds in the environment as well,which can interfere with
the individual’s
ability to decipher speech. Individuals
with hearing aids may need speech read-ing to help ﬁll in the gaps in comprehen-sion that still exist. Digital hearing aidshave a unique advantage in that they canprovide more volume to the soft soundsof speech and less volume to the louder,background sounds that can interfere withunderstanding.
Hearing aids should be carefully pre-
scribed to meet individual needs. It is rec-ommended after purchase of hearing aidsthat individuals are provided with a 30-day trial and evaluation period. For bestpractice, hearing aids should be dispensedwith appropriate veriﬁcation and orienta-tion regarding proper care and use. Orien-tation is vital to the success of hearing aiduse because it helps establish realistic ex-pectations. Additional audiological reha-bilitation training or counseling will alsohelp individuals learn ways to enhancecommunication and to minimize commu-nication obstacles. One of the barriers toeffective hearing aid use is the attitude ofthe individual. Some individuals may beresistant to using a hearing aid becausethey believe society will view them as lesscapable. Although smaller, less conspicu-ous hearing aids have improved accept-ance, negative attitudes are still one factorthat may prevent many from beneﬁtingfrom hearing aids.
Hearing aids are delicate devices that
need routine care and maintenance to en-sure maximum function. Batteries must bereplaced regularly. Individuals using hear-ing aids must also be careful to avoid dam-
aging the internal components. They should
refrain from dropping them and from sub-jecting them to extremes in temperature,excess moisture, or exposure to other sub-stances, such as hairspray, that could dam-age the microphone or receiver.
Telephone Devices
There are many different types of tele-
phone devices to assist with telephonecommunication. Some of them are com-patible with hearing aids and work in con-junction with the hearing aid telecoil .
These telephones enable the hearing aiduser to utilize the telecoil circuitry and
therefore receive a clearer signal withoutannoying acoustic feedback or squeal. Thetelecoil circuitry allows the hearing aiduser to tap the electromagnetic signalfrom the telephone. Electromagnetic ener-gy is transferred to the receiver of the hear-ing aid and converted into sound. At
present not all phones are compatible with
telecoil; however, the Federal Communi-cation Commission has made changes instatutes that now require all new phones,including
cell phones, to be hearing aid
compatible.
There are also other telephone devices
that may be used without a hearing aid.Portable telephone ampliﬁers can be slipped
over a telephone receiver and may be use-160
CHAPTER 5 H EARING LOSS AND DEAFNESS

ful for hard-of-hearing individuals whotravel and need a louder signal. Other tele-phone ampliﬁcation devices may be wiredto the telephone handset so that volume is
increased, giving the user more control.
Public telephones equipped with ampliﬁer
handsets, although not always readilyavailable, are becoming more common.These telephones are usually identiﬁedwith an access sign.
Telecommunication Display Devices (TDDs)
Telecommunication display devices (TDDs ),
also called teletypewriters or TTYs , are used
to transmit conversations in printed format
over regular telephone lines. Individualson both ends of the line must have com-patible devices with which to type theirmessages and visualize the printed mes-sage on a screen or paper. If one individ-ual does not have a TDD, a third-partysystem may be used or a relay operator can
transmit the message to the other individ-ual. Telecommunication relay services al-low a person using TDD to communicatewith another person using a voice tele-phone, with the relay operator acting asan interpreter. Special software is alsoavailable that allows a personal comput-er to interact with a TDD and provide asynthesized speech signal. Computers arealso allowing greater access for deaf andhard-of-hearing individuals with e-mail.
Assistive Listening Devices
Assistive listening devices include a wide
variety of equipment other than hearingaids that can be used by persons withhearing loss. Some may be used independ-ently, and others supplement the hearingaid. Since individuals with hearing lossmay have more difﬁculty perceiving thehigh-pitched sounds common in speech,or hearing in background noise, assistivelistening devices help to improve thesignal-to-noise ratio by facilitating listen-ing and reducing background noise andreverberation.
Hard-Wired Systems (Personal Listening
Systems)
Hard-wired systems are individual devices
that amplify speech and minimize outside
noise so that speech can be more easily un-
derstood. The systems must have a directconnection to the sound source usingeither a microphone or a direct plug-inwire to convey ampliﬁed speech signaldirectly to the receiver (a hearing aid, ear-phone headset, or neck loop) worn by theindividual. When a microphone is used,the speaker speaks into the microphone,speech travels through a cord, and thendirectly reaches the receiver worn by thelistener. When a plug-in is used, a wire isplugged into the sound source (such as atelevision or radio) and the sound travelsthrough the wire directly to the individ-ual’s personal receiver. Using hard-wired
systems with television or radio enables in-
dividuals with hearing loss to increase thevolume on their personal receiver without
altering the volume for others in the room.
Hard-wired systems are considered per-
sonal listening systems and are more use-ful in one-to-one communication than ingroup settings. The systems are small, with
the ampliﬁer being contained in a pocket.
Large Area Systems
Background noise competes with speech
sounds, creating a more challenging lis-tening environment for individuals withhearing loss. Additional reverberation af-fects sound quality in large groups andbrings a more distorted signal to hard-of-hearing individuals. Finally, distance is thethird factor that has a negative effect forTreatment of Hearing Loss and Deafness 161

those with hearing loss. A number of large
area devices to enhance hearing in group
settings are available:
• Audio loop systems • FM systems• Infrared systems
Audio Loop. Audio loop systems are made
up of a microphone, ampliﬁer, and coil of
wire (also called induction coil) that loops
around the seating area. Electricity ﬂowsthrough the coil, creating an electromag-netic ﬁeld that can be picked up by thetelecoil of a hearing aid that has been acti-vated through the T-switch or push but-ton. The telecoil acts as an antenna andpicks up the electromagnetic energy, de-livering it to the user’s hearing aid. In-dividuals using the audio loop must sitwithin or near the loop for it to operateeffectively. Audio loop systems can be per-manently installed in public meetingrooms, churches, or theaters or can be setup as needed.
FM Systems. FM systems are wireless and
work much the same as listening to FMradios. Sound is picked up and transmit-ted through a frequency-modulated banddirectly to a receiver worn by the individ-ual with hearing loss. Wireless FM systemshave greatly reduced the hardware need-ed, especially with regard to hearing andcompatibility. FM systems enhance listen-ing in noisy environments by improvingthe signal-to-noise ratio.
Infrared Systems. Infrared systems require
the installation of an infrared light emit-ter that is usually piggybacked onto anexisting public address system. Sound istransmitted by invisible, harmless infraredlight waves and picked up by a receiver,which can be a headset for use without ahearing aid or a receiver that can be usedwith hearing aids equipped with a T-switch. These systems are best suited inrooms or meeting areas without windows,since sunlight affects the signal.
Caption Services and Telecaption Adapters
Closed captioning may be used for televi-
sion or movies in which printed dialogue
appears on a corner or at the bottom of the
screen. Real-time caption services displaythe text on the video monitor immediate-ly. All televisions manufactured after July1993 that are 13 inches or larger must be
equipped with a closed caption option. Old-
er models can utilize a decoder that is con-nected to the television. Captioned featureand educational ﬁlms are also availablethrough various distribution services.
Alerting Devices
Hearing enables individuals to respond
to sounds such as sirens, the horn of anapproaching car, the doorbell, or a baby’scry. Hearing loss can hamper individuals’ability to respond to everyday environ-mental sounds, potentially increasing therisk of accidents and possibly increasingfeelings of insecurity. Various devices andsystems are available commercially to alertindividuals with hearing loss to these cues.They may use visual cues , such as ﬂashing
lights; auditory cues , such as increased
ampliﬁcation of sound; or tactile cues , such
as a vibrator. Certiﬁed Hearing Guide Dogs
(International Dogs, Inc.) are dogs that aretrained to react to certain environmentalsounds (a telephone ringing, etc.). The dogdoes not bark but rather makes physicalcontact with the individual and then runsto the source of the sound.
Speech Reading (Lip Reading)
Speech reading is a communication skill
in which individuals with hearing loss162 CHAPTER 5 H EARING LOSS AND DEAFNESS

watch for clues from the lips, tongue, andfacial expression of the speaker. Only one-third of the English language is visi
ble on
the lips; therefore, individuals who speech-
read often supplement meaning by ob-serving the facial expressions of thespeaker and gathering conceptual cues(Myers & Thyer, 1997). Speech reading
may also be supplemented with a manual
communication system such as cued speech .
Speech reading requires good lighting.
The speaker must face the individual whois speech reading and must be closeenough
to enable the individual to see the
speaker’s lip formation. The speakershould use a natural speaking voice andexpression,
avoiding distortions of the
mouth through movements such as gri-
macing. Speech reading is more difﬁcultwhen the speaker speaks very rapidly orenunciates poorly, or when distractinghand movements, a beard, or a mustacheobstructs view of the lips. Speakers
should
avoid chewing, turning away from the lis-
tener, or moving about while talking. Thespeaker should obtain the listener’s atten-tion before beginning to speak and clari-fy statements as necessary. Considerableconcentration is required for most peoplewith hearing loss to grasp the spokenword. It is a complex process that can be
very tiring when conversation is extended.
Sign Language
Language is a set of symbols combined
in a certain way to convey concepts, ideas,
and emotions. There are many ways of
transmitting language. Speech is the verbal
expression of language concepts. Sign
language is a means of communication in
which speciﬁc hand conﬁgurations sym-bolize language concepts.
There are several types of sign lan
guage,
the two most common being Amer ican
Sign Language (ASL) and Signed English.American Sign Language is a distinct and
complete language that contains linguis-tic components that constitute a sophis-ticated, independent language. It is thenative language of Deaf culture. ASL hasits own grammar and syntax, idioms andmetaphors. It has no written form. More-over, it is conceptual in nature, ratherthan word oriented. The signs of ASL areabstract symbols that are capable ofexpressing multiple elements simulta-neously. Signed English , in contrast, follows
the syntax and linguistic structure ofEnglish. Often, people who use SignedEnglish also mouth the words that theysign. This process is called simultaneous
communication . Cued speech , another sys-
tem of communication, is phoneticallybased and uses hand shapes to representspeech sounds.
Interpreters
Certified interpreters can provide an
important communication link betweenthe deaf or hard-of-hearing individual andthe hearing world. In both group settingsand one-to-one interactions, the
inter-
preter is able to translate information so
that accurate communication can takeplace. In situations where it is importantfor the translation to be precise and accu-rate, such as professional medical orcounseling situations, it is crucial that theinterpreter be properly trained and be cer-tiﬁed by the Registry of Interpreters for the
Deaf. In particular situations, such as med-
ical or counseling interactions, it is bene-ﬁcial to use a professional interpreter withexperience in mental health or medicalparadigms who can properly assist withcomplex communication needs. Althoughfamily members sometimes serve as inter-preters for deaf or hard-of-hearing individ-uals in more informal situations, inprofessional situations, their use mayTreatment of Hearing Loss and Deafness 163

obscure objective information and there-fore should be avoided if possible.
The presence of an interpreter can be
intrusive and alter the dynamics of themedical or counseling interaction. Use ofinterpreters means that a third party willbe present, reducing the sense of privacythat is normally expected in a number ofprofessional situations. Certiﬁed interpre-ters, however, practice under a stringent
Code of Ethics that requires that all trans-
actions must be strictly conﬁdential. Ittakes some adjustment for the employer,counselor, physician, or other personworking with the deaf or hard-of-hearingindividual to become accustomed to hav-ing a third party present in situations that
normally take place on a one-to-one basis.
PSYCHOSOCIAL ISSUES INHEARING LOSSDeafness and Deaf Culture
The needs of people who are deaf or
hard of hearing are different from thoseof individuals with other disabilities.Hearing is vital to verbal communicationand to perception of environmental cues;thus all hearing loss interferes with dailyfunction to some degree. The ability ofindividuals to cope with hearing lossdepends on the type and degree of loss,the age of onset, and the extent to whichit interferes with daily communicationand activity.
Deafness pervades every aspect and
activity of an individual’s life. It affectsspeech intelligibility and other basic as-pects of hearing, such as localization, re-cognition, or identiﬁcation of sound.Severe hearing loss or deafness experi-enced congenitally or in early childhoodalso has developmental implications.Hearing loss occurring prior to languagedevelopment affects individuals’ experi-ence and opportunity to gain conceptsgenerally taken for granted in the hearingworld. Children learn many conceptsfrom overheard conversations, back-ground information from radio or televi-sion, and a multiplicity of other sources.Through this peripheral, daily communi-cation, children learn cultural norms andexpectations, generate and shape ideas,and form and enhance values and beliefs.Children who have severe hearing loss orare deaf are not exposed to many elementsof communication that enrich the lan-guage base, help to formulate concepts,and impart social norms.
Individuals with congenital deafness or
with hearing loss acquired before speechdevelopment require special programs tohelp them learn to communicate. Unfor-tunately, hearing loss in the very youngis not always recognized immediately and
may be misinterpreted as intellectual deﬁ-
cits, mental retardation, or behavior dis-orders. Normal develop
ment and healthy
adjustment of children with hearing loss
are dependent to a large degree on earlydiagnosis and treatment, early social andcultural inﬂuen
ces, and parental attitudes
and acceptance.
The diagnosis of deafness in a child of-
ten results in parental guilt, overprotec-
tion, or rejection. Professional assistance for
the family of a newly diagnosed deaf in-fant may be critical to their acceptance ofthe child’s needs and to their competencein providing a nurturing environment forthe child’s emotional development.
Individuals who have acquired a hear-
ing loss during adulthood have memoriesof sound, language, and previous func-tion. Speech patterns have already beenlearned and can be maintained throughspeech and conversation therapy. Indi-viduals who have acquired hearing loss inadulthood may, however, feel uncomfort-able and fear that others will reject them164
CHAPTER 5 H EARING LOSS AND DEAFNESS

if they admit their disability. They maydeny that hearing loss exists or may devel-op strategies to hide it, such as dominat-ing the conversation to minimize thenecessity of understanding anyone else, oraccusing others of not speaking clearly ormumbling. Some individuals exhibitaggressive and dominating behavior as areaction to their hearing loss. Individualswith hearing loss may withdraw complete-ly from situations in which they have dif-ﬁculty hearing. Being unable to under-stand what is being said, individuals withhearing loss may believe that the laugh-ter and talk of others are being directedtoward them.
Hearing loss can lead to isolation, loneli-
ness, and frustration, as well as to sensory
deprivation. Hearing helps individuals com-
municate on a daily basis with family andfriends, and in the social and work setting.At the most basic level, hearing helps indi-
viduals keep in touch with the environment.
Background sounds, such as the wind in
the trees, children playing down the street,
or a train whistle in the distance, keepindividuals aware of what is happening inthe outside world. Hearing also acts as a
signal to action. The sounds of a telephone
ringing or a baby crying, the horn of anapproaching car, or the sound of footstepsfrom behind are all cues for some type ofaction. Thus, not only must individualswith a loss of hearing alter activities forwhich hearing is vital, but also they car-ry a sense of vulnerability because of theirinability to hear sounds that once servedas cues to action or danger.
There is a distinction between Deaf cul-
ture and the Deaf people who are in it andthose who are deaf. Using a capital D indi-
cates individuals who are part of a Deafculture and describe themselves as a lin-
guistic minority sharing a culture, not a
medical condition (Phillips, 1996; Porter,1999). From the vantage point of Deaf cul-ture, deafness is not a disability, not a deﬁ-ciency, not a handicap, but rather a cul-ture (Lane, 1995).
Culture inﬂuences knowledge, beliefs,
attitudes, values, and perceptions. It under-
lies the meaning given to action and themeans by which experiences in the worldare organized and understood. One of thekey components of culture is language,which is necessary for communication.Language is an important part of everycultural identity and determines to a greatextent who talks with whom and what isdiscussed (Rendon, 1992). For those withhearing loss, there is no common lan-guage. Not all individuals with severe orprofound hearing loss use the same lan-guage to communicate. Some use SignedEnglish; some use ASL; some rely to a largeextent on speech reading. Especially forprelingually deaf individuals who use ASLas their primary language, English may beviewed as a foreign language.
ASL is a source of pride for the Deaf
community. It is a language with its ownstructure and syntax that has developedover time and that has no written form(Filer & Filer, 2000). Consequently, thosewho use ASL and especially those who areprelingually deaf frequently become partof the larger Deaf culture.
The Deaf community has its own the-
ater, literature, and schools, along withsocial rules that are different from those
of the hearing community (Barnett, 1999).
These differences, especially in social
norms, patterns, and traditions, can cause
misunderstandings and misperceptions bythose in the hearing world. Members ofthe Deaf culture have rules for behaviorssuch as getting the attention of individu-als with whom they would like to commu-nicate. Stomping a foot or tapping thehand of an individual to get his or herattention may be perfectly acceptablesocial behavior in the Deaf communityPsychosocial Issues in Hearing Loss 165

but may be viewed as rude by those in thehearing world. Consequently, individualsin the Deaf community may tend to asso-ciate with others in the Deaf communityrather than with those in the hearingworld, may prefer state residential schoolsfor educating the deaf rather than main-streaming, and may reject efforts to incor-porate them into the hearing world. Insome instances, pride in the Deaf culturemay even preclude procedures such ascochlear implant that could improve theability to hear. Deaf individuals maybelieve that attempts to correct their hear-ing is an implication that they have amedical condition that needs a cure, per-petuating the view of the Deaf as disabled.Some may go as far as to view efforts tocure deafness as an attempt to obliterateDeaf culture (Tucker, 1998).
The Deaf culture does not exist in a vac-
uum. Individuals may be imbedded in theDeaf culture, but they are also imbuedwith values, attitudes, and behaviors thatare part of a larger national culture as wellas often part of ethnic
minority cultures
(Moore, 2001). This fact creates another
layer of diversity.
Psychological Issues in Hearing Loss
Hearing loss is often associated with iso-
lation because of the very nature of thedisability itself. Grief reactions are notuncommon for individuals with acquiredhearing loss because they have memoriesof sounds. Their inability to hear cherish-
ed sounds, such as the voices of loved ones,
music, or the chirping of birds, may be adifﬁcult loss to accept.
Because hearing loss is an invisible dis-
ability, denial is common, especially forthose who acquire a hearing loss later inlife. They may react with increased sensi-
tivity or irritability when they do not under –
stand words. The increased social pressureto understand may cause anxiety and frus-tration, and they may avoid activities andinteractions that they once enjoyed. Un-willingness to acknowledge hearing lossmay result in individuals’ refusal to par-ticipate in hearing evaluations or reluc-tance to wear hearing aids.
Individuals with an acquired hearing
loss, especially if the onset is sudden, mayexperience depression. The suddenness ofthe loss does not give individuals theopportunity to adapt gradually as hearingdiminishes; consequently, they are unlike-ly to have developed signing skills. De-pression can also interfere with learningand using new communication skills. Itseffects are circular; depression is a barrierto communication, thus intensifying feel-ings of isolation and making the individ-ual more depressed. Counselors trained insign language may not be readily avail-able, and the use of an interpreter forcounseling sessions may increase thereluctance to participate or to disclose feel-ings openly.
Any and all of the emotional states
experienced by the adult with hearing lossmay be experienced by the parents of chil-dren who have been identiﬁed as hard ofhearing or deaf. Just as adults with hear-ing loss must work through their feelingsto achieve a healthy adjustment, so mustparents before they can be of optimalassistance to their child.
Individuals who have hearing loss
depend heavily on visual channels and onmanual means of communication. Thedevelopment of additional medical condi-tions that threaten these resources is ofincreased concern. A visual impairment orconditions that affect the hands, such asrheumatoid arthritis, can serious
ly hamper
individuals’ accustomed means of commu-
nication if they are using ASL or Signed
English, necessitating additional training in
new ways of communicating.166 CHAPTER 5 H EARING LOSS AND DEAFNESS

Lifestyle Issues in Hearing Loss
Many daily activities involve the sense
of hearing. For individuals with hearingloss, simple transactions, such as purchas-ing items from a local store, communicat-
ing with a repair person, or obtaining direc-
tions, require additional means of commun-
ication. In some instances, use of a thirdparty as an interpreter may be a solution;
however, individuals who are hard of hear-
ing or deaf may resent the loss of privacyor the sense of independence associatedwith the use of an interpreter.
Signal dogs trained to alert their deaf
owners to environmental sounds or sig-nals are increasing in popularity. Specialdevices are necessary to make daily envi-ronmental sounds, such as a knock on thedoor, known to individuals with hearingloss. Technology and special aids become,in many instances, a necessity.
Everyday activities with family members
may require more effort on the part of allinvolved. For instance, without awarenessand sensitivity of other family members,individuals with hearing loss may be un-
able to participate actively in family conver-
sations at mealtime or engage in small talkwhile performing various tasks. They mayalso ﬁnd themselves increasingly left out
of family decision making and discussions.
Depending on the degree of hearing
loss, special activities, such as watching
television or attending movies, plays, and
concerts, may also be affected. Special de-vices mentioned previously may help in-dividuals participate more fully in suchactivities. In addition, television decodersthat provide captioned programming maybe available to enable individuals withhearing loss to enjoy television.
Although hearing loss does not direct-
ly affect sexual activity, verbal communi-cation during lovemaking may no longer
be possible, which may be viewed by someindividuals as an emotional loss. Indi-viduals who are single may have more dif-ﬁculty meeting potential partners andestablishing communication that couldlead to a more intimate relationship.
Social Issues in Hearing Loss
The social environment of individuals
with hearing loss is profoundly alteredbecause of the need for alternate means ofcommunication. Individuals who havebeen deaf since birth or early childhoodmay integrate well with the Deaf commu-nity, where a common language is shared.Those who acquired hearing loss later inlife, however, frequently do not join theDeaf community and may feel more iso-lated, feeling they ﬁt neither into the Deafcommunity nor into the hearing world.
Language plays an important role in reg-
ulating social play interactions and is para-mount for framing and setting up playactivities. Children who are deaf or hardof hearing may have difﬁculty developingcooperative play with hearing playmates.Promoting socialization between childrenwith hearing loss and hearing peers in-cludes building on the strengths of thechildren and fostering a shared commu-nication system that encourages social
integration. Hearing loss at an early age also
has implications for literacy. Individualswho have been deaf from an early age usu-ally have a literacy level of fourth to ﬁfth
grade after high school (Barnett, 1999). Low
literacy rates may be attributed to lack ofconsensus on educational methods. Em-
phasis is often placed on techniques ofcommunication, with less emphasis oncontent
matter. In addition, since English
is often a second language to individualswho are deaf, children who are deaf mayface the same educational barriers as thoseexperienced by other minority groupswhose primary language is not English.Psychosocial Issues in Hearing Loss 167

People with acquired hearing loss in
adulthood are more likely to maintain
social and cultural contacts with the hear –
ing world because they have grown upwith the language and culture of the hear-ing world. Many people, however, whowere deaf from an early age and use ASLas their primary language feel part of theDeaf community, which has established aculture in which there are, in addition tolanguage, speciﬁc norms and characteris-tics. Individuals in this culture identifywith the Deaf community and tend toview deafness not as a disability but ratheras an alternative culture and associatedlifestyle. Individuals who do not speak thelanguage are frequently viewed as out-siders and are not readily integrated intothe Deaf culture.
Individuals with hearing loss may lim-
it social contacts to family members anda few close friends, or they may avoidsocial contacts altogether because of theirinability to understand what is being said.Difﬁculty in understanding ver
bal com-
munication can cause withdrawal from
social situations to avoid the embar-
rassment of giving inappropriate responses
to questions or statements. Lack of under-
standing by others can contribute to social
isolation. New acquaintances, unfamiliarwith hearing loss or unaware of the indi-vidual’s inability to hear, may perceivethem as aloof or even rude be
cause of their
failure to respond to a friend ly statement
that they did not hear.
Individuals may have more difﬁculty
keeping up with conversations in groupsettings, especially if others in the groupare unaware of or insensitive to theirneeds. Group settings with poor lightingmake lip reading more difficult, andcompeting sounds, such as the rattling ofdishes in a restaurant, may make com-munication difﬁcult even for individualswith milder hearing loss. Engaging in conversation requires coop-
eration from others. Some people may feel
uncomfortable or impatient while attempt –
ing to communicate with individuals with
hearing loss; consequently, they mayavoid contact with them. Some may con-sider deafness a social stigma because ofmyths and misconceptions about hearingimpairments. Such attitudes build a bar-rier to acceptance by others and to inclu-sion in the larger social community.Societal responses can create difﬁcult andstressful situations for individuals with ahearing impairment, discouraging furtherparticipation in social functions. A num-ber of support groups directed towardindividuals with adult or late-onset hear-ing loss are available. One such group isSelf Help for Hard of Hearing People(SHHH). Another example of such agroup is the Association of Late DeafenedAdults (ALDA). In addition to offering sup-port to individuals who are hard of hear-ing or deaf, SHHH and ALDA strive toincrease community understanding aboutthe rights and needs of individuals withhearing loss, as well as to make social envi-ronments more accessible. Many commu-nities also have other types of supportgroups for people with hearing loss.
Although family members serve as a sup-
port group for individuals with hearingloss, their attitudes may also impede indi-viduals’ acceptance of their condition and
subsequent rehabilitation. Family members
may perceive a hearing loss as feigned ormay attribute the difﬁculty to inattention.As a result, they may become angry, ignorethe individual, or exclude him or her fromconversations rather than learn tech-
niques to enhance the individual’s ability
to maintain an active role in conversation.Family members who serve as interpretersfor those with hearing impairments may,on the other hand, begin to resent theirrole, feeling stiﬂed in social interactions.168
CHAPTER 5 H EARING LOSS AND DEAFNESS

VOCATIONAL ISSUES INHEARING LOSS
Individuals with hearing loss face the
same issues with regard to employment asothers with disability; however, there areadditional special vocational issues thatmust be considered. Many jobs requireindividuals to be alert to auditory cues inorder to perform or maintain safety. Mostjobs also require communication withcoworkers and/or customers. People whoare deaf or hard of hearing may have dif-ﬁculty receiving instructions or supervi-sion or participating in staff meetings orin-service training. In addition, they mayhave difﬁculty interacting in work-relatedsocial functions. Assistive devices may beneeded to help
them with basic commu-
nication, or there may be a need for job
restructuring, redesigning procedures, orredelegation of assignment to accommo-date their communication needs.
Just as there are myths and stereotypes
about hearing loss in the social world, sothere are myths and stereotypes in the
world of work. Employers and fellow work-
ers may not understand hearing loss andmay be unaware of the special needs ofdeaf or hard-of-hearing individuals, or ofthe special techniques available toenhance communication with those whoexperience it. Since hearing loss is an in-
visible disability, coworkers or supervisors
may not recognize the need for specialaccommodations or may feel that individ-uals with hearing loss are feigning theirdegree of disability. In some instances,individuals’ lack of ability to hear may beinterpreted as lack of intellectual ability,
and those with hearing loss may be relegat-
ed to jobs requiring less cognitive ability.
In the work setting, individuals with a
hearing loss may need special assistivedevices, communication aids, and signal-ing devices. The use of such devices isoften dependent on the availability andexpense of the purchase and installationof the special items. Equipment may beprioritized according to need if funds arelimited. For example, a signaling devicethat may be crucial for safety may be con-sidered vital, while equipment that wouldenhance individuals’ performance maynot receive as high a priority.
Because visual cues are so important to
communication for individuals with hear-ing loss, good lighting in the workplace isa necessity. Many individuals with hear-ing loss experience discomfort
with loud
noises; therefore, the noise level in the envi-
ronment should be evaluated. In some in-
stances, it may be necessary for individuals
to wear ear protectors to prevent furtherhearing loss. Room acoustics must also beconsidered, because the reverberation ofsound in an environment
can interfere
with hearing aid effectiveness.
Hearing aids can greatly enhance some
individuals’ performance in the work set-
ting. Although technological advances have
made hearing aids more durable, they areintricate devices that may be susceptibleto damage from environmental factors.They are sensitive to extremes of temper-ature, especially extreme cold, and they
re-
quire protection from perspiration in hot
and humid environments.
Individuals with hearing loss are a hetero-
geneous group and should be consideredas such. Although special needs associat-ed with hearing loss should be considered,individuals’ special talents and interestsshould also be considered in helping thosewith hearing loss adjust to the work envi-ronment. With the use of assistive devices,many job opportunities not previouslyavailable to individuals with hearing lossare now possibile.
Central to success in
vocational placement of individuals who
are deaf or have hearing loss is employerfamiliarity with indi-
viduals’ accommoda-Vocational Issues in Hearing Loss 169

tion needs (Schroedel & Geyer, 2001). In
addition, some attitudinal barriers stillexist that may preclude the individualwith hearing loss from obtaining satisfac-tory employment.
CASE STUDIESCase I
Mrs. G. is a 42-year-old woman who has
worked as a public relations specialist ata major university for the past 10 years.In this position Mrs. G. handles commu-nity relations, establishes cooperative rela-tionships with the community and localbusinesses, and keeps university adminis-trators aware of public interests and con-cerns. She also is responsible for preparingwritten press releases and special intereststories that promote activities at the uni-versity. In addition, she is responsible forpreparing reports and writing proposalsfor various projects. Over the past fewyears Mrs. G. has noted a signiﬁcant de-cline in hearing in her left ear with mod-erate loss in her right ear. On evaluationby an audiologist she was found to havesensorineural deafness resulting fromsmall strokes.
Hearing loss in the left ear
was at the 45-decibel range, and loss in the
right ear was at 30 decibels. Mrs. G. has amaster of science degree in public policy.She is married and lives with her husbandin a small city of 50,000. She has twogrown children.
Questions
1. To what extent will Mrs. G.’s degree
of hearing loss impair her ability tocontinue to function in her currentoccupation?
2. Given the type and extent of Mrs.G.’s
hearing loss, what adaptive devices orreasonable accommodations mayhelp her maintain her currentemployment?
3. Should Mrs. G. receive additional
evaluation?
4. How do Mrs. G.’s current skills and
work history affect her rehabilitationpotential?
5. What additional information would
you want to gain about Mrs. G.’s spe-ciﬁc duties at her current job?
Case II
Mr. M. is a 55-year-old male who works
as a printing press operator for a large citynewspaper. While printing presses are run-ning, he monitors the printing process forpaper jams and even ink distribution, andhe attempts to keep the
printing process
running smoothly. There is considerable
pressure on the job because the presseswork under high printing speeds, andpress machinery is potentially hazardousas well as noisy. Mr. M. has experiencedincreasing difﬁculty hearing and on eval-uation is found to have mixed hearingloss, with moderate loss in both ears. Hehas a high school education. He has nev-er been married and lives alone in a smallapartment several blocks from work.
Questions
1. What speciﬁc environmental issues
would you address with Mr. M. and/or his employer regarding his currentjob situation?
2. What speciﬁc issues about Mr. M.’s
type and level of hearing loss wouldyou address?
3. Are there issues regarding Mr. M.’s age
that you would consider in workingwith him on his rehabilitation plan?170
CHAPTER 5 H EARING LOSS AND DEAFNESS

References 171
REFERENCESBarnett, S. (1999). Clinical and cultural issues in car-
ing for deaf people. Family Medicine, 31 (1), 17–22.
Desselle, D. D., & Proctor, T. K. (2000). Advocating
for the elderly hard-of-hearing population: Thedeaf people we ignore. Social Work, 45 (3),
277–281.
Filer, R. D., & Filer, P. A. (2000, Winter). Practical con-
siderations for counselors working with hearingchildren of deaf parents. Journal of Counseling and
Development, 78 , 37–43.
Fischetti, M. (2003, June). Cochlear implants: To hear
again. Scientiﬁc American, 288 , 82–83.
Gates, G. A., & Miyamoto, R. T. (2003). Cochlear
implants. New England Journal of Medicine, 349 (5),
421–423.
Hendley, J. O. (2002). Otitis media. New England
Journal of Medicine, 347 (15), 1169–1174.
Lane, H. (1995). Constructions of deafness. Disability
and Society, 10 (2), 171–189.
Moore, C. L. (2001). Racial and ethnic members of
under-represented groups with hearing loss andVR services: Explaining the disparity in closuresuccess rates. Journal of Applied Rehabilitation Coun-
seling, 33 (1), 15–20.
Moore, D. F. (2001). Educating the deaf: Psychology,
principles, and practices (5th ed.). Boston: Hough-
ton Mifﬂin.
Myers, L. L., & Thyer, A. (1997). Social work prac-
tice with deaf clients: Issues in culturally compe-tent assessment. Social Work in Health Care, 26 (1),
61–74.
Phillips, B. A. (1996). Bringing culture to the fore-
front: Formulating diagnostic impressions of deafand hard-of-hearing people at times of medicalcrisis. Professional Psychology: Research and Practice,
27(2), 137–144.
Porter, A. (1999). Sign-language interpretation is psy-
chotherapy with deaf patients. American Journal of
Psychotherapy, 53 (2), 163–176.
Rendon, M. E. (1992). Deaf culture and alcohol and
substance abuse. Journal of Substance Abuse Treat-
ment, 9 , 103–110.
Saxon, J. P., Holmes, A. E, & Spitznagel, R. J. (2001).
Impact of a cochlear implant on job functioning.Journal of Rehabilitation, 67 (3), 49–54.
Schroedel, J. G., & Geyer, P. D. (2001). Enhancing the
career advancement of workers with hearing loss:Results from a national follow-up survey. Journal
of Applied Rehabilitation Counseling, 32 (3), 35–44.
Steel, K. P. (2000). New interventions in hearing
impairment. British Medical Journal, 320 (7235),
622–629.
Tucker, B. P. (1998). Deaf culture, cochlear implants,
and elective disability. Hastings Center Report ,
July–August, pp. 6–14.
Williams, P. J. (2000). Genetic causes of hearing loss.
New England Journal of Medicine, 342 (15),
1101–1109.

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DEFINING PSYCHIATRIC DISABILITY
Psychiatric disabilities encompass a
broad range of conditions in which there
are behavioral or psychological symptoms
associated with distress and/or alteredfunction. Effective mental functioning de-pends on a variety of social and environ-mental factors, as well as on the efﬁcientfunctioning of structures within the brain.As with physical conditions, the degree ofdisability experienced with psychiatricconditions is variable.
Symptoms of psychiatric conditions
vary widely, consisting of both behavioralmanifestations and subjective feelings.Some psychiatric conditions are charac-
terized by deﬁcits in or loss of intellectual
function, whereas others are associatedwith loss of contact with reality ( psycho-
sis). Some psychiatric conditions do not
have symptoms of psychosis but ratherchanges in mood that cause distress orimpaired function. In still oth
er psychiatric
conditions, intellectual func tion, sense of
reality, or mood may be unimpaired, butsymptoms are manifested by maladaptivebehavior.
The extent of disability experienced as
a result of a psychiatric condition is de-pendent, to a great extent, on the degree
to which the condition interferes with in-dividuals’ ability to function within theenvironment, the degree to which theirbehavior disturbs others, and the degreeto which the condition causes subjectivedistress. The goal of psychiatric rehabili-tation is to help individuals develop skillsand supports that will enable them tofunction at their highest capacity in theresidential, educational, or vocational set-ting of their choice.
Psychiatric conditions are more difﬁcult
to deﬁne and diagnose than are physicalconditions. Causes are not always identi-ﬁable, and there are no laboratory testsreadily available to conﬁrm the diagnosis.In many cases, the primary basis for diag-nosis and prediction of functional capac-ity is the experienced judgment of theprofessionals conducting the evaluation.Moderating variables, such as the ethnicstatus, education, and/or socioeconomicstatus of both the client and the profes-sional, may inﬂuence individuals’ perfor-mance on evaluation as well as profes-sionals’ interpretation of the evaluationresults.
THE DIAGNOSTIC AND STATISTICALMANUAL OF MENTAL DISORDERS
The need for a systematic, more stan-
dardized approach to the diagnosis of psy-Psychiatric DisabilitiesCHAPTER 6
173

chiatric conditions has been recognizedfor well over a century. Although initial-ly psychiatric conditions were codiﬁed forthe purpose of collecting statistical infor-mation, in 1952 the American PsychiatricAssociation Committee on Nomenclatureand Statistics published the Diagnostic
and Statistical Manual of Mental Disorders(DSM-I ), a book that was the ﬁrst ofﬁcial
manual of mental disorders that had clin-ical utility. Since that time, there has beencontinued work to revise and reﬁne thediagnostic manual.
As more empirical research and ﬁeld
trials are available, reliability, descriptivevalidity, and performance characteristicsfor diagnostic criteria have been estab-lished. Updated versions of the manualbear the number of the edition (DSM-II,DSM-III, and DSM-IV). The fourth editionof the manual, the DSM-IV , was publishedin 1994, and in 2000 the DSM-IV-TR (4thedition Text-Revision) was published, rep-resenting the latest effort in empirical doc-umentation on which to base diagnosticdecisions.
The manual is an attempt to establish
objective criteria for the diagnosis of psy-chiatric conditions. In addition to provid-ing speciﬁc criteria by which to diagnose,it provides consistency among profession-als in communicating about psychiatricconditions. The use of the manual fordiagnostic purposes requires specializedclinical training, since the criteria withinthe manual are meant to be guidelines andare not considered absolute. Professionalsworking with indi
viduals with psychiatric
conditions should be familiar with the
manual; however, responsibility for thediagnosis most frequently lies with psychi-atrists, psychologists, and, in some states,social workers.
The DSM-IV-TR, rather than taking a
theoretical approach to deﬁning psychi-atric conditions, attempts to describeconditions by deﬁning observable symp-toms. It should be emphasized that diag-nostic criteria should notbe used to
categorize or label people, but rather toassist in treatment planning.
The DSM-IV-TR uses a multiaxial system
of diagnosis to increase speciﬁcity. Sincepsychiatric conditions, like physical con-ditions, rarely occur in a vacuum and varyfrom individual to individual, the multi-axial approach helps professionals avoidfocusing only on specific symptoms.Rather, it enables professionals to take acomprehensive approach to identifyingother variables that could impact on treat-ment and help to predict outcome.
The manual incorporated multiaxial
diagnosis in 1980 to clarify the complex-ities and relationships of symptoms and,thus, assist professionals in more appropri-ate treatment planning. The DSM-IV-TRuses ﬁve axes:
• Axis I describes clinical syndromes.• Axis II describes personality disorders
and mental retardation.
• Axis III describes medical conditions
that may also be present and relevantto individuals’ treatment.
• Axis IV describes relevant psychoso-
cial or environmental problems thatmay have contributed to the develop-ment of the condition or that mayaffect individuals’ treatment or prog-nosis.
• Axis V is used for reporting individu-
als’ overall level of functioning in thejudgment of the clinician who hasdone the evaluation, in accordancewith the Global Assessment ofFunctioning (GAF) scale.
Level of functioning is determined by
using the GAF scale . The scale is a guide-
line for determining individuals’ psycho-logical, social, and occupational function-ing on a hypothetical continuum of men-174
CHAPTER 6 P SYCHIATRIC DISABILITIES

Common Psychiatric Disabilities 175
tal health and illness. Impairment in func-tioning due to physical or environmentallimitations is not considered. Overall lev-el of psychological functioning is rated ona scale of 1 to 100. The scale ranges fromsuperior level of functioning at the levelof 100 to persistent danger of hurting selfor others at the level of 1.
Table 6–1 Multiaxial Recording of
Evaluation Results
Case I
Axis I 309.81 Posttraumatic Stress
Disorder
305.0 Alcohol Abuse
Axis II V7.09 No Diagnosis
Axis III 491.20 Bronchitis, Chronic
Obstructive PulmonaryDisease (COPD), WithoutAcute Exacerbation
Axis IV Unemployment
Axis V GAF = 53 (current)
Case II
Axis I 295.30 Schizophrenia, Paranoid
Type
Axis II V71.09 No Diagnosis
Axis III 250.01 Diabetes Mellitus, Type I/
Insulin Dependent
Axis IV Abusive Caregiver
Axis V GAF = 27 (on admission)
GAF = 52 (on discharge)
Case III
Axis I 296.23 Major Depressive Disorder,
Single Episode, SevereWithout Psychotic Features
Axis II 317 Mental Retardation
Axis III None
Axis IV None
Axis V GAF = 60 (current)Psychiatric conditions are coded ac-
cording to the International Classiﬁcation
of Diseases , Ninth Revision, Clinical
Modiﬁcation (ICD-9-CM). In reportingevaluation results, the ICD-9-CM code pre-cedes the name of the condition.Diagnostic codes are used for record-keep-ing purposes and for reimbursement.Examples of multiaxial recording of eval-uation results with ICD-9-CM coding canbe seen in Table 6–1.
COMMON PSYCHIATRICDISABILITIESConditions Diagnosed in Infancy,Childhood, or Adolescence
A number of conditions are included in
the DSM-IV-TR diagnostic class of“Disorders Usually First Diagnosed in Infan-
cy, Childhood, or Adolescence .” Some of the
more common conditions in this catego-
ry are mental retardation, autism, Asperger’s
disorder, and attention deﬁcit disorder.The conditions are lifelong. Although usu-ally diagnosed prior to the age of 18, attimes these conditions are not diagnoseduntil adulthood.
Mental Retardation
The term mental retardation describes
conditions in which:
• occurrence is before the age of 18
•intellectual functioning is below average
• adaptive behavior is deﬁcient
(American Psychiatric Association, 2000)
The American Association on Mental
Retardation deﬁnes mental retardation as“a fundamental difﬁculty in learning and
performing certain daily life skills” (1992).
Although many individuals still equate
mental retardation with intelligence quo-tient (IQ), the condition
clearly encom-

passes both intellectual func tioning and
adaptive behavior. Adaptive behaviorencompasses individuals’ social skills andperformance in the social environment,including communication and manage-ment of tasks of daily living. In mostinstances adaptive behavior parallels intel-lectual capacity. In addition to limitedintellectual or adaptive capacity, individ-uals with mental retardation may haveother medical conditions or sensory ormotor deﬁcits that further affect theirfunctional capacity. Any
physical or be-
havioral problems must also be considered
in determining the individual’s ability tofunction within the environment.
Although the exact cause of mental re-
tardation cannot always be identiﬁed, anumber of factors are known to be associ-ated. Prenatal causes of mental retardationcan include maternal infections, maternalnutritional deﬁciency, trauma, exposure totoxic sources during fetal development,
maternal oxygen deprivation, and compro –
mise of the fetal blood supply. A variety
of hereditary disorders, such as metabolic
disorders (e.g., phenylketonuria), chromo-somal abnormalities, or some familial syn-dromes, may also result in mentalretardation. Other speciﬁc conditions thatwere acquired in childhood, such asmeningitis, traumatic brain injury, orexposure to toxic substances, can also leadto mental retardation.
Classiﬁcation of Mental Retardation
There are varying degrees of mental re-
tardation or developmental delay. Degreesof severity of intellectual disability canrange from mild to profound. Individualswith mental retardation have a widerange of abilities as well as disabilities. Theextent of support needed varies with theindividual and with his or her circum-stances. Some individuals with mentalretardation may also have delays in motor-
skill development, speech and languageproblems, and vision or hearing impair-ments. In addition, there may be emotion-al challenges and vulnerabilities that canbe caused by pathologic or environmen-tal factors.
Mental retardation is generally catego-
rized as mild, moderate, severe, or profound
and is expressed in relation to the IQ (seeTable 6–2). The IQ is obtained throughadministration of individualized stan-dardized intelligence tests such as theWechsler Intelligence Scales for Children-Revised or the Stanford-Binet . For a partic-
ular individual, the degree of retardationis classiﬁed according to evaluation andtesting of both the individual’s intellectu-al performance and adaptive behavior.Adaptive behavior refers to the individ-ual’s ability to cope with common life de-mands as would be expected of others intheir age group.
Table 6–2 Classiﬁcation of Mental
Retardation
Classiﬁcation IQ
Mild 50–55 to 70 Moderate 35–40 to 50–55Severe 20–25 to 35–40 Profound Below 20–25
Although a number of scales were
developed to measure adaptive function-ing, assessment information is more use-ful if derived from a number of sources,such as teacher evaluation or education-al development, in addition to adaptivescale scores. A variety of other factors,such as environment and stimulation, alsohelp to determine intellectual functioningand adaptive capability, so test results are176
CHAPTER 6 P SYCHIATRIC DISABILITIES

not always absolute. In all instances, notonly intellectual capacity but also adaptivefunctioning must be considered.
Functional Ability According to
Classiﬁcation of Mental Retardation
Mild Retardation. Generally, individuals with
mild mental retardation are considered
capable of attaining intellectual functionup to a sixth-grade level. During their pre-school years, they are generally capable ofattaining social and communication skillsconsistent with their peers; consequently,some individuals may not be distinguish-able from other children in their agegroup. Individuals with mild retardationmay be able to obtain employment andlive independently or with minimal sup-port and supervision, although they may
need additional support and guidance when
in particularly stressful or new situations.Generally, individuals in this category canlive independently in the community.
Moderate Retardation. Individuals with mod-
erate mental retardation often attain
intellectual function at the second-gradelevel, but they may require more supervi-sion in activities of daily living, althoughthey can usually manage self-care. Pro-cessing abstract information is generallydifﬁcult. Individuals with moderate men-
tal retardation are usually capable of learn-
ing some vocational skills, although theymay function best in a supervised workenvironment, such as a sheltered work-shop or supported employment situation.They may have differing degrees of expres-sive and receptive language skills. They aregenerally able to live in the community,but in supervised settings.Severe Retardation. Individuals with severe
mental retardation generally have limit-
ed communication skills and poorly devel-oped motor skills. During school age, they
may attain some elementary self-care skills
and may learn to read some key words;however, for the most part, they willrequire close supervision for most tasks. Inadulthood,
they may live in community
group homes or with their families. Many
individuals in this category of mentalretardation have an associated centralnervous system disorder, visual and/orhearing impairment, severe motor andphysical disabilities, and lack of expressivelanguage skills. Because of the severity ofthe condition, most individuals requireclose supervision and provision of mostdaily care. They generally respond best toa consistent caretaker and to a low-stim-ulus environment.
Psychosocial Issues in Mental Retardation
The opinions and expectations most
people have about themselves are inﬂu-enced to a great degree by the behavior of
those around them. When minimum expec-
tations or lack of belief in individuals’ abil-ity to achieve are communicated, thechances for individuals to progress in at-taining goals are diminished. Because anumber of inaccurate and stereotypicalideas about individuals with mental retar-dation still exist, barriers to reaching opti-mal function and independence continueto be present. Although societal and em-ployer attitudes are changing slowly,there is continued need for education andintegration of individuals with mentalretardation into society and into the work-place.
Although all individuals with mental
retardation can experience stresses due tosocietal stereotypes and attitudes, individ-uals with mild retardation may confrontspeciﬁc stresses because they may appearnormal to others and consequently limi-tations may not be recognized as a disabil-Common Psychiatric Disabilities 177

ity. Lack of acceptance and devaluationcan resultant in low self-esteem and iso-lation, which in turn can lead to deviantbehaviors or acting out. In more severecases, a psychiatric disorder may be devel-oped as a means of coping.
Vocational Issues in Mental Retardation
The level of occupational functioning
for individuals with mental retardationdepends to some extent on the degree ofdisability. Because mental retardation isoften accompanied by other medical con-ditions, the physical limitations associat-ed with any medical condition must alsobe considered. Individuals with mentalretardation usually perform better in astructured environment. Many individu-als may need to be taught how to functionindependently and may need accompany-ing social skills training.
As with other disabilities, the largest bar-
rier to the individual reaching full poten-tial may be societal stereotypes and preju-dice. Although there has been heightenedeffort and interest toward increasing inte-grated employment opportunities forindividuals with mental retardation, reha-bilitation outcomes, especially for individ-uals of racial and ethnic under-representedgroups, have been less than ideal (Moore,2001). Consequently, continued equalityin service delivery and assurance and edu-cation of potential employers may be cru-cial fac
tors in successful occupational
placement.
Pervasive Developmental Disorders
Pervasive developmental disorders are
conditions in which there is stereotypical
behavior or impairment in several areas of
development, including social interaction
and verbal and nonverbal communication .
Although a number of conditions are in-cluded in this category, two of the most
common are autism and Asperger’s disorder.
Autism
Autism has shown a steady increase in
incidence since it was ﬁrst described in1943 (Merrick, Kandel, & Morad, 2004).Considered one of a family of develop-mental disabilities, autism (autistic disor-der) is a disorder of brain function that hasa broad range of behavioral consequences,including impairment in reciprocal socialinteraction and impairment in verbaland nonverbal communication
(American
Psychiatric Association, 2000). Other
symptoms may include repetitive andstereotyped mannerisms, intense preoccu-pation with a speciﬁc and restricted areaof interest, lack of spontaneous play orshared enjoyment with others, hyperactiv-ity, aggressiveness, or self-injurious behav-iors. Hypersensitivity to sensory stimulimay also be present. Often there is alsoimpairment in cognitive skills and intel-lectual capacity
(Yeung-Courchesne &
Courchesne, 1997). Many individuals with
autistic disorder have an associated diag-nosis of mental retardation. The severityof symptoms varies.
Individuals usually exhibit symptoms of
autism from birth; however, because of the
subtlety of early symptoms, the conditionmay not be diagnosed until symptoms aremore noticeable later in development. By
deﬁnition, onset occurs prior to the age of 3.
Treatment of Autism. There is no cure for
autism. Medications are usually used tocontrol speciﬁc symptoms, such as atten-tion deﬁcit, aggression, or self-abusive orother stereotypic behaviors. The most im-portant intervention for individuals withautism is remedial education directedtoward improving communication skillsand assisting with behavioral disorders.178
CHAPTER 6 P SYCHIATRIC DISABILITIES

Functional Implications of Autism. Each in-
dividual with autism has unique strengthsand interests that should be accommodat-ed to help him or her achieve the maxi-mal degree of functional capacity (Olney,2000). Although symptoms may improveas the child develops, most individualswith autism maintain a degree of depend-ence in their adult years. Those with mild
manifestations of symptoms may, with sup-
port, function independently to somedegree; however, social interaction andcommunication skills may still be proble-matic. Supported work environments andgroup homes may help individuals func-
tion with less dependence on their family.
Asperger’s Disorder
Asperger’s disorder is characterized by the
DSM-IV-TR as “severe and sustained impair –
ment in social interaction and develop-ment of restricted, repetitive patterns of
behavior, interest, and activities” (American
Psychiatric Association, 2000). Controversy
continues, however, regarding the extentto which Asperger’s disorder differs fromautism (Macintosh & Dissanayake, 2004;
Mayes, Calhoun, & Crites, 2001). Individuals
with Asperger’s disorder show core symp-toms of autism in the presence of highverbal intelligence (Frith, 2004), and somequestion whether the disorder (sometimescalled “high-functioning” autism) neces-
sarily leads to disability (Baron-Cohen, 2000).
Individuals with Asperger’s disorder
usually have no signiﬁcant language de-lays or delays in cognitive development,age-appropriate self-help skills, or adaptivebehavior (American Psychiatric Associa-tion, 2000). Diagnosis is often not madeuntil children enter school and have dif-ﬁculties with social interaction. Althoughcognitive function is normal, adults withAsperger’s disorder may continue to havedifﬁculty with social interaction. Attention Deﬁcit/Hyperactivity Disorder
Attention deﬁcit/hyperactivity disorder
(ADHD) is a condition characterized by in-attention, hyperactivity, and impulsivity,with symptoms appearing prior to the ageof 7 (American Psychiatric Association,2000). Symptoms appearing in childhoodcan persist into adulthood, causing adjust-ment problems at work as well as at homeand in other social settings. The cause ofADHD is unknown. Parents may ﬁrst obs-erve symptoms during the toddler years,when the child seems to be constantly “onthe go” and has difﬁculty sitting still dur-ing “quiet” activities. The condition ismore likely to
be diagnosed, however, dur-
ing the school years, when the need for
sustained attention in a more structuredenvironment brings symptoms undercloser attention.
Individuals with the disorder have dif-
ﬁculty paying attention or giving atten-tion to details. They may have difﬁcultywith organizational skills and may be
eas-
ily distracted. In social situations, they may
appear to not be listening to what otherssay, changing the subject of conversationfrequently. Individuals may have difﬁcul-ty sitting still, with frequent ﬁdgeting andsquirming, and difﬁculty remaining seat-ed. They may also have difﬁculty
remain-
ing quiet when appropriate, instead talk-
ing excessively, blurting out respons es at
inappropriate times, and interrupting oth-
ers frequently. Individuals with ADHDmay appear impatient, careless, and disor-ganized. As they reach adulthood, symp-toms may become less conspicuous.
Delirium and Dementia
Delirium and dementia have in com-
mon symptoms in which there is a de-crease in cognitive ability or memory froma prior level of functioning. These condi-Common Psychiatric Disabilities 179

tions are characterized by alteration ofbrain function and are often caused by anidentiﬁable organic factor.
These disorders can occur at any age and
can be secondary to another medical con-dition (e.g., heart disease, in which circula-
tion and consequently oxygen to the brain
are diminished) or can be caused by a sys-temic disease (e.g., thyroid disease), by in-jury to the brain itself (e.g., ministrokes),or by toxic substances (e.g., poisons, alco-hol, or other drugs); several causes may be
present simultaneously. Manifestations of
these conditions may affect psychological,
cognitive, or behavioral function. In previ-
ous editions of the DSM, these conditionswere classiﬁed as organic mental disorders .
The DSM-IV-TR no longer uses this termbecause it implies that other mental dis-orders may not have a biological basis.
The diagnosis of mental conditions in
this category is usually based on a detailedhistory of symptoms, ﬁndings on
physi-
cal and neurological evaluation, and clin-
ical studies and laboratory studies, as wellas neuropsychological assessments.
These conditions affect a variety of cog-
nitive abilities, such as:
• memory• orientation judgment• attention•
computational and organizational skills
There may also be associated psychomo-
tor or language impairments , sleep distur-
bances , and other behavioral manifestations .
Although some of these conditions remainstable, others are associated with pro
gres-
sive deterioration and decline of function.
Conditions classiﬁed in this DSM cate-
gory can be acute or chronic. Symptoms of
acute disorders are sudden in onset, suchas symptoms caused by generalized infec-tion or intoxication. Symptoms of chron-ic disorders generally occur more slowlyand are characterized by the deteriorationof cognitive processes over time, such assymptoms occurring with arteriosclerosisor Alzheimer’s disease.
Mental conditions in this category may
be reversible or irreversible. If the underly-ing cause of the symptoms can be correct-ed and the brain has not been perma-nently damaged, the condition is said tobe reversible . If the underlying cause can-
not be corrected or treated, or if the dam-age to the brain is permanent, the condi-tion is irreversible .
Delirium
Delirium is characterized by difﬁculty in
sustaining attention to external stimuli,difﬁculty in shifting attention to newstimuli, and difﬁculty in maintaining acoherent thought process. Symptoms ofdelirium characteristically develop over ashort period of time and include:
• a clouded state of consciousness• confusion or disorientation
Symptoms of delirium may be caused by:
• infection• the consequences of another medical
condition
• medication side effects or drug inter-
action effects
• substance intoxication or substance
withdrawal
• a combination of causes
If the cause of delirium can be identi-
ﬁed and is appropriately treated, and if nopermanent brain damage has resulted, thecondition is reversible.
Dementia
Dementia is a global deterioration of
multiple intellectual abilities, includingmemory. There are also impairments inother higher intellectual functions, suchas the ability to abstract or make judg-180
CHAPTER 6 P SYCHIATRIC DISABILITIES

ments, and personality variables. There aremany causes of dementia, some of whichare listed in Table 6–3.
Table 6–3 Potential Causes of Dementia
Alzheimer’s disease Anemia Anoxia Binswanger’s diseaseBrain tumor Chronic alcohol/drug use/abuseChronic liver diseaseChronic lung disease Communicating hydrocephalusCreutzfeld-Jakob diseaseDepression HIV infection Huntington’s diseaseInfections of the central nervous system Metabolic disordersMulticerebral infarctsMultiple demyelinating lesions Parkinson’s diseasePick’s diseaseSubdural hematoma SyphilisSystemic lupus erythematosusThyroid disorders UremiaVitamin B12 deﬁciency
Table 6–4 Potential Reversible Causes of
Dementia
Thyroid disorder Anemia Nutritional deﬁciencies Depression
Some types of dementia are reversible,
and some are not. Some potentially reversi-
ble dementias are listed in Table 6–4.Dementias such as those in Alzheimer’sdisease, multi-infarct dementia, or
arterio-
sclerosis are not reversible. Some con ditions
responsible for irreversible dementia are
described below.
Alzheimer’s Disease
Alzheimer’s disease is a progressive,
degenerative type of dementia. Onset isgenerally insidious, with gradual deterio-ration of cognitive function, eventuallyresulting in death. Alzheimer’s disease ac-counts for 50 to 75 percent of all cases ofdementia (Kawas, 2003). Although it hascommonly been thought of as a conditionthat occurs in older age groups, it mayoccur as early as middle life.
Although there are identiﬁable, structur-
al changes of the brain characteristic ofAlzheimer’s disease, there is currently nodeﬁnitive way to make the diagnosis ex-cept by direct examination of the brain it-self at autopsy. Diagnosis is based on docu-mentation of memory impairment,
thor-
ough cognitive testing, a detailed per son-
al and social history, a description of theprogression of symptoms, drug evalua-tion, and elimination of other causes ofsymptoms through laboratory, physical,and neurological examinations.
The progression of the condition and
the severity of symptoms at different stages
vary from individual to individual. Al-
though several drugs to help symptoms are
currently on the market, there is no curefor the disease. Treatment is directed tohelping individuals maintain their gener-al health, well-being, and functional capa-city as long as possible and to supportingthe family responsible for their care.
Multi-Infarct Dementia
Multi-infarct dementia refers to condi-
tions in which deﬁcits in cognitive func-Common Psychiatric Disabilities 181

tion result from small strokes in variouslocations of the brain. Areas of damagecan be identiﬁed through a computedtomography scan or magnetic resonanceimaging. Once permanent damage to thebrain occurs, functional loss of affectedareas of the brain is not reversible. Treat-ment is directed to controlling the under-lying condition responsible for the smallstrokes so as to prevent further damagefrom occurring.
Dementia Due to Other Causes
Arteriosclerosis can contribute to de-
mentia when vessels supplying blood tothe brain become narrowed or occluded,diminishing blood ﬂow and subsequentoxygen to the brain. Larger vessels, suchas the carotid arteries in the neck, areoften affected. Arteriosclerosis is a chron-ic condition (see Chapter 11). Conse-quently, treatment of dementia caused byarteriosclerosis is directed toward control-ling the underlying disease.
Dementia due to human immunodeﬁ-
ciency virus (HIV) disease is experiencedby some individuals with HIV (see
Chapter
8). In this type of dementia, there is
destruction of brain tissue, result ing in
symptoms of forgetfulness, difﬁcul ty with
concentration and problem solving, andgeneral slowness. There may also bebehavioral symptoms of apathy and socialwithdrawal, as well as motor symp
toms
such as tremor or difﬁculty walking.
Dementia due to brain trauma from a
single injury is not progressive, but dam-age to the brain and consequent associat-ed symptoms are permanent (see Chap-ter 2). Individuals who are exposed torepeated head trauma may have increasedsymptoms as additional trauma occurs.The degree and severity of symptoms willdepend on the extent and location ofinjury in the brain. Symptoms may rangefrom severe cognitive, motor, and senso-ry deﬁcits to mild concentration andmemory difﬁculties.
Schizophrenia
Schizophrenia is a chronic, lifelong
mental condition characterized by distor-tion of reality and disturbances ofthought, speech, and behavior. Symptomsof the condition cause impairment inwork or education, interpersonal relations,and self-care.
Symptoms are categorized as either posi –
tive or negative. Positive symptoms refer to
what has been added to the individual’sstate because of the condition; theyinclude symptoms such as distortions orexaggerations of thought, language, orbehavior. Negative symptoms refer to what
has been diminished for the individual,such as social ability, the ability to expe-rience pleasure ( anhedonia ), or move-
ment ability.
Speciﬁc symptoms of schizophrenia
include:
• psychotic symptoms, including delu-
sions and/or hallucinations
• disorders of thought• ﬂattening of affect• disorganized speech and/or behavior
(American Psychiatric Association, 2000)
No speciﬁc cause of schizophrenia has
been found, but it appears that multiplegenetic and environmental factors con-tribute to disturbances in brain function(Tsuang, 2000), and there is some evi-dence of structural or chemical distur-bances in the brains of individuals withschizophrenia (Freedman, 2003).
The ﬁrst episode of schizophrenia occurs
most commonly in adolescence or youngadulthood. The active form of
schizophre-
nia is characterized by the pres ence of psy-
chosis (loss of contact with reality). Prior182 CHAPTER 6 P SYCHIATRIC DISABILITIES

to the appearance of psychosis, individu-als’ daily level of functioning has usuallybegun to deteriorate over several months.Decline in function may be marked by dif-ﬁculty in concentrating or in expressingideas logically.
Individuals with schizophrenia may de-
monstrate emotional responses inappro-
priate to the situation, or they may displaygeneral apathy and indifference. They may
also experience delusions (false beliefs),
such as believing that their thoughts arebeing controlled from outside sources.Delusions may also include ideas of refer-ence in which personal signiﬁcance isattached to events that are unrelated, suchas believing that a presidential address ontelevision contains special coded messagesdirected to them personally.
Persons with schizophrenia may also ex-
perience hallucinations (sensory experi-
ences even though there are no stimulifrom the environment); for example,they may hear voices, see visions, smellodors, and feel sensations even thoughthere are no sources for these sensations.Individuals may experience loosening of
associations , in which there is no logical
progression of thought and rapid shiftingfrom one unrelated idea to the other.There may be poverty of speech , in which
words spoken convey little meaning.Individuals may have ﬂat affect , showing
little emotional responsiveness. They maywithdraw from involvement with the outside
world and exhibit little motivation , having
difﬁculty with self-initiated activity and
decision making . Grooming and hygiene
are also often neglected, and psychomo-tor activity may be slowed.
Subtypes of Schizophrenia
Five subtypes of schizophrenia are de-
scribed in the DSM-IV-TR (American Psy-chiatric Association, 2000):• Paranoid type• Disorganized type• Catatonic type• Undifferentiated type• Residual type
Each subtype shares common symp-
toms of schizophrenia but is differentiat-ed by speciﬁc symptoms. The disorgan-
ized type is characterized by incoherence
of speech, loosening of associations, gross-ly disorganized behavior, and ﬂat or inap-propriate affect. The catatonic type of
schizophrenia includes psychomotor be-havior that is either agitated or so retard-ed that the individual appears to be in astupor. The paranoid type of schizophrenia
is characterized by persecutory or grandi-ose delusions that are often sup
ported by
hallucinations. Individuals with the undif-
ferentiated type have prominent psychotic
symptoms, but the symptoms do not fallinto any speciﬁc category of schizophre-nia. In the residual type of schizophrenia,
individuals have experienced at least one
schizophrenic episode in the past but show
no current prominent psychotic symptoms,
although some residual signs may remain.
Functional Issues in Schizophrenia
The acute or active phase of schizophre-
nia severely impairs personal and socialfunctioning. During this phase, individu-als require supervision and direction in or-der to meet basic needs and to preventself-injury. Depending on individual cir-cumstances and the degree of availablesupport, many individuals are able tofunction independently and obtain em-ployment after the psychosis has beenresolved. The degree of independent func-tion possible depends on the success ofthe chemotherapeutic management of thedisorder, the extent of the individuals’insight into the disorder, and the extentCommon Psychiatric Disabilities 183

to which they continue the treatment pro-tocol. Some individuals need continuedassistance because of repeated exacerba-tion of symptoms, residual symptoms, orimpairment.
Treatment of Schizophrenia
There is currently no cure for schizo-
phrenia. Long-term antipsychotic therapyis the cornerstone of management ofschizophrenia (Ray, Daugherty, & Meador,2003). Treatment is directed toward reduc-ing and/or controlling symptoms throughantipsychotic medications, which reducethe psychotic symptoms and help individ-uals function more effectively and appro-priately. The type of medication and thedose are individually determined. Medica-tions are usually needed throughout life.Individuals taking antipsychotic medica-tions should be carefully monitored todetermine the effectiveness of the medica-tion in controlling symptoms and to iden-tify any side effects or problems.
Although antipsychotic drugs help re-
duce the risk of future psychotic episodesand help individuals function independ-ently, they are not a guarantee against re-lapse. Moreover, the medications used totreat schizophrenia are not without poten-tial side effects. Individuals may experi-ence restlessness, decreased energy, weightgain, muscle spasms or tremors, drymouth, difﬁculty with urination, or con-stipation. Individuals with schizophreniawho experience side effects, who fear thatside effects may occur, or who deny theirneed for medication may discontinue themedication on their own. However,abrupt discontinuation of antipsychoticmedication may not only be potentiallydangerous, but also can cause relapse. In-dividuals expressing concerns about theirmedication should be referred to theirphysician for advice and monitoring.In addition to medication, individuals
are treated with a variety of psychosocialtreatments to improve their functioning.Counseling and individual and/or grouptherapy can help them understand andaccept their condition as well as to buildself-esteem. Case management, behavioral
interventions, social skills training, familygroups, and support groups are other inter-ventions that have been used successfully.
Psychosocial Issues in Schizophrenia
The severity of the symptoms and the
chronicity of schizophrenia have a profound
impact on individuals and their families(Rhoades, 2000). Individuals and familiescan experience social stigma and isolation,disruption of activities of daily life, inter-ruption of future goals, ﬁnancial burden,and other stressors that have effects onhealth and well-being.
Substance dependence and abuse are
also a risk for individuals with schizophre-nia and are associated with poor outcome
(Swofford, Scheller-Gilkey, Miller, Woolwine,
& Mance, 2000). Other risks include suicide
attempts and homelessness. Individual and
family therapy can help individuals andtheir family develop the resources neces-
sary to cope with a chronic lifelong condi –
tion and can also facilitate communication
and enhance problem solving, increasing the
chances of a positive outcome. Al though
medical treatment remains the key in help-ing individuals with schizophrenia achieve
their maximum functional capa city, psy-
chosocial interventions are the key to help-ing individuals and families achieve accept-
ance and ultimate successful outcomes.
Vocational Issues in Schizophrenia
Because individuals with schizophrenia
generally have their ﬁrst symptoms inadolescence or young adulthood when job184
CHAPTER 6 P SYCHIATRIC DISABILITIES

and career choices and skill building are
major tasks, individuals may have limited
work skills. Likewise, they may also havedifﬁculty with social skills. They may need
extensive job training and training in prob –
lem solving, money management, the use
of public transportation, and social skills. In –
dividuals with schizophrenia may have dif-ﬁ
culty coping with stress. Consequently,
the amount of physical and emotional stress
in the workplace and individuals’ abilityto cope with stress should be considered.
Mood Disorders
Mood disorders consist of conditions in
which the characteristic symptom is dis tur-
bance in mood. Symptoms of mood disor-
ders usually occur when individuals are intheir twenties; however, depressive disor-ders may be experienced as early as infan-cy. Hospitalization is frequently necessaryduring the acute phase of mood disordersbecause of the severity of the disturbancethat the disorder creates in interpersonaland/or occupational functioning. Distur-bances in mood can be subdivided intodepressive disorders and bipolar disorders .
Major Depressive Disorder
Major depression is deﬁned by de-
pressed mood or loss of interest in nearlyall activities (or both for at least 2 weeks)that is accompanied by three or more ofthe following symptoms:
• insomnia or hypersomnia (sleeping
too much)
• feelings of worthlessness or excessive
guilt
• fatigue or loss of energy• diminished ability to think or concen-
trate
•
substantial change in appetite or weight
• psychomotor agitation or retardation• recurrent thoughts of death or suicide
(American Psychiatric Association, 2004)
Depression can be an enormous individ-
ual and societal burden in terms of eco-nomic cost, disability days, and pervasiveeffects on physical, mental, and socialwell-being (Kroenke, 2001). Not onlydoes it exist as a primary disability, but italso has the potential to coexist with anychronic illness or disability (Bishop &Sweet, 2000). It is frequently underdiag-nosed because symptoms can be confusedwith symptoms of other medical condi-tions (Whooley & Simon, 2000).
Individuals with a major depressive epi-
sode experience feelings of hopelessnessand discouragement, loss of interest inactivities previously found pleasurable,decreased energy, and difficulty withmemory. They may also express feelingsof worthlessness or guilt and have im-paired cognitive functions, expressingthe inability to concentrate or to make de-cisions. Other symptoms, such as sleepand appetite disturbances (too much ortoo little sleep; weight gain or weight loss),are called vegetative signs .
The degree of impairment due to major
depression varies, although social andoccupational activities are usually affect-ed to some degree. Chronic depressioncauses marked impairment in psychosoci-al function and work performance (Kelleret al., 2000; Scott, 2000). With severe de-pression, incapacitation can be so greatthat individuals are unable to attend totheir own daily needs, such as basichygiene and nutritional needs.
Bipolar Disorders
The diagnostic category of bipolar dis-
order is broken down into several subcat-egories, including bipolar I disorder and
bipolar II disorder .Common Psychiatric Disabilities 185

Bipolar I Disorder
Bipolar I disorders are characterized by
the occurrence of at least one manic epi-
sode or mixed episode . During manic epi-
sodes, mood becomes distinctly elevatedand behavior hyperactive. Individuals ina manic episode appear ﬂamboyant andoverly enthusiastic, often engaging inexcessive activity and needing little sleep.Speech becomes rapid, loud, and difﬁcultto follow because of rapid changes fromone unrelated topic to another. A mixed
episode is characterized by symptoms
involving rapidly changing moods alter-nating between elation and sadness.
Manic episodes impair social and occu-
pational functioning considerably. Duringa manic episode, individuals may be eas-ily distracted. Their attention shifts rapid-ly from one activity to another unrelatedactivity with little provocation. They mayhave grandiose delusions in which theybelieve that they have special
skills, knowl-
edge, or relationships. Halluci nations may
occur during a manic episode and oftenrelate to individuals’
mood or delusions.
Poor judgment during the manic phase
can lead to catastrophic ﬁnancial losses orillegal activities.
Bipolar II Disorders
Bipolar II disorders are characterized by
the occurrence of at least one major de-pressive episode and at least one hypoman-
ic episode . The presence of the hypomanic
episode distinguishes bipolar II disordersfrom major depressive disorders
(American
Psychiatric Association, 2000). As already
described, a major depressive episode is char –
acterized by loss of interest in activities,
sadness, and depressed mood. A hypoman-ic
episode is characterized by elevated or
irritable mood over a period of time that
is not quite as severe as a manic state. If in di-viduals experience a manic or mixed epi-
sode, they are then categorized as havinga bipolar I rather than bipolar II disorder.
Dysthymia and Cyclothymia
Dysthymia is a mood disorder charac-
terized by symptoms similar to those expe-rienced in major depression, but to a lesserdegree. Although symptoms are not sosevere, the chronic nature of the conditionmay impair social and occupational func-tioning. The essential distinction betweenmajor depressive disorder and dysthymiais the severity and duration of the symp-toms. Major depression generally has a
more acute onset, whereas individuals with
dysthymia may be in a depressed moodmost of the time for months or years.
Cyclothymia is a mood disorder char-
acterized by symptoms similar to those ofbipolar disorders, with both hypomanicsymptoms and depressive symptoms. Be-cause symptoms are usually milder, cyclo-thymia causes less impairment in functionthan does a bipolar disorder. The distinc-tion between bipolar disorders and cyclo-thymia is not clearly demarcated, and thediagnosis often depends on the judgmentof the evaluator. Because the condition is
chronic, individuals with cyclothymia can
experience symptoms for months or years.
Anxiety Disorders
There are several different types of anxi-
ety disorders. Their common features in-clude not only anxiety but also increased
arousal and avoidance of situations that the
individual perceives as anxiety provoking.
Panic Disorders
Panic disorders are types of anxiety dis-
orders in which individuals experiencefeelings of intense fear or discomfort; theyare characterized by panic attacks , episodes186
CHAPTER 6 P SYCHIATRIC DISABILITIES

in which the individual has feelings ofintense anxiety or terror, accompanied bya sense of impending doom
(American
Psychiatric Association, 2000). During a
panic attack, individuals experience short-ness of breath, increased heart rate andpalpitations, sweating, and, at times, nau-sea or other physical discomfort. Panicattacks are not triggered by a certain eventand, at least initially, are unpredictable.Attacks usually last from a
few minutes to
a few hours. In themselves, they may be
only mildly debilitating.
Panic disorder is distinguished from gen-
eralized anxiety in that individuals withpanic disorders become preoccupied withthe physical symptoms associated with apanic attack (Mahoney, 2000). Treatmentfocuses on amelioration of symptomsthrough medication and counseling.
Agoraphobia
Panic disorders are sometimes accompa-
nied by agoraphobia , the fear of being in
a situation or place in which it might bedifﬁcult or embarrassing to escape or inwhich there may be no help available ifthe individual experiences a panic attack.Although not all individuals who havepanic attacks experience agoraphobia,those who do may severely restrict theiractivity, hampering both social and occu-pational functioning. They may refuse toventure outside their home alone, or theymay be reluctant to travel by car, bus, orother common means of transportation.
Phobias
The term phobia refers to fear and anxi-
ety related to speciﬁc situations, persons, or
objects. Different types of phobias are cate-
gorized on the basis of the object of fear.
For example, social phobia is a condi tion
in which individuals fear situations that may
potentially result in ridicule or humiliation.Impairments resulting from phobias may
vary from mild to severe. A phobia maybe more of a nuisance than a disability. Onthe other hand, a phobia may be
so dis-
abling that individuals are unable to func-
tion effectively in their day-to-day activ-ities if the phobia causes them to avoidparticular objects or situations or causessuch anxiety that they are unable or un-willing to engage in necessary activities.
Obsessive-Compulsive Disorder
Obsessive-compulsive disorder is a chron-
ic disorder that can cause signiﬁcant dis-ability if not treated, with symptomsfollowing a waxing and waning course(Maj, Sartorius, Okasha, & Zohar, 2002).Individuals with an obsessive-compulsivedisorder have recurrent obsessions
(per-
sistent thoughts) or compulsions (persis-
tent actions) that they are unable to con trol.
For instance, they may have recur-rent
thoughts of the death of a loved one,
or they may have an irresistible urge toperform repetitively some behavior thatseems purposeless, such as turning a lighton and off three times before retiring forthe night. Attempts by individuals to ig-nore the compulsions only increase anx-iety, discomfort, and distress.
Cognitive-behavioral therapy is a major
treatment for obsessive-compulsive dis or-
der (Foa, Franklin, & Moser, 2002). Medi-
cation is often used in combination withcognitive-behavioral therapy, especiallyfor individuals who are unable to functionin their job or in social situations
because
of their symptoms (Jenike, 2004).
Posttraumatic Stress Disorder
Posttraumatic stress disorder (PTSD) was
initially thought to be primarily a disor-der experienced by individuals who hadbeen in military combat; however, it isnow recognized as a condition that affectsCommon Psychiatric Disabilities 187

people in all walks of life (Khouzam &Donnelly, 2001). PTSD is an anxiety dis-order that develops after an individual has
experienced or observed a traumatic or life-
threatening event, such as violence, ﬁre,
natural disaster, or plane crash. It is one of
the few psychiatric conditions whose symp –
toms are attributed to situational causesalone (Hodges, 2003). The
symptoms of
the disorder may include per sistent recol-
lection of the event, sleep difﬁculties andrecurrent nightmares, difﬁculty in concen-trating, and a feeling of hypervigilance orincreased arousal (Khouzam & Donnelly,2001). Individuals may persistently reex-perience the event in distressing images,nightmares, or ﬂashbacks; they may avoidreminders of the event, including personsor places; or they may have hyperarousalsymptoms, such as insomnia, irritability,
impaired concentration, or hypervigilance
(Yehuda, 2002). Individuals may demon-strate little emotion or appear detachedand lose interest in previously enjoyed ac-tivities or in important close relationships.
PTSD, which may occur at any age,
causes varying degrees of impairment.Although many individuals experienceacute forms of PTSD at some time duringtheir life, most recover. When PTSD per-sists, it can be debilitating and require psy-chological and pharmacologic interven-tion (Ursano, 2002). Education and coun-seling can help individuals understand thenature of their condition and can facilitaterecovery. Cognitive therapy and anxietymanagement therapies can also be help-ful. Group therapy has been found toreduce isolation and stigma (Foa, Keane,& Friedman, 2000).
Somatoform Disorders
Somatoform disorders are conditions in
which individuals experience physicalsymptoms for which no organic cause canbe found. Symptoms can cause signiﬁcantdistress and impairment in social, occupa-tional, and interpersonal functioning(American Psychiatric Association, 2000).
There are a number of somatoform dis-
orders, several of which are discussed below.
Somatization Disorder
Somatization disorder is a type of soma-
toform disorder that is characterized byrecurrent, multiple physical complaintsfor which a medical cause cannot befound. Physical symptoms can be so dis-tressing that they impair social or occupa-tional function. Because physical symp-toms are often similar to symptoms of avariety of medical conditions, individualsmay receive medical treatment for theirsymptoms even though no organic causecan be found.
Individuals with somatization disorder
do not consciously produce the symptomsbut truly experience them, even thoughthere is no organic cause of symptomsreadily identiﬁable.
Conversion Disorder
Another type of somatoform disorder is
conversion disorder, in which individuals
lose a physical function, often related to aneurological function (e.g., paralysis, blind-
ness, or numbness of a body part). Symp-toms do not typically follow a pattern thatwould correspond to a speciﬁc disease orinjury. Again, the individual does notintentionally produce the symptoms.
Hypochondriasis
Hypochondriasis, another type of som-
atoform disorder, is characterized by pre-occupation with physical illness. Indi-viduals with this condition may fear orbelieve they have a serious physical illness188
CHAPTER 6 P SYCHIATRIC DISABILITIES

or perceive the symptoms of a coexistingdisease or condition in an exaggeratedway. For example, they may perceive acough associated with a common cold asa sign of tuberculosis or lung cancer.
Pain Disorder
Pain disorder is a preoccupation with
pain that is severe enough to causeimpairment in function at home, school,or work, although no organic cause can befound to explain the pain symptom.Individuals with pain disorder do not con-sciously produce the symptoms of a painand actually experience the pain report-ed. This disorder can be extremely inca-pacitating, often severely limiting socialand work activities.
Factitious Disorders
Although not severely disabling, a vari-
ety of other types of mental disorders mayinterfere with effective functioning. Facti-tious disorders are conditions in which in-dividuals voluntarily produce psychological
or physical symptoms, feigning illness be-cause of a seemingly compulsive need toassume the sick role (American PsychiatricAssociation, 2000). A factitious disorderdiffers from malingering (in which indi-
viduals also produce symptoms intention-ally), in that the goal of a malingerer isusually obvious, such as a desire to receivean insurance settlement or to collect dis-ability payments.
Dissociative Disorders
Conditions in which individuals expe-
rience an alteration in memory, conscious-ness, or identity for no organic reason arecalled dissociative disorders . Dissociative
fugue is a condition in which individuals
leave their environment and assume anew identity without being able to recalltheir previous identity. Dissociative amne-
siais the inability to recall events that
occurred within a certain period of timeor the inability to recall information re-garding one’s own identity. Dissociative
identity disorder , formerly known as multi-
ple personality disorder , is a condition in
which at least two personalities exist with-in the same individual and control theindividual’s behavior.
Personality Disorders
Everyone has personality traits or char-
acteristics. If these traits are maladaptive,they can interfere with the ability to func-tion, especially during times of crisis.Personality disorders describe disorders
characterized by inﬂexible or maladaptivebehaviors that have usually lasted a longtime and that impair interpersonal oroccupational functioning or cause subjec-tive distress (American Psychiatric Asso-ciation, 2000).
Individuals with personality disorders
may have no insight into the role that
their own behavior plays in creating prob –
lems within their environment. They may
rationalize their actions, blaming othersfor their situation or misfortune withoutexamining their own responsibility for thesituation at hand.
There are many types of personality dis-
orders (e.g., paranoid, antisocial, and bor-derline), which cause varying degrees ofimpairment. When a personality disorderexists in combination with other mentaldisorders, the prognosis is more
guarded,
and treatment and management of the
personality disorder are more difﬁcult. Attimes, these individuals may not have afull-blown personality disorder but rathermaladaptive personality traits that mayinterfere with the treatment or diagnosisof the concomitant disorder.Common Psychiatric Disabilities 189

DIAGNOSTIC PROCEDURES INPSYCHIATRIC DISABILITY
The diagnosis of mental conditions is
often an art as well as a science. It requiresskill and experience on the part of thoseevaluating individuals’ symptoms
and
interpreting the results of the various tests
designed to measure psychological or intel-
lectual function. Many professionals maybe involved in testing and evaluation; psy-
chiatrists and clinical psychologists are fre-
quently involved in the diagnosis ofmental disorders. Diagnosis is usuallybased on information from a variety of dif-ferent sources.
Uses of DiagnosticPsychological Testing
Systematic samples of certain types of
verbal, perceptual, intellectual, and motorbehavior under standardized conditionscan be obtained through psycho
logical
testing. Psychological tests may be used to
evaluate intelligence, personality, orbehavior.
The results of psychological tests pro-
vide partial information needed for the ac-curate diagnosis of a mental condition. Nosingle test is adequate to offer a deﬁnitivediagnosis in all situations. Often, becausemental disorders affect a variety of func-
tions, several psychological tests thatmeas
ure different functions may be nec-
essary.
Intelligence Tests
The term intelligence is difﬁcult to deﬁne.
Theoretically, intelligence consists of a num-
ber of skills and abilities, some of whichcannot be measured. Intelligence is a com-bination of individuals’ own unique men-tal structure and processes along withcultural and educational experiences.Psychological science has developed a
number of tests to deﬁne intelligenceoperationally for a variety of capacities.The most commonly used intelligencetests are the Wechsler Intelligence Scale forChildren-Revised, the Wechsler Preschooland Primary Scale of Intelligence, theStanford-Binet, and the Wechsler AdultIntelligence Scale-Revised.
The limitations of intelligence testing
originate from:
• the difﬁculty of tapping all aspects of
intellectual ability
•
the individual’s ability to take the test
• the degree to which the test measures
aptitude rather than prior learningand experience
• the impact of cultural variation on
test results
One way of classifying levels of intelli-
gence is through a numerical value known
as the IQ. There is considerable individualvariability in abilities, however, and resultsof intelligence tests, like results of otherforms of psychological tests, must be eval-uated within the context of the individ-ual’s culture and environment. Muchintelligence testing involves samplingindividuals’ intellectual capacity in a vari-ety of different spheres. Many tests focuson cognitive processes, including problemsolving, adaptive thinking, and otheraspects of performance. Tests alone shouldnot determine a deﬁnitive diagnosis.
Mental Status Examination andAssessment Through Interviews
The structured interview is one way in
which the mental functioning of individ-
uals with a suspected mental disorder may
be assessed during the initial evaluation.Information obtained in this way may aidin determining the diagnosis, as well as inmaking plans for future treatment. 190
CHAPTER 6 P SYCHIATRIC DISABILITIES

Structured interviews provide informa-
tion regarding individuals’ orientation,form and content of thought, speech,affect, and degree of insight. Observationsmade during the interview of individuals’general appearance, behavior, and emo-tional state are also relevant.
The mental status examination is a spe-
ciﬁc type of structured interview used asa screening instrument in assessing intel-lectual impairment. Such an examinationmay be used to detect dementia or im-paired intellectual function, as well as todetermine the severity of the impairment.There are several mental status exam-inations of varying lengths. Althoughsome mental status instruments are partof other instruments that measure func-tional status, a number of short screeninginstruments have been devised especiallyfor the purpose of evaluating mental sta-tus. One widely used mental status test isthe Short Portable Mental Status Question-
naire , which is used to assess orientation,
personal history, remote memory, and cal-culation. Another short mental status ex-amination is the Mini-Mental State Exami-
nation , which is used to assess orientation,
memory, and attention, as well as the abil-ity to write, name objects, copy a design,and follow verbal and written commands.
Personality Assessment
Personality may be assessed by either
objective or projective means. Objective per-
sonality assessment instruments are struc-tured, standardized tests for which clearand concise criteria have been established.These tests have undergone research andscientiﬁc scrutiny to establish their relia-bility and validity. Although numerousobjective personality tests are available, one
of the most commonly used is theMinnesota Multiphasic Personality Inventory(MMPI ). The MMPI has a number of clin-ical scales that can be useful in the diag-nosis of a variety of mental disorders,ranging from schizophrenia to depression,social introversion, and substance abuse.
Projective personality tests , such as the
Rorschach Inkblot Test and the Thematic
Apperception Test , also have criteria on
which interpretations are based, but theyare generally more subjective in nature.Projective testing usually consists of ask-
ing individuals to describe vague and am-
biguous pictures. There are no right orwrong answers. The assumption is that theway in which individuals interpret the pic-tures is a reﬂection of their personality.
Projective tests may be more time con-
suming than are objective tests, and pro-fessionals who administer them requirespecial training. As with all other clinicaldata, the results of personality assessmenttests are only part of the total informationneeded for an accurate diagnosis of a par-ticular mental disorder. No matter whattype of test is used, the accuracy of the re-sults is dependent on individuals’ honestyand care in answering test questions. Ifindividuals answer questions in a social-ly desirable way rather than as an expres-sion of their true feelings, test results canbe invalid.
Neuropsychological Testing
Standardized neuropsychological test
batteries may be used to assess major func-tional areas of the brain. These tests makeit possible to assess a variety of cognitive,perceptual, and motor skills.
Traditionally, neuropsychological test-
ing has been used to identify or localizebrain damage that has behavioral conse-
quences; however, with newer technolog –
ical advances such as computed tomo-graphy and magnetic resonance imaging,this function is now not widely promot-ed. Neuropsychological tests have becomeDiagnostic Procedures in Psychiatric Disability 191

increasingly popular to rule out and/ormonitor the progression of symptoms ofmental disorders that have an identiﬁedorganic basis. Because individual perform-ance on neuropsychological tests changeswith brain function, test results provide abaseline against which future impair-ment of brain function can be measuredand also provide information that can beincorporated into the diagnosis.
A variety of comprehensive standard
neuropsychological test batteries are avail-able for adults; two of the more widely rec-ognized tests are the Halstead-Reitan Bat-
teryand the Luria-Nebraska Neuropsycho-
logical Battery .
Behavioral Assessment
Some methods of assessing mental func –
tion involve direct, systematic observation
of individuals’ behavior. Trained obser-vers, family members, or even individualsthemselves may monitor and record indi-viduals’ behavior. Observation and meas-urement of behavior may take place inindividuals’ own environment or in a
con-
trolled environment. Behavioral assess ment
methods are being applied to an increas-ing number of conditions because theyoffer not only information that can beused in diagnosis but also a method ofmonitoring improvements in behavioronce treatment has been initiated.
GENERAL TREATMENT OFPSYCHIATRIC DISABILITY
Treatment of mental disorders is based
on a comprehensive assessment of the in-
dividual’s problems and needs. It is usu ally
a collaborative effort involving the indi-vidual, the family, and professionals froma variety of disciplines, such as psychia-
trists, psychologists , social workers , nurses ,
and rehabilitation counselors . Treatmentmay be provided in a variety of settings,depending on the individuals’ particularcondition and speciﬁc needs.
Levels of treatment range from the
least
restrictive , such as that provided in an out-
patient setting, to the most restrictive , such
as that provided in an institutional set-ting. Levels of treatment in between in-clude intensive outpatient treatment,residential care, and halfway houses.
Acute episodes of mental disorders may
initially be treated by attempts to alleviatesymptoms. Ongoing treatment is directedtoward preventing recurrence of symp-toms and/or helping individuals attainmaximal functional capacity. Many men-tal conditions require ongoing treatmentor periodic evaluations of the effec
tiveness
of the treatment prescribed. Some mental
conditions, like many physical conditions,require daily medication to control symp-toms and have periods of remission andexacerbation. In many instances, individ-uals’ willingness and ability to adhere tothe prescribed treatment can determinethe success of treatment. A variety of treat-ment modalities, including both nonphar-macologic and pharmacologic methods,may be used in the treatment of mentalillness. More intensive levels of care mayinclude, in addition to psychotherapy andpharmacologic treatment, occupational
therapy , artand music therapy , or recreation-
al therapy . Often different types of treat-
ment are used simultaneously.
Psychiatric Rehabilitation
The purpose of psychiatric rehabilita-
tion is to help individuals with psychia-tric disabilities increase their functional
capacity so they can be successful and satis –
ﬁed in the environment of their choicewith the least amount of ongoing profes-sional intervention (Anthony, Cohen,Farkas, & Gagne, 2001). Psychiatric rehab-192
CHAPTER 6 P SYCHIATRIC DISABILITIES

ilitation is a multidisciplined approach toassisting individuals with chronic psychi-atric disability; it is correlated closely withtreatment and is often offered simultane-ously. The basic goals of psychiatric reha-bilitation include recovery, communityintegration, and improved quality of life(Pratt, Gill, Barrett, & Roberts, 1999). Psy-chiatric rehabilitation is communitybased, client centered, and empower-ment oriented (Leech & Holcomb, 2000).It helps individuals identify and obtainthe resources and support needed toattain their goals (Garske, 1999).
In the 1950s the clubhouse model of
psychosocial rehabilitation was created atFountain House in New York City. This
model provides integrated mental health,
employment, and peer support servicesand has become mandated as a mental
health service under managed care in sev-
eral states (Macias, Jackson, Schroeder, &Wang, 1999). In the 1990s many statesshifted to a case management approach,and by the end of the decade independ-ent employment models were being cre-ated within clubhouses to help membersachieve employment with higher wagesand more advancement potential (Reed &Merz, 2000). The effectiveness of each ofthese programs has been evaluated, andthe results are mixed (Pratt et al., 1999;Reed & Merz, 2000). Continued researchto assess the efﬁcacy of psychiatric reha-bilitation programs in integrating in
di-
viduals into both society and thework
place is essential to effective rehabil-
itation (Accordino, Porter, & Morse, 2001).
Nonpharmacologic Approaches toTreatment of Psychiatric Disability
Psychotherapy and Counseling
Psychotherapy and counseling are con-
versational approaches to treatment inwhich a close relationship between the in-
dividual and the therapist is establishedand used as a therapeutic tool. This ap-proach is used to help individuals exploreand modify their behavior in order todecrease their discomfort and/or increasetheir satisfaction and productivity.
There are numerous schools of thought
regarding psychotherapy and counsel-ing, each having a different approach andtheoretical framework. Examples of someapproaches are psychoanalysis, rational-emotive therapy, Gestalt therapy, realitytherapy, behavior therapy, and transac-tional analysis. Although therapists mayuse a speciﬁc theoretical framework pre-dominantly in their treatment approach,many use a variety of therapeutic ap-proaches, depending on which type seemsmost appropriate for a speciﬁc individual.
Psychotherapy may be conducted on an
individual basis, in a group, with a fami-ly, or between marital partners. Depend-ing on individual need, a combination oftherapies may be included in the treat-ment plan. Individual therapy is directedtoward effecting changes in individuals’behavior, and group therapy is directedtoward helping individuals develop moresatisfying modes of interaction with oth-ers. Group therapy may also be educativeif the content is ﬁxed and the goal of thegroup is to relay information, or support-ive if group members receive and givemutual support and encouragement. Fam-ily therapy is directed toward improvingfamily function as an interdependentgroup, and marital therapy focuses on the
marital relationship and the impact of both
parties’ behaviors on the relationship.
Behavioral Approaches
Behavior is a reﬂection of inner drives,
traits, or patterns of thinking, as well asenvironmental inﬂuences. A number ofGeneral Treatment of Psychiatric Disability 193

treatments designed to help individualsmodify their learned responses and learnnew patterns of more adaptive behaviorhave been used in the treatment of men-tal illness.
Several different forms of behavior ther-
apy have been derived from different the-oretical models, including the respondent
(classical ) model , the instrumental (operant )
model , the observational model , and the cog-
nitive learning model . There are countless
applications of behavioral approaches inthe treatment of mental ill
ness. Behavior
therapy may be used alone or in conjunc-
tion with other treatments.
Social-Skills Training
The purpose of social-skills training is to
identify speciﬁc social-skills deﬁcits and
the circumstances under which these
deﬁcits occur. Educational interventionsare then directed toward correcting thesedeﬁcits. Interventions usually begin withtargeting small elements of behavior andthen gradually adding other elements ofbehavior, always working toward theideal behavior. Through social-skills train-ing groups, individuals learn to make spe-ciﬁc responses to speciﬁc social situations,
as well as to recognize relevant social cues
and to determine appropriate action byusing the cues. Social-skills training may
involve speciﬁc interventions, such as role
modeling, feedback and reinforcement,
and practice, in helping individuals to per-
form speciﬁed behaviors reliably and to
generalize the behavior to other situations.
Specialized Groups
Individuals with some types of mental
illness may neglect their own needs of dai-ly living, including personal hygiene,money management, or housing needs.Special groups (e.g., activities of daily liv-ing groups) may help individuals withthese disorders learn the speciﬁc skillsneeded for day-to-day functioning.
Individuals with some types of mental
illness may require ongoing supervision,and many require a period of transitionfrom the inpatient to the outpatient set-
ting. A variety of therapeutic living arrange-
ments may be used to meet these needs,including group homes, therapeutic com-munities, and transitional living
centers.
Day programs provide a structured environ-
ment in which individuals may participatein the program during the day and returnto the community setting at night. Thegoal of day programs is to facilitate the ad-justment of these individuals to the com-munity setting, to maintain their optimallevel of functioning, and to prevent hos-pitalization.
Pharmacologic Approaches toTreatment of Psychiatric Disability
Antipsychotic Medications
Treatment of psychosis may require the
use of antipsychotic medications (see Table
6–5). Antipsychotic medications, some-times called neuroleptic medications or
major tranquilizers , do not cure psychosis
but rather control the symptoms. The ﬁrstantipsychotic drug, chlorpromazine (Thora-
zine), was developed in the 1950s. Since
that time, numerous other antipsychoticmedications have been developed. Thesedrugs are classiﬁed into different chemi-cal groups. Drugs in each group have vary-ing potency, and individual responses toany of the medications vary.
Duration of treatment with antipsychot-
ic medications is determined individual-ly and based on individuals’ life situationand condition. They may be prescribed forup to a year as a prophylactic measure af-ter psychosis is controlled. All individualsshould have their medications reviewed194
CHAPTER 6 P SYCHIATRIC DISABILITIES

annually by a psychiatrist to evaluate thepossibility of gradual discontinuation.
Table 6–5 Common Antipsychotic Agents
Trade Name Generic Name
Clozaril ClozapineCompazine ProchlorperazineHaldol HaloperidolLoxitane Loxapine Mellaril ThioridazineNavane Thiothixene Prolixin FluphenazineStelazine Trioridazine
It is believed that the symptoms of psy-
chosis may be due to excessive levels ofthe neurotransmitter dopamine . Conse-
quently, it has been postulated that anti-psychotic medications reduce symptomsby blocking the action or transmission of
dopamine. Because of this blocking, how-
ever, one of the side effects of antipsychot-
ic medications may be psychomotor symp-
toms similar to those seen in Par kinson’s
disease (see Chapter 3). These are calledextrapyramidal effects , because changes
take place in the extrapyramidal tracts ofthe central nervous system. The possibleextrapyramidal effects of antipsychoticmedications include dystonia , character-
ized by severe contractions of the musclesof the jaw, neck, and eye so that the headis turned to one side and the eyes look up-ward; akinesia , characterized by decreased
motor activity and apathy; and akathisia ,
characterized by extreme restlessness sothat the individual cannot sit still or re-main in one place for any length of time.The most severe extrapyramidal side effectof antipsychotic medications is tardive
dyskinesia , which consists of abnormal
movements of the mouth, such as chew-ing motions or thrusting movements ofthe tongue. Tardive dyskinesia often indi-cates irreversible damage to the brain.
Antiparkinsonian medications , such as
benztropine (Cogentin ) and trihexphenidyl
(Artane ), are often prescribed along with
antipsychotics to prevent extrapyramidalside effects. Tardive dyskinesia is best treat-ed through prevention, since the occur-rence of the symptom is frequently relatedto drug dosage. Individuals on antipsy-chotic medication must be care
fully moni-
tored by a physician so that the earlysymptoms of tardive dyskinesia may
be
identiﬁed and dosage of the medica tion
adjusted to avoid permanent damage.
Individuals on antipsychotic medica-
tions may also develop photosensitivity ,
which makes them more sensitive to theeffects of the sun and predisposes them tosunburn. Some medications that havepotent sedating effects may decrease alert-ness and produce drowsiness. These symp-toms usually subside within 2 weeks afterthe individual begins to take the medica-tion; if they persist, alteration in medica-tion may be necessary. Individuals mayalso experience orthostatic hypotension ,
in which their blood pressure drops whenthey move from a seated or prone positionto a standing position, resulting in dizzi-ness or lightheadedness. Individuals maycomplain of other uncomfortable side ef-
fects, such as dry mouth, after beginning anti-
psychotic medications. These symptomsgenerally subside within 2 weeks, howev-er. Men on antipsychotic medication maybecome impotent or unable to ejaculate.Reducing the dosage or changing the med-ication may alleviate this side effect. Anymedication change should always be con-ducted under the direction of a physician.
Antidepressants
Conditions in which depression is a
symptom may be treated with antidepres-General Treatment of Psychiatric Disability 195

sants . Although the exact way antidepres-
sants work has not been determined, theyare classiﬁed according to their presumedmode of action. The most widely usedantidepressants, tricyclic antidepressants , are
thought to act by blocking the uptake ofthe neurotransmitters norepinephrine and
serotonin , thus increasing
their concentra-
tion. Levels of both of these neurotransmit-
ters appear to be reduced in depression.
Monoamine oxidase (MAO ) inhibitors ,
less frequently used antidepressants, arethought to act by blocking the action ofthe enzyme monoamine oxidase, whichusually breaks down norepinephrine andserotonin, so that the concentration of theneurotransmitters increases.
The type of depression and the symp-
toms experienced, as well as other individ-
ual factors, determine the type of antide-pressant used. As with all medications,some side effects may be experienced. In-dividuals on tricyclic antide
pressants may
experience symptoms such as orthostatic
hypotension (described previously), dry
mouth , or urinary retention . A more serious
possible side effect is the development ofcardiac arrhythmia , which can result in
myocardial infarction or, in the case ofoverdose, death.
The use of MAO inhibitors has been
limited because of their potential side ef-fects; however, their use is gaining popu-larity. Individuals with chronic alcoholismor liver damage are not good candidatesfor treatment with MAO inhibitors. Inaddition, there are a number of dietaryrestrictions associated with their use, andindividuals who use these medicationsmust follow these restrictions carefully toprevent potentially serious side effects.Monoamine oxidase is essential for themetabolism of a substance called tyramine ,
which is present in a number of foods,including aged cheese, wine, beer, choco-late, coffee, and raisins. If individuals tak-ing MAO inhibitors ingest tyramine-con-taining foods, they may experience ahypertensive crisis in which there is sudden
and extreme elevation in blood pressure,which could result in stroke.
Suicide is always a possibility with indi-
viduals who are depressed. The availabil-ity of antidepressant medication thatcould be used in a suicide attempt is a riskto be considered. The risk of attemptedsuicide may be higher when the antide-pressant begins to take effect because sui-cidal impulses are still present and asindividuals’ energy returns so does theirmotivation to attempt suicide. Althoughantidepressants are an important aspect oftreatment for depressive disorders, psy-chotherapeutic modes of treatmentshould be used in combination with thepharmacologic approach.
Mood Stabilizers
Lithium is an antimanic agent used to
treat the manic symptoms in bipolar dis-order. It is an element that occurs natural-ly as a salt. Use of lithium for treatmentof mental disorders in the United Statesbegan in the 1970s, and lithium is nowwidely used in bipolar disorders, both inthe treatment of symptoms and in the pre-vention of recurring symptoms. In someinstances, lithium has been used alone orin combination with antidepressants totreat depressive disorders. Because not allindividuals respond to lithium in the sameway, lithium use is decided on an individ-ual basis.
The way in which lithium works is un-
clear. It may produce some side effects,including endocrine effects (e.g., hyperthy-
roidism), muscle weakness , or weight gain .
Other common side effects include poly-
uria (excessive urination) and polydipsia
(excessive thirst). Individuals who use lithi-
um should have regular blood tests to196 CHAPTER 6 P SYCHIATRIC DISABILITIES

measure levels of the medication in theblood and must be monitored by a physi-cian on a regular basis.
Antianxiety Medications
Formerly called minor tranquilizers, anti-
anxiety medications are generally used for
mental disorders in which anxiety is thepredominant symptom. These medica-tions are commonly classiﬁed as benzodi-
azepines (e.g., diazepam [Valium], oxaze-
pam [Serax], or lorazepam [Ativan]), bar-
biturates (e.g., phenobarbital), or antihist-
amines (e.g., hydroxyzine [Vistaril or
Atarax]). Antianxiety medications areused mainly for time-limited, short-termtreatment of anxiety. These medicationsshould not be regarded as the mainstay orsole treatment of anxiety disorders, butrather should be used in combinationwith other types of treatment, such as psy-chotherapeutic approaches. Because manyantianxiety agents also have the risk ofabuse or physical dependence, their useshould be carefully monitored. Side effectscan include drowsiness and sedation ormotor difﬁculty.
Electroconvulsive Therapy
Before psychopharmacologic prepara-
tions were readily available, electroconvul-sive therapy (shock therapy) was a majormode of treating some types of mental ill-ness. Its use has diminished with theadvent of a variety of psychotherapeuticdrugs, but some centers continue to use itfor treatment. It may be especially usefulwhen the long-term administration ofmedication is contraindicated (Fink,2000). Although electroconvulsive thera-py does not cure mental disorders, it canbring about a remission of symptoms. Itmay be used in conjunction with psy-chotherapeutic medications or alone.PSYCHOSOCIAL AND VOCATIONALISSUES IN PSYCHIATRIC DISABILITYPsychological Issues
Individuals with psychiatric disability
experience a wide range of symptoms thataffect psychological and cognitive func-tion, and their needs are multifaceted andcomplex (Kress-Shull & Leech, 2000). Al-though the beneﬁts of medication in thetreatment of psychiatric disability are sub-stantial, medication usually does not curethe condition but rather controls thesymptoms. Individuals often have residualsymptoms, deﬁcits, and impairments as aresult of their condition, and many aresubject to periodic relapses with recur-rence of symptoms.
Individuals with psychiatric disability
may be particularly vulnerable to stress
and may lack the ability to withstand pres –
sure or to cope with the normal stressesof everyday life. They may have limitedproblem-solving ability or ﬁnd it difﬁcultto engage in self-directed activity. Someindividuals may become passive, apathet-ic, or oversubmissive as a direct result ofrepeated hospitalizations or as a result ofthe condition itself.
Symptoms experienced vary with the
condition, causing varying degrees of im-
pairment. Although fear and anger are nor-
mal emotional responses, these responsesmay be acutely disproportionate to thestimuli in some psychiatric disabilities.Some individuals’ responses are
covert,
whereas others’ responses are more pro-
nounced. Some individuals manifest theircondition through patterns of behaviorrather than in emotional manifestations.Others experience subjective distress, suchas an inner sense of weakness, jealousy, oranxiety, although function in most oftheir life is minimally disturbed. Somepsychiatric disabilities are characterized byPsychosocial and Vocational Issues in Psychiatric Disability 197

disorganization of mental capacities, which
can affect individuals’ ability to function
in an unstructured environment. Disorders
of memory and perception can severelylimit independent function. Individualsmay fail to carry out age-appropriate rolefunctions and have varying degrees of de-pendence on others.
Symptoms of the psychiatric disability
may cause psychic stress and anxiety, fur-ther compounding the disabling compo-nent of the condition. Individuals’ ownanguish over their impoverished life canbe devastating. Awareness of their ownimpaired function and the impact of theircondition both on others and on theirfuture may cause considerable pain anddiscomfort. In some instances, individu-als with psychiatric disability may be re-luctant to seek appropriate help becauseof their fear of the stigma associated withpsychiatric conditions that require profes-sional help. In other instances, individu-als may not be aware of their symptomsand the effect of their
symptoms on func-
tion, which also hinders them from using
appropriate treatment.
Lifestyle Issues
The degree to which psychiatric disabil-
ity affects individuals’ lifestyle depends toa great extent on the nature of the condi-
tion. Some psychiatric disabilities so severe –
ly impair individuals’ ability to carry on the
activities of daily living that constantsu
pervision or hospitalization is necessary.
Inother instances, individuals are able to
carry on these activities, but in an alteredmanner.
At times, the treatment itself requires
lifestyle changes. Individuals may need torearrange their schedules so that they canattend therapy sessions. Some medicationsused in the treatment of psychiatric dis-ability may require special lifestyle consid-erations. For example, the use of MAO in-hibitors in the treatment of depressionrequires careful monitoring of diet. Othermedications have side effects, such asdrowsiness and sedation, that also affectdaily function.
Either the psychiatric disability or its
treatment may alter sexual function. Indi-viduals with a depressive disorder may lose
interest in sexual activity, whereas individ-
uals with a bipolar depression may have ex-
cessive sexual interests. The side effects of
some medications can alter sexual function
as well. In addition, subjective manifesta-tions of lowered self-esteem and self-con-ﬁdence may make it more difﬁcult forindividuals to form intimate relationships.
Social Issues
The impact of a psychiatric disability on
social function also depends on the natureof the condition. Individuals who
experi-
ence mania as a part of their disabil ity may
enjoy the euphoria and feel that it con-tributes to their social well-being.
Even though attitudes of society have
become more accepting of individualswith mental illness, family members maycontinue to be resistant to recognizing theproblem and pursuing appropriate treat-ment (Hall & Purdy, 2000). If, how
ever,
individuals manifest bizarre, abusive, or
socially offensive behavior, family mem bers
or others within a social group may avoidthe individual altogether, leaving him orher socially isolated.
Other psychiatric disabilities may lead
to social withdrawal. Families of individ-uals with psychiatric disability may expe-rience a variety of stresses engendered bythe condition. These stresses may becaused by their objective problems in deal-ing with the individuals and their condi-tion, as well as by more subjective
psycho-
logical distress (Hall & Purdy, 2000).198 CHAPTER 6 P SYCHIATRIC DISABILITIES

Psychiatric disabilities, especially those
in which individuals need close supervi-sion or long-term care and treatment, mayplace ﬁnancial hardships on their familybecause of medical bills, the indi
vidual’s
economic dependency, and special needs
related to household functioning. In someinstances, the demands of caregiving mayrequire family members to curtail theirsocial activities or alter their relationshipswith friends and acquaintances. The timecommitments of caregiving may lead toneglect of other family members’ needs,further disrupting the family as a unit.
Social barriers are frequently erected
against individuals with a mental disorder
and against their families. Social stigma may
be the result of fear of individuals’ behav-ior, ignorance about psychiatric disabili-ty, or feelings of inadequacy in interactingwith those who have psychiatric disabili-ty. Regardless of the cause, the results can
be a source of continuing stress for individ –
uals and their families, as well as a barri-er to social activity and interaction. Socialstigma and stereotypes can also have aneffect on the extent of the deﬁcits individ-uals experience. Deﬁcits sometimes occurnot only because of the psychiatric condi-tion, but also because of the public’s reac-tion to it (Corrigan & Calabrese, 2001).
Vocational Issues
Individuals with psychiatric disability
have a condition that limits their capaci-ty to perform certain tasks and functionsand their ability to perform certain roles(Farkas & Anthony, 2001). The ability towork depends on the type of disability, thetype of work in which they are involved,and the attitudes of those within the worksetting. Although work is important toincrease self-esteem for those with a num-ber of disabilities, it can be an especiallystrong therapeutic tool for those with apsychiatric disability (Tschopp, Bishop, &Mulvihill, 2001). The skills, aptitude,motivation, and objective symptoms ofindividuals with psychiatric disability areimportant, and their ability to endure andcope with stress and to engage in activeproblem solving also determines their abil-ity to work. Job restrictions may be relat-ed to job pressure or the ability to workwith others, regardless of the individual’slevel of skill or physical and cognitive abil-ity to perform work-related tasks.
Other considerations may relate to indi-
viduals’ treatment. It may be necessary toarrange scheduled absences so that indi-viduals can attend therapy sessions. Somemedications used in treatment may pro-duce side effects, such as drowsiness orsedation, that could adversely affect workperformances. In addition, individuals’level of adherence to the therapeutic reg-imen is especially important if failure todo so means possible relapse and recur-rence of symptoms.
Individuals’ reaction to the work en-
vironment, including noise and distrac-tions, should be taken into account, asshould their level of personal responsibil-ity and ability for self-direction and deci-sion making. Limited interpersonal andcoping skills may make it difﬁcult forsome individuals to adjust to unforeseencircumstances. Individuals’ ﬂexibility totake advantage of chance occurrences
and
their degree of ﬂexibility in the work place
must also be taken into consideration(Szymanski, 2000). Some individuals mayneed a more structured work environ-ment; in some instances, a workshop envi-ronment may be preferable. Some individ-uals’ expectations of work or of their owncapabilities may be unrealistic. Unlessthese unrealistic notions are identiﬁed anddealt with before they enter or reenter thework setting, discouragement, disappoint-ment, or even relapse may occur.Psychosocial and Vocational Issues in Psychiatric Disability 199

Individuals with signiﬁcant mental re-
tardation may need specialized trainingand other assistance in job placement, jobsite training, and long-term support. A job
coach may be utilized to provide individ-
ualized assistance and to act as an advocate .
The job coach provides speciﬁc assistanceby helping individuals ﬁll out an applica-tion, going with them for the job inter-view, and participating in travel trainingand skill training at the job site. The jobcoach gradually reduces involvement overtime as the individual adjusts to the workenvironment.
Supported employment may be successful
for a number of individuals with psychia-tric disability. In supported employment,individuals work in integrated settingswith monitoring, support, and follow-upprovided on a regular basis. Supportedemployment provides permanent jobsthat are based on individuals’ skills andabilities. This model is useful for individ-uals with intellectual disabilities and oth-er types of psychiatric disabilities.
Social skills, aptitude, and the ability to
work are not necessarily concurrent in in-dividuals with psychiatric disability. Em-ployment for each individual must beconsidered in the context of his or her par-ticular symptoms and feelings and thenature of the work environment. The rolethat social stigma plays in individuals’ per-ceptions of their own condition and theirwillingness to accept and follow up withtreatment are crucial aspects in their totalrehabilitation.
The unemployment rate for individu-
als with psychiatric disabilities continuesto be high (Fabian & Coppola, 2001; Kress-Shull, 2000). Although it is believed thatpeople with psychiatric disabilities havebeen signiﬁcantly discriminated against inthe workplace, the extent to which dis-crimination exists is difﬁcult to determinebecause of lack of relevant data (SpiritoDalgin, 2001). Continued advocacy thatincludes educating not only employersbut also individuals with psychiatric dis-ability is necessary in the ongoing processof reducing employment discrimination.
CASE STUDIESCase I
Mr. B. is a 27-year-old individual with
mild mental retardation that has beenestablished through testing. He is a third-generation individual of Chinese descent.
He has never been employed and lived with
his widowed mother until several monthsago, when she died and he moved to agroup home. Mr. B. was very protected byhis mother, and although he went to spe-cial education, he did not build relation-ships with individuals outside his closefamily. Since coming to the group home,he has become increasingly more animat-ed and social and expresses the desire tobe employed. He states he is particularlyinterested in a job like that of one of hisroommates, who is a dining room atten-dant who keeps the serving areas stocked,cleans tables, and removes dirty dishes.
Questions
1. What speciﬁc issues in assessment
would you consider when workingwith Mr. B. to establish a rehabilita-tion plan?
2. Given Mr. B.’s level of mental retar-
dation, what level of function mightyou expect him to attain?
3. What personal and/or social factors
might you consider when workingwith Mr. B. that may be a factor in hisrehabilitation potential?
4. How would you approach a potential
employer in attempting to ﬁnd aplacement opportunity for Mr. B.?200
CHAPTER 6 P SYCHIATRIC DISABILITIES

5. Given Mr. B.’s level of mental retar-
dation, how realistic is his desiredoccupational goal of becoming adining room attendant?
Case II
Ms. S. is a 37-year-old female who was
diagnosed with schizophrenia at the ageof 20 when she was hospitalized in anacute psychotic state. At the time of herdiagnosis, she had completed 2 years ofcollege, where she was studying account-ing. After hospitalization she did notreturn to college and has had a series ofpart-time jobs since that time. Her symp-toms have been fairly well controlled onmedication; however, she tells you thatshe is concerned about side effects and hasconsidered going off her medication.Since her original diagnosis, she has beenhospitalized twice, and on each occasionshe had stopped taking her medication.Over the past 17 years she has had a scat-tered work history and has held jobs as alibrary assistant and as an order clerk. Hercurrent employment is as a ﬁle clerk at aninsurance company, where she stores andretrieves information as needed andupdates ﬁles. She feels her current jobplaces considerable stress on her that attimes she has difﬁculty coping with, andshe requests assistance in ﬁnding otheremployment. She currently lives alone.She is unmarried and her family lives sev-eral hours away.
Questions
1. What factors might you consider in
helping Ms. S. develop a rehabilita-tion plan?
2. How would you handle Ms. S.’s com-
ment that she has considered goingoff her medication because of herconcern about side effects?
3. Given Ms. S.’s diagnosis, are there any
occupations that may not be suitablefor her? Why or why not?
4. Are there other support strategies that
may be helpful to Ms. S. to enhanceher overall rehabilitation potential?References 201
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DEFINING SUBSTANCE USEDISORDERS
For much of human history a wide array
of substances, including plants or plantderivatives, alcohol, nicotine, caffeine, in-halants, and tonics, have been condonedand used by different cultures for thera-peutic, ritualistic, religious, or recreation-al purposes. When society becomes am-bivalent toward the use of a substance or
determines it to be inappropriate, or when
substance use becomes uncontrolled, haz-ardous, or disruptive to individuals or toothers, it is considered to be pathologicaland in some instances becomes illegal.
Conditions related to maladaptive changes
in behavior or health that occur as a resultof the more or less regular use of a sub-stance in this way constitute substance use
disorders .
Substance use disorders reﬂect a combi-
nation of biological, psychological, social,and environmental factors and may in-volve substances that are licit, illicit , pre-
scribed , or not prescribed . The etiology and
treatment of substance use disorders entaila complex interface among all these fac-tors. No one factor explains the develop-ment of substance use disorders.
Just as all chronic illness and disability
affect physical, social, psychological, andvocational aspects of individuals’ lives, sodo substance use disorders. Like otherchronic, relapsing conditions, substanceuse disorders produce a variety of impair-ments. The implications of these disordersmust be evaluated in the context of indi-viduals’ speciﬁc situations. Substance usedisorders can occur alone or in combina-tion with one or more other physical orpsychiatric disabilities. The effects of sub-stance use combined with manifestationof another disability can cause addition-
al physical, psychological, and social com-
plications, adding to the disabling effectsof both.
SUBSTANCE ABUSE ANDDEPENDENCE
A variety of substances are included in
substance use disorders, including bothlegal and illegal substances. Substance use
disorders are classiﬁed either as abuse or
as dependence . Essential features of each are
as follows:
•Substance abuse is a maladaptive pat-
tern of substance use resulting in re-current and signiﬁcant consequencesof substance use, such as neglect ofwork or family obligations, repeated-ly driving under the inﬂuence, recur-Conditions Related to
Substance UseCHAPTER 7
205

rent disorderly conduct or legal prob-lems, or interpersonal problems relat-ed to use of the substance.
•Substance dependence refers to sub-
stance use resulting in physical or psy-chological symptoms related to sub-stance tolerance, symptoms of with-drawal, and a pattern of compulsivesubstance-taking behavior, in whichindividuals become so preoccupiedwith the substance that much of theirdaily activity revolves around obtain-ing it, despite recurrent negative con-sequences (American Psychiatric Asso-ciation, 2000).
Substances of abuse or dependence may
be taken simultaneously or sequentially.With continued substance use, individu-als experience diminished effects with thesame amount so that the amount takenmust be increased to achieve the sameeffects. This is called tolerance . The degree
of tolerance experienced varies from indi-vidual to individual and with the specif-ic substance being used.
Individuals using substances chronical-
ly may behaviorally adapt so that they areable to continue functioning at work, athome, or in social situations, even though
they are under the inﬂuence of a substance.
Although tolerance is not always an indi-cation of dependence, tolerance is a com-mon symptom in individuals with sub-stance use disorders. Furthermore, indi-viduals who develop a tolerance for onesubstance may also develop higher toler-ance for related substances. This conditionis known as cross-tolerance .
The toxic effects of large concentrations
of a substance cause physical disturbancesto occur when the amount is decreased orsuspended. As a result, individuals experi-ence physical symptoms known as with-
drawal . Symptoms of withdrawal depend
on the substance.Intoxication
Intoxication refers to a reversible syn-
drome caused by intake of a speciﬁc sub-stance and characterized by behavioral orpsychological changes related to the effectof the substance on the nervous system(American Psychiatric Association, 2000).The level of drug or alcohol intoxicationis determined by the concentration of thesubstance in the blood. The rate at whichsubstances are absorbed is dependent onthe route. Substances that are injected di-rectly into a vein have an immediate ef-fect. The rate at which substances ingestedorally, such as alcohol, are absorbed intothe bloodstream is dependent on theamount ingested, the presence or absenceof food in the stomach, and the rate ofgastric emptying. Concentration of sub-stances in the blood is also dependent onbody size. For instance, blood alcohol lev-els are proportionately less in large indi-viduals than in small individuals, eventhough they consume equal amounts ofalcohol under similar conditions.
Withdrawal
Consumption of large amounts of alco-
hol or other drugs at frequent intervals forprolonged periods creates a state of phys-ical dependence so that cessation of drugor alcohol intake or reduction in theamount consumed produces distressfuland incapacitating symptoms, known assubstance withdrawal . Symptoms experi-
enced during withdrawal vary in severity.Initial symptoms, regardless of the sub-stance, may consist of dysphoria (exagger-
ated feelings of depression and unrest),
insomnia, anxiety, irritability, nausea, agi-
tation, tachycardia (fast heartbeat), and
hypertension (high blood pressure).
Individuals with mild to moderate with-
drawal symptoms with no preexisting con -206 CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

Substance Use and Chronic Illness and Disability 207
ditions and who have social support mayhave withdrawal managed on an outpa-tient basis. It is important for health pro-fessionals treating withdrawal to know thetype of substance abused, since there aresubstantial differences in complications aswell as management of withdrawal from
different substances. Individuals with more
serious withdrawal symptoms, such as thedelirium tremens associated with alcohol(described later in the chapter) or the psy-chotic symptoms experienced with stim-ulants or opioids, or those who have co-existing psychiatric or medical conditions,usually require inpatient management ofwithdrawal (Kosten & O’Connor, 2003).
Detoxiﬁcation is the ﬁrst step in sub-
stance abuse treatment. The goal of detox-iﬁcation is to initiate abstinence, reducesymptoms of withdrawal, prevent compli-cations, and retain individuals in treat-ment (Kosten & O’Connor, 2003). Afterdetoxiﬁcation, medical treatment consistsof giving medications that act as substi-tutes for the abused substances, with thedosage gradually being tapered off.
Addiction
The American Psychiatric Association
uses the term dependence rather than
addiction ; the term addiction emphasizes
the behavioral component of a substanceabuse disorder rather than physical de-pendence (Maddux & Desmond, 2000).Addiction refers to a chronic, relapsing dis-
order in which individuals exhibit com-pulsive drug-seeking and drug-takingbehavior to induce pleasant states or torelieve stress (Camí & Farré, 2003). Thephysical and psychological craving for thedrug becomes so consuming that individ-uals expend tremendous effort and ener-gy, as well as ﬁnancial resources, to obtainit, often at the expense of the safety andwell-being of themselves as well as others. In addiction, drug-taking behavior be-
comes more than merely “wanting” or“liking” the drug. The brains of individu-als who are addicted become hypersensi-tized, which causes pathological cravingof the drug, independent of physical signs
of withdrawal (Robinson & Berridge, 2001).
Compulsive drug-seeking and drug-taking
behavior is facilitated by difﬁculties in deci-
sion making and the ability to judge theconsequences of this behavior (Robinson& Berridge, 2003).
Several factors appear to predispose
individuals to addiction. Personality traitssuch as a tendency to take risks or to seeknovelty have been found to be more prev-alent in individuals who abuse or aredependent on drugs (Helmus, Downey,Arfken, Henderson, & Schuster, 2001).Individuals with psychiatric disorders,especially schizophrenia, bipolar disorder,and depression, have an increased risk of
abuse. A dual diagnosis also has been shown
to have more unfavorable implications fortreatment and outcome (Kavanagh,McGrath, Saunders, Dore, & Clark, 2002).
SUBSTANCE USE AND CHRONICILLNESS AND DISABILITY
Individuals with chronic illness and dis-
ability can also manifest substance use dis-orders. Substance abuse can be a factor inthe acquisition of a chronic illness ordisability as well as an adjustment to it.Whether substance use was a precursor ofthe acquired chronic illness or disabilityor a coping mechanism after it, a diagno-
sis of two disabling conditions makes treat-
ment of both conditions more complex.
A number of factors may place individ-
uals with chronic illness or disability athigher risk for substance use disorders:
• Medical factors, such as easy access to
prescription medication to alleviatesymptoms such as chronic pain, mak-

ing it easier to use the medicationexcessively; or unnecessary or unwar-ranted prescription of medication forsymptoms that could have been treat-ed by alternative means
• Psychological factors such as depres-
sion, boredom, or frustration, so thatsubstances are used as a means of es-cape from reality
• Social factors such as oppression and
alienation, so that substances are used
recreationally in an attempt to gainacceptance and normalization (Greer,Roberts, & Jenkins, 1990; Watson,Franklin, Ingram, & Eilenberg, 1998)
The coexistence of a substance use dis-
order with other chronic illness or disabil-ity can exacerbate and accentuate symp-toms as well as increase individuals’ vul-nerability to medical complications, lead-ing to the acquisition of additionaldisability. Although substance abuse cancoexist with any disability, comorbiditybetween substance abuse and mental ill-ness ( dual diagnosis ) is very common
(Allen Doyle-Pita, 2001; Volkow, 2001).Whether a substance use disorder is theprimary or secondary disability, appropri-ate intervention and treatment are neces-sary to enable individuals to reach theirfull rehabilitation potential.
PHYSICAL EFFECTS OF ALCOHOLABUSE AND DEPENDENCE
The effect of alcohol on the body, like
the effect of any drug, depends on theinteraction between properties of the spe-ciﬁc pharmacologic agent and the charac-teristics of a speciﬁc individual. There isevidence to suggest that women tend tobe more sensitive to the effects of alcoholand more susceptible to adverse effects ofexcessive alcohol consumption than men(Blume, Counts, & Turnbull, 1992; Harley,1995; Kandall, 1996; Scott-Lennox, Rose,Bohlig, & Lennox, 2000). The medicalcomplications of alcohol abuse and/ordependence result from the direct effectsof alcohol (ethanol) on body tissues andfrom adaptive responses of the body toexcessive exposure to alcohol.
Initially alcohol acts as a stimulant
because it suppresses the central nervoussystem’s inhibitory systems. As alcohollevels increase in the body, however, it hasa sedative effect, causing motor incoordi-nation, ataxia, and impaired psychomotorperformance (Holdstock & deWit, 1998).Alcohol is rapidly absorbed into thebloodstream from the stomach and intes-tines and is rapidly metabolized, makingit a fast-acting drug. Because it diffusesquickly into the water content of all bodytissues, the blood concentration of alco-hol is an accurate reﬂection of the concen-tration of alcohol in other body tissues.
Some alcohol is eliminated through the
kidneys and lungs, but the liver metabo-lizes most. Although a moderate dose ofalcohol normally clears from the blood in
approximately 1 hour, only a ﬁxed amount
of alcohol can be metabolized at a time.When the rate of alcohol consumptionexceeds the body’s ability to metabolize it,alcohol accumulates in the bloodstream,elevating the blood alcohol concentration.
Alcohol has a direct pharmacologic ef-
fect on the nervous system. It is a power-ful central nervous system depressant. Theintoxicating effects of alcohol correlateroughly with the alcohol concentrationsin the blood, which, in turn, reﬂect thealcohol concentration in the brain. At lowlevels of intoxication (0.05 percent), alco-hol may produce a sense of relaxation andwell-being. As the concentration of alco-hol increases (0.11 to 0.20 percent), neu-rological signs of ataxia (defective coor-
dination of muscles, especially with vol-untary movement) occur. Judgment may208
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

also be impaired. Continued elevation ofblood alcohol concentrations (0.31 to 0.41percent) can produce confusion, mild stu-por, and ultimately coma. Blood alcohollevels of 0.51 percent usually lead to deathfrom depression of the respiratory centerof the brain.
Another effect in the spectrum of neu-
rological disturbances associated withintensive alcohol intoxication is theoccurrence of blackouts , periods of am-
nesia characterized by an inability toremember events during the time of theblackout.
Alcohol withdrawal can be complicated
by seizures and delirium. The most severeform of alcohol withdrawal is delirium
tremens . Individuals with delirium tremens
experience signiﬁcant restlessness, grossdisorientation, cognitive disruption, andelevation of temperature and pulse rate.Although delirium tremens can be fatal,the course is often self-limiting. The acuteperiod of delirium tremens usually lastsfrom 2 to 10 days, but it can be more pro-longed if withdrawal is severe. The with-drawal syndrome may be treated medi-cally by the administration of a cross-tol-erant drug, such as a sedative. Initially,sedatives are given in large doses to sup-press the withdrawal symptoms. Then thedose is reduced or the interval betweendoses is increased, or both, so that thedosage is progressively tapered to zero.Because of wide variations in drug toler-ance, treatment is individualized.
Treatment of Alcohol Dependence
Alcohol dependence is a chronic, life-
long disorder. It requires long-term treat-ment that extends beyond the initialperiod of detoxiﬁcation and generallyinvolves a wide variety of services, includ-ing individual, group, and family therapy.In addition, self-help groups, such asAlcoholics Anonymous for alcohol-depend-
ent individuals, and Alanon and Alateen
for their families, are widely recommend-ed. Typically, the goal of treatment is ab-stinence from alcohol and other mood-altering substances. In some circum-stances, drugs are used to discourage andinhibit the use of alcohol. One such drug,disulﬁram ( Antabuse ), interferes with the
normal metabolism of alcohol. Con-sequently, individuals who ingest alcoholafter taking Antabuse have severe gastroin-testinal distress. Thus, the drug acts as adeterrent to alcohol intake.
Alcohol-Related Medical Illness
Medical conditions that can result from
chronic alcohol abuse, other than thosecaused by trauma due to intoxication, aregenerally caused by dietary insufﬁciency,by the direct toxic effects of alcohol onbody tissue, or both. These conditionscan involve all organ systems. The prog-nosis of alcohol-related medical illnessdepends on the nature of the illness andits severity. Although some alcohol-related medical illnesses are reversible,almost no alcohol-related illness can becured if the individual continues to abusealcohol.
Nervous System
Korsakoff’s Syndrome
Associated with an excessive intake of
alcohol, chronic malnutrition, and a deﬁci-
ency of the B vitamins (thiamine in par-ticular), Korsakoff’s syndrome is charac-
terized by gross disturbances in formingnew memories and recalling past memo-ries. The use of confabulation, in whichindividuals make up experiences to ﬁllmemory gaps, is a common characteristicof those with Korsakoff’s syndrome. InPhysical Effects of Alcohol Abuse and Dependence 209

addition to abstinence, treatment consistsof the administration of thiamine. Somecognitive improvement is possible, but fullrecovery is unlikely. Several months maybe required before improvement is notice-able.
Wernicke’s Encephalopathy
(Wernicke’s Disease)
Although Wernicke’s encephalopathy can
occur in other conditions, it is most com-monly associated with chronic alcoholabuse. It is characterized by the suddenonset of confusion, double vision, and dif-ﬁculty with balance. It often occurs incombination with Korsakoff’s syndromeand, like Korsakoff’s syndrome, is relatedto thiamine deﬁciency. Treatment consists
of the replacement of thiamine. Earlytreatment is mandatory to prevent perma-nent deﬁcits. Prompt treatment resolvesmany of the symptoms. When Korsakoff’ssyndrome accompanies Wernicke’s en-cephalopathy, however, memory deﬁcitsremain.
Peripheral Neuropathy
Although there are many causes of
peripheral neuropathy, a number of indi-viduals who chronically abuse alcoholdevelop disorders of the peripheral nerves(see Chapter 3). Peripheral neuropathyassociated with chronic alcohol abuse isthought to be the result of inadequatenutrition, speciﬁcally inadequate amountsof thiamine and the other B vitamins. The
condition affects the extremities andincludes symptoms such as numbness,painful sensations, weakness, and musclecramps. Burning pain of the feet may alsooccur. Good nutrition and the administra-tion of supplemental B vitamins canbring about improvement, but theimprovement may be slow.Cardiovascular System
Cardiomyopathy
Alcoholic cardiomyopathy occurs after
long-term chronic use of alcohol. It resultsfrom the direct toxic effects of alcohol onthe heart muscle itself. The heart may be-come enlarged ( cardiomegaly ), and the
heart muscle may become more ﬁbrous.The heart’s ability to pump effectively maybe compromised so that symptoms of con-gestive heart failure, such as difﬁculty in
breathing and swelling (see Chapter 11), may
occur as the cardiac damage increases.
Beriberi Heart Disease
A deﬁciency in thiamine is thought to
contribute to the development of beriberiheart disease. Individuals with the condi-tion have a high cardiac output, even atrest, because of the dilation of the periph-eral small blood vessels. Beriberi heart dis-ease responds well to the administrationof supplemental thiamine.
Alterations in Heart Rate and Rhythm
Alcohol can affect both the speed at
which the heart beats and the rhythm thatit maintains. The direct long-term effecton blood pressure is variable. Alcoholwithdrawal can put a heavy load on theheart, sometimes compromising cardiacfunction so severely during detoxiﬁcationthat death can result. Consequently,detoxiﬁcation should be conducted undercareful medical supervision.
Alterations in Blood
Alcohol can have a direct and adverse
effect on the development of red bloodcells, white blood cells, and platelets,resulting in subsequent anemia, lower210
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

resistance to infection, and interferencewith blood clotting. One of the mecha-nisms by which alcohol affects blood cellformation is by interfering with the use offolic acid, a nutritional substance thatbone marrow requires to manufacturehealthy cells effectively.
Megaloblastic anemia (the presence of
large abnormal red blood cells) withleukopenia (an abnormal decrease in the
number of white blood cells) and throm-
bocytopenia (an abnormal decrease in the
number of platelets) occurs frequently inindividuals with low folic acid intake.Treatment with the administration of sup-plemental folate , proper nutrition, and
abstinence from alcohol can generallyreverse these abnormalities.
Respiratory System
Alcohol has a direct toxic effect on lung
tissue. In combination with cigarettesmoking, a higher incidence of chronicobstructive pulmonary disease (see Chap-ter 12) can result from chronic alcoholabuse. In addition, because chronic alco-hol abuse affects some of the lungs’ nat-ural defenses, individuals who abusealcohol have a greater tendency to devel-op lung infections.
Musculoskeletal System
Regardless of the nutritional status, alco-
hol has a direct toxic effect on skeletalmuscle; destruction of muscle ﬁbers leadsto weakness, pain, tenderness, and swel-ling of affected muscles. Myopathy (dis-
ease of muscle) due to alcohol abuse maybe acute or chronic. The more commonform is chronic alcoholic myopathy , which
evolves over months to years. Pain may beless severe in chronic myopathy, althoughmuscle cramps can occur. In addition,muscles may atrophy (shrink or becomesmaller) and become weak. Most symp-toms of myopathy improve with the ces-sation of alcohol abuse, but continuedalcohol abuse leads to continued deteri-oration. Excessive alcohol consumptioncan also contribute to osteoporosis
(reduction in bone mass), causing bonesto become weakened, fragile, and easilybroken; see Chapter 14). Osteoporosisoccurs not only because calcium intake isinsufﬁcient but also because alcohol in-terferes with the absorption of calciumfrom the intestine.
In addition to the direct effect on the
musculoskeletal system, alcohol can alsocontribute to major injury. Individualsunder the inﬂuence of alcohol may havedecreased balance and coordination anddemonstrate impaired judgment. As aresult, they may be injuried in falls, ﬁres,or motor vehicle or pedestrian accidents.
Gastrointestinal System
It is possible for alcohol to affect almost
every organ of the gastrointestinal tract.Individuals who consume alcohol exces-sively have an increased incidence of can-cer of the throat and esophagus (seeChapter 16). Whether the increased inci-dence of cancer is due to direct contact ofalcohol with the tissues, the presence ofcarcinogenic substances in some alcoholicbeverages, or a combination of the two isunknown. Despite the fact that alcohol isconsidered a hepatotoxin (substance that
is harmful to the liver), individuals whochronically abuse alcohol differ widely intheir susceptibility to liver disease.
Esophagitis and Gastritis
Esophagitis is inﬂammation of the
esophagus. Gastritis is an inﬂammation of
the stomach. Both can occur with theacute and chronic abuse of alcohol. ThePhysical Effects of Alcohol Abuse and Dependence 211

severity of these conditions depends onthe individual. In some instances, the con-ditions produce only a mild discomfort,but in other instances, the irritation andinﬂammation produce ulcerations andbleeding. Treatment is directed toward re-
ducing the inﬂammation. Obviously, absti-
nence from alcohol is a major treatmentobjective.
Esophageal Varices
Some individuals who abuse alcohol
develop esophageal varices , “varicose” veins
of the esophagus, a condition in whichthe veins become dilated and tortuous.Esophageal varices are usually a complica-tion of cirrhosis. They may cause nosymptoms. If the varices become ulcerat-ed due to irritation, however, or if thereis increased strain from coughing or vom-iting, the distended veins may rupture,causing serious hemorrhage.
Treatment is directed toward controlling
hemorrhage, usually by inserting a specialtube ( Sengstaken-Blakemore tube ) into the
esophagus. A balloon on the tube is then
inﬂated to exert pressure against the bleed-
ing vein. Because the esophagus needs restin order to heal, other types of feedingmay be instituted until the esophagus ishealed (see Chapter 10).
Alcoholic Hepatitis
During alcohol metabolism, fat is de-
posited in the liver. When individuals con-sume excessive amounts of alcohol, theaccumulation of fat enlarges the liver, acondition called fatty liver . If individuals
continue to consume alcohol, liver cellsmay die, causing the liver to becomeinﬂamed. This inﬂammatory condition, inwhich the liver is usually enlarged andpainful, is known as alcoholic hepatitis .
Abstinence from alcohol can reverse theeffects of both fatty liver and alcoholichepatitis. Individuals who continue toabuse alcohol, however, have a highchance of developing cirrhosis.
Cirrhosis
Cirrhosis is most frequently caused by
either hepatitis C or alcoholism (Ginès,Cárdenas, Arroyo, & Rodés, 2004). Itinvolves the reaction of the liver to injuryby hepatotoxins (substances that are
harmful to the liver), in this case, alcohol.When alcohol injures the liver repeated-ly over a period of time, ﬁbrous tissuereplaces liver cells. Circulation within theliver becomes less efﬁcient, resulting inobstructions and thus increasing pressurein the vessels.
All blood from the gastrointestinal
tract, spleen, pancreas, and gallbladder iscarried to the heart through the liver bythe portal system . Because of the ﬁbrous
changes that occur in the liver with cirrho-sis, there is increased pressure in the por-tal vein, a condition known as portal
hypertension . Backﬂow of blood results in
the enlargement of the spleen ( splenom-
egaly ), accumulation of fluid in the
abdominal cavity ( ascites ), and develop-
ment of esophageal varices .
Some individuals with cirrhosis experi-
ence no symptoms. Others experienceweakness, nausea, loss of appetite ( ano-
rexia ), and jaundice (yellow discoloration
of the skin and whites of the eyes due tothe accumulation of bile pigments in theblood). Treatment of cirrhosis is largelysymptomatic, but abstinence from alcoholis a necessity for survival. Individuals withcirrhotic changes in the liver have anincreased risk of cancer of the liver. Thosewho continue to abuse alcohol despite cir-rhotic changes in the liver, or despite oth-er complications, have a signiﬁcantlydecreased survival rate.212
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

Pancreatitis
A variety of conditions other than alco-
hol abuse may cause pancreatitis (inﬂam-
mation of the pancreas). Alcoholic
pancreatitis , however, is a form of pancre-
atitis that develops in susceptible individ-uals after chronic alcohol abuse. In thiscondition, the pancreatic ducts becomeobstructed. The enzymes that the pancreasnormally secretes into the small intestineto aid in digestion become active whilethey are still in the pancreas (see Chapter10). As a result, the pancreas essentiallybegins to digest itself, causing progressivedegeneration with scarring and calciﬁca-tion of pancreatic tissues. Pancreatic func-tion is often severely curtailed. Chronicpancreatitis can lead to severe disabilityfrom pain, malabsorption of nutrientsresulting in weight loss, and diabetes mel-litus secondary to the destruction of theislets of Langerhans (see Chapter 9).
Treatment of pancreatitis is directed
toward halting destruction of tissue andalleviating the symptoms. As with otherconditions of the gastrointestinal tract,effective treatment requires that individ-uals abstain from alcohol. If they nolonger consume alcohol, many individu-als recover from alcoholic pancreatitis tolive a normal life. If they continue todrink, however, the prognosis is general-ly poor.
Reproductive System Problems
Excessive alcohol use has been found to
lower the level of the male hormonetestosterone , which, in turn, has been relat-
ed to decreased libido and, in someinstances, impotence. Excessive alcoholintake also increases the level of epineph-
rineand other hormones. The toxic effects
of alcohol on the developing fetus duringpregnancy can result in a deformity of theinfant called fetal alcohol syndrome . The
amount of alcohol that pregnant womenmust consume before the fetus is injuredis unknown and appears to vary with theindividual. Fetal alcohol syndrome ischaracterized by prenatal and postnatalgrowth retardation , microcephaly (abnor-
mal smallness of the head), abnormalities
of the nervous system , and facial disﬁgura-
tion. Other congenital anomalies may
include mental retardation , as well as mus-
culoskeletal and cardiac abnormalities .
USE DISORDERS INVOLVING OTHERSUBSTANCESCaffeine and Nicotine
Tolerance and dependence have been
established for both caffeine and nicotine,although these substances are not com-monly thought of as substances of abuse.Caffeine is commonly obtained from cof-fee or tea, but it may also be consumed insoft drinks, chocolate, and many over-the-counter drugs. Caffeine is a powerful cen-tral nervous system stimulant (Ochs,Holmes, & Karst, 1992) that can also affectcardiac muscle, elevate blood pressure,increase gastric acid secretion, and have adiuretic effect. It can exacerbate existingdisabling conditions as well as generatenew symptoms.
Caffeine can produce both psychologi-
cal and physical dependence. Headachesthat are not relieved by regular analgesicsare a manifestation of withdrawal fromcaffeine. Although caffeine abuse in itselfis not usually disabling, it may aggravatepreexisting conditions, such as ulcer dis-ease, hypertension, or cardiac arrhythmia.The availability of a large number of decaf-feinated products makes it possible to de-crease caffeine consumption, if necessary.
Nicotine is a highly dependence-produc-
ing drug (Christen & Christen, 1994). TheUse Disorders Involving Other Substances 213

amount of dependence is proportional tothe quantity of the drug used. Nicotineconsumed through smoking, chewing, orsnufﬁng tobacco is absorbed through themucous membranes or surfaces of thelung. Taken into the body, nicotine pro-duces initial stimulation, followed by se-dation. Withdrawal effects of nicotine in-clude restlessness, irritability, and tension.
The health consequences of tobacco use
can be severe. Cancer of the lung or oralcavity and a variety of other lung diseaseshave been linked to tobacco use. In addi-tion, tobacco use has been shown toaggravate other preexisting conditions,such as heart disease and hypertension.The addictive nature of nicotine can alsointerfere with treatment of smoking-relat-ed diseases. An early study reported thatat least 50 percent of individuals recover-ing from surgery for a smoking-relatedcondition such as lung cancer or cardio-vascular disease continued to smoke whilethey were hospitalized or resumed smok-ing shortly after they were discharged(Burling, Stitzer, Bigelow, et al., 1985).Although smoking was once socially ac-ceptable, pressure from various groups andpublic awareness of the health hazards ofsmoking have resulted in sanctions onpublic smoking behavior. Treatment ofnicotine dependence varies widely, rang-ing from the use of nicotine-containinggum to hypnosis to behavioral and groupprograms. The success of most programsdesigned to stop tobacco use is directlyrelated to the smoker’s motivation to stop.
Sedatives
Sedation implies calmness and tranquil-
ity. Sedatives are classiﬁed according to the
pharmacologic action they produce,namely, depression of the central nervous
system . Examples of sedative drugs are
alcohol , barbiturates , diazepam (Valium ),and alprazolam (Xanax ). If taken in high-
er doses to produce sleep, they are calledhypnotics. Sedatives are sometimes alsocalled minor tranquilizers or antianxiety
agents . Whether they have been pre-
scribed for treatment of a speciﬁc condi-tion or symptom or whether they havebeen obtained illegally, sedatives may beassociated with abuse, tolerance, anddependence.
Individuals commonly abuse sedatives
in combination with alcohol, and theyoften abuse opiates and stimulants con-currently. Commonly abused sedatives arebarbiturates (e.g., phenobarbital, seco-
barbital, and amobarbital sodium), benzo-
diazepines (e.g., chlordiazepoxide hydro-
chloride [Librium], diazepam [Valium],
and chlorzepate dipotassium [Tranzene]),
and other central nervous system depressants
(e.g., methaqualone [Quaalude], meproba-mate, and ethchlorvynol [Placidyl]).
Withdrawal from sedatives is similar to
withdrawal from alcohol. Some sedatives,such as benzodiazepines, may have adelayed withdrawal effect, beginning sev-eral days after ceasing to take the drug. Ifindividuals have become sedative depend-ent on lower doses of the drug, withdraw-al symptoms may consist only of irri-tability, sleep disturbance, and generalizedanxiety. If, however, individuals becamedependent on higher doses, withdrawalcan be life-threatening. Sudden with-drawal, especially from barbiturates, canresult in acute psychosis and seizures.Therapeutic withdrawal from a sedative,like the therapeutic withdrawal fromalcohol, usually involves the administra-tion of a cross-tolerant drug to suppresswithdrawal symptoms with gradual taper-ing of the dosage. The drug being with-drawn determines the length of timerequired for tapering. For some sedatives,7 to 10 days is sufﬁcient for detoxiﬁcation.Longer-acting drugs that have been used214
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

at high dosages may require 14 or moredays for detoxiﬁcation.
Opioids
Because opioids (narcotic drugs such as
morphine, meperidine [Demerol], pro-poxyphene [Darvon], oxycodone [Perco-dan], and codeine) are frequentlyprescribed for pain, addiction can occurthrough regular prescription use. In oth-er instances, these medications areobtained illegally. A commonly used ille-gal opioid is heroin.
In addition to producing pain relief,
narcotics produce euphoria, sedation, anda feeling of tranquility. At ﬁrst, individu-als may take illegal narcotics primarily forthe feeling of euphoria. Repeated admin-istration rapidly produces tolerance andintense physical dependence. Eventually,as the dosage and/or frequency of drugadministration increases, individuals needto continue to take the drug regularly toavoid symptoms of physical withdrawal.
There are numerous negative health
consequences related to opiate use, espe-cially to long-term use of heroin (Fiellin& O’Connor, 2002; Gonzalez, Oliveto, &Kosten, 2002), including lethal respirato-ry depression with overdose. Drugs thatare injected increase individuals’ risk ofcontracting HIV infection or hepatitis C if
needles are shared. Adding adulterants tosubstances or using nonsterile techniquesof injection may also produce medicalcomplications. Skin abscesses, cellulitis
(inﬂammation of tissues), thrombophle-
bitis (inﬂammation of a vein with associ-
ated clot formation), septicemia (presence
of toxins in the blood), and bacterialendocarditis (inﬂammation of the inner
lining of the heart) are frequent compli-cations.
Withdrawal symptoms vary in severity
and duration, depending on the particu-lar drug abused. Withdrawal from nar-cotics is generally not life-threatening.Many symptoms of withdrawal are ﬂulike,although they may include anxiety, irri-tability, and restlessness.
Opiate substitution drugs are sometimes
used in treatment of opiate addiction andmay be used for either detoxiﬁcation ormaintenance. Methadone and anotheropiate-substitute, levomethadyl acetate,may be used to reduce the use of illicit opi-ates and the high-risk behaviors associat-ed with drug use (Fudala et al., 2003;O’Connor, 2000), as well as to providemedical assistance with withdrawal of opi-ates. When used for detoxiﬁcation, thedrug dosage is gradually tapered duringthe withdrawal period. Some individualsmay be enrolled in a maintenance program
in which they do not undergo detoxiﬁca-tion but rather receive maintenance dos-es of an opiate substitute along withcounseling. The goal of such programs isﬁrst to help individuals return to a social-ly rehabilitated state and then to helpthem achieve a drug-free state.
Opiate substitution therapy is provided
only in a strictly regulated environment
in which medication is taken under clinical
observation and supervision (Clark, 2003).
Stimulants
Acting directly on the central nervous
system, stimulants create an increased state
of arousal and concentration and speed up
mental and motor activity. Individuals may
take stimulants for such effects as in-creased alertness and increased sense ofwell-being, increased conﬁdence, reduc-tion of fatigue, or decrease in appetite.Amphetamines (Benzedrine or Dexedrine),
methylphenidate (Ritalin), cocaine , and caf-
feine are all stimulants. They can be taken
orally , topically , intravenously , or by inhala-
tion. In addition to central nervous systemUse Disorders Involving Other Substances 215

effects, stimulants have generalized sys-temic effects, including an increase in heart
rate, an increase in blood pressure , a rise in
body temperature , and the constriction of
peripheral blood vessels (Sarnyai, Shaham,
& Heinrichs, 2001). Cocaine can also cause
cardiac arrhythmia (irregular heartbeat)
and can increase the respiratory rate (Kloner
& Rezkalla, 2003). Long-term use of stim-ulants can cause irritability, aggressivebehavior, and paranoid psychosis (Camí &Farré, 2003).
In recent years, cocaine has become one
of the most widely abused stimulants. Itmay be taken orally, used intranasally(snorted), smoked, or injected intraven-ously. The technique of free-basing
cocaine, which gained popularity in the1980s, involves heating a ﬂammable sol-vent such as petroleum or ether, and thenusing it to heat cocaine. The process“frees” cocaine hydrochloride from itssalts and adulterants, converting it to aform of cocaine that will vaporize. Thefree-base cocaine can be inhaled orsmoked, usually with a water pipe, fordirect absorption through the alveoli inthe lungs. The technique rapidly delivershigh concentrations of cocaine to thebrain and results in blood levels as highas those for self-injection.
The free-basing technique can cause
additional disability caused by the burnsfrom ﬁres started during the free-basingprocess. The level of tolerance for cocainerapidly increases, and the need for addi-tional cocaine to function normally canrapidly lead to the use of crack . Crack, a
solid form of cocaine free base, is thoughtto be one of the most addictive substancesyet encountered. Dependence is producedvery rapidly. Crack is smoked rather thansniffed. Its concentrated form and its routeof administration make its potency manytimes greater than that of cocaine alone.The euphoric effect produced by cracklasts only a matter of minutes, however,and is often followed by irritability, rest-lessness, and depression. The aftereffects
of crack can be so intense that individuals
continue to smoke it, despite obviousadverse consequences.
Individuals using cocaine, especially at
higher dosages, may use depressant drugsto counterbalance the stimulant effects.For example, alcohol and cocaine are com-monly combined for this purpose. Thesimultaneous injection of cocaine andheroin ( speedballing ) is also common.
Aside from its psychological, social, andvocational consequences, cocaine use canhave serious medical consequences. Free-basing or smoking crack can lead to pul-
monary complications . Chronic use of
intranasal cocaine may cause ulceration or
perforation of the nasal septum. Cocaine
intoxication can produce neurologic effects ,
such as confusion, anxiety, hyperexcitabil-ity, agitation, and violence. More seriouseffects are the result of acute cocaine toxi-
city, which is dose related, in which indi-
viduals can experience stroke or seizures ,
severe hyperthermia (increased body
temperature), arrhythmia (irregular heart-
beat), myocardial infarction (heart
attack), and, in some instances, sudden
death . Cocaine psychosis , another side
effect of cocaine use, is manifested byparanoia, panic, hallucinations, insomnia,and picking at the skin. The psychoticepisode can last from 24 to 36 hours.Individuals with this condition are usual-ly hospitalized and treated with antipsy-chotic medication.
Substances added to adulterate cocaine
to increase its weight, thereby increasingproﬁt on its sale, may cause additionalmedical complications. Problems canresult from the nature of the substanceused to cut the cocaine or from the dosagetaken. Adulterants such as talc or corn-starch can cause complications ranging216
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

from inﬂammation to embolus (undis-
solved matter in the blood).
Procaine , PCP, or heroin , which also may
be added to cocaine, may potentiate theeffects. Because the user can never be cer-tain of cocaine’s potency, the effects arenot always predictable. The withdrawal
syndrome from cocaine consists of a crav –
ing for more cocaine, depression, irritabil-ity, sleep disturbances, gastrointestinaldisturbances, headaches, and possiblysuicidal ideation. Because it is not unusu-al for individuals who are cocaine depend-ent to also be dependent on other drugs,a withdrawal reaction from other sub-stances may be experienced as well.
A newer street drug classiﬁed as an
amphetamine is crystalline methampheta-
mine (“ice”), which is highly addictive
physically and psychologically (Lukas,1997). Like crack, it can be heated andinhaled. The technique is similar to thatof smoking free-base cocaine. Ice hasgreater strength and more enduringeffects, lasting from 8 to 24 hours. Greaterstimulation to the brain makes it moredangerous mentally because it creates acraving that can continue years after ces-sation of use (Wermuth, 2000). Toxic lev-els can produce severe paranoid thinkingwith hallucinations. There is also greaterrisk of suicidal depression. Chronic use ofmethamphetamine in any form can resultin serious psychiatric, cardiovascular,metabolic, and neuromuscular changes.
Cannabis
When cannabis ( marijuana ) is smoked,
the active compound (THC) that it con-
tains produces euphoria, relaxation, dream –
like states, and sleepiness. It also impairscognitive function and performance ofpsychomotor tasks (Camí & Farré, 2003).Some individuals report enhanced percep-tions of colors, tastes,
and textures. Thepsychoactive responses to the drug depend
to a great extent on the dose, the person-ality and the experience of the user, andthe environment in which the drug isused. Often, users report a sense of timeslowing and an impairment in their abil-ity to learn new facts while they are underthe inﬂuence of the drug.
Overdose can produce anxiety, panic
states, and psychosis (Hall & Solowij,1998). Systemically, cannabis producesan increase in heart rate, dilation of thebronchioles, and dilation of the peripher-al blood vessels. Because of the stimulato-ry effect on the heart, cannabis use maylead to cardiac complications in individ-uals with heart disease. Chronic smokingof cannabis produces inflammatorychanges in the lungs that contribute to thedevelopment of chronic conditions suchas emphysema (see Chapter 12). Further-more, the use of other drugs, includingalcohol and tobacco, may compoundthe adverse effects of cannabis. For exam-ple, the combination of tobacco andcannabis use is thought to increase the riskof lung cancer.
Although cannabis may be ingested
orally, oral consumption can delay itseffects for up to an hour, and the effectsare less potent. Hashish , the concentrated
form of THC, is also smoked and has con-
siderably more potency than does cannabis.
Some individuals use cannabis only on
special occasions, but others become com-pulsively preoccupied with daily use. Thelong-term effects of cannabis use remaincontroversial. The degree to which can-nabis creates physical dependence has notbeen established; however, it may be pos-sible to develop psychological dependenceon cannabis (Hall & Solowij, 1998).Symptoms of withdrawal including rest-lessness, irritability, and insomnia havebeen noted in heavy users (Budney,Hughes, Moore, & Novy, 2001). Use Disorders Involving Other Substances 217

There is no speciﬁc medical treatment
for cannabis abuse. When cannabis useseverely hampers individuals’ functioning,treatment most often involves psycho-therapeutic techniques directed at under-lying problems. Because cannabis may beabused in combination with other drugs,treatment may occasionally be multifocalin nature.
Hallucinogens
Sometimes called psychedelics , hallu-
cinogens are drugs that, at some dosage,
produce hallucinations or distortions in per-
ceptions or thinking . Individuals under the
influence of hallucinogens report in-creased awareness of sensory input and asubjective feeling of enhanced mentalactivity. Common hallucinogens are LSD,
PCP (angel dust ), and mescaline .
Controlled substance analogues, or
designer drugs , can have dangerous, perma-
nent effects. Users of one class of thesedrugs, the methamphetamines such as
MDMA ( ecstasy ) or MDA ( street name
Adam ), may be especially susceptible to
permanent brain damage because theamount that produces psychological ef-fects is not far from the dosage that pro-duces neural damage. Designer derivativesof amphetamines (MDMA) produce eu-phoria but can also have hallucinogeniceffects; they may also cause cerebral hem-
orrhage (stroke), hyperthermia (elevated
body temperature), altered mental status,panic, and psychosis.
Hallucinogens are usually taken orally.
Although the use of hallucinogens hasdeclined somewhat, patterns of use varywidely. Their use is now often concurrentwith the use of other drugs. One of themost powerful hallucinogens is LSD. Its
effects vary with the individual, the dose,and the environment in which the drugis used. Generally, the effects developwithin several hours and last up to12 hours. Individuals may report height-ened sensitivity and clarity, increasedinsights, a sense of time moving moreslowly, and distortions of visual images.Some individuals experience adverseeffects from LSD, such as a panic statewith severe anxiety.
The physical consequences of hallucino-
gen abuse in and of themselves are not sig-niﬁcant. The psychological consequences,however, can be severe. Adverse effects ofhallucinogens vary from acute psychosis to
self-mutilation or suicide . Accidents can
result from misjudgment or impairment.Some individuals experience “ﬂashbacks”in which hallucinations reappear brieﬂyeven months after the last drug dose. Anoverdose of hallucinogens can result inexceedingly high body temperatures,seizures, and shock.
Because hallucinogens produce no
physical dependence, there is no speciﬁcmedical regimen for treatment. Adverseeffects such as panic episodes are usuallytreated with a supportive environmentand observation.
Inhalants
Substances that cause perceptible
changes in brain function through inhala-tion are called inhalants . Inhalants are gen-
erally classiﬁed into four categories:
• Aerosols• Gases• Solvents• Nitrites
(Ballard, 1998)
A wide variety of substances are abused
in this way, often because they are readily
accessible and inexpensive. For example,commonly used inhalants are airplane
glue,typewriter correction ﬂuid , marking pen-
cils, industrial and household chemicals ,218
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

gasoline , nitrites (poppers, snappers , or rush),
and nitrous oxide . Although individuals of
all age groups practice inhalant abuse, itis especially prevalent among adolescentsand preadolescents.
Although the effects of inhalants are
brief, they can be serious, especially withprolonged or long-term use. Adverse ef-fects of inhalants vary according to thetype of substance inhaled. Organic sol-vents such as airplane glue can producecardiac arrhythmia , bone marrow depression ,
damage to the kidney and liver , and, in some
instances, death .
Prolonged use of nitrites is thought to
suppress the immune system , increasing the
individual’s susceptibility to infection.Nitrites are frequently used to enhancesexual pleasure; consequently, individualswho use nitrites in this way and are alsoexposed to human immunodeﬁciencyvirus (HIV) may be at greater risk for devel-oping HIV infection because of their sup-pressed immune system and subsequentincreased vulnerability to infection.Chronic abuse of nitrous oxide can result
in nerve damage , seizures , bone marrow
changes , respiratory depression , or death .
Because nitrous oxide distorts specialsenses, driving during intoxication is haz-ardous. Even though the effects ofinhalants are brief, their use can result independence. No speciﬁc medical treat-ment is usually indicated for inhalantabuse, but specific psychotherapeuticmeasures may be used to prevent relapseand to help individuals discontinueinhalant use.
MEDICAL CONSEQUENCES OFABUSE OF OTHER DRUGS ANDSUBSTANCES
Substance abuse leads to psychological,
social, and vocational impairments and of -ten to crimes committed to obtain drugs or
to obtain money for additional drugs. Sub-
stance abuse also has medical consequences.
Drug-Related Illness
Dermatologic Complications
Many of the medical complications
related to drug abuse result from nonster-ile injections or from adulterants, ratherthan from the drug itself.
Abscess
Bacterial infection may cause pus to col-
lect in the tissues, forming an abscess. Inassociation with drug use, improper
cleansing of the skin before injection or the
use of a nonsterile needle may lead to an
abscess. Skin at the site becomes warm, red,
swollen, and painful with a purulent (pus)
discharge. Skin around the area frequent-ly becomes necrotic (dies). If the abscess
goes untreated, individuals may developsystemic symptoms of fever, loss of appe-tite, and fatigue. Infection may spread tothe bloodstream, creating a generalizedsystemic infection ( bacteremia ). Treat-
ment of an abscess consists of draining thepurulent material and debriding (remov-
ing) the area of dead tissue. Antibiotics areusually prescribed, especially if individu-als demonstrate systemic symptoms.
Cellulitis
An acute inﬂammation of the tissues
without necrosis (tissue death) is called
cellulitis. When associated with intra-venous drug abuse, cellulitis is caused bythe invasion of a variety of organisms orby irritation of the tissues from the drugitself. The tissue becomes red and tender,and there may be adenopathy (swelling
of lymph nodes). Treatment of cellulitisMedical Consequences of Abuse of Other Drugs and Substances 219

depends on the cause. Occasionally, cel-lulitis progresses to abscess formation.
Other Dermatologic Complications
Injections with nonsterile needles or in-
jections of drugs that have been contam-inated by adulterants may leave needle
track scars . Injections cause a mild inﬂam-
matory reaction and, with subsequentinjections, produce scarring at the injec-tion site. Injection of a drug into an arteryinstead of a vein can cause an extremereaction of intense pain, swelling, andcoldness of an extremity. If not treatedproperly, gangrene may develop, necessitat-
ing amputation.
Cardiovascular Complications
Other than direct effects on the heart
from the drug itself, most cardiovascularcomplications that result from drug useare related to the use of nonsterile injec-tion techniques or to contamination ofthe drug with adulterants. A commoncomplication is endocarditis (inﬂamma-
tion of the inner lining of the heart),which affects the valves of the heart andcan lead to potentially serious conse-quences (see Chapter 11).
Some drugs have a direct toxic effect on
the heart muscle or may directly affectheart rhythm. In some instances, inﬂam-mation of the veins with clot formation(thrombophlebitis ) may occur because of
the toxic effects of the drug.
Respiratory Complications
The intravenous injection of drugs to
which adulterants such as talc, starch, orbaking soda have been added may resultin pulmonary complications. Becausethese substances do not dissolve, they cir-culate in the blood and may becomelodged in lung tissue. The lodged particlescause an inﬂammatory reaction in thelungs, resulting in ﬁbrosis of the lung tis-
sue. If the ﬁbrous changes are extensive,they affect the oxygen-exchanging abili-ty of the lungs. Symptoms similar to thoseof emphysema may develop. Changes inlung elasticity can eventually result in pul-
monary hypertension and subsequent heart
failure . (See Chapter 12 for a discussion of
the symptoms of emphysema and Chap-
ter 11 for a discussion of pulmonary hyper –
tension and heart failure.)
Lung infections or lung abscesses may
occur if organisms localize in the lung after
the nonsterile injection of a substance.Aspiration pneumonia , an inﬂammation of
the lung, may result from the inhalationof foreign substances or chemical irritants.Aspiration of gastric contents is also acommon cause of aspiration pneumonia.Individuals who become unconscious be-cause of a drug overdose may, in theirunconscious state, vomit and subsequent-ly inhale the vomitus. If they inhale alarge quantity, the results can be fatal.
Individuals who abuse drugs, including
alcohol, may also develop tuberculosis (see
Chapter 12). Rather than being a directresult of drug use, tuberculosis is morelikely the consequence of the generallifestyle and living conditions of individ-uals who abuse drugs. Malnourishment,poor hygiene, and overcrowding all con-tribute to development of the disease. Inaddition, because some drugs have an im-munosuppressant effect, individuals maybe more susceptible to the infection. Anoverdose of narcotics or sedative/hypno-tics can severely depress the respiratorycenter, causing cessation of breathing andconsequent death. Overdoses of narcoticshave also been associated with develop-ment of severe pulmonary edema (collec-
tion of ﬂuid in the lungs), which, withouttreatment, can also result in death.220
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

Gastrointestinal Complications
Because the liver acts as the detoxiﬁca-
tion center for the body, individuals whochronically abuse drugs may damagetheir liver. Some substances appear to bemore directly harmful to the liver thanothers. Chronic, excessive abuse of sol-vents, for example, can cause liver necro-
sis(tissue death). Other substances may
cause liver abnormalities such as inﬂam-mation or ﬁbrosis.
Hepatitis is a common complication of
drug abuse. Hepatitis A may be related topoor hygiene habits and poor envi-ronmental conditions. More commonly,hepatitis B (serum hepatitis ) occurs as the
result of nonsterile or contaminated intra-venous injections. (See Chapter 10 for adiscussion of hepatitis A and hepatitisB.) Hepatitis C is caused by the hepatitis C
virus (HCV). HCV causes what was pre-viously called non-A , non-B hepatitis and
is transmitted through infected blood.Consequently, individuals who use in-travenous drugs and share needles are athigh risk for developing the disease.Hepatitis C generally becomes a chronicdisease and can predispose the individ-ual to cirrhosis (Ginès et al., 2004). Theonly treatment currently used for hepati-tis C consists of injections of interferon ;
however, about half of people treatedrelapse.
Neurological Complications
Seizures may result from an overdose of
drugs or a hypersensitivity to adulterants.Seizures are especially prevalent after anoverdose of amphetamines, heroin, or hal-lucinogens. In some instances, strokemay also accompany an overdose. Thetoxic effects of adulterants on the nervoussystem can lead to blindness and periph-eral nerve damage.Other Complications
The chronic use of some drugs may
result in nystagmus (involuntary eye
movement). Use of solvents can producebone marrow changes and aplastic anemia .
An overdose of drugs can result in acute
renal failure , which can progress to per-
manent kidney damage (see Chapter 13).Individuals who abuse drugs also have ahigher incidence of venereal disease , such
as gonorrhea and syphilis, related to theirgeneral lifestyle and sexual practices. Oneof the most serious and hazardous com-plications of drug use in the past fewyears has been infection with HIV (seeChapter 8), which is related to both in-travenous drug use and unsafe sexualpractices.
Drug abuse during pregnancy has seri-
ous implications for the offspring. Somefetal hazards are related to the lifestyle ofthe mother, which results in poor prena-tal care, poor nutrition, and a generallypoor health status. The direct toxic effectsof drugs on the developing fetus ( tetrato-
genic effects) can include neurological
and/or physical abnormalities, as well asthe dangers of the withdrawal syndrometo the infant after birth.
DIAGNOSTIC PROCEDURES
Diagnosis of substance use disorders is
often delayed or symptoms overlooked.The result is continued disabling effects,further development of medical complica-tions, and progression of dependence.Denial and resistance to acknowledgingthe problem are universal symptoms ofsubstance abuse and dependence. Con-sequently, even if family members or asso-ciates have identiﬁed a substance useproblem, the individual who abuses sub-stances may deny the condition andrefuse to seek treatment. Diagnostic Procedures 221

Substance use disorders are frequently
associated with other health and person-al concerns. Consequently, many individ-uals presenting at health or counselingfacilities may have coexisting or second-ary substance use problems that are notidentiﬁed or diagnosed. Some profession-als may feel uncomfortable questioning orconfronting individuals about substanceuse disorders, in which cases diagnosis ortreatment of the problem is further de-layed. Undetected substance use problemshave signiﬁcant effects on the health andwell-being of individuals as well as theirfamily and others.
Screening Instruments
Routine screening of individuals pre-
senting for health care or counseling helpsprofessionals determine whether a prob-lem exists and whether there is need formore in-depth assessment. Several types ofscreening instruments are available. Oneof the best-known and most widely usedinstrument is the Michigan Alcoholism
Screening Test . Others include the CAGE ,
the T-ACE , the TWEAK , the Alcohol Use
Disorders Identiﬁcation Test , the Substance
Abuse Life Circumstances Evaluation , the
MacAndrew Scale and MacAndrew Scale-
Revised , and the Substance Abuse Subtle
Screening Inventory (Piazza, Martin, &
Dildine, 2000). Each screening test has itsown assets and limitations. The type ofscreening test chosen should be based onthe circumstances under which the test isused as well as on speciﬁc factors relatedto the individual.
Direct Drug Screening
Direct testing for the presence of the
substance in the body may involve breathanalyzers and blood alcohol tests. Bothtests serve as a measurement of intoxica-tion, but they do not reveal the extent ofabuse or dependence. Screening of bloodor urine samples is also used to verify sus-pected substance use. As with any labora-tory test, there is a possibility of false-negative or false-positive results. Newerscreening methods are designed to bemore sensitive and produce more accurateresults. Two common methods of urinetesting available in most laboratories arethin-layer chromatography and gas chro-matography. Drug testing is valid, howev-er, only if accomplished under strictlycontrolled conditions. Many individualswho abuse or are dependent on drugs areaware of a variety of methods to invalidatetest results, such as substituting specimensfrom a drug-free individual for their ownspecimen. The appropriate methods andtimes of drug screening are highly contro-versial. Routine screening for drugs with-out the individual’s knowledge andconsent evokes a variety of legal and eth-ical concerns.
Medical Evaluation
Medical diagnosis of substance use may
be attained from several sources. The phys-
ical manifestations of substance abuse and/
or dependence may include a variety ofdisorders. Questions about substance usepractices should be routinely asked in theexamination of individuals with gastro-in
testinal disturbances, hypertension or
heart disease, liver disease, neurological
changes, or a history of traumatic injuries.Blood cell abnormalities, such as decreasednumber of platelets or signs of bone mar-row depression (see Chapter 8), or otherindirect clinical laboratory signs, such aselevated levels of gamma-glutamyltrans-ferase or gamma-glutamyltranspeptidase
,
or an elevated red blood cell mean corpus-
cular volume, may suggest problems withsubstance abuse. Elevated levels of en-222
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

zymes such as serum glutamic-oxaloacetictransaminase (SGOT) and serum gluta-mate pyruvate transaminase ( SGPT), mayalso be associated with substance abuse;however, increased concentrations ofenzymes such as SGOT and SGPT can alsobe associated with other conditions (e.g.,myocardial infarction).
Behavioral and PsychologicalScreening
Investigation of subtle psychological or
behavioral symptoms is also important inthe diagnosis of a substance use disorder.Depression, hyperactivity, sleep distur-bances, anxiety, sexual problems, or per-sonality changes are common manifes-tations of substance use disorders. In addi-tion, the incidence of accidents andinjury is often increased.
TREATMENT OF SUBSTANCE USEDISORDERS
The ﬁrst step in the treatment of sub-
stance-related disorders is identifying and
acknowledging the problem. Screening may
be hampered by several barriers, including:
• denial of the problem by individuals
or family members
• reluctance of medical and counseling
personnel to confront or discuss theproblem
Once the problem is identiﬁed and con-
fronted, individuals should be assessed formedical and psychosocial problems thattypically accompany it as well as for theirmotivation for change (O’Connor, 2000).Successful treatment of substance use dis-orders generally requires more than onelevel of care during the long recovery
pro-
cess. Treatment may involve outpatient or
inpatient care and continued aftercare.
Most individuals in treatment for substanceuse consider themselves as “recover ing,”
denoting the long-term and chronicnature of the recovery process. Relapse is
a common part of recovery and, rather than
being thought of as failure, can be viewedas an opportunity for learning and growth(American Academy of Pediatrics, 2000).
Many individuals with substance use
disorders eventually experience physical,social, or psychological crises that requireinpatient or residential treatment. Thetype of treatment received varies greatlyfrom facility to facility and depends on theparticular type of crisis experienced. Somefacilities provide treatment for substanceuse disorders solely on an outpatient basis.Others provide a combination of inpatientor residential and outpatient treatment.
Treatment usually begins with detoxiﬁ-
cation, which may or may not involveinpatient or residential treatment, depend-ing on the individual, the speciﬁc sub-stance, and the presence of additionalcomplications. Detoxiﬁcation is only aninitial step in the treatment of substanceuse disorders, however. Ongoing therapythat includes a variety of rehabilitationstrategies, such as psychotherapy, familytherapy, and self-help programs (e.g.,
Alcoholics Anonymous or Narcotics Anon-y
mous), is necessary to prevent relapse.
Several psychotherapeutic approaches tothe treatment of substance abuse exist.The speciﬁc type of therapy used is oftendependent on the facility in which theindividual is being treated and the over-all philosophy of professionals conductingthe treatment. In almost all instances,abstinence is a treatment goal.
In some instances, drugs are prescribed
in the ongoing treatment of substancedependence. Antabuse and methadone (or
other opiate substitute), which were dis-cussed earlier, are drugs commonly usedin the treatment of alcohol and opiatedependence, respectively. Treatment of Substance Use Disorders 223

Individuals with a substance use disor-
der may also require ongoing medicaltreatment for any medical complicationsthat have resulted from the substance use.Because nutritional deﬁciencies frequent-ly accompany substance use disorders,most detoxiﬁcation centers and residentialfacilities provide nutrition therapy as apart of the treatment. Educational pro-grams that stress the importance of nutri-tion, as well as other aspects of a healthylifestyle, are often incorporated into thegeneral treatment program.
PSYCHOSOCIAL AND VOCATIONALISSUES IN SUBSTANCE ABUSEPsychological Issues
The extent to which psychological dis-
ability is the direct result of a substance-related disorder or the cause of the dis-order is not easily determined. Individualswith substance use disorders frequentlyhave low self-esteem and experiencedepression. They may have feelings of in-adequacy, loneliness, and isolation thatlead to increased substance use. Inﬂuencedand controlled by the substance, they mayrely on it rather than on their ownresources. Doubt that they will be able tocope without the substance may erodetheir self-conﬁdence and self-esteem evenmore.
Individuals who are psychologically
dependent on a substance feel a need andlonging for the substance and become irri-table, depressed, anxious, and resentfulwhen the substance is not available. Indi-viduals with a psychological craving for asubstance may attribute their need to apersonal ﬂaw in their character or mayconsider their need as a negative reﬂectionon themselves. Either interpretation fur-ther contributes to lowered self-esteemand self-deprecation. Individuals may use denial or rational-
ization as a form of self-protection andas a way to minimize substance use prob-lems. They deny that a substance useproblem exists or may rationalize theirbehavior by redeﬁning their substanceuse so that it appears to be acceptable.Some individuals become aggressive orperform violent acts when they are underthe inﬂuence of certain substances. Thosewho are predisposed to this type of reac-tion may become involved in criminalacts, such as brawls, homicide, rape, orchild abuse.
As individuals become increasingly
dependent on the substance, the conceptof living without it produces fear anddread. Individuals interpret removal of thesubstance as removal of all joy and excite-ment from life. As with all types of per-ceived loss, individuals may experiencegrief and bereavement.
Recovery from a substance use disorder
involves restoration of self-esteem andconfidence, as well as willingness toaccept responsibility for personal behav-ior. Individuals need assistance to acceptthe losses they have experienced and todevelop skills for coping in the future.Recovery is a continuing process thatincludes long-term vigilance and a contin-uing commitment to remain drug-free.
Lifestyle Issues
A substance-related disorder affects
every aspect of individuals’ daily life. Asdependence on the substance becomesmore pronounced, individuals may loseinterest in self-care, may show a decreaseddesire for food, and may have a variety ofsleep disturbances, resulting in sleep dep-rivation. Daily activities may becomefocused on obtaining more of the sub-stance. Activities once enjoyed may offerlittle joy or stimulate little interest.224
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

Substance use can affect individuals’
ability to drive. Poor driving performancecan result in accidents or arrests, whichcan lead to the loss of a driver’s license.Therefore, transportation may become aproblem, so that individuals must dependon others for their transportation needs.
Sexual dysfunction is common in indi-
viduals with substance use disorders.Women may experience decreased libidoor may become promiscuous. Men mayexperience not only decreased libido butalso adverse effects on sexual perform-ance, including impotence, a commonside effect of chronic alcohol abuse. Indi-viduals recovering from a substance usedisorder may need to learn or relearn thecomponents of a healthy lifestyle, such asgood hygiene and grooming, proper diet,and the importance of exercise. Theseaspects of daily living may be a vital partof an individual’s rehabilitation.
Social Issues
The social effects of substance-related
disorders are widespread, touching fami-ly relationships, relationships with friendsand associates, and general functioning asa member of society. Individuals’ ability tofunction as a member of a social groupmay gradually deteriorate as substance useincreases. To some extent, social factorsmay determine the social implications ofsubstance use. For example, the availabil-ity of substances within a group or as partof a social event may determine whetheror not individuals participate in thatgroup or event.
The extent of the social tolerance of in-
dividuals’ behavior while intoxicated mayeither curtail or enhance substance use at
ﬁrst. As individuals become increasingly sub-
stance-dependent, however, the substancebecomes more important and social con-tacts and activities become less important.Individuals with a substance use disor-
der may be unable to function withintheir social network. Repeated, heavy useof the substance often leads to upheavalsin relationships. Social and family rela-tionships are strained and often destroyedif individuals become abusive and violentor if they engage in socially unacceptablebehavior while under the inﬂuence of thesubstance. Their behavior often alienatesothers, leading to social isolation. Decreas-ing reliability in performing social rolesand continued inability to maintain com-mitments cause those affected by the indi-vidual’s deterioration to feel disappointedand angry. Others in the social environ-ment may have to alter their own roles toincorporate duties the individual oncehad. This places additional burdens on allconcerned and may eventually lead to re-sentment or even banishment from thegroup. Family members and associatesmay begin to withdraw from the individ-ual emotionally. As individuals becomeincreasingly more isolated, feelings of self-loathing, guilt, and shame may develop.Feeling rejected by family and associates,they may limit their social contacts to rela-tionships with others who also focus onsubstance use.
The broader social consequences of sub-
stance use disorders may have legal andeven criminal implications. There is astrong relationship between substance use
disorders and a variety of accidents; motor
vehicle accidents, for example, can lead tophysical disability not only for the indi-vidual with the substance use disorder, butalso for others. Thus, the loss of a driver’slicense and more serious criminal chargesare potential effects of substance abuseand/or dependence. Furthermore, individ-uals who become dependent on illegalsubstances may engage in illegal activitiesto gain money for the purchase of addi-tional drugs. Even if they do not face crim-Psychosocial and Vocational Issues in Substance Abuse 225

inal charges, they can become focused onobtaining the drug rather than on func-tioning in a productive social role.
In some cases, family and social rela-
tionships can be salvaged in the recovery
process. In other instances, the loss of these
relationships is permanent. Depending onindividual circumstances, therapeuticrecovery may involve the development ofnew social roles and relationships or thereestablishment of old ones.
Vocational Issues
In the early stages of a substance-related
disorder, individuals may be concernedthat the use of the substance will interferewith their work. If substance use progress-es to abuse or dependence, however, con-cern may be reversed so that individualsbecome more concerned that their workwill interfere with the use of the sub-stance. The substance becomes ultimate-ly important, thus drastically affectingwork performance.
Although early identiﬁcation of and
intervention with workers with a sub-stance use disorder are most desirable, theproblem may not be recognized untilthere is a progressive deterioration of workperformance, increased absenteeism, or anincrease in job-related accidents. Fearthat they will lose their jobs if their em-ployers become aware of these indicatorsmay motivate individuals with a sub-stance use disorder to seek treatment.
The ability of individuals to return to
their former employment after treatmentfor substance abuse and/or dependencedepends on the circumstances. In someinstances, the stress and tension imposedby the job may be beyond individuals’ tol-erance and coping ability. It may be ben-eﬁcial to ﬁnd a less stressful work setting,especially in the early stages of recovery,until their tolerance for stress graduallyincreases. Physical disability resultingfrom substance abuse and/or dependencemust also be considered when evaluatingvocational potential.
It is essential to identify past work prob-
lems that may extend beyond issues ofsubstance abuse and/or dependence. Someindividuals may need to learn socialskills, work-appropriate behaviors, or good
hygiene or grooming practices; some need
to improve their work skills. Individualswho began abusing substances at an ear-ly age may not have developed sufﬁcientwork skills or work history to obtainemployment and may require additionaleducation or job training. If they return tothe same work setting that originally pre-cipitated feelings of inadequacy and thatcontributed to the development of sub-stance abuse and/or dependence, return towork may increase the risk of relapse. Insome cases, learning new skills or copingstrategies may enable individuals to returnsuccessfully to the work setting. In otherinstances, however, a new work environ-ment may be necessary.
Loss of a driver’s license because of a
substance use disorder may make trans-portation to and from work more difﬁcult.In addition, if driving a motor vehicle hadbeen part of the former employment, jobrestructuring or job change may be nec-essary. Some occupations require profes-sional licensure. Therefore, revocation ofindividuals’ licenses may limit their abil-ity to work in their former occupation.Many professional licensing boards haveprovisions for the reinstatement of licen-sure after documented rehabilitation. Ifthe professional license is reinstated, theremay be a probationary period in whichindividuals’ work performance is closelyobserved and monitored.
Conviction of criminal charges, espe-
cially felony charges, may prohibit em-ployment in some occupations. Although226
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

decisions may be made on a case-by-casebasis, such charges and their impact onemployment in different ﬁelds and in dif-ferent locations must be considered. Aswith most disabilities, the attitudes andconcerns of employers must be addressed,especially since social stigma is oftenattached to substance use disorders. Em-ployers may require particular encourage-ment to reinstate or hire individuals whohave been convicted of criminal charges.Knowing the potential for rejection byemployers based on these attitudes, recov-ering individuals may be reluctant to sharetheir complete history with employers ormay become defensive when asked ques-tions about substance use. Fear of rejectionbecause of prejudice must be consideredwhen the individual returns to work. Withincreasing awareness of substance abuseand dependence and with educationalefforts directed toward employers, howev-er, individuals may encounter decreasinglevels of prejudice.
Many individuals who are recovering
from substance abuse and/or dependencereturn to their employment and lead fullproductive lives. In all instances, howev-er, abstinence is a prerequisite for contin-uing productivity. Ongoing long-termtreatment or involvement with self-helpgroups may also be necessary to preventrelapse.
CASE STUDIESCase I
Ms. S. is a 35-year-old unmarried moth-
er of an 18-month-old son. She beganusing cocaine when she was 20 while stillin college, where she majored in businessmanagement. Her use continued after col-lege and intensiﬁed after she obtained aposition at a major real estate ﬁrm. Shelost her job after 2 years because of heraddiction, which by then had gone on toinclude heroin. She has been in and outof treatment for the past 10 years, and shehas been drug-free since she became preg-nant with her son; however, during herdrug use she also developed hepatitis C,which is currently in remission. Ms. S. hasbeen uneasy about returning to work butknows she needs to support her son. Herparents live nearby and have offered tocare for her son while she works or goesback to school.
Questions
1. What factors would you consider
when working with Ms. S. to devel-op a rehabilitation plan?
2. How does the diagnosis of hepatitis
C affect Ms. S.’s rehabilitation poten-tial?
3. What social factors are important to
consider when helping Ms. S. devel-op her plan for rehabilitation?
4. Given Ms. S.’s disability, education,
social situation, and work history,what might be some appropriateoccupations to consider?
5. How would you discuss Ms. S.’s dis-
ability with a prospective employer?
Case II
Mr. W. is a 45-year-old white male who
began drinking alcohol at age 16. He com-pleted high school and obtained a job asa yard laborer at a railroad. He married atthe age of 30, and he and his wife had fourchildren who are now ages 14, 12, 8, and5. Mr. W. was promoted to the position ofbrake operator at the time of his marriage
and later became a rail yard engineer whose
responsibility was to move cars within theyard, where they were then repaired. Hisalcohol use did not interfere with his workperformance initially, although he drankCase Studies 227

heavily after work every day and was abinge drinker with his wife on days off.Five years ago his wife stopped drinking,and 3 years ago she ﬁled for divorce. Mr.W.’s drinking intensiﬁed, and after sever-al accidents that occurred at work becauseof his drinking he was ﬁred. He has spent3 months in a residential treatment facil-ity, where he was treated for Wernicke’sencephalopathy; however, Korsakoff’s syn-drome was not present. He is now in out-patient treatment and says his goal is toreturn to his former job. He also express-es a desire to reunite with his former wife,although he is concerned that she hasbegun drinking again.Questions
1. When working with Mr. W. to devel-
op a rehabilitation plan, what signif-icant factors would you considerabout Mr. W.’s situation?
2. What ramiﬁcations does the diagno-
sis of Wernicke’s encephalopathyhave for Mr. W.’s rehabilitation?
3. How do social factors inﬂuence Mr.
W.’s effective rehabilitation?
4. What types of services might be help-
ful for Mr. W. in his rehabilitation?
5. To what extent is Mr. W.’s desire to
return to his former employmentrealistic?228
CHAPTER 7 C ONDITIONS RELATED TO SUBSTANCE USE

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30(4), 423–433.

NORMAL STRUCTURE ANDFUNCTION
Blood is a combination of different
types of cells and liquid that circulatescontinuously through the body. Thequantity of blood in the adult bodyremains constant under normal condi-tions. Blood cells are produced in the bonemarrow, as well as in lymphoid tissue andorgans. All blood cells are formed fromspecial cells called stem cells . The bone
marrow is especially rich in stem cells.
Blood has many important functions:
• It carries oxygen and nutrients to the
body tissues.
• It facilitates communication between
the endocrine glands and other bodyorgans by transporting hormones.
• It carries waste products from the tis-
sues to the organs of excretion, suchas the lungs and the kidneys.
• It protects the body from dangerous
organisms (immune function).
• It promotes clotting to minimize
excessive bleeding.
•
It helps regulate the body temperature.
Several different types of cells make up
the blood. Approximately two-ﬁfths of thetotal blood volume is composed of cellsthat are formed by a process called hemo-
poiesis or hematopoiesis . The types of cellscontained within the blood are red blood
cells(erythrocytes ), white blood cells
(leu-
kocytes ), and platelets (thrombocytes ).
More than 99 percent of the cells in bloodare red blood cells.
The number of circulating white blood
cells under normal circumstances is min-imal. When infection or other foreignstimuli are present, white blood cells pro-liferate so that there are large numbers ofthem circulating in the bloodstream. Thiscondition is called leukocytosis .
The number of platelets circulating in
the blood normally does not change. Ifthere should be a decrease in the numberof platelets, however, the condition iscalled thrombocytopenia ; an increase in
the number of platelets is called throm-
bocytosis . The liquid portion of the
blood is a watery, colorless ﬂuid calledplasma . It contains no blood cells but is
essential for carrying blood cells and nutri-ents through the circulation and fortransporting wastes from the tissues.Approximately three-ﬁfths of the totalblood volume is plasma.
Normal Structure and Function ofRed Blood Cells
Erythrocytes (red blood cells) carry oxy-
gen to the tissues. When mature, they aredevoid of a nucleus. They are normallyConditions of the Blood and
Immune SystemCHAPTER 8
231

disk shaped, with a thin center and thick-er edges. Their ﬂexible shape allows themto ﬁt through blood vessels of differingsizes. Hemoglobin is the red-pigmented
protein contained within the erythrocytesand is the speciﬁc part of the red blood cellthat carries oxygen. Hemoglobin alsocontains iron.
Special cells in the bone marrow pro-
duce erythrocytes. Several vitamins, suchas vitamin B12 and folic acid (which is part
of the vitamin B complex), are necessaryfor the formation of erythrocytes. They areobtained from the diet. Iron, which is alsoobtained from the diet, is important forthe formation of hemoglobin. Excessamounts of iron and vitamin B12 arestored in the liver.
New red blood cells are constantly being
formed. Although most erythrocytes arereleased into the blood, some are taken upby the spleen to be stored for emergencyuse when the red blood cell count dropssigniﬁcantly below normal levels, such asduring hemorrhage . Newly formed red
blood cells enter the bloodstream beforethey are totally mature. At this stage, theyare called reticulocytes . Within several
days, the cells mature to become erythro-
cytes. The life cycle of erythrocytes is
approximately 120 days. As the erythro-cytes reach the end of their life cycle, theybecome more fragile and rupture. Some ofthe old erythrocytes are destroyed in thespleen . Special cells within the spleen and
liver absorb the old erythrocytes, makingroom for more new cells.
A decrease in the quantity of oxygen
supplied to the tissues results in anincrease in the number of red blood cellsproduced. For example, at higher alti-tudes, where less oxygen is available in theair, the bone marrow reacts by producingmore red blood cells, even if there is anadequate number of red blood cells in thecirculation.Normal Structure and Function ofWhite Blood Cells and Immunity
The immune system is a complex organ-
ization of specialized cells and organs thatdistinguishes between self and nonself,defending the body against “foreign” in-vaders. Although the body is exposed toa number of microorganisms each day, theimmune system helps it ﬁght off bacteria,viruses, and other microbes. Althoughconstantly bombarded by microorganismsor trauma that can result in infection, dis-ease, or injury, the body has speciﬁc de-fenses to protect it against such invasions.
The immune system has traditionally
been divided into innate and adaptive com-
ponents, each with a different role and
function (Medzhitov & Janeway, 2000). The
body’s ﬁrst line of defense against foreignmaterial is called nonspeciﬁc or innate im-
munity . This type of immunity includes the
protection provided by the skin, whichacts as a barrier to organisms, and by themucous membranes, gastric secretions,and tears, which contain special chemicalsthat destroy potentially harmful organ-isms. Innate immunity requires no previ-ous exposure to the foreign substance orrecognition of any speciﬁc properties ofthe foreign material. When, despite exter-nal and chemical barriers, an organism orother foreign material gains entry into the
body, an inﬂammatory response results (the
adaptive component ). The main purpose of
the inﬂammatory response is to bringphagocytes (cells that destroy and ingest
foreign material) to the area to destroy orinactivate the foreign substances so thatthe repair of tissue can begin.
Also important to the body’s defense is
a circulatory system called the lymphatic
system . The lymphatic system is a circula-
tory system separate from the general cir-culation and consists of lymph vessels ,
circulating lymph ﬂuid (clear ﬂuid that232
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

Normal Structure and Function 233
bathes the body’s tissues), and lymph nodes
(small glands of the immune system thatare located throughout the body and actas ﬁlters). Lymph nodes also serve as tem-porary storage reservoirs for lymphocytes
(white blood cells that ﬁght infection)and, with appropriate stimulus, as manu-facturers of lymphocytes.
The lymphatic system is crucial to
the body’s defense against invading organ-isms and other foreign substances. Itdepends on muscle movement to circulatethe ﬂuid within it. Other organs and tis-sues important to the body’s defense arethe spleen , thymus , and bone marrow .
Located in the upper left quadrant of theabdomen, the spleen is an organ com-posed of tissue that disposes of worn-outblood cells and lymphatic tissue thatﬁlters blood and helps to trap and destroymicroorganisms. The thymus, which liesin the upper portion of the chest, is alymphoid organ that produces a hormoneimportant in controlling the developmentof lymphocytes. Bone marrow also pro-duces lymphocytes and, consequently, isalso classiﬁed as a lymphoid organ.
White blood cells ( leukocytes ), formed
in the bone marrow, have the predomi-nant role in the body’s defense system.Leukocytes take action when body tissueshave been damaged or invaded by organ-isms or other foreign material. Any infec-tion or invasion by foreign substancescauses a dramatic increase in the numberof white blood cells in the blood. A typeof leukocyte called a phagocyte is a scav-
enger that ingests bacteria and foreign par-ticles. Phagocytes consist of microphages ,
which ingest bacteria, and macrophages ,
which ingest dead tissue. The process ofingesting cells and foreign objects iscalled phagocytosis .
In addition to phagocytosis activity,
white blood cells ﬁght infection through a
process called acquired immunity (the abil-ity of cells to recognize an organism to
which there has been previous exposure and
to neutralize or destroy the invading organ –
ism). This is part of the adaptive component
of the immune system. Lymphocytes arewhite blood cells formed by the lymphnodes, spleen, thymus, and sometimes thebone marrow. They circulate throughout
the bloodstream and the lymphatic system
and are important to acquired immunity.
The two major types of lymphocytes are B
lymphocytes and T lymphocytes. B lymph-
ocytes migrate to lymphoid tissue, such asthe lymph nodes and spleen. When theyare exposed to a foreign substance ( anti-
gen), they produce special substances
called antibodies (immunoglobulins) that
enter the bloodstream to lock with theantigen and destroy it. This type ofimmune response is called humoral immu-
nity. Antibodies do not penetrate cells, but
rather interact with circulating antigens.T lymphocytes are the regulators and con-trollers of the immune system. When Tlymphocytes are exposed to an antigen,rather than producing antibodies, theyreact to the antigen directly, attackingbody cells that have been invaded by theforeign substance or malignancy. Thisresponse is called cellular immunity .
T cells have different subsets of cells that
behave differently. Regulatory T cells ( helper/
inducer cells ) help to coordinate the im-
mune response, helping activate B cells tomake antibodies against antigens and acti-vating other T cells, natural killer cells , and
macrophages . These helper cells contain
markers (T4/CD4) that recognize speciﬁc
types of antigens. Other types of T cellsdeactivate or suppress B-lymphocyte andother cell activity when appropriate anti-body levels have been reached. Normally,helper cells outnumber suppressor cells2:1. Some T lymphocytes become memo-ry cells so that if the speciﬁc organisminvades the body again, it is “remem-

bered” and the immune response is moreintense. Another type of T cell, the killer
cellor cytotoxic T cell, carries the T8 mark-
er. Killer cells, in addition to working torid the body of infected cells, are alsoresponsible for the rejection of grafts ortransplants. Lymphocytes called natural
killer cells target tumor cells as well as oth-
er infectious organisms.
Cells carry markers ( allogens ) to ensure
that the body recognizes its own tissue asself and not foreign. Sometimes the im-mune system becomes unable to recognizethe body’s own tissue and begins to pro-duce antibodies and T cells that attack the
body’s own cells as if they were foreign sub-
stances; in these cases individuals are saidto have an autoimmune disease . Examples
of autoimmune diseases are systemic lupus
erythematosus and rheumatoid arthritis (see
Chapter 14).
A variety of conditions can alter the
body’s immune response and leave indi-viduals more susceptible to disease. Be-cause it is necessary to suppress the im-
mune system of individuals who are about
to receive an organ transplant to preventrejection of the donor tissue, these indi-viduals are more prone to infections.Individuals with certain types of cancers,such as lymphoma and leukemia (seeChapter 16), may become immunodeﬁcient
and develop serious infections. Overuse orabuse of narcotics or steroid drugs can alsoalter the immune response.
Normal Structure and Function ofPlatelets and Coagulation
The term hemostasis refers to a series
of events that stop bleeding from dam-aged vessels. Platelets are involved in the
important ﬁrst step in preventing exces-sive bleeding after an injury. Formed byspecial cells in the bone marrow, plateletsare the smallest of the cells in the blood.They are disk-shaped and contain nohemoglobin but are concerned with theclotting of blood. When injury occurs, thewalls of the blood vessels contract, andplatelets adhere to the site of the injury.They release a special substance thatcauses other platelets to collect at the site;thus, they “plug” injured blood vessels tostop the bleeding momentarily.
Platelets alone cannot stop the bleeding
indeﬁnitely. The formation of the plug ac-tivates clotting factors (coagulation factors
from the liver, plasma, and other sources) so
that a clot forms to control the bleeding.
There are intrinsic and extrinsic blood clotting
factors , most named by Roman numerals
designated from I to XIII, in which differ-
ent sets of substances play major roles. Vita-
min K is necessary for the formation of
some clotting factors. To prevent excessiveclotting, other body mechanisms are alsoactivated. For example, basophils (a type of
white blood cell) are thought to havesome role in stopping the coagulationprocess once the bleeding is under control.
CONDITIONS AFFECTING THE BLOODOR IMMUNE SYSTEM
The term blood dyscrasias is used to
describe a large group of disorders thataffect the blood. Disorders of the blood orblood-forming organs may arise from anumber of different sources; may be man-ifest in a number of different ways; andmay involve abnormalities of erythro-cytes, leukocytes, platelets, or clottingmechanisms. These disorders may becharacterized by the overproduction of cells ,
the underproduction of cells , or defects in the
clotting mechanism .
Anemia
Although not thought of as a disability
per se, anemia is associated with a num-234 CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

ber of chronic illnesses and disabilities andalso is a side effect of many conditions andtheir treatment. Anemia may be a compli-cation associated with cancer or cancertreatment (Bokemeyer & Foubert, 2004),a symptom of dietary deﬁciency (Stabler& Allen, 2004), a symptom of gastrointes-tinal disorders, or a side effect of treatmentof gastrointestinal disorders (Andres,Loukili, Ben, & Noel, 2004; Bodemar,Kechagias, Almer, & Danielson, 2004).Fatigue is a major symptom of anemia andcan be debilitating, reducing individuals’ability to work, decreasing their physicaland emotional well-being, and interferingwith their cognitive ability, all of whichcan lead to anxiety and depression(Bokemeyer & Foubert, 2004).
Conditions that fall under the general
term anemia are characterized by a reduc-
tionin the amount of hemoglobin or the
number of red blood cells . Anemias are
sometimes classiﬁed by the size and col-or of the red blood cell. For example,healthy, normal-sized cells are called nor-
mocytic ; normal cells that are of normal
color are called normochromic . Anemias in
which the color of the red blood cells ispaler than usual are called hypochromic
anemias . Anemias in which the red blood
cells are larger than usual are calledmacrocytic anemias , and those with cells
smaller than usual are called microcytic
anemias .
Anemias may also be classiﬁed accord-
ing to their causative mechanisms. Forexample, anemias may result from anexcessive blood loss, from decreased orabnormal red blood cell formation, orfrom the destruction of red blood cells.Destruction of red blood cells is calledhemolysis . Anemias caused by excessive
and/or premature destruction of red bloodcells are called hemolytic anemias . A vari-
ety of abnormal conditions can cause redblood cell destruction. Hemolytic anemiamay occur in association with some infec-tious diseases or with certain inherited redblood cell disorders, or it may develop asa response to drugs or other foreign or tox-ic agents. Anemia results when the rate ofdestruction is greater than the ability ofthe body to produce red cells. The degreeof anemia reﬂects the ability of the bonemarrow to increase the production of redblood cells. The spleen usually becomesenlarged ( splenomegaly ) in chronic hem-
olytic conditions because of the need toremove an excessive number of damagedred cells.
Aplastic anemia (sometimes called pan-
cytopenia ) is caused by inadequate func-
tioning of the bone marrow in manu-facturing red blood cells. Aplastic anemiacan occur spontaneously, or it can resultfrom damage to the bone marrow throughdrugs, chemicals, or ionizing radiation.
Iron deﬁciency anemia , one of the most
common types of anemia (Shah, 2004), isoften caused by a deﬁciency of iron in thediet. It can also result from the body’s fail-ure to absorb iron, excessive or chronicblood loss, or increases in the body’s ironrequirements.
Pernicious anemia is a chronic condition
caused by the inadequate secretion by thestomach of a substance ( intrinsic factor )
that is necessary for the intestine to absorbvitamin B12. It may also be caused bydietary deﬁciency of vitamin B12, especial-ly in vegetarians (Stabler & Allen, 2004).The deﬁciency of vitamin B12 impairs pro-duction and maturation of blood cells.Consequently, the body is unable to pro-duce adequate numbers of red blood cells,resulting in anemia.
Regardless of the cause, anemia disrupts
the transport of oxygen to tissues through-out the body. Severe anemia increases theworkload of the heart. Common symp-toms of anemia are pale skin ( pallor ),
weakness, fatigue, difﬁculty in breathingConditions Affecting the Blood or Immune System 235

(dyspnea ), and fast heart rate ( tachy-
cardia ). Other possible symptoms of
anemia include the inability to concen-trate, irritability, and susceptibility toinfection.
Treatment must be speciﬁc to the cause.
If anemia is the result of blood loss, bloodreplacement through transfusion may be
necessary. In other instances, dietary, vita-
min, or iron supplements may be necessary.
Thalassemia
The thalassemias are inherited hemolyt-
ic anemias common in individuals from
the Mediterranean region, Africa, theMiddle East, India, and Southeast Asia(Olivieri, 1999). Thalassemias are charac-terized by the production of thin, fragilered blood cells and defective hemoglobinsynthesis. As a result, the hemoglobincontent of the red blood cells is inade-quate. In addition, there is often someinterference with erythrocyte metabo-lism that causes the red blood cells to bedeformed and decreases their survivaltime. Thus, the anemia associated withthalassemia can result both from theincreased destruction of red blood cellsand from the impaired production ofhemoglobin.
Symptoms of thalassemia are similar to
those of other types of anemias. Indi-viduals whose symptoms are severe andconsequently diagnosed early in life mayrequire regular transfusions to survive(Olivieri, 1999). Even individuals with amild form of the condition may experi-ence complications, such as osteopenia
(decreased bone density) in later age. Inaddition to transfusion therapy, ironchelation therapy is often necessary toprevent iron overload (Rodgers, 2000),and in some instances bone marrowtransplantation may be indicated(Giardini, 1997). Polycythemia
In polycythemia, there is an increase in
the number of red blood cells and in theconcentration of hemoglobin within theblood. There are several types of poly-cythemia. One type, polycythemia vera , is
associated with an overproduction ofboth red and white blood cells. The causeof polycythemia vera is unknown. Becauseof the increased number of cells in the
blood, individuals with this condition may
experience hypertension, congestive heartfailure, stroke, or heart attack (see Chapter11), or they may experience a hemorrhagebecause the congestion in the blood ves-sels may cause the vessels to rupture.
Secondary polycythemia occurs in con-
junction with another disease. When thebody’s demand for oxygen increases, thebone marrow produces additional redblood cells to meet the increased demand.Chronic obstructive pulmonary disease isa condition in which secondary poly-cythemia may occur (see Chapter 12).Treatment focuses on the underlying con-dition.
Individuals with conditions in which
there has been a loss of plasma without aloss of red blood cells, such as burns, maydevelop a state similar to polycythemia.Although there is no actual increase in thenumber of red blood cells, loss of ﬂuidincreases the proportion of red blood cellsin the blood. In these cases, treatmentinvolves ﬂuid replacement to decrease theviscosity of the blood.
Agranulocytosis (Neutropenia)
Agranulocytosis is the marked reduc-
tion in the level of a speciﬁc type of leuko-cyte. This reduction in leukocytes is calledleukopenia . A common cause of agranu-
locytosis is toxic reaction to certain medica-
tions used in the treatment of chronic236
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

disorders, such as medications used totreat epilepsy or medications used to treat
certain mental disorders . Agranulocytosis
may also result from exposure to certain
chemicals or to ionizing radiation .
Because white blood cells are important
in ﬁghting infection, a reduction in thenumber of these cells increases individu-als’ susceptibility to infection. Agranulo-cytosis is a potentially serious conditionand, without prompt treatment, can resultin death. Treatment is directed towardremoving the toxic agent responsible andproviding medications (e.g., antibiotics) totreat resulting infections.
Purpura
Purpura is a condition characterized by
hemorrhage of small blood vessels intothe skin. Small amounts of blood leak intovarious tissues of the body. It can becaused by an allergic response or a deﬁ-ciency in platelets, or it can be associatedwith other disorders in the body.
Leukemia
The leukemias are caused by the cancer-
ous production of lymph cells or whiteblood cells. They are discussed in greaterdetail in Chapter 16.
Hemophilia
Hemophilia is an inherited, potentially
disabling condition associated with highﬁnancial costs (Beeton, 2002). Severalinherited blood disorders make up thecondition known as hemophilia , a chron-
ic bleeding disorder in which there is adeﬁciency in or absence of one of the clot-ting factors (Bolton-Maggs & Pasi, 2003).Individuals with hemophilia have a bleed-ing tendency. Although they do not ini-tially bleed faster, the normal clottingmechanism is disturbed so that bleedingis prolonged or the oozing of blood maypersist after injury. Because the plateletcount is normal in hemophilia, bleedingfrom a small cut or scratch does not posea severe problem. However, the deﬁcien-cy in clotting factors does pose the dan-ger of bleeding into the internal organs,joints, or the brain.
Hemophilia is transmitted from moth-
er to son. Women do not develop the dis-
ease, but they can inherit the trait and pass
it to their sons. If a woman is a carrier,each of her sons has a 50 percent chanceof developing hemophilia; each of herdaughters has a 50 percent chance of be-coming a carrier. None of the sons of a
man with hemophilia will have hemophil –
ia; however, all of his daughters will becarriers.
There are several different types of
hemophilia, which are differentiated bythe speciﬁc clotting factor that is deﬁcient.The most common type is hemophilia A ,
also known as classical hemophilia . In this
type of hemophilia, a protein in clotting
Factor VIII is deﬁcient. The next most com-
mon type is hemophilia B , also called
Christmas disease , in which clotting Factor
IXis defective. The rarest type of hemo-
philia is von Willebrand’s disease , in which
Factor VIII manifests platelet dysfunction
(Bolton-Maggs & Pasi, 2003).
The severity of hemophilia varies along
a continuum from a tendency toward slow,
prolonged, persistent bleeding to a ten-dency toward severe hemorrhage, and itis categorized as mild, moderate , or severe
depending on the level of clotting factorpresent. Individuals with mild hemophilia
will probably experience abnormal bleed-ing only after major injuries or minor sur-gery, such as a tooth extraction. Individ-uals with moderate hemophilia may have
prolonged bleeding after major trauma or
surgery. Individuals with severe disease mayConditions Affecting the Blood or Immune System 237

bleed spontaneously and have hemor-rhages into deep muscles and joints.
Bleeding into the joint ( hemarthrosis )
is extremely painful and can cause signiﬁ-cant joint destruction (Elander & Barry,2003; Shapiro & Hoots, 2000). Knees andankles are affected most frequently, al-though elbows may become involved lat-er. Joint deformity and crippling mayresult from damage to the joint structureand from atrophy (wasting) of surround-
ing muscles. Bleeding into the muscle, ifsevere, may exert pressure on nerves and
cause a temporary sensory loss. If the hem-
orrhage damages muscle tissue, ﬁbrous tis-sue may form, causing varying degrees offunctional loss.
Treatment for Hemophilia
Hemophilia is not curable and requires
treatment for bleeding problems through-out the individual’s life. With proper careand treatment, however, individuals withhemophilia can manage their chronic dis-ease, and their life expectancy approach-es normal (Teitel et al., 2004).
To prevent damage from abnormal bleed-
ing, signiﬁcant blood loss, and chronicjoint disease, all bleeding must be detect-ed early and treated promptly. There areover 100 comprehensive hemophilia treat-ment centers throughout the country thathelp individuals with hemophilia managetheir condition physically and psycholog-ically. These care centers emphasize earlyintervention for bleeding episodes andtrain individuals to administer replace-ment therapy at home, thus markedlyimproving both school attendance andthe amount of absences from work (Teitelet al., 2004).
Because there is no cure for hemophil-
ia, treatment is directed toward prevent-ing any injury that could precipitatebleeding and toward controlling bleedingepisodes when they do occur. The main-stay of treatment for hemophilia is re-placement therapy with plasma or plasmaconcentrates that contain the clotting fac-tors in which the individual’s blood is deﬁ-cient. Because of the higher concentra-tions of clotting factors, plasma concen-trates are given more frequently than isfresh plasma. Clotting factors are usuallyreplaced through intravenous infusion(infusing substance directly into a vein).The amount, type, and duration of theinfusion depend on the individual’s clot-ting deﬁciency and the size and severityof the bleeding problem. Treatment maybe instituted prior to surgery to preventexcessive bleeding.
Early treatment of bleeding helps to pre-
vent complications. Consequently, learn-ing to administer clotting factor concen-trates at home is beneﬁcial. To do so, how-ever, individuals must be able to calculatethe appropriate dose and mix and admin-ister the concentrate intravenously. Hometherapy is appropriate for mild bleedingbut is not sufﬁcient when major bleedingoccurs. Major bleeding requires medicalevaluation.
There are possible complications associ-
ated with replacement therapy. As with alltherapies that involve intravenous infu-sion, there is the chance of the transmis-sion of infection such as hepatitis andhuman immunodeﬁciency virus (HIV)(Parish, 2002). Needles and equipmentshould always be sterile and never shared.Although most blood products are nowcarefully screened for disease, individualswho received replacement therapy before1985 may already have been exposed toHIV , which can be a persistent source ofanxiety and concern. Individuals whoreceive blood products intravenously canalso develop an allergic reaction to the in-fusion. Such reactions should be reportedto a physician promptly.238
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

Individuals with bleeding into a joint
may require joint immobilization for sev-eral days in addition to replacement ther-apy. Joint pain may be treated with anti-inﬂammatory medications and analgesics.Medications that contain aspirin shouldbe avoided, however, because aspirininterferes with platelet function and cancause increased susceptibility to bleeding.Physical therapy or prescribed exercise car-
ried out at home may be necessary to main –
tain the range of motion of the affectedjoints. If the joints undergo severe degen-eration, reconstructive orthopedic surgery,such as joint replacement, may also benecessary (see Chapter 14). Individualswith hemophilia should always wear aMedic Alert identification bracelet ornecklace to alert others to their conditionin case of an emergency.
Psychosocial Issues in Hemophilia
How individuals respond to having
hemophilia as adults is dependent to agreat extent on their experiences with thedisease during childhood. Because hemo-philia is present from birth, the attitudesdisplayed by parents and signiﬁcant oth-ers during individuals’ development havea signiﬁcant impact on their self-view andview of their condition. If parents are over-protective, individuals’ social and psycho-logical development may be stunted anddependency may result. Likewise, individ-uals may not have had the opportunity toparticipate in sports or other activities inwhich physical and motor skills arelearned and mastered. If many hospitaliza-tions are required because of the conditionand individuals have had multiple schoolabsences, their educational achievementmay also be lower than that of others ofthe same age.
In some instances, the inability to pre-
dict when bleeding may occur or fear ofbeing unable to control bleeding mayresult in passivity and inactivity. At oth-er times, if individuals have had difﬁcul-ty adjusting to the condition, are uncer-tain about the future, or deny the serious-ness of hemophilia or the precautions thatneed to be taken to control it, they mayindulge in excessive risk taking.
Although replacement therapy and home
treatment have done much to improvethe lives of individuals with hemophiliaand to decrease the disability resultingfrom the condition, treatments are veryexpensive. This expense may be an addi-tional source of stress.
Individuals with hemophilia may expe-
rience both acute and chronic pain if therehas been bleeding into the joints. Conse-quently, pain medications are frequentlyused. If medications are not carefully usedand monitored, drug dependence mayresult, sometimes necessitating drug reha-bilitation. Individuals who have not ad-justed well to their condition and mayself-medicate to alleviate emotional dis-comfort may be at particular risk.
Sexual issues may also be of concern for
individuals with hemophilia. The mostserious complication of replacement ther-apy is the possibility of being exposed toHIV through contaminated concentrates.Although precautions have now been tak-en in the preparation of concentrates tomake infection with HIV rare, individualswho were infused prior to 1985 had muchgreater chances of being exposed to HIV.For those individuals who have HIV as aresult of replacement therapy, not onlythe stress and anxiety produced by hav-ing the disease, but also the anger, resent-ment, and depression because of beingexposed to HIV may complicate individ-uals’ ability to cope with the disease, aswell as their ability to establish or main-tain sexual relationships. Some individu-als may feel stigmatized by their condi-Conditions Affecting the Blood or Immune System 239

tion, especially the public’s awareness ofthe link to HIV , and attempt to hide theircondition. Even in the absence of HIVconcerns, complications of hemophiliamay affect sexual activity. Joint disability,medication side effects, and other compli-cations can interfere with sexual function(Parish, 2002).
Hemophilia is a hereditary condition.
The potential impact on long-term rela-tionships and the decision of whether tohave children may be troublesome forindividuals with hemophilia. In someinstances, they may avoid developingclose, meaningful relationships because oftheir discomfort with having a hereditarycondition.
Vocational Issues in Hemophilia
Improved medical technology and the
availability of self-infused coagulation fac –
tors have greatly increased the ability ofindividuals with hemophilia to decreasetheir disability and maintain employmentin a variety of settings (Teitel et al., 2004).Individuals with severe hemophilia may
also be able to perform a variety of job tasks
without limitations; however, in severedisease, there is the increased unpredicta-bility of when the bleeding will occur.
When bleeding does occur, individuals
should be able to self-infuse the concen-trates in 15 to 30 minutes; however, theywill need to take a break from work to per-form the replacement therapy. A semipri-vate place to perform the infusion, as wellas a place to store the equipment and con-centrate, will also be needed.
Usually, individuals with hemophilia
have few functional limitations in thevocational setting, unless they experiencejoint complications. Especially those withmoderate to severe disease should avoidemployment in which there is a directthreat of physical injury. Injuries that maybe minimal by most standards can haveserious implications for individuals withmore severe forms of hemophilia. Jointdamage and/or subsequent joint replace-ment due to complications of hemophil-ia may impose the same limitations as do
joint disorders from other causes. In some
instances, surgical correction of damagedjoints may be indicated (see Chapter 14).For the most part, however, the vocation-al functioning of individuals with hemo-philia is determined primarily by theirabilities and interests.
One barrier to employment may be lack
of understanding on the part of the em-ployer about the few limitations that areactually associated with hemophilia. Be-cause the public has now connectedhemophilia and the potential for HIV con-tamination, there may be fear and anxi-ety from coworkers when working withindividuals with hemophilia, especially ifbleeding occurs. Likewise, coworkers whoobserve individuals administering self-infusing concentrates and who do notunderstand replacement therapy maydraw false conclusions about the activity,causing further discrimination. Educatingemployers and coworkers about hemo-philia and its treatment may be one of themost crucial links to vocational success forthe individual with hemophilia.
Sickle Cell Disease
Sickle cell disease is a heredity condi-
tion that occurs primary in individuals ofblack African descent, but it can also occurin those with Mediterranean ancestry.Sickle cell disease occurs because of a gene-
tic mutation of hemoglobin. Normal hemo –
globin is called hemoglobin A . Individuals
with sickle cell disease have an abnormalhemoglobin called hemoglobin S , and the
disease gets its name from the shape thatthe red blood cell assumes when hemoglo-240
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

bin S is present. Hemoglobin S is protective
against malaria and so is more prevalentin areas of the world where there is malar-ia (Lewing & Woods, 2000).
When hemoglobin S is present, individ-
uals are said to have sickle cell trait . Indi-
viduals may only be carriers of the abnor-
mal gene that causes the hemoglobinabnormality. Although carriers have nosymptoms, they may pass the abnormalgene to their offspring. The offspring of anindividual with sickle cell trait and anindividual with normal genes have a 50percent chance of being carriers of theabnormal gene. When both parents have
the sickle cell trait, the chances with eachpregnancy are 1 in 4 that the baby willhave normal hemoglobin, 2 in 4 that thebaby will have sickle cell trait, and 1 in 4that the baby will have sickle cell anemia(Wang, Grover, & Gallagher, 1993). Chil-dren of one parent with sickle cell anemiaand one with normal hemoglobin will allhave sickle cell trait. If one parent has sick-le cell trait and one has sickle cell anemia,there is a 50 percent chance with eachpregnancy that the child will have sicklecell trait and a 50 percent chance that thechild will have sickle cell anemia. Whenboth parents have sickle cell anemia, sowill all of their children (Lukens, 1993).
Sickle Cell Anemia
Sickle cell anemia is the most severe form
of sickle cell disease. It is characterized bylifelong hemolytic anemia and a widevariety of painful and debilitating vaso-occlusive events (Mentzer & Kan, 2001).When hemoglobin S molecules interactwith each other, they become stacked up,especially when the oxygen concentrationin the blood is low. The red blood cellsbecome deformed so that, instead of beingdisk-shaped, they assume the abnormalshape of a crescent or sickle. Because ofthis distortion, the red blood cell becomesrigid and is unable to adapt its shape toﬁt through tiny blood vessels. The abnor-mal sickled cell becomes very fragile andis easily destroyed, which severely curtailsits normal life span. As a result, the bonemarrow drastically increases its produc-tion of red blood cells to keep up with therate of destruction. Because the rate of pro-duction cannot keep up with the rate of
destruction, however, individuals with sick-le
cell disease can become severely anemic,
thus developing sickle cell anemia .
Manifestations of Sickle Cell Disease
Individuals with sickle cell disease have
an unpredictable course that can range from
mild to severe (Cooper-Effa, Blount, Kaslow,
Rothenberg, & Eckman, 2001). There may
be varying physical limitations from sick-le cell disease at each developmental stage(Westerdale & Jegede, 2004). The growth
and development of individuals with sickle
cell anemia are signiﬁcantly impaired, al-though the exact way the disease contri-butes to delayed growth is still unclear.Although there is delay in physical and
sexual maturation, individuals do reach full
maturity (Scott & Scott, 1999).
Sickle cell crisis is a manifestation of sick-
le cell disease that occurs when blood ﬂowto a body part becomes obstructed byrigid, sickled red cells. This is called a vaso-
occlusive crisis . The affected body part does
not receive adequate oxygen, resulting insevere pain. If blood ﬂow is severelydiminished, the affected tissue may under-go necrosis (tissue death). Any part of the
body, including organs, may be affected;the resulting damage may be mild to
severe, depending on the degree and length
of blockage. The most common body partsaffected are the chest, legs, arms, back, andabdomen. Sickle cell crisis can result instroke (Gebreyohanns & Adams, 2004);Conditions Affecting the Blood or Immune System 241

cardiovascular dysfunction, includingmyocardial infarction (heart attack)(Assanasen, Quinton, & Buchanan, 2003);chronic lung disease; or kidney failure(Lewing & Woods, 2000).
Individuals with sickle cell disease have
multiple bouts of sickle cell crisis duringtheir lives, which lead to chronic organdamage. If the spleen is repeatedly in-volved in crisis, it may become signiﬁcant-ly enlarged and removal of the spleen(splenectomy ) may be indicated. A con-
dition called aplastic crisis , or megaloblas-
ticcrisis, may also be a manifestation of
sickle cell disease. In this condition, thereis a rapid onset of anemia so that bloodtransfusions are also indicated.
Because of the lowered resistance due to
anemia, as well as the altered spleen func-tion, serious infections may also become
problems. Associated chronic anemia caus-es
the heart to pump faster in an attempt
to supply additional oxygen to the tissues.Increased heart action can contribute toenlargement of the heart ( cardiomegaly )
and decreased cardiac efficiency. De-creased oxygen supply caused by thechronic anemia can also produce symp-toms of fatigue and difﬁculty in breathingon exertion ( exertional dyspnea ).
Occlusion of blood ﬂow during a sickle
cell crisis can damage bones and joints,leading to pain, swelling, and limitedmobility of the joints, as well as resultingdeformity. Occlusion of vessels in thebrain can cause a stroke (see Chapter 2).Increased blood viscosity (thickness) may
also cause sickle cell retinopathy in
which there is damage to the vessels in theretina of the eye, which results in dimin-ished vision and, possibly, retinal detach-ment (see Chapter 4).
Some individuals develop leg ulcers
because of the interruption of circulationduring sickle cell crisis. Ulcers may notheal and may become infected, necessi-tating bedrest and, in some instances, skingrafts.
The speciﬁc causes of sickle cell crisis are
unknown; however, certain factors, suchas heavy exertional stress (Assanasen et al.,2003), mental stress, infection, dehydra-tion, or extremes in temperature, may pre-cipitate a crisis (Lewing & Woods, 2000).
The prognosis of individuals with sick-
le cell anemia is dependent on the indi-vidual and the degree of organ damage. Inthe past, many individuals with sickle cellanemia did not live to adulthood, butmany now reach midlife and beyond, liv-ing productive lives (Cooper-Effa et al.,2001; McKerrell, Cohen, & Billett, 2004).The prediction of outcome is individual-ly determined.
Diagnosis of Sickle Cell Disease
Deﬁnitive diagnosis of sickle cell disease
or sickle cell trait is based on a blood test
called hemoglobin electrophoresis . Blood tests
such as sickle cell prep are screening tests
that can detect the presence of abnormalhemoglobin but cannot distinguish be-
tween sickle cell disease and sickle cell trait.
Treatment for Sickle Cell Disease
For the most part, sickle cell disease is a
chronic, lifelong disease without cure.Treatment is directed toward controllingits symptoms. Good nutrition is essentialto combat anemia and to maintain thebody’s resistance to infection. Because ofthe propensity of those who have sicklecell anemia to develop infections,prophylactic antibiotics may be given onoccasion.
Maintaining adequate ﬂuid intake is
also important for individuals with sicklecell disease, because adequate hydrationcan minimize the sickling of red bloodcells and decrease the blood viscosity. The242
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

anemia associated with this conditionsometimes necessitates transfusion thera-py. A medication used to treat cancer,hydroxyurea, has recently been tested foruse in the treatment of sickle cell anemiaand has been found to prevent sickling ofred blood cells in some individuals(Lewing & Woods, 2000). Other therapies,including the use of antisickling agentsand bone marrow transplantation, mayalso be used in some instances.
Individuals with sickle cell disease who
experience sickle cell crisis often requirehospitalization. During the crisis, treat-ment focuses on restoring ﬂuids if dehy-dration has occurred, and relieving thepain associated with the crisis, whichusually requires the administration of nar-cotics. If another condition, such as an in-fection, has precipitated the crisis,treatment of that condition may also beinstituted. Organ damage as a result ofsickle cell disease is treated in a similarfashion to chronic organ disease from oth-er causes.
Comprehensive medical care, preventa-
tive care, and health maintenance havebeen shown to increase the life expectan-cy of individuals with sickle cell disease.Care should be taken to avoid any factorsthat precipitate a sickle cell crisis.
Psychosocial Issues in
Sickle Cell Disease
Like hemophilia, sickle cell disease usu-
ally manifests itself in childhood, neces-sitating medical attention and, possibly,frequent hospitalizations, which can dis-rupt social development and educationalprogress. Psychological coping patterns arerelevant both to the experience of painand to broader adjustment issues (Anie,Steptoe, & Bevan, 2002). Coping with sick-le cell disease may be especially difﬁcultduring adolescence as individuals struggleto maintain a “normal” life and minimizetheir difference from peers (Atkin &Ahmad, 2001). Adherence to prophylac-tic measures may be especially difﬁcultand require signiﬁcant support (While &Mullen, 2004).
Because it is a hereditary disease, parents
of children with sickle cell disease mayexperience guilt or fear of the loss of theirchild. As a result, they may become over-ly protective, promoting abnormal depen-dence in the child. At the same time, thechild may learn manipulative behaviors togain attention. These maladaptive meansof coping may persist throughout life, cre-ating a greater barrier than the conditionitself.
Sickle cell disease carries the addition-
al stress of unpredictability. Althoughsome of the factors that provoke a sicklecell crisis may be identiﬁable, crises areoften unpredictable and beyond individ-uals’ control. Not only are the crises pain-ful and debilitating, but there is also thepotential for organ damage each time acrisis occurs. The lack of control over thefrequency or severity of sickle cell crisescan lead to feelings of hopelessness anddepression.
Individuals with sickle cell disease can
usually maintain regular schedules; how-ever, the onset of sickle cell crises is unpre-dictable and may necessitate hospitali-zation. In most instances, individuals withsickle cell disease do not need to alteractivities, unless activities appear to pro-voke a sickle cell crisis. Most activities, ifperformed in moderation, can be tolerat-ed. The unpredictability of sickle cell crisescan alter social functioning for individu-als with sickle cell disease, who may haveto cancel or alter plans at the last minuteif a crisis should occur. The role of stressas a precipitating factor in sickle cell cri-sis must also be considered. Althoughstress is frequently associated with nega-Conditions Affecting the Blood or Immune System 243

tive events, stress can also be associatedwith positive events, such as a graduationcelebration or a wedding.
Vocational Issues in Sickle Cell Disease
Individuals with sickle cell disease must
consider not only the physical demandsof the job as related to stamina, but alsothe role that strenuous exertion has in pre-cipitating sickle cell crises. Because sicklecell disease is a lifelong condition, mostindividuals learn, over the years, whichtype of activity and how much activitythey can usually tolerate. Despite thepotential relationship of overexertion andsickle cell crisis, most individuals withsickle cell disease are capable of perform-ing moderate and, in some instances, evenheavy work.
Individuals who have experienced speci-
ﬁc organ or joint damage as a result of re-peated sickle cell crises have many of thesame limitations as those who have simi-lar conditions for other reasons. In addi-tion, individuals with sickle cell diseaseshould avoid extremes in temperature.Very hot weather places extra strain on theheart and predisposes to dehydration,which can precipitate a crisis. Very cold,damp environments can also precipitatea crisis. Consequently, it may be beneﬁcialfor individuals with sickle cell anemia towork in indoor or controlled environ-ments.
Stress in the work environment and its
contribution to the development of sick-le cell crisis is another factor individualswith sickle cell anemia must consider. Notall individuals react to stress in the sameway, nor are perceptions of stress alwaysthe same. Consequently, the importanceof stress must be determined on an indi-vidual basis. The degree to which absencesdue to sickle cell crises become a hin-drance to work performance is dependenton the individual, the frequency, and theseriousness of the crises when they occur.
Human Immunodeﬁciency Virus (HIV)Infection
Not all viruses are harmful to humans,
but some viruses can cause disease.Diseases that result from viruses rangefrom the common cold and commonchildhood illness to more serious diseases,such as poliomyelitis and acquiredimmune deficiency syndrome (AIDS).
A virus can be deﬁned as an infectious
organism that cannot grow or reproduceoutside living cells. In order to survive, avirus must enter a living cell and use thereproductive capacity of that cell for itsown replication. Consequently, when avirus enters a cell, it instructs the cell toreproduce the virus. Normally, the bodyrecognizes viruses as foreign and activatesthe immune system to attack and destroythe offending agent. Of those viruses thatare not destroyed, some remain inactive(dormant ) for long periods without caus-
ing problems; however, they remain inte-grated within the genetic material of thecell, and they are capable of replicatingwhen triggered to do so. The direct dam-age the virus does to the cell itself mayvary from slight damage to total destruc-tion. Some cells are able to reproduce afterbeing damaged, but others, especiallythose of the nervous system, are not ableto reproduce and, consequently, are notreplaced after invasion by a virus.
HIV infection is caused by a virus called
the human immunodeﬁciency virus . It is
called a retrovirus because it uses a compli-
cated process called reverse transcription .
This process uses a viral enzyme calledreverse transcriptase to integrate its genet-
ic material into the genetic material ofother cells. In so doing, the HIV essential-ly takes over other cells and makes them244
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

produce other infected cells, each ofwhich is slightly different.
There are two viruses that cause HIV
infection: HIV-1 and HIV-2. HIV-1 is themost common and is responsible for mostof the cases of HIV infection (Kilby &Eron, 2003). HIV-2 is conﬁned largely towestern Africa (Kalichman, 1998). HIVdestroys a subset of helper T cells andimpairs the ability of cells to recognizeantigens. As a result, there is profounddeterioration of the immune system sothat the body has no defense against eventhe least aggressive organism. The virusreproduces within the T cell itself, produc-ing additional HIV , which in turn, invadesother T cells. The normal 2:1 ratio ofhelper cells to suppressor cells becomesreversed. The increased number of sup-pressor cells severely limits normal B-cellfunction, and they fail to respond to newantigens. The normal immune responsebecomes dysfunctional.
Most individuals with HIV infection
exhibit no symptoms until the later stagesof the disease. AIDS is the ﬁnal stage ofHIV infection and is characterized bysymptoms of severe failure of the immunesystem.
Transmission of HIV
HIV is found in the blood, as well as in
body secretions such as sperm. Trans-mission can occur in a variety of ways. Thevirus can be transmitted through:
• infusion of infected blood or blood
products
• an accidental stick with an infected
needle
• intravenous drug use and sharing of
equipment
• anal, oral, or genital intercourse • contact with a cut or open wound on
the skin• fetal transmission from a woman
with HIV infection to her unbornchild
There is no evidence that transmission
can occur in any way other than throughdirect blood-to-blood or sexual contactwith an infected individual. The virus doesnot appear to be transmitted throughcoughing, sneezing, or casual contact.Moreover, because all viruses require liv-ing tissue to survive and multiply, thevirus dies quickly once outside the body.
Diagnosis of HIV/AIDS
The most common procedures for diag-
nosing HIV infection are blood tests thatidentify not the virus itself but the pres-ence of antibodies that the body has pro-duced against the virus. The enzyme-linked immunosorbent assay ( ELISA ),
which is a blood test, is usually performedﬁrst because of its level of sensitivity toHIV antibodies. If the ELISA test is posi-tive, it is repeated, since false-positiveresults are more common than false-neg-ative results. If the ELISA is positive thesecond time, the test is conﬁrmed by usinga second procedure, the Western blo t. A
repeated positive ELISA and positiveWestern blot conﬁrm the diagnosis of HIVinfection. AIDS is usually diagnosed whenHIV is present and the individual hasdeveloped an opportunistic infectionand/or has a T-helper cell count that hasfallen below a certain level (see below).
Stages of HIV/AIDS
Infection with HIV can be separated
roughly into several stages, although there
are no ﬁrm guidelines that distinguish the
different phases. During the early or acute
phase, symptoms may be subtle or non-existent. Initially, some individ
uals mayConditions Affecting the Blood or Immune System 245

experience mild ﬂulike symptoms that sub-
side, leaving them symptom-free, although
the virus is still transmissible to others.
In the second stage, as the HIV infection
progresses, the levels of circulating virusincrease. At the same time, there is adecline in the number of helper T cells ofthe immune system. Infected individualsmay experience some or all of the follow-ing symptoms.
• Weight loss of 10 or more pounds in
less than 2 months for no apparentreason
• Loss of appetite • Unexplained persistent fever• Drenching night sweats • Severe fatigue that is unrelated to
exercise, stress, or drug use
• Persistent diarrhea• Swollen lymph nodes ( lympha-
denopathy )
Stages of HIV infection can be charac-
terized with greater precision by using T-helper lymphocyte cell counts. HIVinfections can generally be classifiedaccording to three stages:
• Early-stage disease (generally asymp-
tomatic) = T-helper cell counts above500
• Middle-stage disease (swollen lymph
nodes, fatigue, intermittent fever) = T-helper cell counts between 500 and200
• Later-stage disease (presence of oppor-
tunistic infections such as pneumocys-tis
carinii or tuberculosis) = T-helper
cell counts less than 200 (Bartlett,2000; Clement & Hollander, 1992)
Symptoms of Advanced HIV Disease
and AIDS
Many individuals with HIV infection
remain asymptomatic (without symp-toms) until they develop opportunistic
infections (infections that would not
occur in individuals with normal immunesystem function) and malignancies, orwhen the T-helper cell count falls below200/mm. Many organisms commonlyfound in the environment pose no threatunder normal circumstances because thefunctioning immune system resists them.When individuals have HIV infection, theimmune system is no longer able to act asa defense, and individuals are susceptibleto disease and infections that under nor-mal circumstances would not become full-blown. These diseases and infections arecalled “opportunistic.” Death in AIDS isnot caused by the dysfunction of theimmune system per se, but by complica-tions of conditions that develop becauseof inadequate immune system function.
One common opportunistic infection
associated with HIV infection is Pneumo-
cystis carinii pneumonia , a parasitic infec-
tion of the lung. This condition is highlyuncommon in healthy individuals, al-though it may be found in other immuno-compromised individuals, such as inthose who have cancer or those who havereceived immunosuppressants in associa-tion with organ transplantation. Pneumo-
cystis carinii pneumonia is one of the most
common manifestations of HIV infec-tions. Symptoms usually begin with a drycough and difficulty in breathing.Although some drugs are available to treatthe disease, they can have toxic side ef-fects that can further jeopardize the indi-vidual’s condition.
Another type of opportunistic infection
is candidiasis (yeast infection). The fungus
Candida frequently invades the oral cavi-
ty of the HIV-infected individual, causinga superﬁcial infection in the mouth andthroat that is manifested by pain andwhite plaques. This condition, also knownas oral thrush , may be the ﬁrst clue that the246
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

individual is infected with HIV. Althoughit is uncomfortable and difﬁcult to cure,infection with Candida is not likely to be
fatal. Individuals with debilitating condi-tions other than HIV infections may alsodevelop candidiasis.
In addition to opportunistic infections,
an otherwise rare form of cancer calledKaposi’s sarcoma is frequently associated
with HIV infection; this is considered anopportunistic cancer. Kaposi’s sarcomacauses pink, brown, or purplish blotcheson the skin.
Many individuals with HIV infection
develop neurological symptoms at sometime during the course of the disease.These may be mild, such as a headache,or more severe, such as aphasia (inabili-
ty to communicate through speech, writ-ing, or signs due to brain dysfunction),seizures, gait disturbances, visual distur-bances, and incontinence. Pain is a preva-lent feature among individuals withHIV/AIDS (Marcus, Kerns, Rosenfeld, &Breitbart, 2000). This chronic pain oftendoes not receive optimal treatment.
Individuals with HIV infection may also
experience a type of dementia called AIDS
dementia complex , which may include
cognitive symptoms such as poor concen-tration or forgetfulness, motor symptomssuch as loss of balance or clumsiness, andbehavioral symptoms such as apathy andsocial withdrawal. The precise mechanism
by which HIV causes dementia is unknown.
Treatment for HIV/AIDS
Currently, there is no means of restor-
ing the damaged immune function char-acteristic of HIV infections. However, ad-vances in the medical management of HIVinfection have improved the life expectan-cy of individuals living with HIV infec-tion. Antiretroviral drugs directly inhibit
HIV by disrupting its replication cycle orby interrupting the ability of HIV to bindwith other cells. New antiviral treatmentsreferred to as highly aggressive antiretroviral
therapy , which includes a class of drugs
called protease inhibitors , can be taken in
combination with other antiviral drugssuch as AZT. This combination therapy has
offered dramatic improvements in themedical treatment of individuals withHIV/AIDS (Britton, 2000; Shernoff &Smith, 2001; Tashima & Carpenter, 2003).
Resistance to antiretroviral drugs, how-
ever, is a growing problem for individualswith HIV (Gerberding, 2003). Because ofthe toxicity of some of the medications,individuals may experience toxic side ef-fects such as headache, nausea and vomit-ing, insomnia, diarrhea, and muscle pain.In addition, some individuals experiencebone marrow suppression, which necessi-tates immediate discontinuation of themedication. Additional side effects ofperipheral neuropathy, ulcerations of themouth, and skin rashes may also be expe-rienced.
The regimen of medications used in the
treatment of HIV infection can be cumber-some as well as expensive, making thetreatment not accessible to many individ-uals with HIV infection who are withoutinsurance or who are underinsured. Newerantiviral drugs can cost up to $20,000 peryear, which is more than twice that of thenext most expensive antiretroviral drug(Steinbrook, 2003). In addition, the poten-tially serious side effects associated withthe newer antiretroviral drugs can be ofconcern (Tashima & Carpenter, 2003).
In addition to medication, much of the
treatment for individuals with HIV infec-tion is geared toward supportive care andprevention of opportunistic infections.Individuals should have adequate rest,should engage in a program of moderateexercise, and should maintain adequatenutrition. As the condition progresses,Conditions Affecting the Blood or Immune System 247

individuals may need to modify their exer-cise program and allow for more frequentrest periods to conserve energy. As muchas possible, individuals with HIV infectionshould attempt to prevent opportunisticinfection. In addition to maintaininggood health practices, they should avoidcrowds and people with known infectionssuch as colds and ﬂu. If they developsymptoms of infection, they should con-sult a physician immediately.
In the later stages of HIV infection,
when opportunistic and/or neurologicalsymptoms occur, treatment is directedtoward the speciﬁc infection or symptommanifestation. It is not unusual for indi-viduals with later stages of HIV infectionto experience a number of hospitalizationsfor acute opportunistic infections.
Individuals with HIV infection should
take precautions not to transmit thevirus. They should fully understand theimportance of practicing safe sex; ofinforming sexual partners of their condi-tion prior to sexual activity; and of notsharing needles, razors, toothbrushes, orany other item that could be contaminat-ed with blood.
Psychosocial Issues in HIV/AIDS
Distress and preoccupation with illness
and imminent death may characterizeindividuals with any fatal disorder. Copingwith a diagnosis of HIV and the changesthe disease precipitates can be both phys-ically and mentally exhausting (Bower &Collins, 2000). Individuals with HIV infec-tion face an even more grim realizationthat, to date, no one with later stages ofHIV infection has survived.
Individuals infected with HIV are con-
fronted with ongoing stressful situationsinvolving noxious symptoms, treatmentwith unpleasant side effects, periods ofphysical disability, potential loss of em-ployment, possible rejection by family orfriends, economic stress, and potentialpremature death (Fleishman, Donald-Sherbourne, Crystal, Collins, et al., 2000).Consequently, those who have testedHIV positive are likely to experience con-siderable anxiety.
In addition to the grave prognosis, there
is much ambiguity associated with posi-tive test results. It is impossible to predictwhen or how rapidly the infection willprogress to the later stages. Individualslive with total unpredictability. Followingperiods of being very unwell, they maythen recover and experience periods ofwell-being only to then develop anotherinfection and return to an illness state(Cochrane, 2003). Living with the po-tential for progressing to the later stageand the uncertainty about the disease’sprogression often leads to additionalstress and anxiety. Individuals may retreatfrom most of their former activities andmay ﬁnd it difﬁcult to set goals for thefuture. They may put aside their person-al aspirations and focus on the struggle tosurvive.
Individuals with HIV infection often
bear the additional stress of the stigmaand fear associated with the disease, bothof which can lead to rejection and aban-
donment by others. Feelings of depression,
despair, and hopelessness are common.Individuals may also experience consider-able anger. There may be anger andresentment toward the society-imposedisolation that hampers HIV-infected indi-viduals in their efforts to obtain social sup-port and, at times, even the medical careafforded to individuals with other life-threatening conditions. Individuals whohave become infected with HIV throughmedical treatment, such as blood transfu-sions, may experience additional anger atcontracting the disease as “innocent vic-tims.” Those infected through contact248
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

with others may direct their anger againstthe individual or individuals from whomthey contracted the disease.
If the HIV infection is the result of indi-
viduals’ past behavior or lifestyle, theymay also experience guilt and self-incrim-ination. Guilt, self-blame, fear of abandon-ment, and fear of imminent painful deathcan lead to self-destructive behaviors, in-cluding attempted suicide. For individu-als whose families and friends had notbeen aware of their lifestyle, exposure mayresult in increased anxiety and fear ofabandonment. In other instances, HIV-infected individuals may experience guiltbecause of the fear that they have been thesource of contagion to others.
There may need to be a balance between
periods of activity and rest to avoid be-coming too fatigued. A moderate, regularprogram of exercise can help individualswith HIV infection maintain optimalemotional as well as physical health. Asthe condition progresses and staminadecreases, they may need to modify theiractivities. Individuals in the later stages ofHIV infection often need assistance witheveryday activities, including at firsthousekeeping chores and later extendingto personal care.
The social effects of HIV infection are as
varied as the symptoms associated withthe condition. HIV infection is a diseasethat many fear and perceive as shroudedin mystery. Many social implications ofHIV infection are related to this fear andmisunderstanding.
Some segments of society believe that
the illness is “deserved” because it hasbeen associated with behavior that theyconsider unacceptable. Others avoid indi-viduals with HIV infection because theyfear contagion and are not aware of theactual modes of transmission. Such soci-etal concepts can result in ostracism anddiscrimination against individuals withHIV. They may ﬁnd activities or socialinteractions at school, work, and socialfunctions restricted because of social prej-udice. The social stigma attached to thecondition may be particularly overwhelm-ing and traumatic if family and friendsalso express such reactions. Individualswith HIV infection are often left with lit-tle social support at a time when theyneed it most. Support groups, althoughbeneﬁcial in many chronic diseases, areeven more important for individuals withHIV infection.
Vocational Issues in HIV/AIDS
Maintaining vocational roles despite
signiﬁcant health issues is important inmeeting individuals’ emotional as well aseconomic needs (Lynch Fesko, 2001;McReynolds, 2001). With the advent ofnew therapies to treat HIV and the asso-ciated increase in life expectancy forsome, individuals with HIV may also gaina more positive outlook. Despite increasedfunctional capacity and longevity, how-ever, many individuals living withHIV/AIDS remain unemployed or losetheir jobs (Glenn, Ford, Moore, & Hollar,2003). Barriers to returning to or main-taining employment are numerous andrequire motivation and commitment toovercome (Maticka-Tyndale, Adam, &Cohen, 2002).
Many psychosocial, ﬁnancial, medical,
and legal factors affect individuals’ abili-ty and willingness to maintain employ-ment (Kohlenberg & Watts, 2003).Individuals are frequently confrontedwith conﬂicting pressures about whetheror not they should continue to work(Nixon & Renwick, 2003). Contextual fac-tors, such as disability and health insur-ance or drug plans, often influenceindividuals’ decision (Ferrier & Lavis,2003).Conditions Affecting the Blood or Immune System 249

For individuals with HIV infection, the
most serious impediments to successfulfunctioning in the workplace are the fear,discrimination, and prejudice that theyencounter. Many individuals who are HIVpositive encounter discrimination at work,and as a result they may withdraw fromthe workplace altogether (Hunt, Jaques,Niles, & Wiezalis, 2003). Individuals withHIV frequently fear that they will losetheir jobs as a result of their diagnosis, re-gardless of their continued mental andphysical ability to work.
When individuals with HIV do main-
tain their employment, there are usuallyno special restrictions, especially in theearly stages of the condition; however,because of the mode of transmission ofthe virus, they should avoid occupationsin which their blood may contaminate theblood of others. Because infection canhave such serious consequences for indi-viduals with HIV infection, they shouldalso avoid job situations in which they arelikely to be exposed to infection. As theHIV infection progresses and individualsexperience increasing fatigue, they mayneed to undertake less strenuous work orarrange for shorter work schedules ormore frequent rest periods. In the laterstages of the disease, mental changes mayaffect an individual’s capacity to functionin the work setting.
DIAGNOSTIC PROCEDURES FORCONDITIONS AFFECTING THE BLOODOR IMMUNE SYSTEMStandard Blood Tests
The diagnosis of many blood disorders
is dependent on laboratory analyses of theblood itself. A complete blood count is a test
used to evaluate a number of different com-
ponents in the blood. Sometimes thesevarious components are also measuredseparately. The components of a completeblood count include the following:
•Red blood cell count: measurement of
the total number of red blood cells ina cubic millimeter of blood
•White blood cell count: measurement of
the total number of white blood cellsin a cubic millimeter of blood
•Differential: measurement of the pro-
portion of each type of white bloodcell (i.e., neutrophils, eosinophils,basophils, lymphocytes, monocytes)in a sample of 100 white blood cells
•Hemoglobin: evaluation of the amount
of hemoglobin content of erythro-cytes in 100 milliliters of blood
•Hematocrit: measurement of the per-
centage or proportion of red bloodcells in the plasma (based on theassumption that the volume of plas-ma is normal)
Other types of blood tests used to meas-
ure speciﬁc components of blood are asfollows:
•Reticulocyte count: assessment of bone
marrow function by measuring itsproduction of immature red bloodcells ( reticulocytes )
•Platelet count: measurement of the
number of platelets in a cubic mil-limeter of blood
•Mean corpuscular volume (MCV ):calcu-
lation of the volume of a single redblood cell by dividing the hematocritby the red blood cell count
•Mean corpuscular hemoglobin concentra-tion: calculation of the amount of
hemoglobin in each red blood cell bydividing the hemoglobin concentra-tion by the hematocrit
Bleeding Time Test
A bleeding time test measures the length
of time it takes for bleeding to stop after250 CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

a small puncture wound. The test deter-mines how quickly a platelet clot forms.An abnormal bleeding time would in-dicate a tendency toward prolongedbleeding such as that found in condi-tions in which there is an abnormally lownumber of platelets circulating in theblood.
Prothrombin Time (PT, Pro Time) Test
A prothrombin time test measures the
length of time that a blood sample takesto clot when certain chemicals are addedto it in the laboratory. It tests for very spe-ciﬁc factors involved in clotting and maybe used diagnostically to identify patho-logic clotting disorders, such as may befound with liver dysfunction or in theabsence of vitamin K. The test may alsobe used to monitor the effectiveness of cer-tain anticoagulant medications used inthe treatment of conditions in which clotformation is or has been a problem. Pro-longation of clotting time indicates thatindividuals may be prone to abnormalbleeding. If the test indicates that clottingtime is reduced, there may be blood hy-percoagulability, which could contributeto the formation of blood clots.
Partial Thromboplastin Time (PTT) Test
A partial thromboplastin time test is used
to evaluate a special part of the clottingmechanism not evaluated by prothrom-bin time. As with the prothrombin timetest, certain chemicals are added to ablood sample in the laboratory, and theamount of time it takes a clot to form ismeasured. Prolongation of time in whichit takes a clot to form is indicative of ableeding disorder, such as that found inhemophilia . Prolongation of clot formation
may also be found with the use of theanticoagulant heparin, which affects aspeciﬁc part of the clotting mechanismthat is not measured by the prothrombintime test.
Bone Marrow Aspiration
Bone marrow aspiration involves remov-
ing a sample of bone marrow by insertinga special needle into the marrow space ofthe bone and then aspirating a small sam-ple. The bone marrow is then examinedmicroscopically for various abnormalitiesin the number, size, and shape of the pre-cursors of red blood cells, white bloodcells, and platelets.
ELISA and Western Blot
Both the ELISA and Western blot are
blood tests that screen for HIV antibodies.If the initial screening with the ELISA testproduces positive results, a second ELISAtest is performed. If the result of the sec-ond test is negative, the test result is con-sidered negative. If the result of thesecond test is positive, the Western blottest is usually performed as a conﬁrmato-ry test. If the result of the Western blot testis positive, it is highly suggestive that theHIV antibody is present and that the indi-vidual has been exposed to HIV.
Hemoglobin Electrophoresis
Hemoglobin electrophoresis is a blood
test by which a deﬁnitive diagnosis ofsickle cell disease or sickle cell trait can bemade.
Sickle Cell Prep
Sickle cell prep is a blood test used in sick-
le cell screening. The test can detect the
presence of abnormal hemoglobin but can –
not distinguish between sickle cell diseaseand sickle cell trait.Diagnostic Procedures for Conditions Affecting the Blood or Immune System 251

GENERAL TREATMENT FORCONDITIONS AFFECTING THE BLOODOR IMMUNE SYSTEM
For many disorders of the blood, treat-
ment is directed toward symptoms and/orthe underlying cause. If a blood disorderis caused by a toxic substance, the ﬁrst lineof treatment is to remove the offendingagent. Anemia that is caused by a deﬁcien-
cy may be treated by supplementation orreplacement therapy. For instance, iron
deﬁciency anemia may be treated by the
administration of oral or injectable ironpreparations. Pernicious anemia may be
treated with injections of vitamin B12.When there is an overproduction of redblood cells, as in polycythemia , treatment
may involve the removal of blood. Vene-section ( phlebotomy ) is a procedure in
which quantities of blood are removed toreduce the volume of blood.
Transfusion
Part of the treatment for a number of
blood disorders may be the transfusion of
whole blood or a blood component, such as
packed red blood cells, plasma, or plate-lets. Because blood is living tissue, trans-fusion can be thought of as a form oftransplantation, carrying the same risks ofimmune response as do other types oftransplantation. For this reason, the exactmatching of a number of factors in theblood between the donor and the re-cipient is crucial to prevent serious aller-gic reactions, which could be fatal. Inaddition to the risk of such a reaction,there is a risk that a blood transfusion willtransmit a disease, such as hepatitis orHIV , although careful screening byblood banks has signiﬁcantly reducedthis risk.Bone Marrow Transplant
Bone marrow transplant is a procedure
in which individuals’ bone marrow iseradicated and healthy bone marrow istransplanted. Bone marrow transplants areused when the immune system is severe-ly deﬁcient or when certain types of can-cers exist (see Chapter 16). Bone marrowcells are received from a donor, and a care-ful match is made to decrease the chancesof rejection of the transplant and to pre-vent a reaction in which the transplant-ed cells attack the cells of the individualwho has received the transplant.
PSYCHOSOCIAL ISSUES INCONDITIONS AFFECTING THE BLOODOR IMMUNE SYSTEMPsychological Issues
Disorders of the blood and immune sys-
tem have a variety of psychological impli-cations. The speciﬁc implications for aparticular individual are dependent on thecondition. Some conditions may be con-trolled relatively easily, whereas others re-quire constant vigilance. Although some
conditions may be treated and, in some in-
stances, cured, others require lifelong treat-
ment and carry a more ominous prognosis.
Individuals with conditions affecting
the blood and immune system generallyhave no visible reminders of their disabil-ity. Without external adaptive devices,such as wheelchairs, crutches, or canes, orany other signs of disability, individualsmay react by denying the seriousness oftheir condition and resist medical direc-tives. For example, individuals with hemo-
philia may engage in risk-taking behaviors,
even though injury and subsequent bleed-ing could occur. Individuals with sickle252

cell anemia may engage in a ﬂurry of ac-tivity, even though the associated stressand fatigue may precipitate a sickle cellcrisis. Individuals with HIV infection maywithhold their diagnosis from others withwhom they engage in sexual activity, eventhough their behavior could put thoseothers at risk.
Some disorders occur later in life, neces-
sitating adjustment at the time the disabil-ity occurs. Disorders such as sickle cellanemia and hemophilia are lifelong disor-ders, however. Consequently, individualswith these disorders have had to copewith their condition in one way or anoth-er from childhood into adulthood, andmost of them have experienced frequentillness and medical care throughout theirchildhood and adolescence. Althoughthese experiences can build conﬁdence inthe ability to cope with adversity, they canalso have a negative impact on develop-ment. Individuals may carry the copingbehaviors and attitudes learned in child-hood into the adult years, where they con-tinue to affect their perception of them-selves, their condition, and their abilities.Depending on the constructiveness of thecoping strategy used, such behaviors maybe an asset or a hindrance.
The possibility of early death, a source
of anxiety and depression for those withany disorder, is a reality for individualswith hemophilia, sickle cell anemia, andHIV infection. Although hemophilia canbe controlled to some degree, there isalways the fear that an accident or trau-matic event may occur in which bleedingmay not be controlled. Individuals withsickle cell anemia are aware of the possi-bility that sudden death will occur as aresult of a sickle cell crisis or complica-tions. Individuals with HIV infectionknow that their progression to AIDS willprobably result in death. Individuals maycope with the threat of early death in avariety of ways, ranging from the adop-tion of a philosophical view toward life topassivity and withdrawal.
The way in which individuals cope with
a condition they have had since child-hood depends on a wide variety of factors,some of which relate to the coping mech-anisms learned in childhood. Individuals’reaction as an adult to their condition isdependent to some extent on how welltheir psychological adjustment was man-aged throughout development. Childrenwho were encouraged to live as normal alife as possible, despite their condition,may exhibit a greater sense of self-esteemand autonomy as adults than do thosewho were kept in a dependent, overpro-tected state.
Lifestyle Issues
Different conditions affecting the blood
or immune system affect activities of dai-ly living in varying degrees, depending onthe associated symptoms. Symptoms offatigue or difﬁculty in breathing with exer-tion may require individuals to pace theiractivities throughout the day to conserveenergy. Individuals may need more fre-quent rest periods, or they may need todivide activities into smaller steps thatthey perform throughout the day, ratherthan completing a task all at once.
Good health practices are important to
everyone; however, because of the in-creased susceptibility to infection that ispart of many conditions affecting theblood or immune system (especially HIVinfection), individuals must take extra careto have well-balanced diets and well-bal-anced regimens of rest and activity. Exer-cise is especially important to individualswith hemophilia. Regular, moderate exer-cise can build the muscles that protectjoints and decrease the incidence ofbleeding into the joints. However, activi-Psychosocial Issues in Conditions Affecting the Blood or Immune System 253

ties that carry a higher probability of in-jury, such as contact sports, should beavoided.
The degree to which individuals can
maintain their routine daily schedulesdepends on the speciﬁc condition, its pro-gression, and its complications. For themost part, individuals with hemophilianeed not interrupt their daily schedules.The use of home self-infusion therapy hasgreatly reduced their incapacity by provid-ing prompt and early treatment of spon-taneous bleeding.
Although neither hemophilia nor sick-
le cell anemia alters sexual function, both
are inherited disorders, and individualsmay wish to consider genetic counselingbefore deciding to have children. There isno direct effect on sexual function asso-ciated with HIV infection; however,because of the possibility of transmittingthe virus to others, individuals with HIVinfections should inform their sexualpartners about their diagnosis prior to sex-ual contact and should engage only in safesexual practices. When women with HIVinfection become pregnant, the childmay be born HIV infected.
Social Issues
The social effects of conditions affecting
the blood or immune system vary withthe condition, the individual, and the par-ticular circumstances. The fatigue and sus-ceptibility to infection characteristic ofmany conditions affecting the blood orimmune system may alter social function-ing to some degree.
Because many conditions affecting the
blood or immune system have no readily
observable outward cues and signs, and be-
cause symptoms are often intermittent,others may not understand why individ-uals with these conditions must adhere tocertain restrictions or why they are undercontinuing medical care. Because theseindividuals do not appear to be legitimate-ly ill and, in many instances, have little
physical impairment, they may receive less
social support and understanding thanindividuals with more visible disabilities.
Conditions that are hereditary and those
that occur in childhood can impair thesocialization necessary for functioning in
adulthood. Recurrent hospitalizations may
affect children’s school performance and,consequently, their sense of industry andachievement. In addition, frequent schoolabsences, hospitalizations, or the inabili-ty to engage in some activities may affectchildren’s interactions and relationshipswith peers, which, in turn, could affecttheir self-esteem and sense of self-worth.
Some children, as a means of dealing
with the stress inherent in their condition,may learn to use their condition to mani-pulate and control the behaviors of oth-ers. The parents of a child with an inher-ited disorder, such as hemophilia or sick-le cell anemia, may experience guilt, reactwith overprotectiveness, or foster a senseof dependency in the child. They mayadopt a permissive or indulgent attituderather than correcting the child when heor she misbehaves. They may also excusethe child from the normal responsibilitiesor the limits established for the child’s sib-lings. Such parental reactions can impedethe child’s ability to function adequatelyas an adult in society.
VOCATIONAL ISSUES IN CONDITIONSAFFECTING THE BLOOD OR IMMUNESYSTEM
The cause and symptoms of a condition
affecting the blood or immune systemdetermine its vocational impact. If, forexample, the condition has been causedin part by exposure to toxic substanceswithin the environment, the hazards254
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

should be removed before individuals re-turn to the workplace. If fatigue or dysp-nea is a symptom of the condition, as inthose conditions characterized by anemia,it may be necessary to consider the phys-ical demands of the job and the need formore frequent rest periods. When infec-tion is a potential complication of the dis-order, individuals should avoid exposureto factors and environments that may pre-cipitate infection. The functional impactof immune disorders is also dependent onthe stage of the condition. With HIVinfection, for example, prior to the devel-opment of severe immunodeﬁciency, nodisability may be present. In the milderstages of the condition, individuals maybe able to perform all but the mostdemanding tasks at work. As the immunesystem becomes more compromised, how-ever, opportunistic infections may resultin prolonged periods of illness and hospi-talization, interfering with individuals’ability to work. In addition to the physi-cal disability resulting from opportunisticinfections, individuals with HIV infectionsmay also develop central nervous systemsymptoms, which can affect their cogni-tive, motor, and behavioral abilities.
CASE STUDIESCase I
Mr. G. is a 26-year-old male who is HIV
positive. He contracted HIV from his part-ner, who died of the disease last year. Mr.G. has a high school education and is acertiﬁed nursing assistant working in anursing home, where he performs routinecare for nursing home residents, such asbathing, lifting, turning, and feeding. Hehas been employed at the nursing homefor the past 10 years. He tells you he loveshis work and very much wants to contin-ue as long as possible, both for ﬁnancialreasons and because his health insuranceis tied to his employment. He also tellsyou that work has been therapeutic forhim after the loss of his partner. Mr. G.states that his employer is unaware of hisillness because it has not interfered withhis job performance; however, lately hestates he has had more difﬁculty keepingup with the physical demands at workbecause of fatigue and in the past fewweeks he has developed lymphadenopa-thy. He has begun a new experimentalmedication that he also believes mighthave some side effects that could interferewith his ability to work. He has a strongsupport group of friends; however, hisfamily has severed all ties with him.
Questions
1. Is it appropriate for Mr. G. to not
inform his current employer abouthis diagnosis and to continue to workin the current setting? Why or whynot?
2. What is Mr. G.’s rehabilitation poten-
tial?
3. What factors will inﬂuence Mr. G.’s
rehabilitation potential?
4. What medical factors related to Mr.
G.’s condition would you considerwhen helping him develop a rehabil-itation plan?
Case II
Ms. S. is a 19-year-old African American
female with sickle cell disease. She is ahigh school graduate and is currentlyenrolled in a junior college, where she isstudying to be an X-ray technician. Sinceentering school at the junior college, shehas had a number of sickle cell crises thathave necessitated hospitalization. Ms. S.has developed severe damage to joints inher lower extremities as a result of her dis-Case Studies 255

ease. Although her physician has recom-mended that Ms. S. reconsider her occu-pational goal given her series of sickle cellcrises since being in school, she is deter-mined to pursue her education and tobecome an X-ray technician. She contin-ues to push herself even when she doesnot feel well.
Questions
1. How would you approach Ms. S.
about her vocational plans given herphysician’s recommendation?2. How realistic is Ms. S.’s vocational
choice?
3. What medical, physical, and psycho-
logical issues would you considerwhen working with Ms. S. to devel-op her rehabilitation plan?
4. What is the general prognosis for Ms.
S.’s condition?
5. What general lifestyle issues might
you address with Ms. S. that couldcontribute to her rehabilitationpotential?256
CHAPTER 8 C ONDITIONS OF THE BLOOD AND IMMUNE SYSTEM

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NORMAL STRUCTURE ANDFUNCTION OF THE ENDOCRINESYSTEM
The endocrine system works together
with the other communication system,the nervous system , to regulate or direct
various body functions. The endocrinesystem is composed of ductless glands(endocrine glands ) scattered throughout the
body (Figure 9–1). The endocrine glandsproduce chemical substances called hor-
mones that are secreted directly into the
bloodstream and act as messengers on tar-get cells in other parts of the body. Endo-crine glands include the:
• thyroid gland , located in the neck, in
front of and on either side of the tra-
chea (windpipe).
• parathyroid glands , small, bean-shaped
glands buried within the thyroid gland.
• adrenal glands , small glands lying on
top of the kidneys. Each adrenalgland has two parts, the medulla and
the cortex . Each part has a different
function.
• pituitary gland , located in the skull just
above the roof of the mouth and con-nected to the brain by a slender stalk.It is divided into two parts, the ante-
riorand the posterior lobes .• hypothalamus , a area of the brain that
coordinates functions of the nervoussystem and endocrine system.
• islets of Langerhans , special cells
embedded in the pancreas.
• testes in males and ovaries in females.
The main function of the endocrine sys-
tem is regulatory, with different hormonesaltering various body processes so that thebody’s internal balance ( homeostasis ) is
maintained. Although each endocrinegland has its own unique and independ-ent function, endocrine glands oftenwork in concert. The hormones secretedby the endocrine system control and inte-grate a variety of body activities, establish-ing a delicate chain of communicationbetween various body systems and inﬂu-encing and regulating a number of phys-iologic processes:
•Growth and development of the bodyand brain
•Reproductive maturity and function
•Metabolism
•Adjustment to internal and externalstress
•Water and electrolyte balance
Overproduction or underproduction of
one hormone can affect a number of oth-er endocrine glands and a variety of bodyEndocrine ConditionsCHAPTER 9
259

functions. Some hormones have the solefunction of regulating the production andsecretion of another hormone.
The hormone thyroxine , which is secret-
ed by the thyroid gland , regulates the rate
of metabolism and also inﬂuences nervoussystem maturation. When the level of thy-roxine in the blood is high, metabolism
speeds up; when it is low, metabolism slows
down.
Parathyroid hormone , which regulates the
concentrations of calcium and phosphatein the body, is secreted by the parathyroid
glands . Excessive amounts of the parathy-
roid hormone in the blood can result in the
demineralization of bone, causing bones tobecome fragile so that they are easily bro
-ken. Insufﬁcient amounts of the parathy-
roid hormone in the blood can cause spasm
and involuntary contraction of the mus-cles ( tetany ). If parathyroid hormone is to
be effective, vitamin D must be present.
The inner part of the adrenal gland
(medulla ) secretes the hormones epineph-
rineand, to a lesser extent, norepinephrine
at times of stress to enable the body to pre-
pare physiologically for emergencies. These
hormones increase the heart rate, increasemuscle tone, and constrict blood vesselsin times of stress. The outer portion of the
adrenal glands (cortex ) secretes hormones
called steroids , which regulate many essen-
tial functions, such as electrolyte andwater balance, metabolism, immune re-260
CHAPTER 9 E NDOCRINE CONDITIONS
Thyroid
PancreasPituitary
Parathyroid glands
AdrenalglandKidneyOvaries(female)Testes(male)
Figure 9–1 Endocrine System.

Conditions of the Endocrine System 261
sponses, and inﬂammatory reactions. Theadrenal cortex is essential to life. If it is dys-
functional, death will occur within a fewdays unless the hormones that it normal-ly secretes are replaced.
The anterior lobe of the pituitary secretes
thyroid-stimulating hormone (TSH) (which
is necessary for thyroid function), growth
hormone , hormones that control reproduc-
tive function, and corticotropin (a hormone
necessary for the function of the adrenalcortex). The posterior lobe of the pituitary
gland stores hormones produced in the
hypothalamus. Antidiuretic hormone (ADH),
which increases water reabsorption by the
kidneys, is produced by the hypothalamusbut is stored in and secreted by the pos-terior lobe of the pituitary gland.
Special cells within the islets of Langer-
hans in the pancreas produce the hormones
insulin and glucagon , which are necessary
for the metabolism of carbohydrates, pro-teins, and fats. Hormones produced by thetestes and ovaries are important not only
to reproductive function, but also to nor-mal growth and development.
CONDITIONS OF THEENDOCRINE SYSTEM
A number of medical conditions result-
ing from endocrine dysfunction constitutea major health problem (Wilson, 2001).Because symptoms of endocrine condi-tions are often similar to those associatedwith a number of mental disorders, someendocrine conditions may go unrecog-nized or misdiagnosed as psychiatricdisorders. Likewise, administration of hor-mones in treatment of an endocrine deﬁ-ciency may have side effects similar tothose of some mental disorders. Clearly,the endocrine system, in addition to reg-ulating internal body functions and main-taining homeostasis, has a role in humanbehavior and emotions.Hyperthyroidism (Graves’ Disease,Thyrotoxicosis)
Manifestations of Hyperthyroidism
Hyperthyroidism is the overproduction
of thyroid hormone because of hyperfunc-tion of the thyroid gland. Hyperthyroid-ism results in an increased metabolic rate.
The term thyrotoxicosis is often used in-
terchangeably with hyperthyroidism ; how-
ever, thyrotoxicosis results from over-
ingestion of the hormone or inﬂammation
of the thyroid gland ( thyroiditis ) rather
than from an overactive thyroid gland(Woeber, 2000).
The overactive thyroid gland may
become so enlarged that there is a visibleswelling in the neck, called a goiter . Other
symptoms of hyperthyroidism includerestlessness, irritability, nervousness, andweight loss. The increased rate of metab-olism causes intolerance to heat; thus,environmental temperatures that seemcomfortable to others seem unbearablywarm to individuals with hyperthy-roidism. Exophthalmos (abnormal pro-
trusion of the eyeball) may also developwith hyperthyroidism. Once exophthal-mos develops, the effects are permanent,giving individuals a wide-eyed and startledappearance. With early diagnosis andtreatment, hyperthyroidism usually caus-es no permanent disability.
Treatment and Management of
Hyperthyroidism
In hyperthyroidism, treatment is direct-
ed toward curtailing the secretion of thethyroid hormone. Antithyroid medicationthat blocks the production of the hor-mone may be used. Symptoms usuallysubside within weeks or months after thetreatment begins. Treatment does not,however, alleviate exophthalmos.

Some physicians recommend oral ad-
ministration of 131I (radioactive iodine).Radioactive iodine destroys cells that pro-duce thyroid hormone, and symptomsusually subside within weeks or months.This type of treatment causes some indi-viduals to become hypothyroid , however,
which requires that they take thyroidmedication for life. Surgical treatmentof hyperthyroidism is sometimes indicat-ed. In these instances, a subtotal thyroidec-
tomy , which involves the removal of
most, but not all, of the thyroid gland, isperformed. Because some of the thyroidgland is left in place, replacement thera-py with thyroid hormone is not usuallynecessary.
Hypothyroidism (Myxedema)
Manifestations of Hypothyroidism
Hypothyroidism is the most common
thyroid disorder in the adult population(Wu, 2000). Individuals with hypothy-
roidism have inadequate production of
thyroid hormone. The symptoms of hypo-thyroidism are in many ways the oppositeof those of hyperthyroidism. Individualshave a slowed metabolic rate, and may feeltired, lack energy, and gain weight. Theirhair becomes dry, brittle, and thin, andtheir voice may be slow, low-pitched, andcoarse. Emotional responses are subduedand mental processes slowed. Complica-tions of hypothyroidism include the rap-id development of atherosclerotic heartdisease, including angina pectoris , myocar-
dial infarction , and congestive heart failure
(see Chapter 11). Individuals with severehypothyroidism can also develop psycho-
sis, with associated paranoia and delusions .
Unless complications develop, however,appropriate treatment usually preventsany permanent disability.Treatment and Management of
Hypothyroidism
The goal of treatment of hypothy-
roidism is to correct thyroid hormone deﬁ-ciency. Consequently, a primary mode oftreatment is replacement therapy. Themedication of choice for thyroid hormonereplacement is levothyroxine (Synthroid ), a
synthetic thyroid preparation. Individualswith hypothyroidism need to remain onthis medication for life. Appearance andlevel of physical and mental activity usu-ally improve gradually as the level of thy-roid hormone rises. Blood levels of thyroidhormone and TSH should be measuredregularly when individuals are on thyroidhormone replacement therapy. Theyshould not alter the medication regimenwithout consulting a physician.
Cushing’s Syndrome(Adrenal Cortex Hyperfunction)
Manifestations of Cushing’s Syndrome
Overproduction of hormones by the
adrenal cortex leads to Cushing’s syndrome ,
which is characterized by pufﬁness and arounded moon face, obesity of the trunkof the body, fat pads at the back of theneck ( buffalo hump ), and weakness. The
skin becomes thin and fragile, woundhealing is poor, and bruising is frequent.Women with Cushing’s syndrome mayhave menstrual irregularities and facialhair growth. Mood and mental acuity mayalso be altered.
Treatment and Management of
Cushing’s Syndrome
Treatment involves prescribing medica-
tion to reduce the production of corticos-teroids. Treatment may involve surgicalintervention, pituitary irradiation, hor-262
CHAPTER 9 E NDOCRINE CONDITIONS

mone replacement therapy, or other med-ications to suppress hormone production.
Despite treatment, Cushing’s syndrome
can have profound physical and emotion-al effects, requiring ongoing assessment ofpsychological well-being and functionalcapacity (Sonino, Boscaro, Fallo, & Fava,
2000). Full recovery may be slow, and there
may be neuropsychological (cognitive and
emotional) as well as physical (osteoporo-sis, hypertension) residual impairments(Boscaro, Barzon, Fallo, Sonino, 2001).
Addison’s Disease(Adrenocortical Insufﬁciency)
Manifestations of Addison’s Disease
In contrast to Cushing’s syndrome,
Addison’s disease results from underproduc-
tion of hormones by the adrenal cortex .
Weakness and fatigue are early symp-toms; skin pigmentation may becomedarker. Individuals with Addison’s diseasemay also experience weight loss, loss ofappetite, and decreased cold tolerance.Because hormones secreted by the adrenalcortex play a prominent role in thebody’s adaptive response to stress, individ-uals with Addison’s disease may havesevere, potentially life-threatening reac-tions, such as extremely low blood pres-sure and severe electrolyte imbalance, insituations (e.g., uncomplicated surgicalprocedures) that do not normally elicitsuch a response.
Treatment and Management of
Addison’s Disease
Although Addison’s disease was once
fatal, replacement therapy with synthet-ic corticosteroids now enables individualsto live full, normal lives. Replacementmedication must be taken daily, howev-er. Careful monitoring for the develop-ment of the symptoms of excessive corti-costeroid ingestion is necessary.
Diabetes Insipidus
Manifestations of Diabetes Insipidus
Diabetes insipidus is a condition in
which there is inadequate secretion ofADH from the hypothalamus . Although
there are a number of causes of diabetesinsipidus, the most common cause is dam-age to the stalk connecting the hypothal-amus to the posterior lobe of the pituitarygland (where ADH is stored), which pre-vents ADH from being secreted. As aresult, excessive water is “lost” by the kid-neys ( polyuria ). Individuals have exces-
sive and constant thirst ( polydipsia ),
consuming as much as 30 quarts of waterper day. Diabetes insipidus may be a tem-porary condition or can become chronic.The condition is permanent, but symp-toms can be controlled with medication,enabling individuals in most instances tolive a normal life.
Treatment and Management of
Diabetes Insipidus
Depending on the cause, different hor-
monal preparations may be used to cor-rect diabetes insipidus or to treatsymptoms. If the condition has beencaused by a pituitary tumor (a rare occur-rence), surgical resection of the tumor maybe indicated.
Diabetes Mellitus
Deﬁning Diabetes Mellitus
A chronic, incurable disorder of carbo-
hydrate metabolism, diabetes mellitus
involves an imbalance of the supply ofand demand for insulin and is the mostConditions of the Endocrine System 263

common of all endocrine conditions(Olefsky, 2001). Every body system isaffected by the condition. The impact ofdiabetes is immense. Over the last twodecades the prevalence of diabetes in theUnited States has doubled (Centers forDisease Control, 2002). Over 18 millionindividuals (6.3 percent) of the populationin the United States have diabetes (Ameri-can Diabetes Association, 2004a). Diabetesmellitus is a leading cause of heart disease,stroke, hypertension, blindness, kidneydisease, amputation, and nervous systemdamage (American Diabetes Association,2004a; Taylor, 2004).
The cause of diabetes mellitus is un-
known, but there may be a familial ten-dency to develop the disease. Obesity alsogreatly increases the risk of diabetes(Tataranni & Bogardus, 2001). Diabetesmellitus can also occur as a complicationof other conditions, such as pancreatitis
(inﬂammation of the pancreas) or tumorsof the pancreas; as a side effect of medica-tions that cause an abnormal tolerance toglucose (sugar); or as a result of speciﬁc
conditions that increase the body’sdemand for insulin, such as gestational
diabetes (diabetes that occurs during
pregnancy). In these cases, the correctionof the underlying cause may reverse thediabetes.
Mechanisms of Diabetes Mellitus
Food that is ingested is eventually con-
verted to glucose (sugar), where it is car-
ried in the blood to nourish all cells of thebody. Certain tissues, such as muscle andfat, need insulin to use glucose as a sourceof energy and to store glucose for futureuse. In diabetes mellitus, insufficientinsulin is available to meet this need. Thereason may be:
• failure of the islets of Langerhans to
produce enough insulin• destruction of insulin before it can be
used
• inability of body tissues to use the
insulin that is present
When there is insufﬁcient insulin, cells
are unable to utilize glucose, so largeamounts accumulate in the blood. Thiscondition is known as hyperglycemia .
As blood is ﬁltered by the kidney, glu-
cose is normally channeled back into theblood. Because individuals with diabetesmellitus have such a large amount of glu-cose in the blood, however, some glucosespills over into the urine ( glycosuria ). Be-
cause of the large concentration of glucosein the urine, the kidney excretes largequantities of water, a symptom calledpolyuria . As a result, individuals need to
drink large quantities of water to replacethe excess ﬂuid lost ( polydipsia ). The
body’s inability to use glucose means thatthe food or energy available to body tis-sues is inadequate. To compensate, indi-viduals with diabetes increase food intakedramatically ( polyphagia ). Despite this
increased food intake, however, lack ofinsulin prevents the body from using foodas an energy source. Consequently, indi-viduals begin to lose weight and becomeincreasingly weak. Unless supplementalinsulin is available, they literally enter astate of starvation. Because the body’sneed for energy remains unmet, it metab-olizes its own stores of fat and proteins forenergy. As a result, ketones , the byproducts
of fat metabolism, are formed. Normally,ketones are broken down and excreted. Inindividuals with diabetes mellitus, howev-er, they accumulate more rapidly thanthey can be excreted. When ketone levelsbecome toxic, a condition called ketosis
or ketoacidosis (diabetic coma ) occurs.
Having too little or no insulin available forthe amount of food ingested may alsocause a diabetic coma.264
CHAPTER 9 E NDOCRINE CONDITIONS

Types of Diabetes Mellitus
There are two types of diabetes mellitus,
Type I (insulin-dependent diabetes mellitus ,
or IDDM ) and Type II (non-insulin-depend-
ent diabetes mellitus , or NIDDM ). Type I
accounts for about 10 percent of all dia-betes, and Type II accounts for the re-mainder, or about 90 percent (AmericanDiabetes Association, 2004a). In Type I thebody produces little or no insulin, so thatindividuals require external sources ofinsulin for their survival. In Type II thebody produces insulin, but the insulinproduced is insufﬁcient to meet the totalbody needs or the body is unable to usethe existing insulin adequately. Externalsources of insulin may or may not be tak-en to control the symptoms of Type II, butsurvival does notdepend on an external
insulin source. Obesity is a major risk fac-tor in development of Type II diabetes(Ludwig & Ebbeling, 2001; Tataranni &Bogardus, 2001).
Treatment of Diabetes Mellitus
There is no cure for diabetes mellitus.
Treatment is directed toward controllingthe levels of glucose in the blood and pre-venting complications (Hoffman, 2001). A
landmark study (The Diabetes Control andComplications Trial Research Group, 1993)
demonstrated that strict control of bloodglucose could signiﬁcantly reduce thecomplications of diabetes. Regardless ofthe type of diabetes, diet is an importantpart of treatment (Chandalia et al., 2000;Rendell, 2000). Individuals with Type II
(non-insulin-dependent) diabetes may be able
to control their blood glucose level with
diet alone or with a combination of diet and
oral hypoglycemic agents and, at times,insulin. Individuals with Type I (insulin-
dependent ) diabetes control blood glucose
levels through diet and the use of insulininjections. Individuals with either type of
diabetes must consider the amount of ener-
gy expended through exercise and balanceit with calories available from food. Alldiabetic diets are designed to balance thenumber of proteins, carbohydrates, andfats ingested and to exclude foods thatcontain large amounts of sugar.
Pancreas transplantation has been used
in some individuals with Type I diabeteswho have poor glucose control and whosequality of life has been significantlyimpacted by their condition (Robertson,
2004). Transplantation of islets alone shows
promise and can result in insulin indepen-dence and good glucose control (Shapiroet al., 2000), although it is still consideredto be experimental (Robertson, 2000;Stevens, Matsumoto, & Marsh, 2001).
Type I (Insulin-Dependent
Diabetes Mellitus)
Type I (IDDM ) is the most severe form
of the disease. Insulin is the primary modeof therapy for all individuals with Type Idiabetes. Gastric juices inactivate insulin.Consequently, insulin cannot be takenorally, so individuals who are insulin-dependent must inject insulin into thesubcutaneous (fatty) layer of tissues.
The goal of insulin therapy is to main-
tain blood sugar levels as close to the nor-mal range as possible and to delay orprevent complications. There are a num-ber of different commercial insulin prepa-rations from a number of different sources(beef, pork, beef-pork, or human synthet-ic insulin). Some are rapid acting , some
intermediate acting, and others long act-
ing. Rapid-acting insulin usually acts with-
in 30 minutes to 1 hour after injection,and intermediate types work within 1 to 2
hours. Long-acting insulin works within 4
to 6 hours. Each type of insulin also has a
different time of peak action and duration. Conditions of the Endocrine System 265

Because of body responses to insulin,
absorption differences, and other factors,insulin absorption varies considerablyfrom individual to individual, as well asin the same individual from day to day.Most individuals require more than oneinsulin injection per day. Some may berequired to take several different types ofinsulin. Individuals must rotate the injec-tion site to avoid a buildup of scar tissue,which can interfere with the absorption ofinsulin. They may use a regular syringeand needle for insulin injections, or someﬁnd it more convenient to use a devicecalled an insulin injector , which resembles
a pen. The device consists of a cylinderinto which a cartridge ﬁlled with a prede-termined dose of insulin and disposableneedles is placed. The advantage of thedevice is that it is relatively reliable andaccurate in delivering the amount of in-sulin injected, as well as convenient. Thesedevices may be carried unobtrusively in apurse or pocket for use away from home.Individuals using the insulin injector donot need to carry extra syringes andinsulin bottles. When at a social event,business meeting, or family outing, theycan easily give themselves injections withminimal disruption.
Disposable syringes eliminate the need
for cleaning and decrease the possibilityof contamination and subsequent infec-tion. For the most part, insulin no longerrequires refrigeration for storage, but ex-posure to extremes in temperature and tointense light should be avoided. Otherindividuals may choose an insulin pump ,
which provides a slow, continuous subcu-
taneous infusion of insulin throughout the
day, thus avoiding the need for numerousinjections. Insulin is delivered to subcuta-
neous (fatty) tissue in the abdominal wall
through a needle and an open loop delivery
device consisting of a small insulin pump,about the size of a pager, that is worn 24hours a day. Although more expensivethan other methods, the pump providesmore ﬂexibility relative to meal timing.
Regardless of the method of insulin
delivery, the amount and type of insulinare balanced with the number of caloriesconsumed and the amount of physicalactivity performed daily. Because insulininjected into the body must be balancedwith the amount of glucose available,individuals cannot, after receiving insulin,decide to “skip a meal.” Likewise, sincephysical exercise burns glucose for ener-gy, a drastic increase in activity, eventhough adequate amounts of food wereconsumed, may mean that the rapid con-sumption of glucose for energy will leavetoo much insulin in the body for theamount of glucose left.
Conditions that increase the metabo-
lism rate or cause the body to consumemore of the available glucose, such asstress, illness, infection, and pregnancy, allalter insulin requirements and, conse-quently, may necessitate an alteration inan individual’s insulin dosage. Conse-quently, individuals with IDDM who be-come ill with ﬂu, fever, or other types ofillness should consult their physicianregarding adjustments to their normalinsulin dosage.
Dietary treatment is also an integral part
of treatment. The primary goal of diettherapy is to optimize blood levels of glu-cose. Individuals with diabetes mellitusmust consider, in addition to propernutrition, the total number of caloriesingested as well as the distribution of calo-ries consumed throughout the day.Because those with IDDM take a predeter-mined amount of insulin, they must beespecially careful to consume a speciﬁednumber of calories at consistent timesthroughout the day to maintain a balanceof insulin and glucose in the blood. Forthe most part, calories should be distrib-266
CHAPTER 9 E NDOCRINE CONDITIONS

uted evenly throughout the day so thatthere is not a large concentration of calo-ries at any one time. Because the onlysource of insulin for individuals withIDDM is that which they administer ex-ternally, they must pay close attention tothe timing of meals and must be sure thatthere is correct timing between the inges-tion of food and the time course of actionof the insulin they have injected. In addi-tion to being cautious of the caloric val-ue of food, individuals must also monitorthe types of foods and their balance with-in the diet, since some types of foodsaffect the absorption and metabolism ofothers. Counseling by a dietitian or
nutritionist (individuals who study and
counsel individuals on the therapeutic useof food) is imperative in the treatment ofdiabetes. Diabetic diets are individualizedbased on many personal factors, such asweight, age, and type of daily activity (e.g.,sedentary, moderately active, very active).Individuals who are overweight may beplaced on a low-calorie reduction diet sothat the body will need less insulin.Because of their growth needs, adolescentsmay be placed on a higher-calorie dietthan an older individual of the same size.Individuals who engage in sedentaryactivities throughout the day do notrequire as many calories as do individualswho are very physically active in their jobor at home. Compliance with the pre-scribed diet is usually better if lifestyle,religious, and cultural habits are consid-ered as much as possible when dietary rec-ommendations are made.
Exercise is important for the general
health and well-being of all individuals.For individuals with diabetes mellitus,however, calories must be balanced withthe amount of activity to be performed aswell as with the amount of insulin taken.Unplanned exercise that is not coordinat-ed with caloric intake can create an im-balance between the amount of insulinpreviously taken and the amount of glu-cose remaining available in the blood.Individuals with IDDM must learn to bal-ance exercise, insulin, and blood glucoselevels to prevent hypoglycemia (insulin
shock ). Considerable time and effort may
be spent in learning how exercise of a giv-en intensity and duration affects bloodglucose levels, and what adjustmentsmust be made in eating patterns andinsulin dosages to compensate.
Self-monitoring of blood glucose levels
is also important in the overall treatmentof diabetes. Monitoring of blood glucoselevels helps to determine the efﬁciency ofthe insulin dosage prescribed. Individualswho take insulin should monitor bloodglucose levels at least several times a day.Many individuals monitor glucose levelsbefore breakfast, lunch, and dinner, as wellas at bedtime. Monitoring gives theminformation about the level of sugar in theblood and consequently changes in treat-ment that may be appropriate. Forinstance, if the blood sugar level is toolow, they may need to ingest a “quick”sugar such as orange juice to preventsevere hypoglycemia. If the blood sugarlevel is too high, they may need to injectadditional insulin.
There are a variety of different types of
techniques available for testing blood sug-ar. Individuals may monitor their ownblood glucose levels by lancing their ﬁn-ger and using a small portable machinecalled a glucometer to assess the glucose
content of the blood. Blood glucose mayalso be monitored through a device forcontinuous monitoring that uses a tinysensor inserted just beneath the skin, usu-ally on the abdomen. The monitor recordsup to 288 readings per day for up to 3days. At the end of 3 days the sensor isremoved and the stored data are down-loaded to a computer. The data enableConditions of the Endocrine System 267

physicians to make appropriate changes ininsulin doses based on the glucose read-ings (Bode, Sabbah, & Davidson, 2001).During the time the continuous monitor-ing device is being used, individuals con
tinue to use standard methods of meas-
uring blood glucose, since the monitor does
not display real-time glucose levels. Indi-viduals may learn to alter their own in-
sulin levels in accordance with their home
blood glucose reading; however, suchalterations should always be done with theadvice and supervision of a physician.
Type II (Non-Insulin-Dependent
Diabetes Mellitus)
Although many of the same aspects of
treatment for Type I diabetes also apply toindividuals with NIDDM, some individu-
als with NIDDM may control blood sugar
levels with diet alone. In other instances,weight loss may help to control the con-dition. When blood sugar levels are not
controlled by following a carefully planned
diet, individuals may need to take hypo-
glycemic agents/oral agents (oral medica-
tions that are effective in lowering bloodsugar). There are several different types oforal medications available. When oralmedications do not adequately controlblood sugar, individuals with NIDDM mayneed to also take supplemental insulin.
Diabetic Coma and Insulin Shock
Careful control of blood sugar is impor-
tant to prevent complications, as dis-cussed later in the chapter; however,another major concern is the potentiallyfatal acute conditions of diabetic coma or
insulin shock . Diabetic coma occurs when
there is too much circulating glucose inthe blood. The onset of diabetic coma maybe gradual. Few symptoms may appear
until the blood sugar level becomes severe-ly elevated. Individuals may become con-fused, drowsy, and then eventually slipinto unconsciousness. They may have dif-ﬁculty breathing or experience nausea,vomiting, and ﬂushing of the skin, whichremains dry. Water depletion and dehy-dration are common. Characteristically,the breath of individuals in diabetic comahas a fruity odor. Diabetic coma is a med-ical emergency that can result in death ifappropriate treatment is not initiated.Medical treatment is directed toward low-ering the level of blood sugar through theinjection of insulin and correcting dehy-dration and electrolyte imbalance throughthe intravenous infusion of ﬂuids.
Insulin shock is the opposite of diabet-
ic coma, occurring when there is toomuch insulin in the blood for the amountof glucose present. Insulin shock mayresult from injecting too much insulin,from engaging in an unusual amount ofexercise that burns up the glucose normal-ly available, or from failing to take in suf-ﬁcient amounts of food for the amount ofinsulin injected. Individuals going intoinsulin shock may feel hungry, weak, andnervous. They may perspire profusely,although their skin is cold to the touch.Confusion and personality changes mayalso occur. If insulin shock is untreated,individuals may lapse into unconscious-ness. If it continues to go untreated, braindamage and eventual death can result.Treatment of insulin shock is directed to-ward raising blood sugar levels. If individ-uals are conscious, simple sugars such ascandy, orange juice, or honey may beingested orally; if individuals are uncon-scious, glucose must be infused intra-venously.
Complications of Diabetes Mellitus
Individuals with diabetes mellitus,
whether Type I or Type II, are susceptible268 CHAPTER 9 E NDOCRINE CONDITIONS

to a number of complications that canaffect a number of different body systemsand result in major disability (Stevens etal., 2001; Strauss, 2001). The exact reasonindividuals with diabetes mellitus devel-op these complications is unknown, al-though there does appear to be a link tothe length of time they have had diabetesmellitus and the degree to which glucosehas been controlled.
Some complications are related to the
circulatory system. Vascular changes can
contribute to myocardial infarction
(heart attack; see Chapter 11) or cerebro-
vascular accident (stroke; see Chapter 2).
They may also lead to poor circulation inthe extremities ( peripheral vascular in-
sufﬁciency ; see Chapter 11), so that even
minor injuries are prone to become infect-ed and may become so severely infectedthat amputation (see Chapter 14) is nec-
essary. Vascular changes may also deprivethe kidney of an adequate blood supply,causing kidney failure and requiring dialy-
sis(see Chapter 13). Changes in blood
vessels in the retina (retinopathy ) can
result in blindness (see Chapter 4). Other
complications associated with diabetesmellitus may involve changes in the nerv-
ous system . Changes in the peripheral
nerves , or peripheral neuropathy , may
result in the loss of sensation in theextremities, so that the protective sensa-tion of pain is absent, further makingthem prone to injury. Inappropriatefootwear is the most common source oftrauma to the feet of individuals with dia-betes, resulting in foot ulcers that can leadto need for amputation (Boulton, Kirsner,& Vileikyte, 2004). Consequently, appro-priate foot care is a necessity to preventserious complications.
Other effects of neuropathy may be sex-
ual impotence in men and decreased gen-ital sensation in women. Individuals withdiabetes mellitus have a higher incidenceof surgery (such as cardiovascular surgery,amputation, or ophthalmological proce-dures) related to their complications andare also at higher risk for postsurgical com-plications because of poor wound healing,increased infection, and increased risk of
acute renal failure (Plodkowski & Edelman,
2001).
The risk that individuals with diabetes
mellitus will develop complications is vari-able. Factors such as the type of diabetes,the age of onset, the duration of the dis-ease, and the degree to which individualsfollow the prescribed protocol must beconsidered.
Psychosocial Issues in Diabetes Mellitus
Diabetes mellitus not only involves life-
long multifaceted treatment, but it alsosigniﬁcantly affects individuals’ daily livesand futures, especially if complications de-velop. Psychological as well as physiologi-cal factors frequently determine the courseof diabetes mellitus. Psychological factorsmay affect the management of diabetesdirectly by inducing metabolic changesthat can affect individuals’ ability to con-trol blood glucose levels or indirectly byaltering the degree to which individualsfollow instructions related to medication,diet, and exercise. Motivation to followthe prescribed treatment is paramount inthe control of diabetes mellitus.
Diabetes mellitus is a hidden disability,
since its symptoms are not visible. Othersmay see no indication of chronic illnessor disability that imposes restrictions, andtherefore they may have no expectationsthat individuals may be restricted regard-ing some aspects of lifestyle or activity. Ifthe individuals with diabetes have notadapted to their condition or if they fearsocial rejection because of their condition,they may attempt to hide their diagnosisConditions of the Endocrine System 269

from others, ignoring dietary restrictionsor engaging in activities outside theirtreatment plan. Some individuals maybelieve that following a diabetic diet drawsattention to the condition and, therefore,may neglect their diet.
In some instances, the beneﬁt of care-
ful adherence to the recommended regi-men is not always apparent to individualswith diabetes mellitus. Even though in-structions have been followed carefully,the blood glucose level may remain elevat-ed, or complications may still develop.Such occurrences can result in discourage-
ment and depression. If emphasis is placed
on the restrictions associated with treat-ment of diabetes, individuals may feeldepressed and hopeless.
Fear of complications that may lead to
blindness or possible amputation may cre-ate additional anxiety. For some individ-uals, these feelings are overwhelming.Self-destructive behaviors, such as skip-ping insulin injections and/or abandoningthe diet, both of which can imperil theirlife, may result.
Many lifestyle changes are necessary for
individuals with diabetes mellitus, espe-cially for those with IDDM. Although dietand insulin dosage can be adjusted toaccount for different types of activities,advance planning is essential. Activities,including exercise and meal times, shouldgenerally be consistent from day to day.Eating on the run or skipping meals isnot feasible. If the schedule changes, foodintake and insulin dosage must bechanged accordingly. If activities involveadditional walking, comfortable and well-ﬁtting shoes should be worn to avoid for-mation of blisters that could becomeinfected.
Individuals with diabetes mellitus
should check with their physician aboutinsulin and food schedules before travel-ing, especially across time zones. If trav-eling by plane, they should request specialmeals ahead of time, and they should beserved at the time required for the regi-men. They should carry insulin with themand should protect it against extremes oftemperature. With guidance from physi-cians or dietitians, individuals can learn toaccommodate meals served at restaurantsor in other people’s homes. The quantityand types of foods must be taken intoaccount, however. Individuals with dia-betes must learn to judge calories and por-tions, and fatty, rich foods should beeliminated from the diet. Although con-centrated sweets and alcohol should usu-ally be avoided, planning may permit theincorporation of small quantities into thediet for special occasions.
Diabetes mellitus does not usually affect
sexual activity unless there are complica-tions. Neuropathy may be the cause ofimpotence in men and decreased sensa-tion in women. Frequent vaginal infec-tions in women with diabetes may alsoalter sexual activity because of the physi-cal discomfort involved. Reproductivefunction is not affected in men who arenot impotent. Women with diabetes mel-litus who become pregnant generallyhave more complicated pregnancies andneed special medical attention to monitorthe progress of the pregnancy and to alterinsulin and caloric requirements.
The effects of any chronic disease are
not limited to the individuals with thecondition. This is especially true of dia-betes mellitus, because so many lifestylefactors are involved in the adequate man-agement of the condition. Often, thedegree to which individuals follow theprescribed treatment protocol depends onthe degree of social support they receive.The eating habits of family members, aswell as their understanding of the impor-tance of the diet prescribed for the indi-vidual with diabetes, can contribute270
CHAPTER 9 E NDOCRINE CONDITIONS

signiﬁcantly to the individual’s willingnessto adapt to and follow the diabetic diet.Acceptance and understanding of diabetesand its restrictions by friends and col-leagues also contribute to individuals’ self-concept and subsequent acceptance oftheir condition.
The effect that a diagnosis of diabetes
has on the family of individuals with dia-betes depends on family composition, thefamily’s usual coping mechanisms, the ageof the individual at the onset of diabetes,the regimen prescribed, perceptions offuture disability, and how the family func-tioned before the diagnosis was made. Ifindividuals with diabetes do not controltheir diet or prepare their own meals, thefamily member assuming this responsibil-ity has new status and inﬂuence. This cancreate another source of support or, insome instances, a source of sabotage of theregimen itself.
The impact of diabetes on other social
relationships varies. In social situationswhere food and alcohol are the majorfocus of activity, individuals with diabetesmellitus may need to modify their par-ticipation, although they need not total-ly avoid such situations. Depending onthe individual and others in the social set-ting, modiﬁcations may or may not havean impact on the social relationshipitself.
Individuals with diabetes mellitus are
constantly aware of the need to complywith dietary restrictions, the need to eatat regular times, the need to balance activ-ity with calories, and the need to stickthemselves several times a day to injectinsulin or to test their blood glucose lev-el. These factors can make them feel aloneand different if they do not have socialsupport at work or at home.
Since diabetes mellitus is an invisible
disability, couples planning to marry maynot discuss diabetes and its effect on themarital relationship or on plans for chil-dren. Depending on the maturity, under-standing, and expectations of both indi-viduals in the marital relationship, prob-lems related to the presence of diabetesmay emerge, especially in the decision tohave children or in the management ofcomplications, should they arise.
Vocational Issues in Diabetes Mellitus
The type of diabetes, the demands of
the job, individuals’ willingness and abil-ity to carry out treatment recommenda-tions, and the degree to which the pre-scribed protocol controls their diabetesdetermine the special needs of individu-als in the work environment. Certainmodiﬁcations in employment may be nec-essary to accommodate their condition.First, the activity level should be consis-tent as much as possible, or activityshould be planned so that it is balancedwith food intake and insulin or thedosage of oral hypoglycemic agents. If atall possible, rotating shifts or irregularschedules should be avoided because ofthe alterations in insulin and food sched-ules that would be required for individu-als with Type I diabetes.
Work in which there is risk of even
minor cuts and scratches, especially to thefeet, should be avoided because of the riskof infection. Emotional stress has a directimpact on the blood glucose level.Consequently, individuals with diabetesmellitus should learn coping strategiesthat enable them to deal effectively withjob stress, or they should avoid overlystressful job situations, if possible.
Despite the ability of many individuals
to effectively control their diabetes, dis-crimination in employment still occurs(American Diabetes Association, 2004b).Employers may fear that individuals are aConditions of the Endocrine System 271

safety risk or that ﬂuctuations in bloodglucose levels may cause unexpected inca-pacity. Many individuals are able to rec-ognize early warning signs of high or lowglucose levels and are therefore able totake steps to counteract physical reactionsso that risk is minimal. Symptoms ofinsulin reactions are, however, variablefrom individual to individual. In someinstances, individuals may become desen-sitized to symptoms and therefore not rec-ognize the need to intervene before thereaction occurs (Martz, 2003).
Employers should generally be informed
of an employee’s diagnosis of diabetesmellitus so that misunderstandings aboutthe need for regular meal schedules, rou-tine activities, and avoidance of injury do
not develop. In addition, employers should
be alerted to the symptoms of diabeticcoma or insulin shock so that appropriateaction may be taken if either of theseevents occurs.
The potential for complications should
be considered in vocational planning.Although following treatment protocolprecisely does not guarantee that compli-cations will not develop, maintaininggood control of
blood glucose levels can
decrease the num ber of days lost from
work due to minor complications. Whencomplications do develop, alterations inemployment are speciﬁc to the type ofcomplication. For example, individualswith peripheral neuropathy or poor circu-lation to the lower extremities may needto avoid occupations that require exces-sive walking or standing. Individuals whodevelop diabetic retinopathy may needspecial low-vision aids. Development ofperipheral neuropathy of the upperextremities may interfere with sensationand manual dexterity. Because of the pos-sibility of diabetic coma or insulin shock,individuals with diabetes mellitus shouldnot work in isolation.DIAGNOSTIC PROCEDURES FORCONDITIONS OF THE ENDOCRINESYSTEMBlood Tests for Thyroid Function
A number of tests are available to assess
thyroid function. Examples of blood testsare the serum thyroxine (T4) and free thy-
roxine index tests. These tests measure
either the exact or relative amount of thy-roid hormone in the blood. In addition,a blood test that measures the level of TSHin the blood is an accurate assessment ofthyroid hormone levels.
Blood Tests for Diabetes Mellitus
The major blood tests used in the diag-
nosis of diabetes mellitus are determina-tions of the fasting blood glucose and
postprandial plasma glucose levels, and the
oral glucose tolerance test . In the fasting
blood glucose test, blood is drawn after the
individual has not eaten for a number ofhours. For a postprandial plasma glucose
test, blood is drawn several hours after
individuals have eaten. Blood is drawn forthe oral glucose tolerance test while individ-
uals are fasting. Individuals are then giv-en concentrated glucose in liquid form to
drink, and blood samples are drawn at 1-,
2-, and 3-hour intervals. All three testsmake it possible to compare the level ofglucose in individuals’ blood with the lev-el expected in persons without diabetesmellitus under similar circumstances.
GENERAL TREATMENT OFENDOCRINE CONDITIONS
For many endocrine conditions, treat-
ment involves replacement of hormonesif there is insufﬁcient production, or ad-ministration of medication to decreaseproduction of hormones if hormones are272
CHAPTER 9 E NDOCRINE CONDITIONS

being overproduced. Although in someinstances surgery may be indicated, it isnot always curative.
PSYCHOSOCIAL AND VOCATIONALISSUES IN ENDOCRINE CONDITIONSPsychological Issues
The changes in hormonal patterns asso-
ciated with conditions of the endocrinesystem may cause behavioral changes thatresult in misdiagnosis, delayed treatment,and subjection of individuals to unnec-essary hardships. Individuals with treat-able endocrine disorders have beendiagnosed as having mental disorders andat times even placed in institutions, whilethe real cause of their symptoms is leftuntreated.
Endocrine disorders can cause a broad
range of emotional and psychiatric symp-toms. For example, individuals with thy-roid disease may experience emotionaloutbursts, irritability, or anxiety symp-toms that are not always recognized asmanifestations of their disease. Olderadults with a thyroid disorder maydemonstrate memory impairment, whichis misdiagnosed as Alzheimer’s disease oranother dementia that goes untreated. Inmost cases, changes in behavior are tem-porary and steadily improve as theendocrine condition is corrected. In chil-dren, unrecognized endocrine disorderscan cause permanent disability, such asmental retardation. Recognition of therole of the endocrine system and varioushormones in children’s cognitive develop-ment has resulted in earlier recognitionand treatment, in many cases preventingdisability from occurring.
Changes in physical appearance, such
as the exophthalmos associated with thy-roid disease or the physical changes asso-ciated with Cushing’s syndrome, candisturb individuals’ body image, causingsubsequent emotional reactions. Treat-ment of many endocrine conditions in-volves long-term or lifelong ingestion ofmedications. For some individuals, takingmedication daily creates frustration andresentment, which lead to noncompliancewith treatment and the development ofsubsequent
complications or a recurrence
of the disease.
Lifestyle Issues
For most individuals with endocrine dis-
orders, after the condition has been stabi-lized and barring complications, primarylifestyle changes involve remembering totake medications at the same time everyday. The exception is, of course, diabetesmellitus, in which lifestyle changes are asigniﬁcant part of the treatment of thecondition and are necessary for survival.
Social Issues
Many social issues associated with endo-
crine disorders depend on the speciﬁccondition. For example, individuals withhyperthyroidism may experience socialisolation because of associated behaviorchanges that occur before treatment isinstituted. Physical changes caused byendocrine conditions, such as those asso-ciated with Cushing’s syndrome, may leadto self-consciousness and cause individu-als to withdraw from social activities. Thedemands of diabetes can also cause stressin families, especially to siblings of chil-dren with Type I diabetes, causing isola-tion and resentment (Hollidge, 2001).
VOCATIONAL ISSUES INENDOCRINE CONDITIONS
In most instances, individuals with
conditions of the endocrine system thatVocational Issues in Endocrine Conditions 273

have been identiﬁed and are being treat-ed have no special vocational needs.When hormone replacement therapy ispart of the treatment, however, the impor-tance of complying with the prescribedmedical regimen cannot be overstated.This is, again, especially true of individu-als with diabetes mellitus, the vocationalimplications of which were discussed ear-lier in the chapter.
CASE STUDIESCase I
Ms. T., a legal secretary with an associ-
ate’s degree, is 35 years old and hasworked for a law ﬁrm for the past 15 years.Over the past year her employer expressedincreasing concern about her job perform-ance, citing her decreased ability to keepup with work, and she was ﬁnally termi-nated. Ms. T. states that over the past yearshe had become increasingly fatigued andadmits she had difﬁculty keeping up withwork. She is married with two childrenwho are ages 12 and 14. Her family hadalso noticed a change in her behavior andencouraged her to seek medical attention.Her physician attributed her symptoms todepression and prescribed antidepressantmedication. Her symptoms continued toworsen over the next 3 months until dur-ing a routine eye examination the oph-thalmologist noticed changes suggestiveof thyroid disease and recommended thatshe have a blood test. She was found tohave severe hypothyroidism, and whenshe was placed on thyroid medication, hersymptoms improved dramatically.
Questions
1. Are there limitations resulting from
her condition that will affect Ms. T.’srehabilitation potential?2. How would you assist Ms. T. in deter-
mining her vocational goals?
3. Would you approach Ms. T.’s former
employer about reinstating her in herformer job?
4. What other factors might you consid-
er in helping Ms. T. develop her reha-bilitation plan?
Case II
Mr. W. is a 49-year-old jewelry distrib-
utor and works for a major jewelry com-pany. He lives in Minnesota with his wifeand one daughter, who is 12. Mr. W.’s jobis to supply jewelry to major departmentstores in a 300-mile radius of his home,which involves frequent trips away fromhome. He travels alone by car and mustengage in frequent business lunches anddinners with clients. Mr. W. was diagnosedwith Type I (insulin-dependent diabetes)when he was 22. He has a bachelor of sci-ence degree in business administration.His diabetes has been under moderatecontrol; however, he recently has hadincreasing visual difﬁculty from complica-tions of diabetic retinopathy and also hashad difﬁculty with circulation in his low-er extremities.
Questions
1. What factors regarding the demands
of his current job would you consid-er given his diagnosis?
2. What impact might his complica-
tions have on his ability to continuein his current line of employment?
3. What other factors should you con-
sider when estimating Mr. W.’s reha-bilitation potential?
4. Are there other issues or concerns
regarding his diagnosis that should beconsidered when working with Mr.W. to develop his rehabilitation plan?274
CHAPTER 9 E NDOCRINE CONDITIONS

References 275
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American Diabetes Association. (2004b). Hypogly-
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Bode, B. W., Sabbah, H., & Davidson, P. C. (2001).
What’s ahead in glucose monitoring. Postgraduate
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Boscaro, M., Barzon, L., Fallo, F., & Sonino, N. (2001).
Cushing’s syndrome. Lancet, 357 (9258), 783–791.
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Chandalia, M., Garg, A., Lutjohann, D., von
Bergmann, K., Grundy, S. M., & Brinkley, L. J.(2000). Beneﬁcial effects of high dietary ﬁberintake in patients with type 2 diabetes mellitus.New England Journal of Medicine, 342 (19),
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The Diabetes Control and Complications Trial
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Hoffman, R. P. (2001). Eating disorders in adolescents
with type I diabetes. Postgraduate Medicine, 109 (4),
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Hollidge, C. (2001). Psychological adjustment of sib-
lings to a child with diabetes. Health and Social
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Ludwig, D. S., & Ebbeling, C. B. (2001). Type 2 dia-
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Martz, E. (2003). Living with insulin-dependent dia-
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Olefsky, J. M. (2001). Diabetes mellitus. Journal of the
American Medical Association, 285 , 628–632.Plodkowski, R. A., & Edelman, S. V. (2001). Pre-sur-
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G. S., Toth, E., Warnock, G. L., Kneteman, N. M.,& Rajotte, R. V. (2000). Islet transplantation in sev-en patients with type 1 diabetes mellitus using aglucocorticoid-free immunosuppressive regimen.New England Journal of Medicine, 343 (4), 230–238.
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NORMAL STRUCTURE ANDFUNCTION OF THEGASTROINTESTINAL SYSTEM
The gastrointestinal tract ( alimentary
canal ) is a hollow, muscular tube approx-
imately 30 feet long (Figure 10–1). Its prin-cipal purpose is to provide a mechanismwhereby nutrients and liquids can be tak-en into the body for energy and tissuegrowth, and through which wastes fromthe digestive process can be eliminated.
The digestive process begins in the
mouth, sometimes called the oralor buc-
cal cavity , where teeth break food into
smaller particles. The teeth at the front ofthe mouth ( incisors ) provide a cutting
action, while the teeth at the back of themouth ( molars ) provide a grinding action.
Chewing is important to the digestiveprocess. Breaking food into smaller parti-cles facilitates the passage of the food intothe stomach and also enlarges the surfacearea available for the gastric juices as thedigestive process continues in the stom-ach. While still in the mouth, smaller par-ticles of food are mixed with saliva , a ﬂuid
secretion in the mouth that lubricates andsoftens food and that also facilitates itspassage down the throat. Produced by theparotid glands , submaxillary glands , and sub-lingual glands , saliva contains an enzyme
that begins the breakdown of starches.
Food passes from the throat ( pharynx )
into a muscular tube called the esopha-
gus, which leads from the mouth to the
stomach. The esophagus and windpipe(trachea ) have a common opening at the
pharynx. Consequently, a ﬂap called theepiglottis closes over the opening to the
windpipe when food is swallowed, ensur-ing that food will pass into the esophagusrather than the windpipe. The esophagusis approximately 10 inches long andmoves food along through rhythmic,muscular movements called peristalsis .
The esophagus passes through a muscu-
lar wall called the diaphragm that sepa-
rates the thoracic (chest) cavity from the
abdominal cavity . The abdominal cavity
contains the stomach, intestines, and oth-er abdominal organs and is lined with athin membrane called the peritoneum .
The esophagus passes through thediaphragm in order to reach the stomach .
Food enters the stomach from the esoph-agus through an opening called the low-er esophageal sphincter (LES), sometimescalled the cardiac sphincter . Pressure gradi-
ents around this opening prevent thebackﬂow of food and gastric juices intothe esophagus from the stomach.Conditions of the
Gastrointestinal SystemCHAPTER 10
277

The stomach is a muscular organ that
stores, mixes, and liqueﬁes food. It con-tains gastric juices that continue the di-gestive process. One component of gastricjuice, hydrochloric acid, has a sterilizingeffect, killing most organisms that enterthe stomach. Pepsin, the primary enzymeof gastric juice, digests protein in the pres-ence of hydrochloric acid. Also producedin the stomach is a substance called theintrinsic factor that is necessary for theabsorption of vitamin B12. Gastic secre-tion is stimulated by the vagus nerve , as
well as by the presence of food in thestomach. The stomach lining is protectedfrom irritation and from the action of thegastric enzymes by a thin layer of mucussecreted by tiny glands within its lining.Although some alcohol, water, sugars, anddrugs are absorbed in the stomach, mostdigestion and absorption take place in thesmall intestine.
From the stomach, food passes through
an opening called the pyloric sphincter into
the small intestine. The small intestine isapproximately 22 feet long and is divid-ed into three parts. The ﬁrst part of thesmall intestine, the duodenum , is approx-
imately 10 inches long and is connectedto the stomach at the pyloric sphincter . The
middle section, the jejunum , is approxi-
mately 8 feet long. The last part of thesmall intestine, the ileum, connects to thelarge intestine , and is approximately 12 feet
long. Digested food continues to movethrough the gastrointestinal tract by peri-
staltic movements . Most nutrients are ab-
sorbed in the small intestine. Some ﬂuid278
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM
Pharnyx
Trachea
Liver
Gallbladder
Duodenum
Common Bile ductEsophagus
Diaphragm
StomachPancreasPyloric SphincterJejunumColonIleumSigmoid Colon
Figure 10–1 Gastrointestinal System.

Conditions of the Gastrointestinal System 279
is also absorbed in the small intestine;however, most ﬂuid is absorbed in thelarge intestine . Thus the contents of the
small intestine tend to be liquid in nature.
The small and large intestines are con-
nected by the ileocecal valve , which allows
the contents of the small intestine to ﬂowinto the large intestine but prevents back-ﬂow. The large intestine ( colon ) is only
about 5 feet long, but, like the small intes-tine, it is divided into parts. The partattached to the small intestine at the ileo-cecal valve is the cecum , to which the
appendix is attached. The major portion of
the large intestine, the colon , is divided
into the ascending colon , the transverse
colon , the descending colon , and the sigmoid
colon . The sigmoid colon leads to the rec-
tum, which leads to the anus, the open-ing through which solid waste is excretedfrom the body. The large intestine collectsfood residue and is the site of most waterabsorption from intestinal contents.Consequently, waste products ( feces ) con-
tained in the large intestine are more sol-id. The brown color of feces is dueprimarily to bile pigments .
The liver, gallbladder , and pancreas ,
sometimes called the accessory organs of
digestion , are located together in the upper
abdominal cavity . The liveris the largest sin-
gle organ in the body and is necessary forsurvival. In addition to aiding in diges-tion, the liver is important to carbohydrate ,
protein , and fat metabolism . The liver:
• converts glucose, a product of carbo-
hydrate metabolism, into an energysource, glycogen.
• stores glycogen until the body needs
it.
• converts the end products of protein
metabolism into urea, which is laterexcreted by the kidneys.
• manufactures and secretes bile for the
digestion and absorption of fat.
• breaks down red blood cells.• produces substances important for the
clotting of blood.
• acts as a detoxiﬁcation center of the
body, detoxifying poisonous chemi-cals and drugs.
Two major blood vessels enter the liv-
er. The hepatic artery carries oxygenated
blood for the liver itself. The portal vein
carries blood to the liver from the pancreas ,
spleen , stomach , and intestine . Blood in the
portal vein contains nutrients and toxinsfor either metabolism or detoxiﬁcation by the
liver. The gallbladder , a small sac that stores
bile, is located on the underside of the liv-er. Bile leaves the liver via the hepatic ducts
and enters the gallbladder through the cys-
tic duct . When the gallbladder contracts,
bile ﬂows through a structure called thecommon bile duct into the small intestine.
Bile, along with bile salts, contains biliru-
bin, an orange pigment formed from the
breakdown of red blood cells. Bile salts areimportant to fat digestion and absorption.
The pancreas, in addition to its endocrine
function of producing the hormone insulin
(see Chapter 9), also plays an importantrole in digestion. The pancreas lies behindthe stomach and produces pancreatic juice ,
which contains enzymes to digest fats, car-bohydrates, and proteins. Pancreatic juicesenter the common bile duct through thepancreatic duct and then continue to the
small intestine.
CONDITIONS OF THEGASTROINTESTINAL SYSTEMConditions of the Mouth
Although not always disabling, disor-
ders of the mouth can contribute to illnessand disability by interfering with nutri-tion. Tooth decay ( dental caries ) and peri-
odontal disease (disease of the tissues that
surround and support the teeth) can leadto the loss of the teeth. Periodontal disease

can also contribute to other systemic dis-eases, such as cardiovascular disease(Janket et al., 2004). With periodontal dis-ease, gum tissue may separate from thetooth, leading to the destruction of under-lying tissues. The early form of the diseaseis called gingivitis (inﬂammation of the
gums). If untreated, periodontitis (a more
severe form of gum disease) may develop.
Periodontitis can affect the supporting
structures of the teeth, causing the teethto become loose and possibly fall out. Lossof teeth has implications not only for cos-metic appearance but also for nutritionand general health. The inability to chewfood adequately may limit the food typestaken in as well as interfere with the be-ginning digestive process. Although den-tures may help cosmetically and increasethe ability to chew food, they are not aseffective as natural teeth for chewing.Dental caries and periodontal disease arebest treated through prevention, earlydetection, and early treatment.
Other disorders of the mouth that in-
terfere with proper nutrition are stomati-
tis(inﬂammation of the mouth) and
parotitis (inﬂammation of the parotid
glands). Stomatitis can be the result ofinfection, injury, toxic agents, or systemicillness. Parotitis can result from inactivi-ty of the glands due to lack of oral intake,can be caused by infection, or can be aside effect of medications or general anes-thesia. Treatment of both stomatitis andparotitis is directed toward correcting oralleviating the underlying cause.
Conditions of the Esophagus
General Conditions of the Esophagus
Dysphagia (difﬁculty in swallowing) is
a major symptom of a variety of disorders.One cause of dysphagia is stricture (nar-
rowing) of portions of the esophagus be-cause of injury or obstruction. When dys-phagia is caused by narrowing or constric-tion of the esophagus, the goal oftreatment is to widen the opening of thepassageway. The opening may be dilatedrepeatedly with a dilating instrument, orsurgical repair may be necessary.
When narrowing is due to a tumor , sur-
gical removal of the tumor or part of theesophagus may be indicated. Dysphagiamay also be caused by neurologic disorders ,
such as stroke or multiple sclerosis ; or car-
diovascular conditions , such as an enlarged
heart. Achalasia (cardiospasm) is a type of
dysphagia believed to be caused by degen-eration of the nerves that supply the mus-cles of the esophagus. As a result, themotility of the lower portion of theesophagus is decreased, and food is unableto pass into the stomach efﬁciently andaccumulates within the lower esophagus,causing esophageal irritation (esophagitis )
and regurgitation . Emotional upsets can
aggravate the problem. In addition to thediscomforts of esophagitis and the embar-rassment of regurgitation, aspiration ofundigested food particles into the lungsmay occur, resulting in atelectasis (see
Chapter 12).
The aim of the treatment of achalasia is
to reduce the amount of pressure at thelower end of the esophagus, thus reduc-ing the extent of the obstruction. Theopening between the stomach and esoph-agus may be dilated mechanically with theuse of a dilating instrument, or, in moresevere cases, surgery that involves cuttingthe muscle ﬁbers of the sphincter of thelower esophagus may be indicated.
Dyspepsia (indigestion) is also a com-
mon symptom of esophageal disease.Dyspepsia may be experienced alone or incombination with dysphagia. Among thecauses of dyspepsia is esophageal reﬂux , in
which stomach contents ﬂow back intothe esophagus, irritating the esophageal280
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

lining. Esophageal reﬂux may be treatedby using medications (e.g., antacids or
cimetidine ) that decrease acidity, by avoid-
ing smoking, and by avoiding foods orbeverages that seem to increase gastricacidity and discomfort. Mechanical meas-ures may include sleeping with the headof the bed raised to minimize the amountof reﬂux by gravity.
Hiatal Hernia (Esophageal Hernia;
Diaphragmatic Hernia)
The esophagus passes through an open-
ing in the diaphragm to the stomach.When the opening becomes stretched orweakened, the stomach may protrudethrough the opening in the diaphragminto the thoracic cavity. This condition iscalled a hiatal hernia . It allows gastric
juices to come into contact with theesophageal wall, causing esophagitis
(inﬂammation of the esophagus), dyspep-
sia(indigestion), and possible ulceration
of the esophagus. Individuals with ahiatal hernia may experience mild tosevere pain and discomfort with thedevelopment of esophagitis.
Although a hiatal hernia may not cause
extensive debilitation, the discomfort andpotential complications may interferewith individuals’ sense of well-being andsubsequent productivity. If symptoms aremild, treatment of hiatal hernia may besimilar to the treatment of esophagealreﬂux, as described above. To decrease thefrequency of symptoms, individuals witha hiatal hernia may need to refrain fromany activity that increases intra-abdomi-nal pressure, such as strenuous exerciseand bending. In addition, they may needto modify the timing and size of meals(such as having four to six small meals aday) to decrease the amount of gastric acidthe stomach produces. Raising the head ofthe bed approximately 6 inches whilesleeping may also improve symptoms. Inother instances, hiatal hernia may berepaired surgically. Surgery returns thestomach to its normal position and makesthe opening in the diaphragm smaller sothat the stomach cannot again moveabove the diaphragm.
Gastroesophageal Reﬂux Disease
(GERD; Reﬂux Disease)
Gastroesophageal reﬂux disease (GERD)
is a condition of the digestive tract thataffects the LES, which connects the esoph-agus and the stomach. During normaldigestion, when food is swallowed, theLES opens to allow food into the stomach.After the food passes the sphincter, theLES normally closes like a door to preventfood and stomach acids from coming incontact with the esophagus. When thesphincter becomes weakened, it may notclose adequately, allowing stomach con-tents to ﬂow backward ( reﬂux ) into the
esophagus ( acid regurgitation ), causing
inﬂammation of the esophagus ( esophagi-
tis). When this happens, individuals expe-
rience symptoms commonly known asheartburn or acid indigestion . The symp-
toms are pressure and burning chest pain,often moving upward to the neck and thethroat.
A variety of lifestyle and dietary factors
have been implicated as playing a role inthe cause of GERD; however, there are stillconﬂicting ﬁndings regarding the impactof most of these factors, including the roleof alcohol and tobacco (Meining &Classen, 2000). Fatty meals, sweets, car-bonated beverages, juices and citrus prod-ucts, large meals, and obesity have all beenimplicated as potential causes. GERD mayalso be related to hiatal hernia, as de-scribed above.
In addition to causing significant
discomfort, GERD symptoms have beenConditions of the Gastrointestinal System 281

found to precede the diagnosis of cancerin about 60 percent of individuals withesophageal adenocarcinoma (see Chapter
16) (Lagergren, Bergstro, Lingren, et al.,1999).
Diagnosis is based on symptoms, and in
some instances an endoscopy (examina-
tion of the esophagus through a hollowtube) is performed, usually by a physicianspecializing in conditions of the gastroin-testinal tract ( gastroenterologist ). Short-
term treatment of GERD usually consistsof using medications such as H
2receptor
antagonists (cimetidine) or medications
called proton pump inhibitors (PPIs) (Cohen
& Parkman, 2000). Lifestyle modiﬁcationssuch as avoiding foods, exercises, or posi-tions that seem to aggravate the conditionare also incorporated into treatment. Insome instances, treatment remains longterm and is directed to helping individu-als control symptoms when they occur(Dent, 2001).
Conditions of the Stomach
Gastritis
Gastritis is an inﬂammation of the lin-
ing of the stomach that can be caused bya variety of irritants or infectious agents.Acute gastritis is of short duration, with
symptoms of nausea, vomiting, and pain.It is generally self-limiting, requiring lit-tle except symptomatic treatment. Chron-ic gastritis, which is of longer duration,may consist of nondescript upper abdom-inal distress with vague symptoms. Ex-tensive evaluation may be necessary toidentify causative factors. It may be due toirritation of the stomach from medica-tions used to treat another condition, orit may be a symptom of a more serious ill-ness. Untreated, chronic gastritis canprogress to scarring of the stomach lining,ulceration, or hemorrhage.Peptic Ulcer Disease
Types of Peptic Ulcers
Peptic ulcer disease (PUD ) is a chron-
ic inﬂammatory gastrointestinal disordercharacterized by ulcer (sore) formation inthe esophagus, stomach, or duodenum.Peptic ulcers in the upper portion of thesmall intestine are called duodenal ulcers ;
those in the stomach are called gastric
ulcers . Duodenal ulcers occur more fre-
quently than gastric ulcers.
Until the 1980s, spicy food, acid, stress,
and lifestyle were considered major caus-es of ulcers. In 1982 the bacterium Helico-
bacter pylori (H. pylori ) was discovered and
was found to cause more than 90 percentof duodenal ulcers and up to 80 percentof gastric ulcers (Centers for Disease Con-trol and Prevention, 2004). Other risk fac-tors for PUD include the use of aspirin ornonsteroidal inﬂammatory drugs, chron-ic renal failure, chronic obstructive pul-monary disease, hyperparathyroidism,renal transplantation, and alcoholic cir-rhosis (Laird, 1999).
Although some foods and beverages
(e.g., alcohol and caffeine-containing bev-erages) increase gastric secretion and canirritate the lining of the gastrointestinaltract, there is no evidence to suggest thatthe intake of these substances causes ulcerdisease.
Another type of peptic ulcer, a stress
ulcer, may develop after an acute medical
crisis, such as a severe injury or a cata-strophic illness. Special names are given tostress ulcers that develop with some con-ditions. For example, stress ulcers associ-ated with burns are called Curling’s ulcers ;
those associated with head injury arecalled Cushing’s ulcers . The reason that
these ulcers develop is unknown; howev-er, they develop rapidly, sometimes with-in 72 hours of the injury or illness. Symp-282
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

toms may not appear until the ulcer perfo-
rates and massive gastric hemorrhage occurs.
Symptoms and Complications of
Peptic Ulcer Disease
The most common symptom of a pep-
tic ulcer is epigastric pain , a gnawing or
burning pain located in the lower chestabove the heart. Often, pain occurs sever-al hours after eating when the stomach isempty, and it is relieved by the ingestionof food, especially in the case of a duode-nal ulcer. Bleeding of the ulcer may alsooccur, causing symptoms of hemateme-
sis(vomiting of blood) or melena (black,
tarry bowel movements). Individuals maybecome anemic from blood loss, and insome instances hemorrhage, a severecomplication of a peptic ulcer, may occur.Another serious complication of a pepticulcer is perforation , the erosion of the ulcer
through the gastric lining. Perforation ofthe gastric lining allows the contents ofthe gastrointestinal tract to escape into theperitoneal cavity , causing irritation of the
peritoneal lining. The resulting inﬂamma-tion of the peritoneum ( peritonitis ) can
be fatal. The complications of hemorrhageand perforation are medical emergencies.
Diagnosis of Peptic Ulcer Disease
Several types of diagnostic procedures
can be used to diagnose peptic ulcer dis-ease and to determine whether individu-als have been infected with H. pylori .
Blood tests may be used to determinewhether individuals have the organism. Abreath test can also determine if individ-uals are infected with H. pylori . In the
breath test individuals are given a drink ofa special liquid and an hour later theirbreath is tested for H. pylori . In some
instances individuals may have an endo-
scopy , in which a tube with a camerainside is inserted through the mouth andinto the stomach to look for evidence ofulcers. During the endoscopy, biopsies ortissue samples of the stomach lining maybe obtained and examined for H. pylori .
Treatment of Peptic Ulcer Disease
The overall goals in the treatment of
peptic ulcer are to relieve discomfort, toheal the ulcer itself, and to eradicate theorganism H. pylori . Once the organism is
eradicated, reinfection rates are low(Suerbaum & Michetti, 2002). A majortreatment is the use of antibiotics andmedications to suppress stomach acidsecretion, such as H
2blockers or PPIs
(Cutler, 2001). Since the H. pylori organ-
ism is difﬁcult to eradicate, partly becauseit is protected by the stomach lining, it isespecially important that the individualtakes medications as prescribed and com-pletes the entire regimen of medication toprevent the organism from developingresistance. Resistance to antibiotics andnoncompliance with the medical treat-ment are the two most common reasonsfor treatment failure (Centers for DiseaseControl and Prevention, 2004).
There is little evidence that dietary
intake causes PUD or that dietary therapyis useful in its treatment. Even so, individ-uals are generally encouraged to avoidfoods that produce discomfort and to usealcohol and coffee only in moderation.Other substances that irritate the stomachlining, such as tobacco, aspirin, and non-steroidal anti-inﬂammatory medications,are generally discontinued. Individualswho must continue using aspirin, such asfor the treatment of arthritis, may beencouraged to use aspirin that is bufferedor that has a special enteric coating.
Although surgical treatment of PUD is
rare today, if the ulcer does not respond
to medical therapy or if there are complica -Conditions of the Gastrointestinal System 283

tions such as uncontrollable bleeding orperforation, surgery is indicated. Severaltypes of surgery may be performed. Oneprocedure, a vagotomy , involves cutting
the vagal nerve to eliminate its ability to
stimulate acid secretion in the stomach.Another procedure, pyloroplasty , involves
widening the opening between the stom-ach and the small intestine to facilitatestomach drainage. A gastroenterostomy is a
surgical procedure in which the bottom ofthe stomach and the small intestine areboth opened. The two openings are thenconnected, creating a passage between thebody of the stomach and the small intes-tine to facilitate stomach drainage. Insome instances, the acid-secreting por-tions of the stomach are removed; thisprocedure is called an antrectomy or subto-
tal gastrectomy .
Surgical resection of the stomach has
several possible consequences. One ofthese is a condition known as dumping syn-
drome , which occurs when food enters the
small intestine too rapidly and is not ade-quately mixed. Individuals with dumpingsyndrome may experience dizziness,sweating, fainting, rapid heartbeat, andnausea 5 to 30 minutes after eating. Thetreatment of dumping syndrome involvesdecreasing the amount of food taken atone time, lying down after meals, and nottaking liquids with meals. Dumping syn-drome usually subsides 6 months to 1 yearafter surgery.
Another possible consequence of surgi-
cal resection of the stomach includes per-
nicious anemia (vitamin B12 deﬁciency).
Pernicious anemia may occur after theremoval of a portion of the stomachbecause of the absence of the intrinsic fac-
tor, a substance necessary for the absorp-
tion of vitamin B12. In this case, lifelongtreatment with injections of supplemen-tal vitamin B12 is necessary. Other nutri-tional problems, such as reduced absorp-tion of calcium or vitamin D, may also beexperienced because of the rapid empty-ing of food into the bowel.
Psychosocial Issues in Peptic Ulcer Disease
In the past, individuals with PUD were
advised to avoid certain foods and oftenwere placed on restricted diets. Evidencenow shows that special diets have nogreater beneﬁt for the treatment of ulcerdisease than regular meals. Individualsmay need to avoid foods or drinks thatappear to be aggravating their symptoms.
Stress had been viewed as a major con-
tributor to PUD prior to the discovery ofthe organism H. pylori . Although treat-
ment now focuses on medical treatmentof the condition rather than lifestyle mod-iﬁcation, stress as a contributing factor tothe development of the peptic ulcers is fre-quently ignored (Levenstein, 1998), but itcannot be dismissed. An increase in bothgastric and duodenal ulcers has beenfound in survivors of a number of natu-ral disasters and crisis situations (Aoyama,Kinoshita, Fujimoto, et al., 1998; Nice,Garland, Hilton, Baggett, & Mitchell,1996). Although H. pylori may still be
present in these individuals, the impact ofthe organism and stress may be additive,promoting growth of the organism(Levenstein, Ackerman, Kiecolt-Glaser, &Dubois, 1999). Moreover, even if stressmay not be a causative factor in PUD, itmay worsen symptoms. Consequently,minimizing stress in general or learningstress reduction techniques may still be animportant part of overall treatment formany individuals with PUD.
Vocational Issues in Peptic Ulcer Disease
In most instances, disability from PUD
alone is nonexistent. In the past, the dis-ability experienced was mostly related to284
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

the side effects of surgery performed forthe disease. Now that medications areused predominantly in the treatment ofPUD, the incidence of resulting disabilityhas decreased signiﬁcantly. Most individ-uals, if undergoing appropriate and time-ly medical treatment, will be able tocontinue in their employment. However,those with additional chronic diseases,those who do not have access to appropri-ate health care, or those who are noncom-pliant with the treatment and recommen-dations prescribed have a greater chanceof experiencing a reoccurrence and thesubsequent disabling effects.
Conditions of the Intestine
Hernia (Rupture)
Protrusion of an organ through tissues
that normally hold it in place is called ahernia . The most common types of
abdominal hernias are the inguinal and
femoral hernias , in which the intestine pro-
trudes through a weakened part of thelower abdominal wall. Men are more like-
ly to develop inguinal hernias , and women
are more likely to develop femoral hernias .
Symptoms are often mild, consisting of
little more than a lump or swelling on theabdomen underneath the skin. Theprotrusion may appear when the individ-uals cough or lift something heavy, butapplication of manual pressure over thearea often pushes it back into place(reduces it ). The protruding structure can
become swollen and constricted by theopening, however, making it impossible tomove the protrusion back into place. Ifthis condition, called incarceration , is not
treated, the blood supply to the herniat-ed portion of the intestine may be cut off,causing tissue death. This condition iscalled a strangulated hernia and is a sur-
gical emergency.Uncomplicated hernias cause little dis-
ability, although it may be necessary toavoid activities such as lifting or pushingheavy objects. Even though there may belittle discomfort or disability, because ofthe danger of hernia strangulation it isimportant for individuals to seek treat-ment, even though they have no pain.This is especially true if they engage instrenuous work.
The surgical procedure used to repair
hernias is called a herniorrhaphy . In this
surgery, the protruding organs are replacedand the weakened area in the abdominalwall is repaired.
Inﬂammatory Bowel Disease
Inﬂammatory bowel disease refers to a
group of disorders that cause inﬂamma-tion and/or ulceration in the lining of thebowel. Inﬂammatory bowel disease ischronic and long term, with an unpre-dictable course. Symptoms usually consistof fever, weight loss, diarrhea, tendernessin the abdomen, and sometimes blood inthe stool. Some people experience longperiods of remission (times when symp-
toms subside) that alternate with periodsof exacerbation (times when symptoms
become worse). The exact cause of inﬂam-matory bowel disease is unknown, but itappears that susceptibility is inherited inat least some inﬂammatory bowel diseases(Podolsky, 2002). Two of the most com-mon conditions classiﬁed as inﬂammato-ry bowel disease are Crohn’s disease and
ulcerative colitis .
Crohn’s Disease (Regional Enteritis)
Crohn’s disease is a lifelong, relapsing
and remitting condition characterized byinﬂammation of segments of the ileum
(small intestine). It results in scarring,thickening, and small inﬂammatory nod-Conditions of the Gastrointestinal System 285

ules of the intestinal wall that can causestenosis (narrowing) of the intestine. It is
characterized by chronic diarrhea , abdomi-
nal pain , fever, loss of appetite , and weight
loss. The condition’s disabling and unpre-
dictable recurrence pattern causes restric-tions in lifestyle and can interfere withwork attendance. Three contributing in-teracting factors—genetic susceptibility,environmental triggers, and altered im-mune response—appear to be indicated inthe development of Crohn’s disease(Shanahan, 2003).
Crohn’s disease may be complicated by
obstruction of the intestine because ofstenosis or by the formation of abscesses.In addition, an abnormal tubelike passage(ﬁstula ) may form between the small in-
testine and other parts of the abdominalcavity. If there are no complications, com-plete recovery may follow a single isolat-ed attack; however, Crohn’s disease isfrequently characterized by lifelong exac-erbation.
Treatment of Crohn’s disease is aimed at
managing symptoms, improving qualityof life, and minimizing complications.Treatment varies according to severity andcomplications. In severe exacerbations ofthe condition, medications, includingantibiotics, steroids, and sulfa preparationsto reduce inﬂammation, are often used inaddition to nutrition support through spe-cial feedings or total parenteral nutrition
(discussed later in the chapter) if individ-uals are unable to tolerate an oral diet forlonger than 5 to 7 days (Ireton-Jones,George, Day, & Zeiter, 2000).
Ulcerative Colitis
In contrast to Crohn’s disease, which
affects segments of the small bowel, ulcer-
ative colitis is an inﬂammatory condition
of the colon (large intestine). It starts at
the rectum or lower end of the colon andspreads upward, at times involving theentire colon. The colon lining becomesedematous (swollen), thickened, and con-
gested with small ulcers that ooze blood.Ulcerative colitis may develop slowly orrapidly. Symptoms usually include crampyabdominal pain and bloody diarrhea. Insevere cases, shock may result.
Ulcerative colitis, as a condition with
periods of remission and exacerbation, canbe a serious, debilitating disease with sys-temic complications that range from mal-nutrition to arthritis and ankylosing
spondylitis . Treatment consists of medica-
tions, such as steroids, to control inﬂam-mation or immunosuppressive drugs toinduce remission. There is no evidencethat dietary intervention has any speciﬁctherapeutic effect. Nearly one-third ofindividuals with ulcerative colitis eventu-ally require surgical intervention, such asa colectomy (removal of the colon),
which is curative. Removal of the colondoes, however, require permanent ileosto-
myor the creation of a pouch or reservoir
for solid waste ( ileoanal pouch ), both of
which are discussed below. Because indi-viduals with ulcerative colitis have anincreased risk of developing cancer of thecolon, regular cancer screening is essential(Ghosh, Shand, & Ferguson, 2000).
Medical Treatment of
Inﬂammatory Bowel Disease
The treatment of inﬂammatory bowel
disease depends on the location, severity,and chronicity of the disease and onwhether it is Crohn’s disease or ulcerativecolitis. Steroid therapy may be used toreduce inﬂammation in acute exacerba-tion of the disease. A sulfonamide knownas sulfasalazine is frequently prescribed forindividuals with inﬂammatory bowel dis-ease to prevent or control infections, sincethe inﬂamed bowel is susceptible to infec-286
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

tion. During acute attacks, individualswith inflammatory bowel disease aredirected to keep physical activity to a min-imum. They may continue working, butthey may need rest at intervals. Some indi-viduals with severe symptoms may bedebilitated to the extent that bedrest isindicated.
Speciﬁc dietary restrictions vary with
different individuals. In general, individ-uals with inﬂammatory bowel diseaseneed to avoid foods that cause ﬂareups.Low-ﬁber diets may be appropriate forthose who have a propensity towardbowel obstruction, whereas a high-ﬁberdiet that stimulates the bowel may beadvisable for others.
Surgical Treatment of
Inﬂammatory Bowel Disease
When medical management fails to re-
solve inﬂammatory bowel disease or ifcomplications occur, surgery may be indi-cated. The type of surgery depends on thelocation and severity of the disease. InCrohn’s disease, surgery is not curative butrather is indicated for complications suchas obstruction or abscess formation.Surgical treatment of Crohn’s disease mayinvolve removing or resecting the diseasedportion of the intestine and surgicallyconnecting the two ends of the intestine.This surgical connection is called ananastomosis .
The most common surgical procedure
for ulcerative colitis is the removal of allor part of the colon, a procedure called acolectomy . If removing the entire colon,
the surgeon passes a portion of the smallintestine ( ileum ) through a surgically cre-
ated opening to the outside of theabdomen and establishes an ileostomy .
The part of the intestine that is exposedto the outer surface of the abdomen iscalled a stoma . In this instance, the ileos-tomy is permanent, and all waste from thesmall intestine passes through this open-ing rather than through the rectum. Theremoval of the entire colon is curative forulcerative colitis.
If only part of the colon is removed, a
surgically created opening between theremaining portion of the colon and theexternal surface of the abdomen is formed.This opening, called a colostomy , is the
opening through which solid wastes(feces ) will be excreted. A colostomy may
be temporary or permanent.
Because the stoma of either an ileosto-
my or a colostomy has no sphincter, indi-viduals have no control over the elim-ination of waste through the stoma.Individuals with an ileostomy have more
liquid and more frequent bowel move-ments than do individuals with a colosto-my, because a great deal of liquid isremoved from waste products in the largeintestine. Thus, although individuals witha colostomy may be able to control thetiming of their bowel movements throughregular daily colostomy irrigation, individ-uals with an ileostomy may have more dif-ficulty regulating elimination by thismeans. Individuals with either a colosto-my or ileostomy may wear ostomy pouch-
es, which are small plastic bags placed over
the stoma to collect fecal waste (see Fig-ure 10–2). The bag is attached by a sepa-rate base plate that is individualized to ﬁtsnugly around the stoma. A skin barrierpaste is usually used to ensure a tight sealand prevent leakage. A variety of productsare also available that may be placed inthe bag to neutralize odor. For some indi-viduals, especially those with colostomywho are able to control elimination withirrigation, small security pads may be allthat are needed over the stoma betweenirrigations.
Some individuals with an ileostomy
have a continent ileostomy , in which anConditions of the Gastrointestinal System 287

intra-abdominal pouch , or Kock pouch , is sur-
gically constructed from a portion of thesmall intestine. Fecal waste collects in thepouch until individuals drain the pouchthrough the stoma with a catheter. Thosewho have such a pouch need not wear anexternal appliance. Individuals insert acatheter three or four times a day, as need-ed, to remove the waste.
Some individuals are able to have a sur-
gical procedure that creates an ileoanal
pouch . In this procedure, after the colon
is removed, the small intestine is suturedto the anal opening. An internal pouch forstoring feces is created from the ileum sothat individuals are able to have bowelmovements through the anus. A tempo-rary ileostomy may be necessary until thearea around the ileoanal pouch heals, but,after 2 to 3 months, the ileostomy may beclosed and anal elimination resumed.
Having a colostomy or ileostomy not
only alters body function, but also altersbody image. Ostomy support groups are use-
ful to help individuals learn to live witha stoma and to overcome the self-con-sciousness that may be associated withhaving an ostomy. Ileoanal pouches havegained increasing popularity, and forsome individuals they have improved thequality of life; however, they have high-er complication rates (Seidel, Newman, &Sharp, 2000).
Psychosocial Issues in
Inﬂammatory Bowel Disease
The chronic nature of inﬂammatory
bowel disease, with its associated remis-sions and exacerbations, may cause signif-icant stress, since individuals are unable topredict when there will be a ﬂareup. Whensurgery is required and a colostomy orileostomy result, individuals’ body imageis altered. Since the stomas of a colosto-my and ileostomy have no sphincter, andthus no control over elimination, individ-uals may be concerned about odors orembarrassing sounds when in social situ-ations, and consequently may avoid them.Because of an alteration of body imageand the fear of an “accident” during sex-288
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM
Figure 10–2 Colostomy with Bag. Copyright © 1999 Rachel Clarke.

ual activity, they may also feel self-con-scious and have concerns about sexualencounters. The reactions of signiﬁcantothers, family, and friends have a signiﬁ-cant impact on individuals’ adjustment totheir condition. An atmosphere of accept-ance and support is important to their self-esteem and ability to live with theircondition.
Vocational Issues in
Inﬂammatory Bowel Disease
When in remission, inﬂammatory bow-
el disease should have little impact onvocational function. When in exacerba-tion, depending on the severity of symp-toms, individuals may have repeatedabsences from work. In some instances, ifthe condition is severe, repeated hospital-izations may be needed.
A colostomy or ileostomy should have
no impact on the ability to work; howev-er, individuals’ own level of comfort ordiscomfort with either may be a majordeterminant of whether they continue towork.
Diverticulitis
A diverticulum is a small balloonlike
sac or pouch that develops in the walls ofthe large intestine. These tiny pouches, ordiverticula , are formed when pressure
causes the inside wall of the large intestineto bulge out through weak spots in theouter wall of the intestine. One of themajor causes of diverticula formation isconstipation. Once diverticula haveformed, there is no way to reverse theprocess; however, a diet that contains ﬁberand bulk to promote regular bowel habitsmay help to control and minimize thecondition. Diverticulosis is the presence
of numerous such outpouchings in theintestinal wall. Individuals with divertic-ulosis are often symptom-free and evenunaware of the condition until it is foundaccidentally through a radiologic exami-nation for another reason. Individualswith no symptoms usually experience lit-tle debilitation and usually require no spe-cial treatment; however, they may beadvised to avoid activities that increaseintra-abdominal pressure, such as bend-ing, lifting, and stooping. They are alsoinstructed to avoid constipation by ingest-ing a high-ﬁber diet and drinking plentyof ﬂuids.
Some individuals with diverticulosis
develop a condition called diverticulitis ,
in which there is obstruction, infection,and inflammation of a diverticulum.Symptoms of diverticulitis include crampypain in the lower abdomen and, occasion-ally, mild fever. Treatment may consist ofproviding the colon with a period of rest.During this time individuals are permittedto have nothing by mouth and may begiven antibiotics. At times diverticula per-forate so that bowel contents spill into theabdomen. The resulting complicationsmay consist of hemorrhage and peritoni-
tis(inﬂammation of the peritoneum).
Individuals who develop complicationsmay require surgery, which usually in-volves a colon resection , in which a portion
of the bowel with the inﬂamed diverticu-la is removed and the healthy portions ofthe bowel are rejoined ( anastomosis ).
Individuals who undergo surgery fordiverticulitis may be able to resume nor-mal activities within 2 to 4 weeks after sur-gery, but they are usually advised tocontinue the therapeutic measures recom-mended for diverticulosis.
Irritable Bowel Syndrome (Spastic Colon)
Irritable bowel syndrome is a chronic or
intermittent condition of the gastrointes-tinal tract in which individuals experienceConditions of the Gastrointestinal System 289

chronic, excessive spasms of the large in-testine, cramping abdominal pain, anddiarrhea, constipation, or both. It isknown as a functional disorder (with no
identifiable organic cause) (Ringel,Sperber, & Drossman, 2001) and, as abiopsychosocial disorder, is thought toresult from the interaction of psychosocialfactors, altered motility of the bowel, andheightened sensory function of the intes-
tine (Camilleri, 2001; Mach, 2004). Psycho-
social factors alone are not the cause ofirritable bowel syndrome; however, theycan worsen the symptoms and inﬂuencethe way the condition is experienced.Although the condition does not causesigniﬁcant functional limitations, it canaffect quality of life and have a large eco-nomic impact because of the increasedhealth care and the indirect cost due toabsenteeism (Camilleri, 2001).
Symptoms of Irritable Bowel Syndrome
In irritable bowel syndrome, the colon
is more sensitive and reacts to mild stim-uli more than the colon of most people,resulting in spasm of the bowel. Indi-viduals may experience cramping abdom-inal pain and a frequent, urgent need todefecate, especially after meals. Symptomsvary in intensity. Although irritable bow-el syndrome can cause signiﬁcant distress,it does not cause permanent harm to theintestines and does not cause ulcerationor bleeding.
Diagnosis of Irritable Bowel Syndrome
Minimal diagnostic tests are advocated
in the initial diagnostic approach to irri-table bowel syndrome. Diagnosis is usual-ly made through a detailed his-tory of abdominal pain or discomfort asso-ciated with chronic altered bowelhabits.Treatment of Irritable Bowel Syndrome
Since there is no known cause of irrita-
ble bowel syndrome, there is also no cure.Treatment is directed toward relieving itssymptoms and eliminating stress. Dietarymodiﬁcation may be indicated. Foods andbeverages that appear to aggravate thesymptoms should be avoided. Individualswho experience constipation may behelped by a diet high in ﬁber.
Medications such as laxatives for con-
stipation or antidiarrheal medication forindividuals who experience diarrhea mayalso be prescribed. Medications may beprescribed to reduce intestinal activity orto relieve tension and anxiety. Medica-tions such as antispasmodics for pain andtricyclic antidepressants (see Chapter 6)may be used as well.
Psychosocial intervention, such as
counseling, psychotherapy, or hypnother-apy, may be necessary in more severe cas-es (Alaradi & Barkin, 2002; Sach & Chang,2002). Individuals may be referred to spe-cial programs where they can learn tech-niques to control emotional tension.Behavioral treatments such as relaxationtherapy, hypnosis, biofeedback, and cog-nitive-behavioral treatments directedtoward reduction of anxiety and promo-tion of healthy behaviors may give indi-viduals a sense of control and help themadapt to the condition.
Individuals with irritable bowel syn-
drome always live with the potential forabnormal function of the colon. If theyare able to identify what triggers the symp-toms, whether it is a certain food or astressful situation, they may be able toprevent the occurrence of symptoms. Be-cause the bowel responds to stress, indi-viduals with this syndrome shouldmaintain a healthy lifestyle that includesadequate nutrition, rest, exercise, andrecreation.290
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

Psychosocial Issues in
Irritable Bowel Syndrome
Irritable bowel syndrome can have a sig-
niﬁcant impact on individuals’ quality oflife, and individuals may have a numberof concerns related to their social activi-ties, home life, and work. The conditioncan have debilitating effects, causing fre-quent absences from work (Camilleri,2001).
Because of their frequent, intense need
to use the bathroom, individuals may beafraid to go to social events or to traveleven short distances. They tend to be veryconcerned about their symptoms and maybe quite sensitive to the physical discom-fort they experience.
Vocational Implications of
Irritable Bowel Syndrome
The prognosis for irritable bowel syn-
drome is often favorable. It is not linkedto other serious diseases, and the mortal-ity rate is zero. Distressing symptoms canbe relieved or eradicated, increasing theindividual’s ability to function.
Conditions of the Accessory Organs ofthe Gastrointestinal System
Pancreatitis
Individuals may develop pancreatitis
(inﬂammation of the pancreas) in associ-ation with gallbladder disease ( biliary
pancreatitis ), certain surgical procedures,
some viral infections (e.g., mumps), trau-ma, chronic alcohol abuse, or drug-induced
pancreatitis due to hypersensitivity. The
most common symptom of pancreatitis issevere abdominal pain, often radiating tothe back and often accompanied by nau-sea and vomiting. Pancreatitis beginswith edema (swelling) in the tissues sur-
rounding the pancreas and may progressto hemorrhage and necrosis (death) of
surrounding tissue. Enzymes produced inthe pancreas for digestion of food maybegin an autodigestive process of attack-ing the pancreas itself.
Pancreatitis may be acute or chronic .
Acute pancreatitis may be mild or may re-
sult in complications such as pancreaticabscess and, in some situations, death. In-dividuals are usually treated in the hospi-tal with intravenous ﬂuids and pain medi-cations during the initial phase of the dis-ease. If there are no complications and ear-ly treatment has been implemented,inﬂammation and symptoms usually sub-side with no long-term effects. Chronic
pancreatitis involves progressive scarring
and calciﬁcation of the pancreas and ismost frequently associated with chronic
alcoholism . The most frequent symptom is
abdominal pain, which can be sudden orchronic. Chronic pancreatitis due to alco-hol abuse can progress even if alcoholingestion is discontinued. With signiﬁcantdamage to the pancreas, individuals withchronic pancreatitis can develop diabetes
mellitus secondary to the pancreatitis (see
Chapter 9). Treatment may involve hos-pitalization for control of pain, althoughdamage to the pancreatic tissue will bepermanent.
Cholecystitis
Although cholecystitis (inﬂammation
of the gallbladder) can occur in individ-uals with severe trauma or other critical ill-ness even if they do not have gallstones ,
an obstruction of the cystic duct by a gall-stone is the most common cause. Thepresence of gallstones is called cholelithi-
asis. Stones may injure the gallbladder
and block passage of the bile that is storedthere.Conditions of the Gastrointestinal System 291

Gallbladder disease can be acute or
chronic . Symptoms of acute cholecystitis in-
clude severe pain in the upper abdomen,often with nausea and vomiting. Whenstones block its passage, bile may back upto the liver, interfering with production ofmore bile. As a result, the level of biliru-
bincirculating in the blood becomes ex-
cessive, causing jaundice (a yellowish
appearance of the skin and whites of theeyes).
Possible complications of cholecystitis
include infection and/or perforation of thegallbladder, damage to the liver, and pan-creatitis. For individuals with cholecystitis ,
treatment may begin with the eliminationfrom the diet of fatty and highly seasonedfoods, which aggravate the condition. Theusual treatment for cholelithiasis is surgi-
cal removal of the gallbladder. The cura-tive treatment is surgical removal of thegallbladder, a procedure called cholecys-
tectomy . Cholecystectomy is often now
performed through a small tube called alaparoscope (in a procedure called laparo-
scopic cholecystectomy ). This procedure
eliminates the need to make large inci-sions through the muscles of the abdom-inal wall. Consequently, cholecystectomyis often now performed in an outpatientsurgical setting, with individuals goinghome 24 to 48 hours after surgery.
Hepatitis
Hepatitis (inﬂammation of the liver)
may be caused by viruses, abuse of alco-hol and drugs, or ingestion of other tox-ic chemicals.
Acute Viral Hepatitis
Several different viruses can cause hep-
atitis. They are transmitted in differentways, but they all produce an inﬂamma-tory process in the liver that interfereswith its effective functioning. Hepatitis iscategorized according to the cause.Hepatitis A. Hepatitis caused by the type
A virus is called hepatitis A . It is highly
contagious and usually transmittedthrough the ingestion of food or waterthat has been contaminated because ofpoor sanitation or poor personal hygiene.When spread through direct person-to-person contact, it may be called infectious
hepatitis . Individuals with hepatitis A usu-
ally experience initial weakness, malaise
(feeling of general fatigue or discomfort),or body aches.
Hepatitis A is diagnosed through blood
tests. There is no speciﬁc treatment, andinfection is usually self-limited. Althoughthe infection can persist for months, itdoes not lead to chronic liver disease(Hoofnagle & Lindsay, 2000). Vaccinationfor Hepatitis A is available for individualsat high risk, such as travelers to areaswhere the rate of infection is high.
There is no speciﬁc medication or treat-
ment that directly affects the viruses thatcause hepatitis A. Usually, the hepatitis re-solves spontaneously after 1 to 2 months.During that interval, the treatment is di-rected toward alleviating the symptomsand maintaining the individual’s state ofhealth so that he or she can withstand theinfection. Rest and adequate nutrition arethe cornerstones of therapy. Individuals
with hepatitis may generally return to work
after jaundice disappears and they feel suf-ﬁciently strong to resume their duties.Hepatitis B. Hepatitis B , sometimes called
serum hepatitis , is caused by the type B virus
and is a major health problem. Initialsymptoms may be ﬂulike. Eventually,jaundice may appear because of hyper-
bilirubinemia (an excess of bilirubin in
the blood). Individuals may also complainof pruritus (itching of the skin).292
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

The hepatitis B virus can live in all body
ﬂuids and is transmitted by blood, semen,and vaginal ﬂuids. Hepatitis B is spreadthrough injection with a contaminatedneedle when injecting drugs or throughtattooing, ear piercing, electrolysis, or acu-puncture. It is also transmitted throughcontact with contaminated body ﬂuidsduring sexual intercourse or throughsharing personal care items such as tooth-brushes.
Pregnant women can pass the hepatitis
B virus to their baby at birth, causing life-long, incurable liver problems for theinfant. Diagnosis of hepatitis B is madethrough a blood test.
Although there is no cure, hepatitis B
can be prevented. Because of the poten-tial serious consequences of hepatitis Binfection, a comprehensive immunizationstrategy to eliminate transmission of thehepatitis B virus has been adopted in theUnited States and includes routine vacci-nation of infants and adolescents and vac-cination of high-risk adults. Althoughmost individuals recover from the symp-toms of hepatitis B in about 6 months,they continue to be carriers of the virus(Zuckerman & Lavancy, 1999). Not all car-riers are infectious; however, they are atrisk for developing chronic hepatitis,which can be associated with cirrhosis, liv-er failure, and liver cancer (Lox, 2002).Hepatitis C. Hepatitis C (formerly called
hepatitis non-A, non-B) is caused by thehepatitis C virus and is contracted prima-rily through the transfusion of contami-nated blood or blood products or frominfected needles. Once an individualbecomes infected, the disease is lifelong.A major complication of hepatitis C ischronic hepatitis. Hepatitis C is the mostcommon cause of chronic liver diseasein the United States, and many individu-als with hepatitis C go on to develop end-stage liver disease (Gaster & Larson, 2000).They are also at risk for developing can-cer of the liver.
Hepatitis C may be asymptomatic
(without symptoms) or may begin withﬂulike symptoms, such as anorexia (lack
of appetite), distaste for cigarettes, chillsand fever, nausea and vomiting, orheadache.
Treatment of hepatitis C consists of a
course of antiviral therapy that includesa combination of medications such asinterferon and ribavirin , usually three times
a week for up to 6 months to a year.Treatment is not curative, but it canhave a beneﬁcial effect on survival anddevelopment of chronic liver disease(Gaster & Larson, 2000). However, treat-ment is expensive and may cause sideeffects so severe that individuals may beprevented from working during treatmentperiods (Yates & Gleason, 1998). Indi-viduals who develop cirrhosis because ofhepatitis C may be candidates for livertransplantation. They are usually requiredto be free of alcohol or illicit drugs for 6months prior to being placed on a trans-plant list.
There is currently no immunization
available to prevent hepatitis C infections.Consequently, the best prevention isavoidance of high-risk behaviors.
Chronic Hepatitis
Chronic hepatitis comprises several dis-
eases that are grouped together because ofsimilar symptoms and because they can alllead to cirrhosis and end-stage liver disease(Lindsay & Hoofnagle, 2000). When liverinﬂammation continues longer than 3 to6 months, individuals are said to havechronic hepatitis. This condition may leadto progressive ﬁbrous changes in the liv-er or cirrhosis. The prognosis is variable,depending on the cause.Conditions of the Gastrointestinal System 293

Toxic Hepatitis
Because the liver metabolizes and detox-
iﬁes many drugs as well as other toxic orpoisonous substances, overexposure to orthe presence of hepatotoxins (substances
that are harmful to the liver) can cause liv-er damage and chronic liver disease. Theprognosis depends on the extent of theliver damage and the prevention of asso-ciated complications.
Cirrhosis
Cirrhosis is a progressive disease of the
liver in which liver function is disorgan-ized and altered because of damage thatproduces ﬁbrous changes in the structureof the liver. Such changes can occur for awide variety of reasons:
• Infection of the liver, as in viral hep-
atitis
• Obstruction of bile ﬂow, as in gall-
bladder disease
• Overexposure to hepatotoxins, such
as toxic chemicals
• Alcohol abuse
Some individuals with cirrhosis have no
symptoms. As the disease progresses, symp –
toms may consist of anorexia (lack of ap-
petite), nausea, and vomiting. Individualswith advanced cases of cirrhosis may gainweight because of their retention of ﬂuid
and the presence of ﬂuid in the abdominal
cavity, a condition called ascites . Finally
there may be vomiting of blood ( hemateme-
sis) and a general bleeding tendency.
Complications of cirrhosis include hem-
orrhage, coma, and eventually death.Treatment of cirrhosis is based on its causeand any complications that may be pres-ent. Treatment is discussed in furtherdetail in Chapter 7. Prognosis depends onthe severity of the condition and the asso-ciated complications.GENERAL DIAGNOSTIC PROCEDURESFOR CONDITIONS OF THEGASTROINTESTINAL SYSTEMBarium Swallow(Upper Gastrointestinal Series)
A radiologic (X-ray) study of the upper
gastrointestinal tract, or barium swallow ,
makes it possible to identify abnormalitiesof the esophagus, stomach, and the upperportion of the small intestine. Immedi-ately before the procedure, individualsdrink a white, chalky liquid called barium
so that the radiologist can visualize thestructures of the upper gastrointestinaltract on X-ray ﬁlm. The test aids in themedical diagnosis of structural abnormal-ities, ulcers, and tumors of the upper gas-trointestinal tract. The test is usuallyperformed by a radiologist (a physician
specializing in diagnostic or therapeuticuse of X-ray ﬁlm).
Barium Enema(Lower Gastrointestinal Series)
Like the barium swallow, a barium ene-
mais a radiologic (X-ray) study. In the
case of the barium enema, however, thelarge intestine is ﬁlled by an enema withbarium. This procedure enables the radi-ologist to visualize the large intestine onX-ray ﬁlm for the diagnosis of structuralabnormalities, diverticula, and tumors.
Esophageal Manoscopy (Manometry)
Although done infrequently, esophageal
manoscopy is a diagnostic procedure to eval-
uate the function of the sphincter be tween
the esophagus and the stomach. Duringthe procedure, individuals swallow a cath-eter that has a small instrument or trans-ducer attached to it. When the transducer
reaches the lower end of the esophagus, the
pressure around the sphincter is measured.294 CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

Endoscopy (Gastroscopy)
When there are indications of abnor-
malities in the esophagus, stomach, orsmall intestine, the walls of these organsmay be visualized directly through a spe-cially lighted, ﬂexible tube called a gastro-
scope or endoscope . This procedure is called
a gastroscopy and is usually performed by
a gastroenterologist (a physician who
specializes in the diagnosis and treatmentof gastrointestinal conditions). During theprocedure, the individual’s throat issprayed with an anesthetic medication tonumb the gagging reﬂex. The gastroscopeis then inserted through the mouth, intothe esophagus, into the stomach, and, attimes, into the small intestine. Throughthe tube, the physician can visualize ulcer-ations or other abnormalities, as well asremove stomach contents for analysis, ifneeded.
Proctoscopy, Colonoscopy, andSigmoidoscopy
These procedures are performed by a
physician, often a gastroenterologist , a fam-
ily physician , or a general internist . The pro-
cedures are performed to identify prob-lems of the rectum and large intestine,including tumors, obstruction, and bleed-ing. The procedure used to detect abnor-malities of the rectum is called aproctoscopy . It involves the direct visu-
alization of the anus and rectum througha special instrument called a proctoscope
inserted into the rectum. Colonoscopy is
a procedure that enables physicians toexamine the lining of the colon (large
bowel) for abnormalities by inserting aﬂexible tube into the anus and advancingit slowly into the rectum and colon.
Similarly, sigmoidoscopy permits direct
visualization of the sigmoid colonthrough a special instrument called a sig-moidoscope inserted through the anus and
rectum up into the colon.
Cholecystography
If gallbladder disease is suspected, a
cholecystogram may be performed to detect
abnormalities, inﬂammation, or the pres-ence of stones. Before the procedure, theindividual swallows special pills or liquidor receives an intravenous injection of aspecial substance that allows the gallblad-der to be visualized on X-ray ﬁlm. A radi-
ologist (a physician who specializes in the
diagnostic or therapeutic use of X-ray)usually performs the procedure.
Cholangiography
A study called a cholangiogram is used to
visualize the bile ducts on X-ray ﬁlm. Dyeis injected into a vein or into a drain calleda T tube that has been inserted into the
bile duct (usually after gallbladder surgery).
A radiologist performs the procedure toidentify any obstruction of the bile ducts.
Ultrasonography(Abdominal Sonography)
In ultrasonography , sound waves are
passed into the body and converted to avisual image or photograph of a bodystructure. Abdominal sonograms focus on
organs contained within the abdomenand can be used to identify disorders ofthe pancreas, liver, gallbladder, or any oth-er abdominal organ.
Computed Tomography(CT Scan, CAT Scan)
A special kind of X-ray procedure, com-
puted tomography , produces three-dimen-
sional pictures of a cross-section of a partof the body. The radiologist studies theGeneral Diagnostic Procedures for Conditions of the Gastrointestinal System 295

image produced to identify problems andto determine if further tests are needed.This procedure can be used to diagnosepancreatic disease, tumors, or abscesses inthe abdominal area.
Radionuclide Imaging
For radionuclide imaging, individuals
are given a small amount of a radioactivechemical ( radionuclide ) that gives off
energy in the form of radiation. Differentradionuclides concentrate in differentorgans. Special types of equipment, suchas counters, scanners, and gamma cam-eras, detect the radiation, producing animage on ﬁlm or on a special type ofscreen. A physician who has specialized innuclear medicine then examines andevaluates the image. In the gastrointesti-nal system, radionuclide imaging is help-ful in detecting tumors, abscesses, orcirrhosis of the liver and in diagnosinggallbladder disease.
Biopsy
The removal of a specimen of tissue
from a speciﬁed site for examination iscalled a biopsy . Common sites of biopsy
in the gastrointestinal tract are the esoph-agus, stomach, rectum and colon, and liv-er. Biopsies are performed by a physicianand can be performed on an outpatientbasis under local anesthesia.
Abdominal Paracentesis
A procedure to remove ﬂuid from the
abdominal cavity, abdominal paracentesis ,
involves puncturing the abdominal cavi-ty with a hollow needle through whichaccumulated ﬂuid can be withdrawn. Aphysician performs the procedure. It maybe done for diagnostic purposes to deter-mine the nature of the ﬂuid present, or fortherapeutic purposes to remove accumu-lated ﬂuid in the abdominal cavity thatmay be causing respiratory difficulty,pain, or other problems.
Laparoscopy
Laparoscopy may be conducted for
either diagnosis or surgical procedures.The abdominal cavity may be directlyexamined through a hollow tube called alaparoscope or peritoneoscope . The instru-
ment is inserted into the abdominal cav-ity through a small incision. Individualswho have undergone laparoscopy mayremain in the hospital overnight forobservation after the procedure.
GENERAL TREATMENT FORCONDITIONS OF THEGASTROINTESTINAL SYSTEMMedications
Various medications are used in treating
conditions of the gastrointestinal system;they may act on either muscular or glan-dular tissues and may be one or more ofthe following:
•Antacids and acid inhibitors to counter-
act excess acidity.
•Antiemetics to prevent nausea and
vomiting. A side effect of these med-ications may be drowsiness.
•Digestants to replace missing enzyme
secretions when there is an enzymedeﬁciency in the gastrointestinal tract.
•Antidiarrheals to prevent diarrhea.
•Laxatives and cathartics to relieve con-
stipation. Generally, laxatives havemild actions, and cathartics havestronger actions.
•Anticholinergics to inhibit the action of
the involuntary nervous system. Ingastrointestinal conditions, thesemedications may be given to reduce296
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

activity of the intestine or to decreasesecretions.
•Histamine H
2receptor antagonists (e.g.,
cimetidine) to inhibit cells in thestomach lining from producing acid.
•Proton pump inhibitors .
•Antimicrobials (e.g., sulfonamides) to
inhibit the growth of microorganisms.
Hyperalimentation(Total Parenteral Nutrition)
When individuals are unable to take
nourishment by mouth, or when nutri-tional status is compromised, it is possi-ble to bypass the gastrointestinal tract inorder to provide nourishment. Hyperali-
mentation is the infusion of a special
nutritional solution into a vein. Becauseof the nature of the solution, infusion usu-ally takes place in a large vessel such as thesubclavian vein , located in the upper body.
Hyperalimentation may be used in thetreatment of any condition that compro-mises the individual’s nutritional status. Itmay also be used when there is a need torest the gastrointestinal tract, as in inﬂam-matory bowel disease, or when there is anobstruction or malabsorption problem inthe bowel.
Stress Management
Although stress management may be
helpful in the treatment of a variety ofchronic illnesses and disabilities, it may beespecially useful in the treatment of con-ditions of the gastrointestinal tract. Stress
itself may not be a direct cause of many con –
ditions of the gastrointestinal tract, but itmay exacerbate or prolong an acute epi-
sode in some patients with existing disease.
The body uses a number of defensive
mechanisms in the face of threat or dan-ger. When stress is encountered, a varietyof physiologic reactions take place in thebody, including in the gastrointestinaltract. The digestive system responds differ-ently to different kinds of emotional stim-uli. For example, it may become more orless active, and it may secrete more or lessgastric juice. The intensity of the physio-logic reaction depends on the individualand on the situation. Stress managementhelps individuals to control their reactionsto stress. Programs in stress managementmay vary from exercise to techniques thatalter the body’s response to stress, such asbiofeedback.
PSYCHOSOCIAL ISSUES INCONDITIONS OF THEGASTROINTESTINAL SYSTEMPsychological Issues
Although not a causative factor in all
instances, there appears to be at least someassociation between psychological factorsand the gastrointestinal system. Psycho-logical factors that signiﬁcantly affect gas-trointestinal conditions may also includenutritional or lifestyle factors, such as alco-hol and tobacco ingestion. In some in-stances, gastrointestinal conditions mayalso be directly related to treatment foranother condition, such as intake ofaspirin for rheumatoid arthritis, whichresults in gastritis.
Conditions that affect the physical
processes of eating and elimination have
many psychological implications. Through –
out life, eating is often associated withpleasure and social interaction. Treatmentof gastrointestinal conditions frequentlyrequires avoiding substances that irritatethe gastrointestinal tract or cause the ex-cessive secretion of gastric juices. Whencertain types of food and beverages arerestricted or when special diets arerequired, individuals may have difﬁcultyin giving up something that they enjoyed.Psychosocial Issues in Conditions of the Gastrointestinal System 297

Elimination is associated with privacy
and personal cleanliness. The modiﬁca-tion of elimination habits is learned inchildhood as part of the socializationprocess. Individuals with problems ofelimination may fear embarrassment andsocial ridicule as a result of their condi-tion. Those with an ileostomy or a colosto-
mymay fear the loss of physical and
sexual attractiveness because of odor orembarrassing sounds. Individuals whohave inﬂammatory bowel disease accom-panied by diarrhea may fear fecal incon-tinence and concomitant humiliation.
There are other reasons for psychologi-
cal reactions as well. Individuals with hep-
atitis may fear transmitting the disease,
and individuals with ulcerative colitis may
be preoccupied with their increased risk ofcancer. Depression is common in individ-uals with irritable bowel syndrome . The
identiﬁcation and resolution of thesereactions may be crucial to rehabilitation.
Emotions affect the involuntary nerv-
ous system, which, in turn, affects the gas-trointestinal tract. Thus, psychologicalfactors may aggravate conditions of thegastrointestinal tract. For example, anxi-ety may contribute to ﬂareups of condi-tions such as inﬂammatory bowel disease.Although rest and relaxation are of primeimportance in the treatment of many gas-trointestinal conditions, individuals mayﬁnd it difﬁcult to modify their schedules,to adjust to new life patterns, or to alter
stressful situations at home or work. Often,
directions to “rest and relax” are uselessunless individuals are assisted with meth-ods and techniques to do so.
Although many conditions of the gas-
trointestinal tract do not affect bodyimage, individuals with an ileostomy or acolostomy may encounter problems withbody image and self-concept. They mayperceive themselves as different fromothers. They may visualize themselves asunattractive and may believe that theymust wear shapeless, dowdy clothes tohide the ileostomy or colostomy bag. It isoften helpful if they are able to meet oth-er persons who have a similar conditionand are leading a normal, active life.
Many conditions of the gastrointestinal
tract require permanent alterations inlifestyle and constant control over emo-tional tension. At times, individuals withsuch conditions exhibit illness behaviorand disability that are out of proportionto the objective ﬁndings. These individu-als should be helped to make the recom-mended alterations and encouraged tomaintain as normal a lifestyle as possible.
Lifestyle Issues
Individuals with any chronic disease
must have a healthy lifestyle, includingadequate nutrition, rest, and exercise, inorder to reach their maximal functionalcapacity. This is especially true of gastroin-testinal conditions, because stress, fatigue,and emotions appear to have some directeffect on the digestive system. Individualswho are accustomed to performing inhigh-pressure, high-stress situations mayneed to learn ways either to decrease thestressful aspects of their daily life or workor to cope better with the stress that ispresent.
Many gastrointestinal disorders carry
notable nutritional implications, and dietis the cornerstone of therapy. Alterationsand restrictions of diet are often based onavoiding foods that appear to cause dis-tress. Depending on the meaning thesefoods have for individuals, it may bedifﬁcult for them to abide by such restric-tions. In most instances, eating well-bal-anced, regular meals is part of the thera-peutic regimen. For individuals whosework or daily schedule is somewhat errat-ic, even this simple task may be difﬁcult.298
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

Alcohol intake should not necessarily be
totally restricted, but it may be limited.Tobacco use is restricted for many individ-uals with gastrointestinal conditions.Depending on the former habits of theseindividuals, both of these recommenda-tions may be difﬁcult to follow.
In most instances, conditions of the gas-
trointestinal tract do not directly affect
sexual function. However, individuals may
be reluctant to engage in sexual activityif their gastrointestinal condition affectstheir body image or if they fear fecalincontinence. Those with an ileostomy ora colostomy may have fears of defecationduring sexual contact or may be self-con-scious about the stoma itself. In some cas-es, men may become impotent as a resultof nerve damage caused by the surgicalprocedure. Open discussion about suchissues is important to uncover such fearsand concerns, as well as to provide infor-mation that can help individuals and theirpartners deal with such issues.
Social Issues
Food is a part of celebration and social-
ization as well as nourishment. When spe-ciﬁc conditions of the gastrointestinal sys-tem prohibit individuals from eating orfrom having foods that have typicallybeen part of their social milieu, their socialinteractions may be affected.
Social situations that are stressful for the
individual with gastrointestinal diseasemay cause a ﬂareup of symptoms. Toavoid such stress, some individuals withgastrointestinal symptoms may withdrawfrom many social activities.
Individuals with ileostomy or colosto-
my bags may fear fecal incontinence,odors, or spillage and may withdrawfrom social interactions to avoid potentialembarrassment. For individuals with anileostomy or a colostomy, problems mayarise if family members are repelled by thecondition or ﬁnd it impossible to ﬁt thecare of a stoma into the household rou-tine. If these individuals have not accept-ed responsibility for their own personalcare, they may become overly demandingor sloppy in the care of the stoma, antag-onizing family members. The acceptanceof the individual by family members andfriends often determines to a great degreethe acceptance of the condition by theindividual.
VOCATIONAL ISSUES IN CONDITIONSOF THE GASTROINTESTINAL SYSTEM
In most instances, special work restric-
tions are not necessary for individualswith gastrointestinal disorders. Thosewith diverticular disease or hernia mayneed to avoid activities that increase intra-abdominal pressure, such as lifting orbending.
Modiﬁcations in the work environ-
ment or work schedule may occasionallybe necessary for those with other gastroin-testinal conditions. For example, erratic orrotating schedules may make it difﬁcultfor individuals with a peptic ulcer to eatregular, well-balanced meals, aggravatingthe condition. Work situations that causeundue stress may contribute to a ﬂareupof the symptoms of some gastrointestinalconditions. If schedules or workload can-not be changed, individuals may need tolearn different ways of expressing tensionand coping with stress.
Special accommodations, such as read-
ily available bathrooms with privacy inthe workplace, may be necessary for indi-viduals who experience diarrhea as asymptom of a gastrointestinal disorder orfor those who have an ileostomy or acolostomy that may need attention dur-ing the day.Vocational Issues in Conditions of the Gastrointestinal System 299

CASE STUDIES Case I
Mr. A. is a 36-year-old musician who
plays with a band at a local club six nightsa week. During the day he gives music les-sons to a number of private music stu-dents. He is unmarried and has a masterof arts degree in music. He was diagnosedwith Crohn’s disease 10 years ago, andsince then his symptoms have becomeconsiderably worse. He has had to cancela number of private music lessons eachweek, and he has been unable to performwith the band several nights a week. Thispattern has continued for several months,and several music students have soughtout other instructors. The band membershave informed him that if the pattern ofabsences continues, they will need to drophim from the band. He is interested inlooking for other career options in theevent this should occur.
Questions
1. What type of medical information
would help Mr. A. evaluate his reha-bilitation potential?2. What factors would you consider in
helping Mr. A. to develop a rehabili-tation plan or to identify his options?
3. Are there any types of referrals that
Mr. A. might ﬁnd helpful?
Case II
Mr. Z., a 17-year-old grocery clerk, was
shot in the abdomen during an attempt-ed robbery. As a result of his injury, Mr. Z.had a large portion of his colon removedand now has a permanent colostomy. It isnow 6 months since his injury, and he isready to graduate from high school. Hehas asked you as his high school vocation-al counselor to help him determine suit-able career options.
Questions
1. Are there speciﬁc limitations associ-
ated with Mr. Z. having a colostomythat you would consider when focus-ing on career goals?
2. In addition to determining Mr. Z.’s
vocational interests and abilities, arethere other issues you may want toexplore?300
CHAPTER 10 C ONDITIONS OF THE GASTROINTESTINAL SYSTEM

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NORMAL STRUCTURE ANDFUNCTION OF THECARDIOVASCULAR SYSTEM
The cardiovascular system consists of the
heart and a network of blood vessels that
carry blood throughout the body. Bloodvessels in the circulatory system are com-posed of:
•arteries , which carry oxygenated blood
away from the heart
•veins , which carry unoxygenated blood
tothe heart
• small branching blood vessels ( arteri-
olesand veniuoles )
• tiny vessels called capillaries , which
provide a link between arterioles andvenules
The heart, acting as a pump, forces
blood through two circuits. One circuitcarries blood toand from the lungs ( pul-
monary circulation ). The second circuit car-
ries blood throughout the body (systemic
circulation ).
The heart is a strong and powerful mus-
cle located somewhat to the left of thecenter of the chest. The heart muscle itselfis called the myocardium . It is enclosed
in an outer covering (the pericardium ) con-
sisting of two layers. The space betweenthe two layers of the pericardium containsa small amount of ﬂuid to lessen frictionbetween the two surfaces as the heartbeats. The inner surface of the heart iscalled the endocardium . The myocardiumis a special type of muscle that has theability to work continuously with onlybrief periods of rest between contractions.This resting period, when the heart isrelaxed and the chambers are ﬁlling, iscalled diastole . The pumping action, or
contraction, of the heart muscle is calledsystole . Diastole and systole produce differ-
ent pressure gradients. The ratio of thesetwo pressures is called blood pressure . The
amount of pressure produced is depend-ent on the force with which the heartpumps and the degree to which the bloodvessels resist blood ﬂow. Blood pressure isexpressed numerically as a fraction, withthe systolic reading being the numerator
and the diastolic reading being the denom –
inator. For instance, in a blood pressurereading of 120/80, the systolic pressure is120 and the diastolic pressure is 80.
Like all muscles of the body, the
myocardium requires oxygen and nutri-ents to survive. A separate network ofblood vessels called the coronary vessels
supplies the heart muscle with blood. Thecoronary vessels consist of coronary
arteries, which carry oxygen and nutri-
ents tothe heart muscle, and coronary
veins , which carry blood used by the heart
muscle and containing waste away from
the heart muscle. Without the blood ﬂowsupplied directly to the myocardium, theheart is unable to carry out its function ofpumping blood to the rest of the body.
The heart contains four chambers. The
two upper chambers are the right and leftCardiovascular ConditionsCHAPTER 11
303

atria , and the two lower chambers are the
right and left ventricles . Four valves help
blood move from chamber to chamber inone direction without backﬂow. Betweenthe right atrium and the right ventricle isthe tricuspid valve . Between the left atrium
and left ventricle is the mitral (biscuspid)
valve. The right atrium receives deoxygenat-
ed blood from the systemic circulation
through a large vein called the vena cava .
The deoxygenated blood is pumped from
the right atrium to the right ventricle through
the tricuspid valve . Blood is then pumped
from the right ventricle through the pul-
monary (semilunar ) valve to the pulmonary
artery, where it is carried to the lungs ( pul-
monary circulation ). The pulmonary artery
is the only artery in the body that carries
deoxygenated blood. In the lungs, wastein the form of carbon dioxide is releasedfrom the blood and excreted from thelungs. Oxygen is taken into the bloodfrom the lungs and the oxygenated bloodis then pumped back to the heart througha vessel called the pulmonary vein (the only
vein in the body that carries oxygenatedblood). Oxygenated blood enters the leftatrium of the heart, moves through themitral valve to the left ventricle, and then
is pumped out of the heart through theaortic (semilunar ) valve to a large blood ves-
sel called the aorta , which carries it to the
systemic circulation . (See Figure 11–1.)
Blood carries oxygen and nutrients to all
parts of the body through blood vessels inthe peripheral circulation (outside the
heart). Arteries diminish in size as theyprogress farther away from the heart,eventually leading to tiny vessels calledcapillaries. Capillary walls are very thin,allowing for the exchange of oxygen andnutrients from the blood with wasteproducts from body tissues. Blood con-taining waste products is then carried backto the heart through small blood vesselsthat increase in size until they reach thevena cava , which returns to the heart, and
the process begins all over again. 304
CHAPTER 11 C ARDIOVASCULAR CONDITIONS
To right
lungTo left
lung
From general
circulationTo general
circulation
From general
circulationVena CavaAortaPulmonary ArteryPulmonary VeinPulmonary Semilunar ValveAortic Semilunar ValveMitral ValveEndocardiumMyocardiumPericardiumRight
AtriumLeft
Atrium
Right
VentricleLeft
VentricleSA Node
AV Node
Tricuspid Valve
Vena Cava
Figure 11–1 The Heart. SA, sinoatrial; AV, atrioventricular.

Cardiovascular Conditions 305
A special nerve conduction system in
the heart maintains its regular, rhythmicbeating. Special cells called the sinoatrial
(SA) node (also called the pacemaker of the
heart), located within the right atrium,initiate the contractions. Impulses fromthe SA node spread over both atria, caus-ing them to contract simultaneously. Theimpulse then reaches special cells in thelower right atrium, the atrioventricular (AV)
node. From the AV node, impulses are
transferred to special muscle ﬁbers ( bun-
dle of His ) located on the right and left side
of the septum separating the two ventri-cles. The bundles branch out to smallbranches (Purkinje ﬁbers), which spreadover both ventricles, causing them to con-tract. At this point, the cycle starts over.Conduction of these nerve impulsesthroughout the heart occurs involuntari-ly. Through communication from the cen-tral nervous system, the heart adjusts tothe changing needs of the body, speedingor slowing the heart rate as needed.
CARDIOVASCULAR CONDITIONSArteriosclerosis (Atherosclerosis)
The general term arteriosclerosis refers
to conditions in which walls of the arter-ies become thickened or less elastic,obstructing circulation and diminishingblood ﬂow to various parts of the body. Itis caused by buildup of plaque along theinterior vessel wall, which causes stenosis
(narrowing) of the vessel, impeding bloodﬂow. Symptoms of arteriosclerosis devel-op slowly and are generally nonexistentuntil blood ﬂow is diminished to theextent that oxygen supply to a body partis hampered. At this point individuals mayexperience pain, fatigue, or altered func-tion in the body part affected.
Plaque can also contribute to thrombus
(blood clot) formation within the nar-rowed vessel, blocking blood ﬂow evenmore. Tissue death can occur if all bloodﬂow to a body part is obliterated. A throm-bus can be dangerous even when bloodﬂow isn’t totally blocked because it canbecome an embolus if it becomes dis-
lodged from the vessel wall and beginstraveling in the bloodstream. (The termembolus can also refer to other substances
traveling in the blood, such as an air bub-ble, fat globule, or other foreign matter.)The embolus can lodge in a blood vesseltoo small to allow its passage, thus occlud-ing blood ﬂow. The effects of an embo-
lism depend on the body part affected. For
example, an embolism of the brain resultsin stroke, whereas an embolism in a coro-nary artery causes myocardial infarction
(heart attack). An embolus lodging in thelungs would be called a pulmonary embo-
lus. In all instances, embolism can result
in severe tissue damage and can be fatal.
Symptoms experienced by individuals
with arteriosclerosis vary depending onthe extent of stenosis of the vessels andthe location of the impeded blood ﬂow.For example, cognitive changes can resultwhen blood ﬂow to the brain is decreased,such as in carotid artery stenosis (narrow-
ing of the vessels carrying blood directlyto the brain). A decreased blood ﬂow tothe kidneys may contribute to kidneydamage, causing chronic renal failure. De-creased blood ﬂow to the heart may causeangina pectoris (chest pain) or, if blood
ﬂow is severely restricted, heart attack.
Treatment of arteriosclerosis is directed
at the complications the condition caus-es, such as angina pectoris, arrhythmias,myocardial infarction, and stroke (see
Chapter 2), kidney failure (see Chapter 13),
and peripheral vascular disease (describedlater in this chapter). Since nicotine fur-ther constricts vessels already narrowed inarteriosclerosis, individuals with arterio-sclerotic disease should avoid tobacco use.

Aneurysm
An aneurysm is a dilation or balloon-
ing out of a weakened arterial wall.
Although often associated with arterio-
sclerosis and hypertension, an aneurysmmay also result from a congenital abnor-mality. The weakened wall of the artery,if under increased pressure (e.g., becauseof hypertension), may burst and lead tohemorrhage. Common sites of aneurysmsare in the brain and the aorta (the major
trunk of the arterial system of the body).A dissecting aneurysm is a tear in the inner
wall of the vessel so that blood leaksbetween the layers of the wall of the ves-sel, moving longitudinally to separate thelayers along the length of the vessel ratherthan rupturing into an open body space.
Symptoms experienced as a result of an
aneurysm vary according to its location.In some instances, there are no symptomsuntil the aneurysm becomes large enoughto create pressure, causing pain at the site.In other instances, there are no symptomsuntil the aneurysm ruptures, which couldresult in sudden death.
Aneurysms may be treated surgically if
they are diagnosed early or if surgery is notcontraindicated because of associatedmedical problems. Surgical procedures tocorrect aneurysms involve removing theweakened area of the artery and then con-necting the two remaining ends. A graftto join the two remaining ends is used ifa large portion of the vessel has beenremoved. Controlling hypertension, ifpresent, is an important aspect of contin-uing treatment after surgery.
Endocarditis
Endocarditis (inflammation of the
membrane that covers the heart valvesand chambers of the heart) is most oftencaused by an infection; however, it canalso be the result of an immunologicalreaction. It is characterized by deposits of“vegetation” on the inner lining of theheart (the endocardium) and most fre-quently on the valves. Damage to thevalves can result.
Endocarditis may be associated with sys-
temic infectious diseases or intravenousdrug abuse, or it may be a complicationof an invasive medical procedure. Some-times even minor trauma can precipitatethe development of endocarditis. Mostpeople have isolated incidents in whichbacteria enter the bloodstream ( bac-
teremia ). Usually the body’s own defens-
es overcome the organism with nountoward results. At times, however,because of the strength of the organism orthe reduced effectiveness of the body’sdefenses, the organism settles on the innerlining of the heart, causing endocarditis.
Individuals with previous cardiac sur-
gery, congenital heart disease, or condi-tions in which the heart valves have beendamaged are more susceptible to develop-ment of endocarditis. Individuals withvalve replacement (discussed later in thechapter), and especially those with pros-thetic valves, are also at higher risk forendocarditis (Levinson, 2000).
Symptoms of endocarditis may be insid-
ious at ﬁrst, mimicking the ﬂu. As the dis-ease progresses, symptoms such as highfever, weight loss, and extreme fatiguebecome more pronounced. The conditionis diagnosed based on history and symp-toms, blood culture, and echocardiogram(discussed later in the chapter). Treatment
consists of administering appropriate anti –
biotics to eradicate the infection before
serious complications develop. When severe
valvular dysfunction results, surgical re-placement of the valve may be necessary.
Complications of endocarditis include
embolism (obstruction of a blood vessel
by a foreign substance), in which some of306
CHAPTER 11 C ARDIOVASCULAR CONDITIONS

the vegetation from the affected valvebreaks away and occludes a blood vesselin another part of the body. Any organ orpart of the body can be affected. De-pending on the part of the body affectedand the extent of the resulting damage,embolism can result in death.
Pericarditis
Inﬂammation of the outer layer of the
heart ( pericardium ) is known as percardi-
tis. Most commonly, pericarditis is caused
by a virus (Manning, 2000). When in-ﬂamed, the pericardial layers can adhereto each other, creating friction as their sur-faces rub together during cardiac contrac-tion. The most common symptom is chestpain that is aggravated by moving andbreathing because of the rubbing togeth-er of the two inﬂamed surfaces. A low-grade fever may also be present.
Diagnosis is often based on symptoms,
physical examination, and at times an elec-
trocardiogram (ECG), which is discussed lat-
er in the chapter. Treatment of pericarditisis directed toward alleviating the paincaused by inﬂammation of the pericardi-um. Medications such as nonsteroidal anti-
inﬂammatory agents are commonly used.
Severe inﬂammation of the pericardium
can result in accumulation of excessiveﬂuid within the pericardial sac, a condi-tion known as pericardial effusion . Excess
ﬂuid in the sac surrounding the heart mayconstrict the myocardium, causing cardiacdysfunction. If constriction of the heart issevere because of increasing amounts ofﬂuid, a condition called cardiac tampon-
ade(severe constriction of the heart that
prevents it from ﬁlling and emptyingproperly) may occur. A procedure calledpericardiocentesis may be performed
during which the physician inserts aneedle into the pericardial sac to drain theﬂuid. After severe inﬂammation, the peri-cardium may become scarred, further con-stricting cardiac function ( constrictive peri-
carditis ). This rare condition may need to
be treated surgically so that a portion ofthe pericardium is removed.
Rheumatic Heart Disease
Rheumatic heart disease is a type of heart
condition brought about by a conditioncalled rheumatic fever . Rheumatic fever is
an inﬂammatory condition that occurs asa type of allergic reaction elicited by anorganism called streptococcus . Since not
everyone develops rheumatic fever after astreptococcal infection, the reason for theallergic type of reaction in some individ-uals is unknown.
Recovery from rheumatic fever can be
complete with no residual effects; however,
some individuals experience permanentcardiac damage as a result. Valves of theheart are most frequently affected, result-ing in stenosis (narrowing), insufﬁciency,
or regurgitation, as described later in thechapter under valvular heart disease.
Hypertension
Individuals with hypertension have a sus-
tained elevation of pressure in the arter-ies. Both systolic and diastolic pressure may
be elevated. Blood pressure normally ﬂuc-
tuates with physical activity, becoming low-
er at rest and higher with changes in pos-
ture, exercise, or emotion. Individuals whose
blood pressure remains high even at rest put
increased strain on body organs. This pro-
longed elevation of pressure can eventually
damage other organs, such as the heart,kidneys, or brain. Although this hyper-
tension (high blood pressure) in and of
itself is not disabling, it is a major healthproblem because of its associated high riskof myocardial infarction (heart attack),stroke, renal failure, and congestive heartCardiovascular Conditions 307

failure, all of which cause signiﬁcant dis-ability (August, 2003; Oparil, 2000).
The most common type of hypertension
is primary , or essential , hypertension , which
has a gradual onset and few, if any, symp-toms. The exact cause of primary hyper-tension is unknown. At other times hyper-tension is a symptom of another medicalcondition, such as kidney disease, endo-crine disorders, neurological disorders, ordrug use or abuse. This type of hyperten-sion is called secondary hypertension . A less
common, but more severe, type of hyper-tension is malignant hypertension , which
has an abrupt onset, more severe symp-toms, and more associated complications.
Often, primary hypertension is discov-
ered for the ﬁrst time during a routinephysical examination. Because symptomsof hypertension are often vague or evennonexistent, hypertension may go unde-
tected until complications such as heart at –
tack, stroke, or visual problems arise (Glasser,
2001; Setness, 2001). Accurate measurement
of blood pressure and veriﬁcation of ele-vated blood pressure on several occasionsare the chief ways hypertension is diag-nosed. Hypertension is most accuratelydiagnosed when blood pressure is meas-ured under similar conditions over a peri-od of time. Since blood pressure ﬂuctuates
throughout the day or may be higher when
measured in a health care setting than itwould be at home, ambulatory 24-hour
blood pressure monitoring is gaining recog-nition as a more accurate appraisal of bloodpressure throughout the day (White, 2003).
The primary goal of treatment of hyper-
tension is to lower blood pressure andreduce the risk of complications such asheart attack, stroke, or kidney failure. Inmild cases of hypertension, lifestyle mod-iﬁcations such as maintaining a properbody weight, engaging in exercise, cuttingdown on alcohol intake, ceasing to usetobacco, and limiting fats, red meat,sweets, and sodium may be sufﬁcient tolower blood pressure (Appel, Moore,Obarzanek, et al., 1997; Sacks, Svetkey,Vollmer, et al., 2001). Although stress itselfmay not directly cause primary hyperten-sion, emotional stress does cause physio-logic changes that raise the blood pressure.Learning how to reduce stress, or learningto avoid chronic stressful situations, mayalso be important in the overall treatmentof hypertension.
When lifestyle modiﬁcation is insufﬁ-
cient to control hypertension or whenblood pressure is too elevated to controlwith lifestyle modification alone, animportant aspect of treatment is medica-tion. A variety of medications, called anti-
hypertensives , are prescribed to control
high blood pressure. However, these canalso cause a variety of side effects that caninterfere with individuals’ willingness totake the medication as prescribed.
Psychosocial Issues in Hypertension
Although there usually are no symp-
toms associated with primary hyper-tension, the consequences of untreatedhypertension can be severe, includingend-stage renal disease, myocardial in-
farction (heart attack), or stroke. Conse-
quently, treatment of hypertension isessential to preventing disability and/ordeath. Individuals with hypertension fre-quently experience few symptoms, sothere may be less motivation to followtreatment. Treatment also often involveslifestyle changes that individuals mayhave difﬁculty accomplishing, such asweight loss, smoking cessation, or exer-cise. Consequently, they may not complywith treatment and medical recommenda-tions that could help them prevent com-plications and further disability.
Compliance is deﬁned as the degree to
which individuals’ behavior corresponds308
CHAPTER 11 C ARDIOVASCULAR CONDITIONS

to the medical advice and instructions giv-en. In treatment of hypertension, compli-ance includes not only taking medicationsprescribed, but also keeping scheduledmedical appointments and making recom-mended lifestyle changes. Identifying thepotential problems that contribute to non-compliance and working toward solutionsto overcome them can be beneﬁcial.
Vocational Issues in Hypertension
There are usually no speciﬁc limitations
associated with hypertension per se; how-ever, isometric activities such as pushing,lifting, or carrying heavy objects canincrease blood pressure during the activ-ity and may need to be avoided. Somedegree of emotional stress is inherent inmany jobs; however, chronic, sustainedstress may also have a detrimental effecton blood pressure. Individuals shouldeither be assisted in learning to managethe stress they experience on the job orshould seek ways of modifying the workenvironment to make it less stressful. Themajor impact hypertension has on em-ployment is the disability that can occurif it is uncontrolled. Consequently, rein-forcing adherence to medical recommen-dations is of major importance to ongoingemployment.
Coronary Artery Disease: AnginaPectoris and Myocardial Infarction
Coronary artery disease is a condition
in which the coronary arteries that supplyblood and oxygen directly to the heartmuscle ( myocardium ) become narrowed.
The condition is usually caused by ather-
osclerosis , in which plaque builds up on
the inner walls of the blood vessels thatsupply the heart muscle. Buildup ofplaque may narrow the coronary arteriesto the extent that insufﬁcient blood pass-es through the arteries to meet the oxy-gen demands of the heart muscle(ischemia ). Lack of oxygen to the heart
muscle results in chest pain ( angina pec-
toris ). Since the heart muscle’s need for
oxygen is greatest when demands areplaced on the heart, angina pain is oftentriggered by physical activity. Decreasingthe activity and thus decreasing the work-load of the heart often causes the chestpain to subside. This type of angina iscalled stable angina . When chest pain oc-
curs at rest, with no precipitating activi-ty, or when pain is more severe, morefrequent, or more prolonged, individualsare said to have unstable angina . Diagnosis
of angina is based on symptoms, labora-
tory evaluations , an electrocardiogram (ECG)
(recording of electrical activity of theheart) or exercise electrocardiogram (stress
test), or an echocardiogram (discussed lat-
er in the chapter).
The heart muscle, like all other muscles,
cannot live without oxygen. When themyocardium receives nooxygen ( anoxia ),
necrosis (tissue death) of part of the heart
muscle occurs. This is called a myocardial
infarction (heart attack), which means
there has been death of part of the heartmuscle. Myocardial infarction may occuranytime the blood supply to the heartmuscle is insufﬁcient. Not all individualswith angina go on to develop myocardialinfarctions, and not all people withmyocardial infarctions have ﬁrst experi-enced angina, where there is only dimin-
ished blood supply and oxygen to the
heart. Total occlusion of a coronary ves-sel so that the heart muscle receives noblood supply can occur because of:
•atherosclerosis , in which the coro-
nary arteries become totally occluded
•a thrombus (blood clot) that has
formed in a coronary artery, occlud-ing blood ﬂowCardiovascular Conditions 309

• an embolus (blood clot, a particle of
vegetation from a diseased valve, orother foreign material that has trav-eled through the bloodstream) thathas lodged in a coronary artery,occluding blood ﬂow
Once a portion of the heart muscle has
been destroyed, it cannot regenerate. Theability of the heart to continue function-ing as a pump is directly related to theamount of heart muscle damage that hasoccurred. Myocardial infarction can resultin arrhythmia (irregular heartbeat), con-
gestive heart failure (discussed later in the
chapter), or death. Individuals with myo-cardial infarction often experience pres-sure in the chest and chest pain that is notrelieved by reducing activity. Myocardialinfarction is a medical emergency and canbe fatal. Consequently, immediate medicalattention is required.
Myocardial infarction is diagnosed by
the individuals’ symptoms and by ECG and
laboratory determinations , which also help
physicians determine the most appropri-ate treatment.
Treatment of Angina Pectoris and
Myocardial Infarction
Angina Pectoris
Treatment of angina pectoris includes
measures to reduce symptoms and preventmyocardial infarction (heart attack), as
well as surgical measures to correct thecause. Treatment includes modiﬁcation of
ongoing risk factors, medication, and eval –
uation of the need for surgical interven-tion. Cessation of tobacco use, treatment
of hypertension (high blood pressure), and
weight loss, if obesity exists, are importantways to modify risk. Angina pectoris maybe helped by nitroglycerin , a medication
that dilates the coronary arteries andenables the heart muscle to receive moreoxygen, thus relieving chest pain.
When angina pain occurs so frequent-
ly that limitation of activity becomes severe-
ly debilitating, or when occlusion of thecoronary arteries becomes so pronouncedthat myocardial infarction is imminent,surgery may be indicated. One procedureused to enlarge a narrowed coronaryartery is coronary angioplasty (percutaneous
transluminal coronary angioplasty , PTCA ). In
this procedure, a long catheter with a bal-loon on its tip is guided into the coronaryartery. The balloon is then inﬂated, com-pressing the occluding material againstthe vessel wall. The vessel is dilated as theinﬂated catheter tip is withdrawn, thusincreasing the blood ﬂow to the myocardi-um. Although helpful for some, the pro-cedure may not be appropriate for allindividuals. Individuals may also have acoronary artery bypass graft (CABG ) to
relieve narrowing or constriction of thecoronary arteries. A graft, usually a veinfrom the individual’s leg, is used tobypass an obstructed coronary artery.Often, several coronary arteries are con-stricted, and more than one graft is need-ed. The bypass increases the myocardium’sblood supply, thus potentially increasingindividuals’ ability to engage in activity.
Myocardial Infarction
Myocardial infarction, a potentially life-
threatening condition, requires immediatemedical attention. Individuals usually re-ceive initial treatment in the emergencydepartment, where the focus is on assess-ing the condition, stabilizing the condi-tion, relieving pain, and preventingsudden death. In the initial stages ofmyocardial infarction, pain may be treat-ed with narcotics, thrombolytic drugs may
be given to dissolve clots, anticoagulants
may be given to decrease the likelihood of310
CHAPTER 11 C ARDIOVASCULAR CONDITIONS

further clot formation, and oxygen maybe given to decrease hypoxemia (lowered
oxygen in the blood).
After emergency department treatment,
individuals are usually hospitalized in ahospital unit called a coronary care unit(CCU), which specializes in critical careof individuals with cardiovascular con-ditions. The goal of treatment in the CCUis to limit the size of heart damage,promote the electrical stability of theheart, promote comfort, and preventadditional damage to the heart muscle.Here individuals are monitored for life-threatening arrhythmias (irregular
heartbeat) and also receive continuedpharmacologic and medical treatmentdirected to these treatment goals. Someindividuals also undergo surgical revascu-larization procedures (PTCA or CABG ) asdescribed above.
Cardiac rehabilitation is crucial in the
treatment of myocardial infarction so thatindividuals can improve their exercisecapacity, return to work, and reduce therisk of mortality. Most cardiac rehabilita-tion programs include educational ses-sions to help individuals achieve necessarylifestyle changes, including dietary restric-tions, smoking cessation, and graduatedexercise training, to help them reach theirmaximum activity level and functionalcapacity. Psychological and vocationalcounseling are also important compo-nents of cardiac rehabilitation programs.
After a cardiac event, the degree of dis-
ability and the type of activities in whichindividuals can engage are based on theenergy expended to perform the activity.Energy is expressed in terms of calories perminute and is based on the equivalent ofone liter of oxygen equaling ﬁve calories.Prior to exercise training, individualsundergo testing to assess their functionalcapacity so that an appropriate level ofactivity may be prescribed. Functionalcapacity is estimated in METs ( metabolic
equivalents ). The MET is the unit or level
used to estimate the oxygen requirements
to perform a task. This measure helps todetermine the energy cost (in terms ofoxygen consumption) of physical activi-ties and provides a method for describingthe functional capacity or exercise toler-ance of individuals and the physicalactivity level in which they can participatesafely. The degree of activity individualsmay participate in after a cardiac event isdetermined by an exercise test that focus-es on the symptoms and signs shown dur-ing exercise. Different types of activityhave been classiﬁed in terms of how manyMETs are required. MET requirementsrange from one (the amount of oxygen anindividual consumes at rest while awake)to nine for the heaviest tasks (such asshoveling heavy snow or carrying itemsover 90 pounds).
Psychosocial Issues in Angina Pectoris and
Myocardial Infarction
The pain associated with angina pec-
toris, as well as limited activity and theanxiety caused by fear of potential heartattack, can limit the individual’s ability toparticipate in a number of activities. As aresult, individuals may experience lowself-esteem and depression.
Stresses experienced as a result of myo-
cardial infarction are greatly inﬂuenced byindividuals’ psychological reactions to theheart attack. When individuals have amyocardial infarction, the realization thatdeath could have occurred as well as therealization of the unpredictability andthreat of sudden death can precipitatesevere depression or anxiety, which canresult in emotional disability. In the ear-ly stages of recovery from myocardialinfarction, denial may be positive becauseit helps individuals reduce the emotionalCardiovascular Conditions 311

distress associated with the knowledgethat their condition is potentially life-threatening (Livneh, 1999). As individu-als progress in recovery, however, denialmay become detrimental if they deny theseriousness of the heart attack and fail toalter their lifestyle or to follow other treat-ment recommendations.
The life-threatening nature of myocar-
dial infarction may cause a variety of reac-tions in family members, which in turnaffect individuals’ adjustment to their con-dition. Because of overwhelming anxietyabout the possibility that another myocar-dial infarction might occur, family mem-bers can overprotect the individual, in-hibiting his or her return to full function-al capacity. The extent to which familymembers believe individuals contributedto the development of their condition byengaging in activities viewed as precursorsof heart disease, such as by smoking, fol-lowing an improper diet, or eating to thepoint of obesity, may further inﬂuencerelationships. Family members mayexpress anger, resentment, or frustrationor blame individuals for their behavior. Inturn, individuals who have had a myocar-dial infarction may experience guilt, lowself-esteem, and self-blame.
Sexual readjustment after myocardial
infarction may also be an issue. Depres-sion and lowered self-esteem may con-tribute to sexual dysfunction. In addition,some medications commonly prescribedafter myocardial infarction may impairsexual function. Individuals and/or theirpartners may be especially anxious aboutengaging in sexual activity after a heartattack, fearing that sexual activity is toostressful and may precipitate anotherheart attack and possibly sudden death.Education and appropriate counseling, aswell as reassurance, may be necessary tohelp individuals and their partners lessentheir fears. Vocational Issues in Angina Pectoris and
Myocardial Infarction
Most individuals after appropriate car-
diac rehabilitation are able to return tomoderate levels of activity; these aredetermined through a medical evaluationof the energy cost of various activities, asdescribed previously. Work activity shouldnot exceed individuals’ limits. For themost part, isometric activities are avoidedbecause of the additional stress they placeon the heart. Because of stress thatextreme temperatures place on the heart,work environments with controlled tem-peratures are preferable. The amount ofstress experienced on the job as well as theindividual’s response to it should also beconsidered.
Cardiac Arrhythmia
An arrhythmia is an abnormality of the
heart rate or rhythm. A dysfunction in theheart’s electrical conduction system caus-es irregularities in its rhythm and/or rate.Arrhythmias decrease the heart’s ability towork effectively and to supply adequateamounts of blood to all of the body’s
organs. The heart may beat too fast ( tachy-
cardia ), too slowly ( bradycardia ), or
irregularly ( dysrhythmia or arrhythmia ).
There are many different causes of
arrhythmia and many different types.Types of arrythmias are usually named forthe type of disorder or the part of the elec-trical impulse system that is affected. Forexample, a sinus bradycardia indicates
that there is an abnormally slow rhythmarising in the SA node of the heart, where-
as an AV block describes an arrhythmia in
which electrical impulses are blocked atthe AV junction . Some arrhythmias may be
the cause of signiﬁcant disability (such asatrial ﬁbrillation , which can cause stroke),
some may be life-threatening (such as ven-312
CHAPTER 11 C ARDIOVASCULAR CONDITIONS

tricular ﬁbrillation ), and others may be rel-
atively minor and require little or no treat-ment.
The symptoms individuals experience
with arrhythmia depend on the type and
extent of the arrhythmia. Individuals may,
for example, experience palpitations (in
which they feel the heartbeat), exertion-
al dyspnea (shortness of breath with
activity), fatigue, vertigo (dizziness), or
syncope (fainting). Severe or prolonged
arrhythmia can result in sudden death.
Treatment of Arrhythmia
Treatment depends on the underlying
condition. It is directed toward correctingor controlling the factors causing thearrhythmia. Some arrhythmias may beprevented by avoiding stimulants such ascaffeine or avoiding alcohol.
Medications
Medications called antiarrhythmics reg-
ulate the heartbeat and are often a centralpart of treatment. Other medications use-ful in the control of arrhythmia are digi-
talis preparations, beta blockers , and
medications called calcium channel block-
ers. Because certain types of arrhythmias
(e.g., atrial flutter ) can contribute to
thrombus (blood clot) formation and pos-
sible embolus (traveling blood clot), some
individuals may also need to be on anti-
coagulant medications (Hart, 2003).
In more severe arrhythmia, an electrical
shock procedure called cardioversion may
be indicated to return the heart to a nor-mal rhythm. For individuals with a severe,recurrent arrhythmia that could result ina life-threatening arrhythmia (e.g., ventric-
ular tachycardia , which can become ventric-
ular fibrillation ), a device called an
implantable automatic deﬁbrillator may be
surgically implanted. The defibrillatordelivers an electric shock automatically tothe heart when an arrhythmia occurs (seethe following section).
Pacemakers and Implantable Deﬁbrillators
When the heart’s ability to maintain an
effective rate or rhythm is altered, an arti-ﬁcial cardiac pacemaker may be used tostimulate the electrical activity of theheart and to maintain function. Implan-table pacemakers and deﬁbrillators areundergoing rapid evolution (Cooper,Katcher, & Orlov, 2002) and can not onlydecrease the incidence of potentially fatalarrhythmias, but can also enhance quali-ty of life for those with problem arrhyth-mias (Newman, Dorian, Schwartzman, etal., 2000). The pacemaker consists of a bat-tery-operated pulse generator and a leadwire with an electrode tip. One end of thelead wire is inserted into a vessel andadvanced into the individual’s heart; theother end is connected to the generator.The generator then sends out an electri-cal stimulus to the heart muscle. The gen-erator may be external if the need forpacing is only temporary. If the pacemak-er is to be permanent, a small battery-operated generator is placed under theskin and fatty tissue of the upper chest orlower thoracic area.
There are various types of pacemakers.
They are usually classiﬁed according to thechamber of the heart that is being stimu-lated, the chamber of the heart that is be-ing monitored, and the response that thepacemaker is expected to deliver. The clas-
siﬁcation system uses a three-letter code to
describe pacemaker function. The ﬁrst let-ter of the code signiﬁes the chamber beingstimulated, the second letter indicates thechamber being monitored, and the third
letter indicates the pacemaker response. For
example, a code VVI would indicate that a
ventricle is being both stimulated andCardiovascular Conditions 313

monitored. The Istands for “inhibited
response,” indicating that the pacemakerwill not allow impulses from the atria tostimulate the ventricle.
There are several modes of pacing that
pacemakers are designed to deliver. Theoldest type of pacing, ﬁxed rate , is rarely
used today. In this type of pacing, thepacemaker is set to ﬁre at a ﬁxed rate, usu-ally about 70 beats per minute, and isunaffected by the heart’s own rhythm.Another type of pacing, demand or stand-
by, is accomplished with a pacemaker that
has a special sensing circuit that is set ata speciﬁc rate. When the individual’s ownconduction system in the heart falls belowthat speciﬁc rate, the artiﬁcial pacemakerﬁres. Other types of pacing, namely, syn-
chronous and bifocal , use pacemakers that
are programmed in similar ways to moni-tor and deliver speciﬁc types of impulses.
The mode of pacing is determined on an
individual basis according to the individ-ual’s speciﬁc arrhythmia. Physicians deter-mine the type of pacemaker to be usedand the amplitude of the stimulus basedon the individual’s condition. For mostindividuals, even permanent pacemakersmay be inserted under local anesthesiawith mild sedation.
Complications related to implantation
of a pacemaker are rare, but they can in-
clude pneumothorax (collapse of the lung),
dislodgement, inﬂammation, or infection
of the surrounding area (Morady, 2000).
The level of activity individuals with a pace-
maker can engage in depends on the under-
lying disease process, age, and the degree of
cardiac functional capacity. Normal dailyactivities can usually be resumed 6 weeks
after the implantation of the pacemaker. Ac-
tivities that could expose the internal pace-
maker to a blow, such as contact sports,should be avoided. Although driving maybe restricted for a short time after the pace-maker is inserted, most individuals canbegin driving in approximately a monthif the pacemaker is functioning well.
Individuals who have a pacemaker
should at all times wear identiﬁcation,such as Medic Alert , or should carry a card
containing information about the type ofpacemaker, the date of implant, and thepacemaker’s programming. Because thepacemaker’s generator is battery-operated,failure of the battery means that the heartreturns to beating at its previously abnor-mal rate or rhythm. Individuals with pace-makers should be aware of the signs ofbattery depletion, such as a change in thecardiac rate or the appearance of symp-toms similar to those experienced beforethe pacemaker was inserted. The length oftime that a battery lasts depends on themodel and can vary from one to severalyears. A physician should evaluate thepacemaker’s function regularly. Periodicevaluations may be conducted with spe-cial telephone monitoring in which infor-mation about the pacemaker’s function istransmitted over regular telephone lines toa special device in the physician’s ofﬁce.
Electromagnetic interference with per-
manent pacemakers and implantabledeﬁbrillators may have deleterious effects(Santucci, Haw, Trohman, & Pinski, 1998).Although the shielding around battery-operated generators has been improvedsigniﬁcantly, individuals who wear thesedevices should be aware of possible inter-ference from a variety of external electri-cal signals in the environment. Since im-plantable deﬁbrillators are designed to bemore sensitive to intracardiac electricalactivity, their sensitivity to electromagnet-ic interference may be increased. Micro-
wave ovens, radar installations, arc welding
devices, antitheft devices, cellular phones,and other sources of electrical signals mayinterfere with pacemaker signals. Thepacemaker may also set off metal detec-tion devices installed at airports.314
CHAPTER 11 C ARDIOVASCULAR CONDITIONS

Psychosocial Issues in Arrhythmia
The psychosocial impact of arrhythmia
can be signiﬁcant. Fearful of triggering apotentially fatal arrhythmia, individualsmay curtail many activities related to bothwork and leisure. In many instances, thefear and anxiety may be more disablingthan the arrhythmia itself.
Since arrhythmias can be triggered by
caffeine, alcohol, or tobacco use, individ-uals may also need to modify their life tosome extent. Commitment to lifestylechanges varies with the degree to which
individuals accept and understand the con-
dition and the necessity for treatment. In-dividuals who become extremely anxiousabout their condition may cope by em-ploying denial as a way of decreasing thelevel of stress. If individuals are in denial,rather than making the necessary lifestylechanges, they may continue activities thatcould have serious consequences.
Vocational Issues in Arrhythmia
Any activity that has been identiﬁed by
the individual as triggering arrhythmiashould be avoided. Avoiding excessiveemotional stress and learning to manageit may be important components of theindividual’s ability to continue to performadequately at work without danger of pre-cipitating an arrhythmia. Individuals be-ing treated with anticoagulants may needto be aware of the potential for excessivebleeding if injury should occur. Excessiveanxiety about precipitating an arrhythmiacan become disabling, immobilizing indi-viduals and preventing them from carry-ing out normal tasks and activities.Individuals may develop chronic depres-sion, which can further interfere withtheir ability to work.
Individuals with pacemakers may need
to avoid activities (such as using an airhammer or shooting a riﬂe) that couldpotentially cause the pacemaker to dis-lodge because of vibrations on the sidewhere the pacemaker is located. Individ-uals should avoid activities such as arc andresistance welding, the use of powertools, or contact with radar transmitters,which could cause the pacemaker to mal-function. A physician should clarify thelevel of activity allowed, and counselingmay be indicated to help individuals dealwith their fears and enhance their abilityto perform work activities.
Valvular Heart Conditions
Damage to the valves of the heart is
most often the result of rheumatic fever(a condition caused by the body’s immuneresponse to a streptococcal infection) orendocarditis (inﬂammation of the inner
membrane of the heart), although valvu-lar abnormalities may also be congenital.Two types of problems generally occur.Valves may become weakened or ﬂoppy,permitting a backﬂow ( regurgitation ) of
blood from the ventricle to the atria, orvalves may become scarred, narrowing thevalvular opening ( stenosis ) and causing
an obstruction of blood ﬂow from a cham-ber of the heart. Although some valvularconditions are minimal and may requirelittle intervention, more extensive valvu-lar damage places an increased burden onthe heart and can lead to dysfunction ofthe myocardium, congestive heart failure
(discussed later in the chapter), and, insome instances, sudden death.
Types of Valvular Conditions
Valvular conditions are classiﬁed accord-
ing to the nature of the abnormality andthe valve affected. One type of valvularcondition, mitral prolapse , refers to bulg-
ing of all or part of the mitral valve intoCardiovascular Conditions 315

the left atrium during ventricular contrac-tion. Mitral regurgitation , mitral insufﬁcien-
cy, or mitral incompetence refers to the
inadequate closing of the mitral valve,which allows blood to ﬂow backward intothe atria. Mitral stenosis refers to narrow-
ing of the mitral valve, which obstructsthe blood ﬂow from the left atrium to theleft ventricle. Tricuspid regurgitation and tri-
cuspid stenosis are conditions similar to the
regurgitation and stenosis conditions de-scribed above but occur on the right in-stead of the left side of the heart. The sameprocess may affect the pulmonary or aor-tic valves.
Valvular defects of the aortic valve, such
as aortic stenosis and aortic regurgitation ,
place additional loads on the left ventri-cle of the heart, possibly resulting in leftventricular heart failure. Symptoms ofvalvular disease vary in severity but ofteninclude fatigue, dyspnea (difficulty
breathing), and palpitations (heartbeat
perceptible to the individual).
Treatment of Valvular Heart Conditions
Speciﬁc treatment depends on the sever-
ity of the problem. Some conditions may
require individuals to avoid strenuous activ –
ity. Others require no treatment or maynot necessitate taking any precautions.Damaged valves are more susceptible to
infection. Consequently, prophylactic anti-
biotics may be given to prevent endo-
carditis when there is the chance for a
generalized bacterial infection, such asafter a dental extraction.
Severe damage to the valve may require
surgery to open or replace the valve.Surgical interventions for valvular abnor-malities are intended either to widen avalve that is narrowed or constricted or,in the case of valvular insufﬁciency orregurgitation, to replace a diseased valvewith an artiﬁcial valve. Individuals withstenosis of a valve may undergo a proce-dure known as valvuloplasty in which
the stenosed (narrowed) valve is dilated
with a balloon that is inserted through aperipheral vessel.
When valves are replaced, artificial
valves, which are mechanical, or valvesmade of tissue may be used. Mechanicalvalves are made entirely from syntheticmaterials, whereas tissue valves may bemade from a combination of syntheticand biologic tissue. Mechanical valvesrequire long-term anticoagulant therapyto prevent thrombus formation. Althoughtissue valves decrease the risk of clot devel-opment, they may not have the long-termdurability of mechanical valves. Becauseprosthetic valves are more vulnerable toinfection, individuals may need to takeantibiotics before procedures in whichinfection is a risk (e.g., dental work).
Congestive Heart Failure
When the heart muscle is weakened or
damaged and it cannot pump an adequateamount of blood to the rest of the body,a condition called heart failure occurs. Thecauses of heart failure include myocardial
infarction (heart attack); damage from
substances toxic to the heart muscle(such as alcohol or other chemicals); hyper-
tension , arteriosclerosis , or valvular dysfunc-
tion; or lung disease such as emphysema , all
of which cause the heart to work harder.When the heart consistently must workharder to pump, over time it becomesenlarged ( hypertrophy ) and ineffective in
its pumping action. As a result, ﬂuid accu-mulates in the lungs, causing congestion,dyspnea (difﬁculty breathing), and difﬁ-
culty breathing when lying down at night
(nocturnal dyspnea ). The decreased pump –
ing action of the heart and the congestionin the lungs result in an inadequate sup-316
CHAPTER 11 C ARDIOVASCULAR CONDITIONS

ply of oxygen to the rest of the body.Individuals with heart failure may conse-quently also experience fatigue and phys-ical weakness. If the oxygen supply to the
brain is inadequate, cognitive changes may
also be present. Because of insufﬁcientpumping and circulation of blood, ﬂuidmay accumulate in the extremities, caus-ing swelling ( edema ). Blood ﬂow to the
gastrointestinal system may be impaired,
causing congestion with resulting anorexia
(loss of appetite) or nausea and vomiting.
Treatment of Congestive Heart Failure
Treatment of heart failure depends on
the type and causes of the condition. It isusually directed toward controlling or cor-recting the cause of heart failure andtoward alleviating the symptoms. Often,medications to lower blood pressure ( anti-
hypertensives ) are prescribed. These med-
ications decrease the vascular resistance,thus decreasing the amount of work thatthe heart must perform to circulate blood.Medications to help the heart muscle workmore efﬁciently by increasing its pumpingaction (e.g., digitalis preparations ) may also
be prescribed. Diuretics (medications that
help rid the body of excess ﬂuid) and alow-salt diet to eliminate some of theexcess ﬂuid may also be part of the treat-ment plan.
Psychosocial Issues in
Congestive Heart Failure
Although some symptoms may be con-
trolled, heart failure usually signiﬁes theend stage of cardiovascular disease.Individuals with heart failure may expe-rience depression and anxiety about theirpresent and future situation. Symptoms ofshortness of breath, fatigue, and edema ofthe extremities can severely limit activities
and increase dependency on others, whichmay in turn lower self-esteem. Sinceheart failure is frequently the result of theheart gradually losing its function, indi-viduals also live with the knowledge thattheir condition can result in increasingdisability and death. If individuals are can-didates for cardiac transplant (discussedlater in the chapter), uncertainty of
whether a donor heart will be identiﬁed be –
fore the condition deteriorates even moremay be another source of continuingstress for individuals and their families.
Vocational Issues in
Congestive Heart Failure
The extent to which individuals are able
to continue to function in the work envi-ronment depends on the severity of thesymptoms and the nature of the work.Individuals in sedentary occupationsrequiring limited activity will be able tofunction longer than individuals in occu-pations in which strenuous activity isrequired.
In general, emotional stress and physi-
cal demands on the job should be mini-mized as much as possible. Extremes intemperatures can put additional strain on
the heart, and therefore temperature-con-
trolled environments are better tolerated.
Congestive heart failure is often associ-
ated with gradual and progressive deteri-oration of cardiac function. Consequently,vocational goals may need to be shortrange to accommodate potential function-al decline if it does occur.
Peripheral Vascular Conditions
Disorders of the peripheral blood vessels
(i.e., those in the extremities) can lead todamage of the tissues supplied by thosevessels. When oxygen supply is inade-quate because of the diminished bloodCardiovascular Conditions 317

ﬂow, extremities feel cold and appear paleor cyanotic (blue). Pain is also char-
acteristic when the oxygen supply isdiminished.
Peripheral Vascular Disease
(Arteriosclerosis Obliterans)
When arteriosclerotic changes have
narrowed or occluded the larger peripher-al vessels, an adequate blood supply can-not reach tissues in the extremities.
Symptoms depend on the extent of the
obstruction, the vessels involved, andwhether alternate blood supply routes,called collateral circulation , have formed.
Exercise requires increased demand foroxygen by muscles. Therefore, individualswho have a deﬁcient blood supply to themuscles because of peripheral vascular dis-ease may, with activities such as walking,experience aching, cramping, or fatigueof the muscles in the legs, a condition
known as intermittent claudication . Stop –
ping to rest decreases the muscles’ needfor oxygen and consequently relieves thepain. If the condition progresses, howev-er, pain in the extremities may occur evenat rest. In severe cases, the feet maybecome numb and cold, and ulcerationsof the foot may appear. Surgical pro-cedures, such as a bypass graft of the
severely affected vessel, may restore vas-cularization to the extremity in selectedcases. Since smoking constricts blood ves-sels, use of tobacco products should beavoided.
Because of the diminished blood supply
in peripheral atherosclerotic disease, eventiny injuries in the extremities maybecome infected and not heal properly. Ifcirculation becomes so severely impairedthat necrosis (tissue death) results, ampu-
tation of the extremity may be necessaryto prevent complications such as thespread of infection throughout the body. Thromboangiitis Obliterans
(Burger’s Disease)
Thromboangiitis obliterans is a rare con-
dition of the small and medium-sizedarteries and superﬁcial veins of the ex-tremities that causes diminished bloodﬂow to the affected part. In contrast toperipheral atherosclerotic disease, throm-boangiitis obliterans occurs predominant-ly in individuals between the ages of 20and 40 who do not have signiﬁcant ath-erosclerosis. Symptoms include numbness,tingling, and pain in the upper or lowerextremities. Although the exact cause isunknown, thromboangiitis obliterans oc-curs almost exclusively in individuals whosmoke. Consequently, the major treat-ment of the condition is to stop smoking.If individuals with thromboangiitis ob-literans continue to smoke, the diseasecontinues to progress and can ultimate-ly require the amputation of affectedextremities.
Raynaud’s Disease
Raynaud’s disease is a condition in
which spasms of the vessels in the ﬁngersor toes impair the blood ﬂow to thoseareas. Occasionally, the condition also af-fects the nose and the tongue. In most in-stances, the cause of the condition isunknown; however, it may be associatedwith other conditions, such as rheumatoid
arthritis or arteriosclerosis obliterans . Attacks
of vasospasm may last from minutes tohours, but they rarely last long enough tocause tissue death. Attacks result in color
changes in ﬁngers or toes, either blanching
(white coloration) or cyanosis (blue color-
ation). Attacks may be precipitated by coldor emotional upsets.
If the Raynaud’s phenomenon is sec-
ondary to another condition, treatment is318
CHAPTER 11 C ARDIOVASCULAR CONDITIONS

directed toward the underlying disorder.In the majority of cases the cause isunknown, and treatment involves takingsteps to prevent an attack from occurring,such as protecting oneself from the coldor avoiding emotional upsets. Smokingconstricts the blood vessels; consequent-ly, tobacco use should also be avoided.Treatment with biofeedback or the use ofrelaxation techniques may sometimes behelpful in reducing attacks.
Venous Thrombosis
(Thrombophlebitis; Phlebitis)
A common and potentially lethal com-
plication of bedrest or inactivity is throm-bophlebitis. In chronic illness or disabilityin which activity is limited, thrombo-phlebitis can be a serious complicationcausing additional disability or potential-ly death. Phlebitis is the inﬂammation of
a vein. Thrombophlebitis is the inﬂam-
mation of a vein with associated clotformation. Although phlebitis and throm-bophlebitis can occur in any vein, theyfrequently occur in the veins of the low-er extremities.
Individuals with thrombophlebitis may
experience pain and tenderness in theaffected area, especially if the lower ex-tremity is affected. Individuals with loss ofsensation as a result of spinal cord injurymay be unaware of the condition, andconsequently it may not be promptlytreated. Other symptoms may includeswelling and redness of the affected part,or at times, depending on the location ofthe inﬂammation, there may be no symp-toms at all. If thrombophlebitis is unrec-ognized or inadequately treated, clots canbreak off and travel to the heart, lungs, orother parts of the body and lodge in a ves-sel, occluding blood supply to the bodypart. Depending on the location of the oc-clusion, individuals can experience stroke,myocardial infarction (heart attack), or
massive damage to whatever body part isaffected. Treatment of phlebitis andthrombophlebitis is directed towarddecreasing inﬂammation and preventingor dissolving clots through use of antico-agulants, antithrombotic agents, or anti-inﬂammatory agents. Bedrest is usuallyprescribed during therapy, along withmedications that decrease clotting.
Varicose Veins
When blood cannot be returned efﬁ-
ciently to the heart, backup of blood caus-es distention and congestion of the veins,or varicose veins. Anything causing stric-ture or pressure on the veins, such as pro-longed standing, obesity, or constrictionof the leg by circular garters, can aggravatethe condition. Symptoms are a sensationof heaviness in the legs, fatigue, and, attimes, pain. In mild cases, treatment mayconsist of using compression hosiery.Surgery to tie and strip the veins may beindicated when the condition involvessevere pain or recurrent phlebitis.
Vocational Issues in
Peripheral Vascular Disease
Individuals with peripheral vascular
conditions may be unable to stand forlong periods of time or may be unableto walk without pain or muscle fatigue.Their stamina during these activitiesshould be evaluated before they return towork. Environmental conditions, such ascold temperatures, or other factors thatreduce blood supply to the extremitiesshould be avoided. The potential for in-fection in the lower extremities because ofinadequate blood supply necessitatesavoiding work environments that containhazards that could cause trauma to thefeet or legs. Cardiovascular Conditions 319

DIAGNOSTIC PROCEDURES INCARDIOVASCULAR CONDITIONS
In addition to a physical examination
and medical history, a variety of tests areused to diagnose cardiovascular condi-tions. These tests may also provide infor-mation that is used to make treatmentdeterminations or to evaluate treatmenteffectiveness.
Chest Roentgenography (X-ray)
A noninvasive radiographic procedure,
roentgenography (X-ray ) makes it possible to
visualize organs in the chest cavity on X-ray
ﬁlm. Films may show evidence of conges-
tion or ﬂuid in the lungs, hypertrophy (en-
largement) of any of the heart’s chambers,
or other abnormalities in the chest cavity.
Electrocardiography
An ECG is a graphic representation of
electrical currents within the heart mus-cle. It is helpful in identifying abnormal-ities of the heart’s rhythm, assessing theamount and location of damage to thecardiac muscle, determining whether thecardiac muscle is receiving an adequatesupply of oxygen, and obtaining informa-tion about the effects of certain medica-tions. An ECG is a painless,
noninvasive
procedure in which electrodes are placed
externally on the skin and then connect-
ed to a special machine that trans forms
electrical impulses from the heart to agraphic printout that records the heart’sactivity. The ECG is usually performed inthe physician’s ofﬁce, hospital, or othermedical setting.
Holter Monitor
The Holter monitor is a form of ECG in
which several electrodes attached exter-nally to the chest are connected to a smallportable device that records the heart’sactivity. The device is worn on the shoul-der or waist so that the individual can goabout regular activities at home or work.The advantage of the Holter monitor isthat the graphic reading of the heart’s elec-trical impulses is continuous rather thanbeing a one-time reading in a laboratorysituation. Readings from the Holter mon-itor enable physicians to assess the heart’sfunctioning during various normal activi-
ties throughout a 24-hour period or longer.
Cardiac Stress Test
The cardiac stress test is a noninvasive
exercise test that provides a graphic recordof the heart’s activity during forced exer-tion. It may be used diagnostically todetermine the extent of cardiac disease. Itcan also be used as a basis for recommend-ing either medical or surgical treatment,as well as for counseling individuals withcardiac disease about the type and amountof physical activity in which they maysafely engage. The stress test is performedin a cardiology unit or clinic by a techni-cian with a physician present.
Electrodes are placed externally on the
chest and connected to an ECG monitor.The individual is then asked to step on amotor-driven treadmill or to sit on a sta-tionary bicycle with an ergometer . Activity
is begun slowly, with a gradual increase ofpace. During this time, the physician ortechnician monitors the individual’s ECG
reading, pulse, and blood pressure. The test
is stopped if the individual is no longerable to keep up the pace or develops chestpain, or if the physician determines thatblood pressure, pulse, or ECG readingsindicate excessive strain on the heart.
The stress test is performed in a con-
trolled, laboratory environment. Consider-
ation must also be given, however, to320 CHAPTER 11 C ARDIOVASCULAR CONDITIONS

additional sources of stress, such as emo-tional stress, extremes in temperature, andphysical terrain (such as steps or ramps),that may be present in the individ-ual’s
natural or work environment and
that may increase the heart’s workload
beyond that experienced in the laborato-
ry situation.
Angiography
When it is necessary to study one or
more blood vessels, an invasive procedurecalled angiography (a series of X-ray pic-
tures that deﬁne the size and shapes ofvessels and/or organs) is used. Anangiogram of the heart enables physicians
to identify abnormalities in the size orshape of the vessels, the extent of narrow-ing or occlusion, and the sequence andtime in which the vessels ﬁll with blood.
Angiograms are named for the speciﬁc
area of the body being studied. If arteriesare being studied, the test is called an arte-
riogram . If veins are being studied, the test
is called a venogram . If vessels of the heart
are being studied, the test is called a car-
diac angiogram . A radiologist (a physician
who specializes in X-ray procedures) per-forms an angiogram. The procedure is usu-ally done in the radiology department.During the procedure, a special catheter isplaced into a vein in the arm or leg anddye is injected. At this time, a rapid seriesof X-rays are taken, enabling the physicianto visualize the vessels.
Echocardiography
Like the ECG, the echocardiogram is
obtained by means of a noninvasive pro-cedure. Ultrasound is used to record thesize, motion, and composition of the heartand large vessels on an echocardiogram.A transducer converts sound waves to elec-trical signals, which are then recorded asvisual images and displayed on a type oftelevision screen called an oscilloscope .
Images can be photographed for furtherevaluation by a radiologist or cardiologist .
Echocardiograms are helpful for identify-ing and evaluating valvular defects or oth-er structural abnormalities of the heart.
Radionuclide Imaging
The procedure for radionuclide imaging
begins with the intravenous injection of a
radioactive substance that localizes in heart
tissue. Multiple views of the heart are tak-
en with a special camera. Additional views
are repeated for comparison hours later.The procedure is most useful in evaluat-ing the myocardium (heart muscle) or
damage to the myocardial tissue. It mayalso be used to evaluate coronary arterydisease or valvular disease. It can be per-formed with the individual either at restor during exercise.
Cardiac Catheterization
An invasive procedure, cardiac catheter-
ization , is performed to study the cham-
bers, valves, and blood supply to the heart.A catheter is passed into a vessel in an armor a leg and then threaded into the heart.A special X-ray machine called a ﬂuoro-
scope enables physicians to visualize the
catheter advancing into the heart. Whenthe catheter is in place, internal pressuresin the heart are measured. Dye is theninjected into the catheter, allowing physi-cians to visualize the pumping action ofthe heart and the blood ﬂow through thecoronary arteries.
Cardiac catheterization may be per-
formed to determine the extent of coronary
artery disease, valvular disease, congenitalheart disease, or damage to the heart mus-cle. Information gained from this proce-dure may be used to determine whetherDiagnostic Procedures in Cardiovascular Conditions 321

cardiac surgery is indicated or to assess thefunction of the heart after cardiac surgery.The procedure may be performed in theradiology department, operating room, orspecial room within a cardiac clinic.
GENERAL TREATMENT OFCARDIOVASCULAR CONDITIONS
Individuals with cardiovascular condi-
tions may receive medical and/or surgicaltreatment. In any case, treatment requiresregular medical follow-up to monitor thesuccess of the treatment and the progres-sion of the disease.
Medical Treatment
Although medical treatment of cardio-
vascular conditions varies with the type of
disorder, treatment generally includes both
medication and lifestyle changes. Hyper-
tension is frequently associated with oth-
er cardiovascular disorders. Consequently,antihypertensives (medications that lower
blood pressure) and/or medications that rid
the body of excess ﬂuid ( diuretics ) are often
prescribed. When arrhythmias (irregular
heartbeat) are present, antiarrhythmic med-
ications may also be prescribed. A medica-tion called nitroglycerin may be taken to di-
late the coronary vessels when there is chest
pain from inadequate oxygen supply toheart muscle. Nitroglycerin may also betaken prophylactically before any activi-ty that may increase the heart’s workload.Anticoagulants may be prescribed to reduce
the coagulability of the blood and, thus,the risk of clot formation. Cardiotonic med-
ications , such as digitalis preparations, may
be prescribed to change heart rhythm or
rate and generally to strengthen the heart.
Regardless of the type of medication
prescribed, in all instances, medicationsare taken under physicians’ direction andsupervision. Physicians also prescribe the degree and
type of activity permissible for individu-als with cardiovascular disease. Becausethe heart responds to different types ofmuscular activity in different ways, whenprescribing activity, physicians take intoaccount the nature of the condition andthe ability of the heart to function undervarious types of muscle action. Exercisemay also be prescribed to increase individ-uals’ tolerance of activity.
Factors that contribute to the develop-
ment of cardiovascular disease or that
place additional burdens on the heart, such
as obesity, tobacco use, and stress, are alsoconsidered in planning treatment goals.Obesity increases strain on the heart. Con-sequently, individuals with cardiac diseaseare often placed on low-calorie diets.Sodium may also be restricted because itcontributes to water retention, which in-creases the heart’s workload. Because cho-lesterol levels have also been associatedwith cardiovascular disease, individualsmay be placed on a low-fat, low-cholester-ol diet. Tobacco use is associated with an
increased pulse rate, blood pressure changes,
and blood vessel constriction. Conse-quently, individuals with cardiovascularconditions should avoid using tobacco.
Surgical Treatment
Angioplasty and Bypass
Surgical treatment of coronary artery
disease consists of PTCA and CABG.
The purpose of PTCA is to reopen a nar-
rowed coronary artery. PTCA, also calledballoon angioplasty , involves putting a spe-
cial catheter (thin ﬂexible tube) into theartery in the leg and then threading it un-der X-ray guidance into the coronary ves-sel that is blocked. A balloon on thecatheter’s tip stretches the vessel and ﬂat-tens the arteriosclerotic plaque against the322
CHAPTER 11 C ARDIOVASCULAR CONDITIONS

wall of the artery. The advantage of PTCAis that it is not an open heart procedure,so the recovery time is shorter. The pro-cedure is, however, a temporary solution.Most individuals who have had PTCAneed to have the procedure repeated orhave a coronary artery bypass at a laterdate. Other devices, such as a coronary
stent, a small mesh tube, may be inserted
and left in the artery to prop it open.
The CABG procedure, although not a cure,
is intended to alleviate symptoms of arte-riosclerotic heart disease, prevent myocar-dial infarction, or, if myocardial has oc-curred, prevent additional damage fromoccurring. In addition, it has considerableimpact on individual’s quality of life as
well as ability to return to work (Charlson &
Isom, 2003; McMurray, 1998). In the pastCABG involved open heart surgery , which
also required the use of a heart-lungmachine. This type of surgery involves alonger recovery time. In recent years lessinvasive procedures called port-access coro-
nary artery bypass surgery and minimally in-
vasive coronary bypass surgery , which require
much smaller incisions and use tiny cam-eras and video monitors to guide
surgical
procedures, have been developed. These min-
imally invasive procedures do not require
a heart-lung machine. Unless complications
develop, recovery time is generally short-er than for the regular CABG procedure.
Individuals with valvular disease may
have surgical treatment to widen a valvethat is narrowed or to replace a valve thatis diseased. All of these procedures aredescribed elsewhere in the chapter. Whenthe heart is severely impaired, cardiactransplantation may be performed, as dis-cussed as follows.
Cardiac Transplantation
Cardiac transplantation is an accepted,
established form of therapy when heartdisease is so advanced ( end-stage heart dis-
ease) that standard therapy is no longer
effective and survival is severely threat-ened (House-Fancher & Foell, 2004).Individuals who undergo successful trans-plantation increase their chance for sur-vival and also their chance to return to anormal, productive life. There are approx-imately 257 transplant centers and 140programs that perform heart transplantsin the United States (United Network ofOrgan Sharing, 2002).
Pre-Transplant Procedures
Individuals selected for transplant often
have end-stage cardiac disease with alife expectancy of less than 1 year with-out a transplant (Rourke, Droogan, &Ohler, 1999). Prior to cardiac transplant,individuals undergo a complete physicaland diagnostic workup. The individualand family also undergo a comprehensivepsychological proﬁle that assesses thecoping skills, family support, and moti-vation in the family to follow the rigor-ous medical regimen that is required.The evaluation period can be extremelystressful, with individuals becoming fear-ful they may be found unsuitable fortransplant.
Not all individuals with heart disease are
candidates for cardiac transplantation.Selection is based on factors such as gen-eral physical condition, absence of othersystemic disease that would in itself lim-it survival, the ability to return to normalfunction after surgery, and the ability tocomply with the complex medical regi-men that necessarily follows transplanta-tion. Usually a history of drug or alcoholabuse, mental illness, severe obesity, oth-er systemic diseases, or end-stage renalfunction or altered liver function is a con-traindication to cardiac transplant (House-Fancher & Foell, 2004).General Treatment of Cardiovascular Conditions 323

To be eligible for transplant, an individ-
ual’s physical condition must be strongenough to survive the transplant proce-dure and to be able to comply with thecomplex medical regimen required aftersurgery. Individuals accepted for trans-plant are placed on a waiting list. A 24-hour national computer network links allorgan procurement centers. When adonor organ is identiﬁed, the computercenter searches the list of potential recip-ients for the best match. Donor and recip-ient match is based not only on bloodtype, but also on body and heart size.
The pre-transplant period may be ex-
tremely stressful for individuals and theirfamilies as they wait for a donor to be
identiﬁed. Many individuals may put their
lives on hold while waiting for a trans-plant, and the pressure of uncertainty asto whether or when a heart will becomeavailable can cause severe stress in fami-ly relationships. Individuals and familieshave no control over when surgery willoccur, and in the interim the individual’scondition may continue to deteriorate.Feelings of anxiety and depression arecommon. Individuals may also have feel-ings of guilt because of receiving a heartdonated as a result of someone else’sdeath. Organ preservation time is limitedfrom the time of procurement to the timeof implant (about 4 to 6 hours), so thatindividuals who are to receive the trans-plant must be readily available for surgeryat short notice. This means that if they donot live near a transplant center, they mayneed to relocate to be closer to the facili-ty. Relocation can be an additional sourceof stress, not only for individuals but alsofor their family.
When a donor heart has been identiﬁed
and matching of donor and recipientblood type has been conﬁrmed, the recip-ient is taken to surgery. The individual’sheart is removed and the donor hearttransplanted. Immunosuppressant thera-py to block the body’s natural response toforeign objects begins immediately.
Post-Transplant Procedures
After cardiac transplant, individuals re-
main in the hospital for approximately 5to 10 days. After discharge from the hos-pital, they are required to have checkupsby the transplant team biweekly and thenevery 6 to 8 weeks for 1 to 2 years after
transplant. Biopsies of the heart and mon-
itoring of blood are conducted frequently
during the ﬁrst 6 months to assess im-mune status; then the frequency of biop-sy decreases over time. Individuals are alsoevaluated for possible medicine toxicity orgraft rejection as well as other complica-tions of transplant. If there are signs ofrejection, additional medications are pre-scribed to augment immunosuppression.
Individuals undergoing cardiac trans-
plantation must follow a complex medicalregimen to prevent rejection of the donororgan and other complications. Becausethe body never really ceases its efforts toreject the donor heart, immunosuppres-sants must be taken indeﬁnitely. Thesemedications are a necessary part of treat-ment, but they have serious side effectsthat must be monitored on a continuingbasis. Too much or too little medicationmay cause the body to reject the trans-
plant. Potential complications of immuno –
suppression include an increased suscept-ibility to infection and an increased rateof malignancy.
Individuals generally take approximate-
ly 3 to 6 months after surgery to becomefully functional and to adjust to the im-munosuppressant medications. Survivalrates after cardiac transplant increase asthe time after transplant increases. Whendeath after transplant does occur in the
ﬁrst year, the most common cause is tissue324 CHAPTER 11 C ARDIOVASCULAR CONDITIONS

rejection or infection (Rourke et al., 1999).
After the ﬁrst year the mortality rate ismore frequently related to malignancy.
The cost of heart transplantation can be
staggering. Not only are there signiﬁcantcharges for the transplant surgery, hospi-talization, and continuing immunosup-pressant medication, but there are alsocosts associated with travel to and fromthe transplant center for checkups, thecost of food and lodging for the familywhile the individual is hospitalized, andand the cost of other support services thatmay be needed when the individualreturns home.
Since the demand for cardiac transplan-
tation far exceeds the number of donorhearts available, mechanical support inthe form of left ventricular assist devicesor artiﬁcial hearts has become a temporarytherapy that can increase survival whileindividuals are waiting for a
donor heart
to become available (Mielniczuk et al.,
2004). The mechanical devices have be-come a reliable bridge until cardiac trans-plant and also have the psychological andsocial beneﬁts of enabling individuals tobe more self-sufficient, possibly evengoing home (Morales, Argenziano, & Oz,2000). These alternative devices do notrequire immunosuppression. The extentto which they may be used over the longterm is, however, still unknown.
Vocational Issues in Heart Transplant
Although after transplant many individ-
uals are able to be physically active andreturn to work, they may experience dif-ﬁculty and require assistance in makingthe transition from their pre-transplantstate to one in which they return to em-ployment. One barrier to employment isprejudicial attitudes of employers, whomay have concerns about individuals’insurability as well as their need for con-tinuing medical care and follow-up aftertransplant. The functional limitations ex-perienced by individuals after transplantare individually determined; however,there may be some restriction with heavylifting or aggressive exercise.
After a transplant, individuals’ immune
status is compromised because of the con-
tinuing use of immunosuppressants to pre-
vent rejection of the transplanted heart.As a result, they may be more prone toinfection and should avoid situations inwhich they may be exposed to contagiousinfections.
Psychological factors may also be a bar-
rier to individuals’ successful return towork. Prior to receiving the transplant,individuals may have been out of work forsome time because of their condition.Adjusting to being employed again maybe difﬁcult. Individuals may also have dif-ﬁculty with body image or may be fearfulthat work-related activity could interferewith their new heart’s effective function-ing. Fear of contracting an infection oranxiety about potential rejection of thetransplant may also be psychologicallydebilitating, interfering with the individ-ual’s ability to work.
Cardiac Rehabilitation
Cardiac rehabilitation has been de-
scribed by the U.S. Department of Health
and Human Services (1995) as a programconsisting of:
• medical evaluation• prescribed exercise• education• counseling
Cardiac rehabilitation is a comprehen-
sive and individualized program, the pur-pose of which is to reverse the limitationsthat have developed following the adverseGeneral Treatment of Cardiovascular Conditions 325

physical and psychological consequencesof cardiac events. Speciﬁc aims are to:
• curtail physical and psychological
consequences
• limit the risk of future cardiac events
from occurring
• relieve symptoms• reintegrate individuals as functional
beings in society and at work
Programs in cardiac rehabilitation use a
multidisciplinary approach and includeexercise training, dietary consultation,
smoking cessation (if needed), patient edu –
cation, and counseling (Balady, Ades,Comoss, et al., 2000). Treatment programsare designed to maximize individuals’physical and psychosocial functioning.Education and increasing awareness of theunderlying condition as well as ways toprevent future cardiac events are a corner-stone of cardiac rehabilitation programs.Since some individuals with cardiac condi-tions become disabled because of excessivefear, anxiety, or depression, interventionsdirected to helping individuals and theirfamilies deal with these feelings are alsopart of the total rehabilitation program.
PSYCHOSOCIAL ISSUES INCARDIOVASCULAR CONDITIONSPsychological Issues
The heart has been given symbolic signiﬁ-
cance for centuries. Consequently, individ-uals’ reactions to conditions involv
ing the
heart can be far-reaching. Sudden death isalso associated with cardiac malfunction.Therefore, fear and anxiety are commonreactions to cardiac conditions. Althoughmany chronic illnesses trigger these reac-tions, because the heart is considered bymany people as the most vital organ, anycondition involving the heart can havesigniﬁcant emotional ramiﬁcations.Most individuals come to accept their
condition and its associated restrictions ortreatment. In other instances, however, in-dividuals’ responses seriously affect treat-
ment and rehabilitation. Reactions of anger,
anxiety, and depression can be the mostdebilitating factors in cardiac disease.Such reactions can contribute to inactiv-ity, social isolation, or withdrawal fromthe activities that were previously enjoyed.Consequently, it is necessary to considerthe impact of emotional reactions on theability to return to a comfortable, produc-tive life.
Individuals with cardiovascular disease
may be immobilized by fear and thereforemay restrict their activities more thanthey need to. Excessive concern that addi-tional stress or exertion may lead to car-diac failure may cause individuals to alterjob, recreational, and family activitiesseverely. Depression may result from thenumerous concerns about work, familyactivities, sexual activities, and lifestylechanges. When cardiovascular conditionsrequire significant modifications inlifestyle or employment, the changesmay be associated with a sense of loss andbereavement.
Denial is part of a normal psychologi-
cal defense that can be used to cope withsevere threat. Although denial can be aneffective mechanism for reducing levels ofanxiety, it can also have a detrimentaleffect on treatment. Symptoms may beignored or trivialized, physical incapacitydenied, or recommendations for treat-ment or lifestyle change ignored. As aresult, care and treatment may be inade-quate, leading to complications or hasten-ing progression of the condition itself.Although the way in which individualsrespond to cardiovascular conditions isdependent to some extent on their per-sonality, the magnitude of the responsemay be due to their personal situation at326
CHAPTER 11 C ARDIOVASCULAR CONDITIONS

the time. Financial, work, and family con-cerns, in addition to the diagnosis of a car-diac condition and its implications, canintensify the response expressed.
Lifestyle Issues
Although not all cardiovascular condi-
tions require signiﬁcant lifestyle changes,
some changes are generally recommended.
Modiﬁcations in diet, a decrease in alco-hol intake, or elimination of tobacco usemay be required. Because smoking con-stricts blood vessels, persons with cardio-vascular disease, particularly peripheralvascular disease, should not smoke toavoid diminishing blood ﬂow to theheart or extremities further. Changes inexercise may also be recommended. Thephysician may prescribe exercise such asdaily walks as a therapeutic activity. Evenwhen recommended changes are mini-mal, individuals may perceive the changesas having a negative effect on the qualityof life; depression or anger may result.
The degree and the way in which stress
contributes to the development of cardio-vascular disease are unknown. Even ifstress itself does not directly cause patho-logic changes in the cardiovascular sys-tem, the behaviors used to cope with stressmay. Overuse of tobacco and alcohol as areaction to stress has adverse effects oncardiovascular function. Individuals whoused tobacco and alcohol as a means ofcoping with stress in the past may needto learn different coping strategies.
Stress is not always associated with over-
commitment and activity. For some indi-viduals, signiﬁcantly cutting down onactivity and involvement can be morestressful than continuing the activityitself. Therefore, helping individuals learnnew ways to cope with stress may be morebeneﬁcial than insisting that potentiallystress-producing activities be avoided. Many cardiovascular diseases require
long-term treatment with medication.Often individuals’ successful rehabilita-tion and subsequent progress depend ontheir willingness and ability to take med-ications accurately. Potential barriers toeffective treatment with medication maybe ﬁnancial, attitudinal, or logistical. Ap-propriate strategies must be developed tomaximize an individual’s ability to com-ply with treatment as prescribed.
After diagnosis of a cardiac condition,
sexual activity may be a special source ofanxiety for individuals, as well as for their
partners. In most instances, sexual activi-
ty can be resumed; however, associated fear
and anxiety can hamper both enjoymentand performance, altering self-esteem andcontributing to depression. Often, lack ofinformation contributes to fear and mis-perceptions. The physician should discussspeciﬁc recommendations regarding theresumption of sexual activity, as well asany restrictions or modiﬁcations.
Social Issues
To some degree, the reactions of those
in the environment inﬂuence the successwith which individuals cope with anychronic condition. The quality of individ-uals’ interpersonal relationships at thetime of the cardiac event and the presenceor absence of social supports can be majordeterminants of individuals’ reaction totheir condition.
Cardiovascular conditions can produce
profound effects on family dynamics.Depending on the condition and extentof disability, there may be a shifting offamily roles and role reversal. Because ofthe invisible nature of many cardiac con-ditions, some family members may notunderstand that individuals may not beable to sustain the activity level they en-gaged in prior to their diagnosis. Lack ofPsychosocial Issues in Cardiovascular Conditions 327

understanding may breed resentment oranger. Family members may be a source ofsupport and consolation. They may also,however, contribute to individuals’ fearand anxiety by being overly protective orshowing anxiety out of proportion to themedical condition. Qualiﬁed professionalsshould discuss such reactions and theirpotential impact on individuals’ return tofunction with family members.
Although those with cardiovascular dis-
ease may continue most forms of recrea-tion, extremely rigorous activities mayneed to be curtailed. When recreationalactivities that were once a major socialoutlet must be restricted, social isolationand depression may result unless anoth-er recreational activity can be substituted.
Cardiac conditions often have few vis-
ible signs of disability. Although this mayseem advantageous at ﬁrst glance, the lackof visible cues may contribute to a misun-derstanding of the activity restrictions thatare part of the treatment protocol. As aresult, individuals with such conditionsmay be pressured into participating inactivities that are more strenuous thanthose at the prescribed level of activity.The absence of outward signs of disabili-ty may also foster denial of the conditionand subsequent noncompliance with themedical treatment plan.
VOCATIONAL ISSUES INCARDIOVASCULAR CONDITIONS
For most individuals, work is a source of
pride as well as a ﬁnancial necessity. Thedegree to which the cardiovascular condi-tion inhibits the return to regular em-ployment can influence individuals’reactions to the condition. In some in-stances, attitudes of employers are barri-ers to the successful return to work.Employers may be reluctant to employ orreemploy individuals with cardiovasculardisease because of fear of liability or of theresponsibility for medical costs if the con-dition should worsen.
Each job must be viewed in relation to
the individual’s physical and emotionalabilities and the effect that the job has onthe individual’s health status. In someinstances, a job change may be necessary,which can be an additional source ofstress. In other instances, individuals canreturn to their former job with little or nomodiﬁcation.
Physicians generally prescribe the de-
gree and type of activity in which individ-uals with cardiovascular disease maysafely engage. Most individuals with heartconditions are able to engage in light tomoderate activity.
Because of the effect on the cardiovascu-
lar system, environmental conditions such
as excessive heat or cold should be avoid-ed. Isometric exercise elevates blood pres-sure and places an extra burden on theheart; consequently, any exertion that in-volves muscular activity against a ﬁxed,unmoving resistance is usually to beavoided. Individuals with pacemakersshould be aware that certain types ofequipment may interfere with pacemak-er function.
In most instances, once cardiovascular
conditions are stabilized and appropriatetreatment is instituted, functional declineis slow or minimal. The greatest barrier toproductive vocational activity may be theindividual’s unwillingness or inability tomake recommended lifestyle changes orhis or her noncompliance with the med-ical treatment prescribed.
CASE STUDIESCase I
Mr. C., a 50-year-old high school teach-
er, has been employed in his current posi-328 CHAPTER 11 C ARDIOVASCULAR CONDITIONS

tion since graduating from college. Heteaches history and physical education.He had a myocardial infarction at the ageof 40; however, he went through intensivecardiac rehabilitation and has returned tomost of his former functional capacity.Over the past year, however, he has hadincreasing fatigue and shortness of breath.Upon evaluation by his cardiologist, Mr.C. was found to have beginning stages ofheart failure. Although his cardiologist iscurrently managing his symptoms withmedication, he has told Mr. C. that if hiscardiac condition continues to deterioratewithin the next 5 years, he may need tohave a heart transplant. Mr. C. is marriedand has three stepchildren.
Questions
1. How would you approach Mr. C.
about his rehabilitation potential?
2. Is there additional medical informa-
tion that would be helpful in estab-lishing Mr. C.’s rehabilitationpotential?
3. What other factors should be consid-
ered in determining his rehabilitationpotential?
4. Is it feasible for Mr. C. to continue in
his current job? 5. If Mr. C. would continue in his cur-
rent job, what modiﬁcations mightbe considered?
6. What would Mr. C.’s rehabilitation
potential be if he had a cardiac trans-plant?
Case II
Ms. B., age 55, had rheumatic fever as
a child and as a result experienced dam-age to the mitral valve. She is unmarriedand lives alone. She has supported herselfby cleaning professional ofﬁces since shegraduated from high school. Over the pastyear she has noticed increasing fatigueand dyspnea. When evaluated by herphysician, she was found to need valvereplacement.
Questions
1. What types of information would
you ﬁnd useful in helping Ms. B.develop a rehabilitation plan?
2. How will Ms. B.’s ability to perform
in her current employment be affect-ed by her surgery?
3. What factors should you consider
when helping Ms. B. develop a reha-bilitation plan?References 329
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House-Fancher, M. A., & Foell, H. Y. (2004). Heart
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Journal of Medicine, 348 (24), 2377–2378.330 CHAPTER 11 C ARDIOVASCULAR CONDITIONS

NORMAL STRUCTURE ANDFUNCTION OF THE RESPIRATORYSYSTEM
The respiratory system consists of air
passages, pulmonary vessels, and thelungs, as well as the muscles involved inbreathing. The respiratory system suppliesoxygen to the blood to be distributed to
body tissues, and it also removes carbon
dioxide , a waste product of tissue metabo-
lism, from the body. Abnormal function-ing of the respiratory system affects everysystem of the body. Diminished oxygen orexcess carbon dioxide can also result inloss of consciousness and death.
Breathing is an involuntary activity and
under the control of the respiratory cen-ter in the brain. Changes in the levels ofcarbon dioxide and oxygen in the bloodbring about automatic changes in the rateand depth of breathing. As the concentra-tion of carbon dioxide in the bloodincreases, the breathing rate increases tohasten elimination of the waste product.
Inspiration refers to the act of breath-
ing in, and ventilation refers to the actu-
al movement of gases (oxygen and carbondioxide) into and out of the lungs. Air ﬁrstenters the respiratory system through thenose during inspiration. Air entering thenostrils comes in contact with a mucous
membrane that warms and moistens it.
Tiny hairs within the nostrils trap dustparticles and organisms before they reachthe pharynx (throat), which serves as a
passageway for both air and food. At thebottom of the pharynx are two openings,one into the esophagus for the passage of
food and the other into the larynx (voice
box) for the passage of air. The larynx con-tains the vocal cords , necessary for speech.
A ﬂap called the epiglottis , located on top
of the larynx, closes over the larynx whenfood is ingested to prevent food fromentering the respiratory system. As air istaken in, it passes through the larynx intothe main airway to the lungs, the trachea
(windpipe). The trachea is a cartilaginoustube lined with special hairlike projectionscalled cilia. These cilia are part of the
body’s defense against foreign objects,such as bacteria, or other particles thathave not been ﬁltered out by the upperpart of the respiratory system. With arhythmic motion, cilia project mucus orother particles up toward the pharynxwhere they can be expectorated. Afterentering the chest cavity, the tracheadivides into two branches, called the rightand left bronchi . Each bronchus, which
also contains cilia, enters a lung and con-Conditions of the Respiratory
(Pulmonary) SystemCHAPTER 12
331

tinues to branch into smaller segmentscalled bronchioles . The bronchioles termi-
nate in tiny sacs called alveolar sacs . With-
in the alveolar sacs are small pockets ofballoonlike structures called alveoli , which
make up most of the lung’s substance. Itis within the alveoli that the exchange ofoxygen and carbon dioxide takes placethrough tiny blood vessels called capillar-
ies. External respiration is the process of
exchanging oxygen and carbon dioxide inthe lungs. The exchange of oxygen andcarbon dioxide at the tissue level is calledinternal respiration . Oxygen diffuses
through the alveolar walls into capillariesso it can be distributed by the blood to tis-sue cells throughout the body. Body cellsrelease carbon dioxide into the blood,where it is then carried to the alveoli.Capillaries in the alveoli release carbondioxide from the blood; it then diffusesacross the alveolar wall and is expelledfrom the lungs through expiration (the
process of expelling air from the lungs).
The lungs are two spongelike structures
contained within the thoracic cavity (chest
cavity) (Figure 12–1). The thoracic cavityis lined by a thin membrane called thepleura , which secretes a thin layer of ﬂu-
id to help minimize friction as the lungsexpand and contract against the chest wallduring respiration. The thoracic cavity issurrounded by ribs. Muscles around theribs ( intercostal muscles ) expand when air
is inhaled and contract when air is ex-haled. The thoracic cavity is separatedfrom the abdominal cavity by the mainrespiratory muscle called the diaphragm .
The left lung contains two lobes, the
upper and lower lobes . The right lung con-
tains three lobes, the upper , middle , and
lower lobes . The heart is located between332
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM
Alveolar sacAlveoli
PleuraThoracic
CavityPharnyxLarnyxTrachea
DiaphragmBronchiBronchiolesEpiglottis
Figure 12–1 Respiratory System.

Conditions of the Respiratory System 333
the two lungs. Pulmonary vessels (the pul-monary artery and pulmonary vein) car-ry blood to and from the lungs from theheart. The pulmonary artery carries deoxy-
genated blood from the heart to the lungs,
and the pulmonary vein carries oxygenat-
ed blood to the heart from the lungs.
After delivering oxygen to the body tis-
sues, blood returns to the heart from thegeneral circulation; at this point, it carriesan excess of carbon dioxide. The blood ispumped from the right ventricle of theheart into the lungs. Here, the thin wallsof the alveoli come in contact with thethin walls of tiny blood vessels called cap-illaries. Carbon dioxide passes from thecapillaries across the alveolar wall to beexpelled through expiration of the lungs.In turn, the oxygen that has been takeninto the alveoli through inspiration pass-es across the alveolar wall into the capil-laries. The red blood cells take up oxygen,and the blood returns to the heart, whereit is pumped into the general circulationto supply body tissues with oxygen.
Air is able to move in and out of the
lungs because of pressure changes thatoccur because of contraction and relaxa-tion of the diaphragm and other breath-ing muscles. The pressure within the lungsand the thorax must be less than the pres-sure in the atmosphere for inspiration tooccur. As air is taken into the lungs, thediaphragm contracts and moves down-ward, increasing the size of the thoraciccavity and lowering the pressure within it.The lungs expand and atmospheric air,which is at higher pressure, ﬂows into thelungs, bringing oxygen as inhalationoccurs. The diaphragm then moves in theopposite direction, relaxing and movingupward and causing the thoracic cavity tobecome smaller, thus increasing intratho-racic pressure. As the lungs are squeezed,air is forced out and carbon dioxide isexhaled. CONDITIONS OF THERESPIRATORY SYSTEMInfections of the Respiratory System
Upper Airway Infections
(Pharyngitis, Laryngitis)
The upper respiratory tract consists of
the nose, the pharynx, and the larynx.Pharyngitis (sore throat) is a condition of
the upper airway that may be caused fromviruses or bacteria. The mucous mem-brane lining the throat becomes inﬂamedand may cause symptoms such as sorethroat, fever, or difﬁculty in swallowing.Laryngitis (inﬂammation of the larynx) is
usually caused by a virus and can producehoarseness, loss of voice, cough, and sorethroat. Both pharyngitis and laryngitis arerelatively minor and self-limiting.
Pneumonia
Pneumonia is an acute illness caused by
inﬂammation and infection that affect thebronchioles and alveolar tissue in thelung. It is characterized by cough, chestpain, fever, and breathlessness. Althoughnot in and of itself a disability, it can bea major and often life-threatening condi-tion when it is superimposed on otherchronic illnesses and disabilities. Individ-uals with conditions such as heart disease,alcoholism, neuromuscular disease (suchas multiple sclerosis), chronic obstructivelung disease, spinal cord injury (especial-ly quadriplegia), dementia, altered im-mune status, or swallowing abnormalitiesare at particularly high risk for developingpneumonia.
The term pneumonia is usually further
qualiﬁed to describe the cause or location.For example, lobar pneumonia refers to
pneumonia that affects one lobe of thelung, and bronchopneumonia refers to
patchy and diffuse inﬂammation and in-

fection of one or both lungs. Pneumoniacan be caused by a number of organisms,including viruses, bacteria, fungi, yeasts,or others. Pneumonia caused by a viruswould be called viral pneumonia , whereas
pneumonia caused by a bacterium wouldbe called bacterial pneumonia .
Usually the defenses of the respiratory
system against infection are sufﬁcient toward off infection. However, when thebody is weakened or when the causativeagent is overwhelming, defenses in therespiratory system cannot withstand theorganism, and pneumonia develops.Individuals with chronic illness or disabil-ity, and particularly those who have lim-ited mobility or prolonged inactivitybecause of bedrest, have an increased sus-ceptibility for developing pneumonia.Pneumonia caused by inactivity or immo-bility so that the lungs do not expand suf-ﬁciently is known as hypostatic pneumonia .
Another type of pneumonia can result inindividuals who have difﬁculty swallow-ing or are unconscious. In these situationsthe epiglottis does not close adequately, sothat food, liquid, or other substances canenter the lungs. The consequent accumu-lation of foreign material in the lung con-tributes to the development of aspiration
pneumonia . Those especially vulnerable to
aspiration pneumonia are individualswith altered mental status, neuromuscu-lar disorders, or abnormalities of theesophagus. Aspiration of toxic materialssuch as oils, bile, gastric acid, or alcoholcauses additional complications becauseof direct damage to the alveolar mem-brane, called chemical pneumonitis .
Inﬂammation and infection of the alve-
oli interfere with oxygen and carbon diox-ide exchange in the lungs. The greater theextent of inﬂammation and infection, thegreater the interference with respiration.Infection triggers changes in the capillarywalls in the alveoli that cause ﬂuid to ﬂowinto the alveoli and accumulate. The accu-mulation of ﬂuid serves as an excellentgrowth medium for organisms, so that theinfection becomes worse. Accumulation ofﬂuid in the lungs further interferes with
the exchange of carbon dioxide and oxygen .
When the infection becomes widespread,there is danger of the organism invadingthe bloodstream, a potentially life-threat-ening complication of pneumonia.
Diagnostic Testing for Pneumonia
Diagnosis of pneumonia is usually
determined by symptoms as well as chestX-ray, which help to identify the locationand degree of lung involvement. Culturesof sputum specimens may be done toidentify the organism responsible for theinfection so that appropriate medicationcan be prescribed. In some instances, if thesputum culture is inconclusive or if theindividual’s condition deteriorates rapid-ly, bronchoscopy (insertion of a special
tube through the mouth extending intothe trachea to examine the bronchi) maybe indicated.
Treatment and Prevention of Pneumonia
Once the cause of pneumonia is identi-
ﬁed, treatment with medication is directed
toward the speciﬁc organism. Medicationsthat facilitate removal of secretions fromthe lungs ( expectorants ) may also be ad-
ministered. Because individuals with
pneumonia may have lowered oxygen con-
tent in the blood ( hypoxia ) due to poor
gas exchange, oxygen may be adminis-tered. Manual chest physiotherapy mayalso be conducted to facilitate drainagefrom the lungs.
Individuals at high risk for developing
pneumonia should be especially vigilantto prevent an infection that might lead topneumonia. Good nutrition, adequate hy-334
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

dration, and adequate sleep help to bol-ster immunity. Individuals should attemptto minimize situations in which they areexposed to infection, especially duringcold and ﬂu season. Exposure to otherswith a cold or ﬂu should be avoided. Theposition of individuals with limited mobil-ity should be changed frequently to facil-itate lung expansion and drainage. Flu andpneumococcal vaccinations may also beimportant to prevent pneumonia fromoccurring. Respiratory irritants such assecondhand cigarette smoke or other pol-lutants can make individuals more sus-ceptible to infection and consequentlyshould be avoided.
Vocational Issues in Pneumonia
Pneumonia can cause signiﬁcant mor-
bidity and consequent loss of workdays,not to mention a threat to life itself.Because individuals with chronic illness ordisability often have increased susceptibil-ity to pneumonia, the steps mentionedabove to minimize the development ofpulmonary complications are especiallyimportant. The presence in the workplaceof conditions that can induce or aggravatelung conditions may require job modiﬁ-cation or complete avoidance or exposureto the risk. Although the risk of exposuremay be present in a number of work sit-uations, speciﬁc exposure to respiratoryirritants or to large numbers of peoplewho may have respiratory symptomsshould be avoided as much as possible.
Tuberculosis (TB)
Tuberculosis (TB) is an infectious and
potentially fatal disease that is caused byan organism called the tubercle bacillus .
Although it occurs most frequently in thelungs, TB can occur in almost any part ofthe body. TB of the lung is called pul-monary tuberculosis . Infection occurs pri-
marily through inhala
tion of infectious
droplets that an infected individual has
released through coughing. As a result ofthe infection, nodules form in the lung.
Exposure to the tubercle bacillus may or
may not lead to infection or to active dis-ease. Whether infection or active TBdevelops depends on individuals’ generalphysical condition and the intensity ofthe exposure. Ordinarily, TB is not con-tracted from brief exposure to a personwith TB. Many factors predispose individ-uals to develop TB. Lowered body resist-ance due to inadequate rest and poornutrition may be one predisposing factor.Persons with other disabling conditions,such as diabetes, alcoholism, HIV infec-tion, and conditions that affect the lungs(e.g., silicosis), are more vulnerable todevelop TB if they should come in contactwith the infectious agent (Poss, 2000).
Individuals infected with TB for the ﬁrst
time are said to have a primary infection.Primary infections may or may not be-
come active. The infection may be dormant
for years until physical resistance is low-ered. The most common form of TB isreinfection, or secondary TB. Individualswith active pulmonary TB may have fewsymptoms until nodules in the lung arelarge enough to be seen on X-ray. Initialsymptoms may be weight loss, anorexia(loss of appetite), and a slight elevation oftemperature. Symptoms may then pro-gress to cough, overproduction of sputum,and hemoptysis (blood-streaked sputum).
Diagnosis of Tuberculosis
Infection with the tubercle bacillus is di-
agnosed through cultures of sputum , chest
X-rays , and tuberculin skin tests . Skin tests
can be a valuable screening tool to deter-mine if the individual has been infectedwith the tubercle bacillus. After beingConditions of the Respiratory System 335

infected by the tubercle bacilli, the bodydevelops an allergic response over time,resulting in tissue sensitivity. This sensi-tivity can be identiﬁed through the tuber-
culin skin test. A skin test consists of an in-
jection of a small amount of ﬁltrate fromdead tubercle bacilli under the skin. If anindividual has been exposed to and infect-ed by the tubercle bacillus, there will be a
local skin reaction at the injection site. Skin
tests are interpreted for reaction at 24
hours and at 48 to 72 hours after injection.
A positive reaction to a skin test indi-
cates that the individual has been exposedto and infected with the tubercle bacillus,but it does not indicate whether the dis-ease is active. Individuals who have a pos-itive skin test but have no symptoms andno evidence of active disease on X-ray orsputum specimens do not have active di-sease, and they are not contagious to oth-ers. It is now recommended that individ-uals with positive skin tests be treatedeven though they have no active signs ofthe disease to prevent the possibility of thedisease becoming active at a later date,when their resistance may be diminishedfrom other causes, such as chronic diseaseor aging (Horsburgh, 2004).
To determine whether the individual
with a positive skin test has active TB,physicians obtain chest X-rays and sputum
specimens from the individual. Identiﬁca-
tion of nodular changes in the lung and/or the ﬁnding of the tubercle bacillus inthe sputum of other body secretions con-ﬁrms the diagnosis of active disease.Individuals with active TB are infectiousand theoretically would be able to trans-mit the disease to others.
Treatment of Tuberculosis
Individuals with active TB should under-
go prompt treatment, not only for theirown well-being but also for the protectionof others. Treatment consists of takingmedication for 6 to 24 months. The aver-age length of treatment with medicationis from 9 to 12 months. Usually, after 2 to4 weeks of intensive treatment with med-ication, individuals are no longer a pub-lic health threat and can return to normalactivities. It is of the utmost importancethat individuals being treated for TB takethe prescribed medication accurately andconsistently if treatment is to be effective.Because individuals have not always beencompliant with the drug regimen pre-scribed, there are more cases in which thetubercle bacillus has become resistant tothe regular medications, which had oncebeen effective. Drug-resistant TB is becom-ing an increasing problem.
TB that occurs outside the lungs is called
extrapulmonary tuberculosis . Possible disease
sites include the lining of the brain andspinal cord, the kidney, the bones, or theabdomen. TB that is widespread through-out the body is called miliary tuberculosis .
Treatment of extrapulmonary TB is simi-lar to that of pulmonary TB, but it maycontinue for a longer period.
Psychosocial Issues in Tuberculosis
Although anyone of any social class or
educational level can be infected by thetubercle bacillus, development of TB
is still
often associated with crowded conditions
and poverty, alcoholism, sub stance abuse,
and homelessness (Campion, 1999). In
many cultures, the social stigma of TB may
contribute to individuals’ denial that they
have the condition and abandonment oftreatment. Individuals with TB who re-
member the social stigma once associated
with the condition may feel ashamed andembarrassed. They may try to hide theirdiagnosis from others, ignore the physi-cian’s recommendations, or discontinuetreatment. Such reactions have serious336
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

consequences for both individuals with TBand those in close contact with them.
Vocational Issues in Tuberculosis
When tubercle bacilli are no longer
present in the sputum after beginningtreatment, individuals are no longer con-sidered infectious to others and are ableto return to work (usually within 2 to 4weeks). Once individuals are treated, pro-viding there have been no associated com-plications, the ability to return to work orto perform tasks performed previouslyshould not be affected. Because of thestigma attached to the condition and themisinformation that employers or cowork-ers may have about it, however, one of themajor barriers to employment may bethe attitudes of employers and fellowemployees.
Chronic Lung Diseases
Asthma
Asthma is a chronic inflammatory
hyperresponsiveness of the airways thatleads to reversible airway obstruction(National Asthma Education and Preven-tion Program, 1997). It is characterized byepisodic attacks of wheezing, dyspnea
(difﬁculty breathing), chest tightness, andcough triggered by a variety of stimuli.Factors such as exercise, emotional stress,inhalation of cold air, or exposure to res-piratory irritants such as fumes from paintor gasoline, cigarette smoke, or perfumesmay precipitate an asthma attack. Somepeople have attacks triggered by foodpreservatives or to substances found insome medications, such as aspirin. Mosttriggers initiate an allergic response thatcauses the immune system to initiate aninﬂammatory response, causing airways toswell and secrete excess mucus that clogsthe passages. At the same time, the mus-cles that control air passages constrict andgo into spasm, causing the airways to nar-row. As a result, individuals have difﬁcul-ty breathing and the lungs work lessefﬁciently.
The severity of symptoms and the fre-
quency of asthma attacks vary with indi-viduals. Some individuals may have aslight cough and shortness of breath dur-ing an attack, whereas others may be sorestricted by cough and shortness ofbreath that they are unable to speak morethan a few words at a time. Chronic bron-
chitis or emphysema may coexist with asth-
ma, especially in older adults.
Asthma may be classiﬁed as mild, mod-
erate, or severe . Individuals with mild asth-
mamay have intermittent brief symptoms
several times a month but have no symp-toms in between attacks, so that medica-tion is required only during an attack.Individuals with moderate asthma may
have an attack several times a week andrequire medication almost daily. Individ-uals are classiﬁed as having severe asthma
when symptoms are almost continuousand physical activities are limited becauseof the symptoms. Severe asthma requiresdaily medication and may be accompa-nied by frequent hospitalizations andpotentially life-threatening exacerbations.
Atelectasis (collapse of the lung) is a
possible complication of asthma. Anotheris a severe, prolonged attack ( status asth-
maticus ). This condition is a severe exac-
erbation of asthma that is unresponsive totreatment methods, and it can be fatal.The condition requires emergency medicalintervention.
Treatment of Asthma
Treatment of asthma is directed toward
the identiﬁcation and avoidance of precip-itating factors, the symptomatic relief ofConditions of the Respiratory System 337

attacks, and the prevention of futureattacks. If asthma is due to allergy, indi-viduals should attempt to rid the environ-ment as much as possible of the substancecausing the allergic response ( allergen ).
Common allergens are dust mites, mold,pollen, and animal dander. When individ-uals know what triggers a response,whether allergic or not, precipitating fac-tors should be eliminated as much as pos-sible. Common precipitating factors thatcan aggravate asthma and possibly bringon an attack are irritants such as dust,smoke, exhaust fumes, chemicals, or per-fumes. Some food preservatives, such assulﬁtes, can also bring on an attack. Insome instances, attacks may be broughton by stress or fatigue.
Several medications are commonly used
to treat asthma. Individuals may takemedications daily or may use medicationsfor relief of symptoms during an asthmaattack. Corticosteroids are substances pro-
duced naturally in the body by the adre-nal glands that perform a number of vitalfunctions, such as regulating metabolism,maintaining proper water balance, andﬁghting against inﬂammation. Cortico-steroids prescribed by a physician for treat-ment of asthma are anti-inﬂammatorymedications that reduce the inﬂammationand swelling of the lining of the bronchialtubes. They are usually taken on a regularbasis to prevent asthma attacks. They maybe inhaled or taken orally or by injection.Cromolyn sodium may be prescribed as analternative to inhaled steroids or to helpreduce the amount of steroid needed. Ithelps to prevent asthma attacks by block-ing the release of substances that narrowairways during an asthmatic reaction andmay be especially helpful when taken pri-or to exercises or prior to exposure to trig-gering factors.
Medications called bronchodilators ,
which dilate the narrow and constrictedbronchioles, are commonly used in thetreatment of asthma attacks. These med-ications dilate the bronchioles by relaxingthe muscles of their walls, thus creating alarger opening for the passage of air. Theymay be used to relieve an acute asthmaattack. Bronchodilators may be used oral-ly or as inhalers or nebulizers . A nebulizer
is a device that converts liquid medicationinto tiny droplets that individuals theninhale. Nebulizers and inhalers delivermedication directly to the lungs so thatthe medication begins to act immediate-ly. Individuals with asthma may use spe-cial instruments such as metered-dose or
aerosol inhalers . When inhalers are used
correctly, medication is delivered directlyinto the lungs for quick relief of symp-toms. Metered-dose inhalers dispenseaerosol medication in measured doses, butto receive the full effect, individuals mustlearn and remember to use the propertechnique with the inhalers. Spacer devices
may also be used. A spacer device is a tubeor bag that is attached to the metered-doseinhaler at one end and that has a mouth-piece for the individual to inhale throughat the other end. The spacer acts as a hold-ing chamber, slowing down the medica-tion so the individual can inhale it moreefﬁciently. A peak ﬂow meter is a device
that determines changes in the size of theindividual’s airways. Individuals are ableto measure and record their peak ﬂow rateto help determine the severity of theirasthma and how well they are respondingto treatment.
Individuals with asthma require period-
ic medical assessment to ensure that thegoals of treatment have been achieved andto monitor their condition. The severityof the condition and the appropriate ther-apy vary widely among individuals, and
their condition can change over time, with
new allergies developing or the severity ofthe asthma increasing or decreasing. One338
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

of the most important aspects of the treat-ment of asthma is patient education andmonitoring (Naureckas & Solway, 2001).Individuals should be aware of how to rec-ognize and manage exacerbations of thecondition as well as to identify and if pos-sible avoid environmental triggers. Asth-ma self-management education should betailored to the needs of each individual
with sensitivity to cultural beliefs and prac-
tices. In addition, an annual inﬂuenza vac-cination may be indicated for individualswith persistent asthma.
Psychosocial Issues in Asthma
Effective management of asthma re-
quires adherence to the medical recommen-
dations to control attacks. However, psy-chological or behavioral responses mayprevent or limit individuals’ ability tocomply with recommendations. Individ-uals may experience a variety of obstaclesthat impede their ability to adhere to med-ical interventions. Resistance to the treat-ment plan may be one of the greatestbarriers to successful asthma control.Asthma, unlike many other chronic con-ditions, has associated symptoms that areusually reversible when treated with medi-cations. Consequently, with proper treat-ment, the condition should only mini-mally affect daily living. Unfortunately,individuals may feel that adherence to thetreatment necessary to control the symp-toms draws attention to them. Conse-quently, they may feel stigmatized andavoid implementing treatment. Individ-uals with asthma may view daily medica-tions as a negative, constant reminder oftheir condition. Social opportunities mayalso be affected by asthma if, dependingon the factors that appear to precipitate anattack, there are limitations on certainphysical activities or on exposure to cer-tain environmental factors because theytrigger attacks. Fear and panic may beexperienced during acute asthma attacks.
Individuals who have had asthma since
childhood may be especially vulnerable tosocial adjustment problems because of thestressors experienced from hospitaliza-
tions and restrictions, which may have im-
peded their social development. Obstaclessuch as lack of medical insurance cover-age may encourage episodic care and inad-equate follow-up and monitoring. Out-of-pocket expenditures for health care andprescription costs may impose an unduehardship on individuals and their family,making it difﬁcult to obtain necessarymedication and medical care. Transpor-tation obstacles that prevent individualsfrom gaining access to follow-up healthcare may also cause difﬁculty.
In other instances, individuals may not
understand the importance of certain rec-ommendations, such as taking medicationdaily even when they are not having anasthma attack, reducing exposure to aller-gens, or modifying the home or workenvironment to reduce irritants that cantrigger attacks.
Individuals with asthma may experi-
ence feelings of anger and low self-esteem, which in turn make it difﬁcult toaccept their condition. They may have dif-ﬁculty coming to terms with the limita-tion of their condition and may have asense of loss of control over themselvesand their life.
Vocational Issues in Asthma
Asthma is a common cause of morbid-
ity and mortality in the United States(Marik, Varon, & Fromm (2002) and amajor cause of missed school or missedwork (Li, 2001). It imposes some limita-tions on individuals regardless of the lev-el of severity. Since exposure to irritantsand allergens can increase asthma exacer-Conditions of the Respiratory System 339

bations in individuals who are sensitive tothese factors, environmental pollutants orallergens to which the individual is par-ticularly sensitive should be avoided. En-vironmental factors that can contribute toasthma include chemicals such as clean-ing solutions, craft supplies, industrial andvehicle emissions, tobacco smoke or woodstove emissions, pollens, and animal dan-der. Exertion should be avoided in situa-tions where air pollution is high. Indi-viduals with asthma should also avoidexposure to individuals with respiratoryinfections.
Chronic Obstructive Pulmonary Disease
(COPD; Chronic Bronchitis and
Emphysema)
The term chronic obstructive pulmonary
disease (COPD ) refers to a collection of dis-
eases with the primary characteristic oflimited expiratory outﬂow. It leads to sub-stantial disability and death (Mannino,Homa, Akinbami, Ford, & Redd, 2002).Cigarette smoking is the major risk factorfor developing COPD (Barnes, 2004; Petty,2001); however, not everyone who hasCOPD has a history of smoking (Rennard,2004). Environmental pollutants, occupa-tional chemicals, passive smoke, and agenetic predisposition to COPD are alsorisk factors (Barnes, 2000; Hogg et al.,2004). Although there is no universallyaccepted definition of COPD (Snider,2003), all instances of COPD involve air-ﬂow limitation that is not fully reversible,that is progressive, and that is associatedwith exposure to noxious particles or gas-es (Pauwels, Buist, Calverley, Jenkins, &Hurd, 2001). Included in this deﬁnitionare chronic bronchitis and emphysema .
Although chronic bronchitis and emphy-
sema are two distinct conditions, they fre-
quently coexist and share similar symptoms,
including dyspnea (shortness of breath),especially on exertion, intermittent cough,and fatigue. This combination of symp-toms is a major contributor to disability,with dyspnea in particular signiﬁcantly al-tering quality of life (Luce & Luce, 2001).Fatigue is a common symptom in most in-dividuals with COPD. The dyspnea ofCOPD can affect individuals’ ability to ex-
ercise or perform tasks of daily living. Those
with early COPD may experience dyspneaafter walking for short distances, andthose in later stages of the condition mayexperience signiﬁcant dyspnea with min-imal activity such as brushing their teeth.
Chronic bronchitis is clinically deﬁned as
a chronic productive cough on most days
for a minimum of 3 months in the year for
not less than 2 consecutive years (Goldman
& Bennett, 2000). Symptoms consist of apersistent cough, often in the early morn-ing, accompanied by an excessive volumeof mucus and expectoration. The lining ofthe air passages becomes irritated, swollen,and clogged with mucus. Mucus obstructsairﬂow in and out of the alveoli (the small
air sacs in the lung where the air exchangetakes place). Sometimes the small musclesaround the air passages tighten. This iscalled bronchospasm and makes breathing
even more difﬁcult. Chronic bronchitisoften leads to emphysema . Although bron-
chitis often predisposes individuals todevelop emphysema, emphysema canalso result from other conditions of thelung, such as occupational lung diseases and
cystic ﬁbrosis .
Emphysema is deﬁned as a permanent
enlargement of the alveoli because of theoverinﬂation of and destructive changesin the alveolar walls (Kerstjens, 1999). Asa result, the alveoli have less surface avail-able for the exchange of oxygen and car-bon dioxide, and the bronchioles close be-fore exhalation is complete. As more andmore alveoli are affected, the lungs losesome of their natural ability to stretch and340
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

relax, thus diminishing the efﬁciency ofexpiration. Airways become obstructed,and stale air, high in carbon dioxide andlow in oxygen, is trapped in the alveoli.The lungs become overinﬂated because allthe air cannot be expelled.
The most important risk factor for the
development of COPD is cigarette smok-ing, even though COPD may develop inthose who have had chronic asthma foryears and do not smoke. Smoking and airpollution in combination may facilitatethe development of COPD. The course ofCOPD varies. In most cases, the conditiondevelops slowly with respiratory functionremaining relatively stable for years. Inother cases, however, respiratory functiondeteriorates rapidly. Many people haveCOPD for years before it is diagnosed.Individuals usually seek medical advicewhen they note shortness of breath withexercise or at rest. At this point, more than50 percent of their lung function mayalready have been lost. Because of airwayobstruction, hypoxemia (decreased oxy-
gen in the blood) may occur. As COPDbecomes more advanced and hypoxemiaincreases, the oxygen supply to the brainmay be inadequate, resulting in impairedjudgment, confusion, or motor incoor-dination. A buildup of carbon dioxide(hypercapnia ) may also occur because of
inadequate gas exchange in the lungs, re-sulting in drowsiness or apathy. To coun-teract the low concentration of oxygen,the body’s production of red blood cellsincreases, resulting in a condition calledpolycythemia . The increased number of
red cells in the blood increases blood vis-cosity, which, in turn, can interfere withblood ﬂow. When polycythemia is severe,periodic phlebotomies (removal of blood)
may be performed to reduce the numberof red blood cells, thus decreasing the vis-cosity of the blood. As COPD advances,individuals may have increased difﬁcultyexpectorating secretions; increased short-ness of breath, especially upon exertion;and increased vulnerability to respiratoryinfections. For individuals with COPD,respiratory infections can be life-threaten-ing, because they further compromise analready diminished gas exchange.
Failure of the right ventricle of the heart
(cor pulmonale ) may develop as a compli-
cation of COPD. As a result of the obstruc-tion of airﬂow in the lungs and thesubsequent breakdown of the alveolarwalls, many capillaries in the lungs aredestroyed. The surrounding capillariesbecome constricted to compensate for thelower concentrations of oxygen. Constric-tion of the capillaries channels addition-al blood ﬂow to areas of the lungs that arebetter oxygenated; however, it also createsa resistance to the blood being pumpedinto the lungs by the right ventricle of theheart. Therefore, the right ventricle mustpump against resistance and becomeshypertrophied (enlarged), losing the abil-
ity to pump effectively (see Chapter 11).Because of the inefﬁcient pumping actionof the enlarged right ventricle, blood re-turning to the right side of the heart fromthe general circulation begins to back up,causing edema (swelling) in other parts of
the body. Organs of the digestive systemmay become engorged with ﬂuid, causingnausea and vomiting. There may also beedema of the lower extremities, predispos-ing individuals to skin ulcerations.
Diagnosis of COPD
Despite the disabling effects of COPD,
it is still grossly underdiagnosed in manyindividuals (Mannino et al., 2000). Al-though a history of smoking, smoker’scough, and excess mucus secretion may beindicative of COPD, spirometric measure-ments are required to diagnosis COPD anddocument the degree of loss of lung func-Conditions of the Respiratory System 341

tion (Ferguson et al., 2000). In an effort toincrease awareness of COPD and to devel-op consensus for the diagnosis of COPD,the Global Initiative for Chronic Obstruc-tive Lung Disease has introduced a ﬁve-stage classiﬁcation for the severity ofCOPD based on measurements of airﬂowlimitation during forced expiration asmeasured through spirometry (Hurd &Pauwels, 2002). Spirometry is discussedlater in the chapter.
Treatment of COPD
COPD is irreversible and incurable. The
major goals of treatment include smokingcessation, symptom relief, improvementin functional capacity, and limitation ofcomplications (Sutherland & Cherniack,2004). Individuals with COPD may bereferred to a pulmonologist (physician
who specializes in evaluation and treat-ment of lung conditions) for evaluationand treatment. Pulmonary rehabilitation is
an important nonpharmacologic treat-ment of COPD and is directed toward in-creasing the ability to compensate for andlive with disease, rather than attemptingto cure it (Rochester, 2000). Pulmonaryrehabilitation consists of a structuredprogram of education, exercise condition-ing, energy conservation, and physiother-apy, as well as psychosocial and vocationalcounseling. The goals are to providesymptomatic relief, decrease disability,and enhance lifestyle. For individuals whosmoke, the most important interventionto alter the clinical course of the conditionis smoking cessation (American ThoracicSociety, 1995; Petty, 1999).
A variety of medications may be used in
the treatment of COPD. Bronchodilatorsthat help to reduce hyperinﬂation andthus dyspnea are a mainstay of treatmentfor COPD (Barnes, 2000). Most individu-als with COPD have chronic inhaled bron-chodilator therapy prescribed. Some in-dividuals with COPD may have features ofasthma, and during exacerbation of symp-toms, systemic steroids may be prescribedfor their anti-inﬂammatory effect (Irwin &Madison, 2003). Individuals with COPDare susceptible to respiratory infectionsand pneumonia, which, because of thealready reduced lung function, may befatal. For bacterial lung infections, antibi-otics are usually prescribed. In addition,individuals with COPD should have annu-al vaccines for both ﬂu and pneumonia.
In addition to medication, other forms
of therapy may include postural drainage
or chest physiotherapy (to remove secretions
from the lungs) or resistive breathing devices
(to increase breathing capacity). Avoid-ance of pulmonary irritants, especiallysmoking, is of primary importance. Manyindividuals with COPD beneﬁt from learn-ing new breathing techniques that stressabdominal, diaphragmatic breathing (to
reduce the use of accessory muscles forbreathing and to conserve energy) orpursed lip breathing (to slow the breathing
rate and help remove trapped air from thelungs). Others ﬁnd it helpful to use a sim-ple resistive breathing device in the homedaily in order to “exercise” the muscles ofrespiration. Physicians may advise individ-uals with COPD to engage in a daily walk-ing or exercise program to keep in shape,to build strength and endurance, and tomaintain their physical condition andimprove their work capacity. Physiciansmay also recommend that individualswith COPD have a series of small mealsthroughout the day rather than a few largemeals because a distended stomach orabdomen can push against the lungs, fur-ther interfering with breathing. Foods thatcause gas and bloating should also beavoided. Adequate ﬂuid intake is impor-tant to facilitate clearance of respiratorysecretions.342
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

Supplemental home oxygen therapy
may be required, depending on theamount of lung damage and the oxygenlevel in the blood. Often these individu-als have right ventricular failure, poly-
cythemia (elevated level of red blood
cells), severe dyspnea, and sleep-associat-ed hypoxemia. Oxygen administrationduring ambulation can reduce the dis-abling effects of dyspnea and increaseendurance and the ability to carry out dai-ly activities. Physicians may prescribehome oxygen therapy for individualswith advanced COPD. The prescription foroxygen therapy usually depends on thedegree of hypoxemia experienced at restor during exercise. The amount of oxygenneeded for each person will vary. Somepeople need oxygen only at night andduring exercise, whereas others will re-quire supplemental oxygen 24 hours aday. Supplemental oxygen provides theadditional oxygen that the lungs cannotprovide.
Oxygen may be supplied from several
different sources. Weight, portability, easeof reﬁlling, availability, and cost are tak-en into account when choosing a system.Compressed gas usually comes in largecylinders, which can weigh up to 200pounds. These cylinders are stationary andmay be mounted near the bed to be usedat night during sleep. Small portabledevices that contain oxygen and that canbe carried and used by the individualthroughout the day are also available.Portable systems generally consist of can-isters containing liquid oxygen. Theyweigh 6 to 9 pounds and can be carriedby the individual. Larger units are alsoavailable, but the difﬁculty of carryingthese devices may make it necessary for
them to be wheeled. Therefore, the larger
device may limit mobility; in addition, there
is the possibility of tripping over the de-vice or becoming entangled in the tubing. Supplemental oxygen reduces dyspnea
and can improve quality of life. However,it is a medicine and should be prescribedand used as such. Although oxygen canimprove individuals’ functional capacityand quality of life, use of portable oxygenmachines can also cause others to perceivethe individual as an invalid, hamperingsocial interaction because of false percep-tions. Because the body’s ability to re
spond
to different concentrations of oxygen
diminishes in some respiratory condi-tions, oxygen should be used only as pre-scribed, and the use should be carefullymonitored. Individuals should neverincrease or decrease the amount of oxygenprescribed without ﬁrst checking withtheir physician. Individuals using oxygenshould not smoke or allow smoking
around it because of the danger of combus-
tion. Oxygen equipment and tubingshould also be kept away from open
ﬂames. Combustible material such as aero –
sol sprays, paint thinners, or petroleum-based products should also be avoided.
Surgical Intervention for COPD
Surgical interventions for COPD are also
available in severe cases. The goal of sur-gical therapy for individuals with ad-vanced COPD is to prolong life bypreventing complications, relieving dysp-nea, and enhancing quality of life byimproving functional status. Until re-cently, lung transplantation was the lastsurgical option available for individualswith limited life expectancy because ofemphysema. Lung transplantation mayinvolve one or both lungs. Selection ofindividuals appropriate for lung transplantis generally based on the individuals’inability to survive without it (O’Brien &Criner, 1998).
More recently, lung volume reduction
surgery has been proposed as a palliativeConditions of the Respiratory System 343

treatment for individuals with severeemphysema (Flaherty, Kazerooni, Curtis,et al., 2001; Geddes et al., 2000; Pompeo,Marino, Nofroni, Matteucci, & Mineo,2000). Since emphysema causes the lungsto become overinﬂated and lose much oftheir elastic recoil, the remaining func-tional part of the lung is essentially com-pressed within the chest wall. Whensome of the functionless area of the lungis removed, lung capacity decreases and amore normal physiologic state is restored.A major requirement for lung reductionsurgery is severe disease in which individ-uals without the surgery are not expectedto live longer than 18 to 24 months.Individuals must be within normal bodyweight, abstain from cigarette smoking forat least 6 months, and have no coexistingmedical problems or severe psychologicalproblems. Prior to the surgery, individu-als are expected to attend pulmonaryrehabilitation for at least 6 weeks. Thisincludes exercises with the treadmill,exercise bike, stair climbing, and the likeunder medical supervision. During exer-cise the oxygen saturation of the individ-ual’s blood is measured. To qualify forsurgery, individuals must build an exercisetolerance to 30 minutes while maintain-ing a predetermined oxygen level in theblood, using supplemental oxygen asnecessary. After lung reduction surgery,individuals undergo pulmonary rehabilita-
tionfor respiratory muscle retraining, in
which they learn how to use the dia-phragm and accessory muscles of respira-tion to assist in breathing. Pulmonaryrehabilitation is also directed to helpingindividuals increase their overall ﬁtnessand endurance. Although overall lung-vol-ume-reduction surgery has been found toincrease the chance of improved exercisecapacity and quality of life (Geddes et al.,2000), it has not been found to increasesurvival rates any more than medical ther-apy (National Emphysema Treatment TrialResearch Group, 2001).
Psychosocial Issues in COPD
Psychosocial issues can manifest as
anxiety, depression, fatigue, and with-drawal from family and social life. Indi-viduals can require extensive psychosocialsupport to deal with these issues. Many ofthe psychosocial issues individuals expe-rience are related to dyspnea. Shortness ofbreath can lead to anxiety and panic,whether a disease process is involved ornot. For individuals with COPD, dyspneamay lead to severe anxiety, fear of death,avoidance of all activities that cause dys-pnea, and preoccupation with bodilycomplaints. Because strong emotions nat-urally raise the respiratory rate, fear ofbecoming short of breath may in itselfincrease dyspnea, causing a vicious cyclethat can be totally incapacitating. Becauseshortness of breath is anxiety provoking,individuals may adopt an abnormally andpotentially unnecessarily restricted lifeeven though they are physically capableof being more active. Maladaptive avoid-ant responses may result, severely limitinginterpersonal activities and causing indi-viduals to become isolated. Individualsmay be unable to work and may feel lessinterested in participating in social andfamily events.
Individuals may also experience intense
emotions such as anger and depression inreaction to coping with their illness.Suppression of emotions can further com-promise individuals’ physical condition,causing increased functional decline andrestricting individuals’ activity and in-volvement with others even more.
Sexual difﬁculties may be a particular
problem for individuals with COPD. Prob-lems may stem from fear of becomingshort of breath rather than from any phys-344
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

ical limitation due to the condition. Al-though it is physically safe for most peo-ple with COPD to engage in sexualactivity, sexual inhibition may be presentif individuals interpret dyspnea that canoccur during sexual activity as an exacer-bation of their condition or as a life-threat-ening symptom. These concerns caninhibit further sexual activity and canaffect intimate relationships with others.In other instances, sexual function may benegatively affected by depression.
Individuals with COPD confront a
num-
ber of losses. Employment, physical inde-pendence, self-esteem, and social inter-actions may be lost or limited because oftheir condition. Some individuals may bereluctant to begin to use portable oxygen
in public because of embarrassment, where –
as others may become psychologically de-
pendent on it and may be reluctant to ven-
ture out without the oxygen source, evenwhen they do not need it. Some insist onusing oxygen even though their difﬁcul-ty in breathing is not the result of loweredoxygen content of the blood. In suchinstances, the psychological dependencyon the oxygen may be more debilitatingthan the respiratory condition itself.
Individuals may ﬁnd themselves exclud-
ed from activities of their families andfriends because of their limited function-al capacity. In some instances, coughingand expectoration of foul-smelling mucusmay interfere with the ability to interactsocially. Counseling and support groupscan help to increase self-esteem and helpfamily members cope with the individual’sillness. Education of both individualswith emphysema and their family mem-bers is important. Both the individual andfamily members should develop reason-able expectations of what can be accom-plished with treatment and should behelped to understand the importance ofadhering to medical recommendations.Vocational Issues in COPD
The most limiting factor in COPD is
dyspnea. Chronic hypoxemia may causeneuropsychological deﬁcits that diminishindividuals’ ability to perform a numberof mental functions, which can furthercontribute to work difﬁculties. In laterstages of the condition, dyspnea may be-come so severe that even walking andcommunicating become difﬁcult.
Until the condition becomes too severe,
individuals may need to learn energy con-servation so that activities are plannedand paced to improve performance with-in the limitations of their condition. Theymay need to change their methods of per-forming more energy-consuming activitiesto improve energy efﬁciency. Proper atti-tude, breathing techniques, body mechan-ics, pacing, and relaxation can all increasework tolerance. Although stress is a partof every job, when demands are too chal-lenging, muscles tense, the heartbeat in-creases, breathing becomes more difﬁcult,and more oxygen is required. Working ina relaxed atmosphere can reduce the emo-tional strain that can contribute to dysp-nea. To expend less energy at work,individuals should sit rather than stand.Ensuring the proper height of stools orchairs in relation to tables or desks andplacing equipment, tools, or supplieswithin easy reach can minimize strain onbreathing. Unnecessary motions or move-ments should be eliminated. Therefore,arranging work to make tasks simpler canincrease functional ability. Pushing or slid-ing objects is easier than lifting, placingcasters on items can facilitate movement,and pushing a wheelbarrel or cart with alight load of items is less strenuous thancarrying the items. Individuals may alsoneed to prioritize tasks. Distributing moredifﬁcult tasks throughout the day andbreaking activities into constituent partsConditions of the Respiratory System 345

with periods of rest in between can enablethem to accomplish tasks more easily. In-dividuals with COPD may also be limitedin the amount they can use their arms andupper body because of the additionalstress placed on accessory muscles of res-piration. For this reason, activities that in-volve lifting or reaching may need to beavoided. Using special long-handled toolsto access materials can help individualsavoid stooping, bending, and reaching.These are all ways to make physical workeasier. If individuals are in sedentary linesof work, they can work even in the laterstages of the condition.
Transportation to and from work should
also be considered. Although many peo-ple with COPD continue to drive, drivingin crowded conditions in which fumesand pollutants are present may be detri-mental. Planning to drive alternate routesthat are less congested or driving earlier orlater in the day can help to conserve ener-gy and decrease exposure to pollutants.The work environment should also be rel-atively free of allergens as well as free ofdust, fumes, or chemicals that are irritat-ing to the airways. Generally, extremes inheat, cold, wind, distance, and durationshould be avoided. The work environmentshould be well ventilated and climate con-trolled so that the temperature is not toohot or too cold.
Occupational Lung Diseases(Pneumoconiosis; Asbestosis; Silicosis;Berylliosis; Byssinosis; OccupationalAsthma)
Some lung disorders are directly related
to matter inhaled from the occupationalenvironment. This group of lung disordersis called occupational lung disease . Although
disabling, occupational lung diseases are
preventable. They are classiﬁed by the type
of material particles inhaled. The termpneumoconiosis refers to a group of lung
diseases in which there has been inhala-tion
of dusts. Examples of types of
pneumoconiosis are silicosis , coal miner’s
pneumoconiosis , asbestosis , berylliosis , and
byssinosis .
Silicosis is a type of occupational lung
disease caused by exposure to silica dust,usually in the form of quartz. It may occurin those with occupations such as quarryworkers, metal mining, foundry work, pot-tery making, sandblasting, or other occu-pations in which there is exposure tosilica. Development of silicosis generallytakes 15 to 20 years of exposure. Whenparticles of silica enter the alveoli, specialcells within the lungs engulf the foreignmaterial and then die. In response, a spe-cial substance is released in the lung, andﬁbrosis (ﬁbrous tissue within the lung)
results. There may be no respiratory im-pairment initially, although damage in theform of nodules may be identiﬁed byroentgenogram (X-ray).
In the early stages of the disease, indi-
viduals may show no symptoms. As dam-
age continues, there is a progressiverestriction of lung function with associat-ed hypoxemia (decreased oxygen in the
blood),
shortness of breath on exertion,
cough, and expectoration. Symptoms are
worse when accompanied by tobaccouse. If the condition progresses further,complications such as right ventricular fail-
ure(see explanation under COPD above)
may result.
There is no effective treatment, other
than removing the individual from theenvironment in which silica is present,although the condition can continue toprogress without additional exposure.Some individuals are able to continue towork in the environment with the use ofan air stream helmet that offers dust pro-tection. Silicosis also predisposes individ-uals to the development of TB. Diagnosis346
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

is based on an occupational history of sil-ica exposure as well as demonstration ofnodule ﬁltration on X-ray. Persons withairway obstruction as a result of silicosisare treated similarly to those individualswith COPD, as described previously.
Coal miner’s pneumoconiosis , or black lung
disease , is an occupational lung disease
in which there has been excessive expo-sure to coal dust. It is characterized by awide distribution of coal dust throughoutthe lungs, leading to a mild dilation ofthe bronchioles and the development ofabnormalities surrounding the bronchi-oles. Although not all cases of coal min-er’s pneumoconiosis progress, a smallpercentage of individuals develop progres-sive scarring of the lung that, in turn,interferes with air exchange. There is nospeciﬁc treatment for coal miner’s pneu-moconiosis. Treatment is similar to thatfor COPD.
Asbestosis is an occupational lung dis-
ease resulting from the long-term inhala-tion of asbestos ﬁbers. Exposure may bethrough the mining, milling, or manufac-turing processes involved in the produc-tion of items such as cement, shingles, orsiding, or through asbestos products suchas insulation. The inhalation of asbestosﬁbers can cause ﬁbrinous changes withinthe lung. Individuals usually notice dys-
pnea (difﬁculty breathing) on exertion.
Treatment of asbestosis is symptomatic.Because asbestosis increases the risk oflung cancer, it is recommended that indi-viduals with the condition abstain fromsmoking.
Inhalation of dust or fumes that contain
beryllium compounds may cause another
type of occupational lung disease, calledberylliosis . Exposure to beryllium is com-
mon in many chemical plants, in factories(e.g., those that manufacture ﬂuorescentlight bulbs), and in the aerospace indus-try. Symptoms of the condition may notappear for as long as 10 to 20 years afterthe exposure. Inhaled beryllium creates aninﬂammatory process in the lungs thatalters the lung tissue. Symptoms mayinclude progressive difﬁculty with breath-ing on exertion, with progressive loss ofrespiratory function. The treatment ofberylliosis is largely symptomatic.
The occupational lung disease byssinosis
occurs primarily in textile workers. It iscaused by the inhalation of dusts fromﬁbers such as cotton, ﬂax, and hemp. Theresulting bronchoconstriction causes chesttightness. Unlike those with other occu-pational lung diseases, which becomeworse with increased exposure, individu-als with byssinosis experience symptomsafter they return to work from days off,but as the week goes on, symptoms grad-ually lessen. With prolonged exposureover a number of years, chest tightnessmay extend for longer periods.
Occupational asthma is a condition char-
acterized by airway restriction and hyper-responsiveness induced by exposure tosensitizing agents in the work environ-ment (Bernstein, Bernstein, Chan-Yeung,& Malo, 1999). The term occupational asth-
mais not used to describe instances in
which environmental factors provoke anattack in someone who already has asth-ma. Rather, it applies to individuals whobecome asthmatic because of exposure toenvironmental agents in the workplace, aprocess that may take from days to years.The list of agents considered potentialcauses of occupational asthma is growingdaily. If asthma is proven to be occupa-tional, exposure must be reduced oravoided completely, depending upon theseverity of the disease. The treatment andmanagement of occupational asthma areindividually determined. In someinstances, even those who leave the workenvironment continue to have symptomsfor a number of years.Conditions of the Respiratory System 347

OTHER CONDITIONS AFFECTINGRESPIRATORY FUNCTIONRestrictive Pulmonary Disease
Restrictive pulmonary diseases prevent
individuals from receiving an adequatesupply of air during inspiration. Con-ditions that cause restrictive pulmonarydisease may include skeletal problemssuch as scoliosis (lateral curvature of the
spine) or kyphosis (forward curvature of
the spine, see Chapter 14) so that chestexpansion is decreased. Other conditionsthat may cause pulmonary restriction arenervous system diseases such as polio,spinal cord injury, or Parkinson’s disease(see Chapter 3), in which the muscles thatassist in respiration are hampered. Obesityalso restricts lung expansion.
Bronchiectasis
Bronchiectasis is a chronic disease in
which there is chronic respiratory tractinfection and increased inflammatoryresponse of the bronchi and bronchiolesso that they become dilated and inﬂamedand permanently vulnerable to recurrentinfection. It is caused by repeated respira-tory tract infections associated with con-ditions such as chronic sinusitis, bacterialinfections, cystic ﬁbrosis, or rheumaticconditions or by a genetic or immune deﬁ-ciency (Barker, 2002). Purulent (pus-con-
taining) material collects in the dilatedairways. Individuals with bronchiectasisexperience cough and chronic sputumproduction. They may also complain offatigue, weight loss, or loss of appetite ormay experience hemoptysis (expectora-
tion of blood).
The condition is usually diagnosed by
symptoms, chest X-ray, and in some in-
stances a computed tomography scan to pin –
point the location and extent of damage. Treatment includes the administration
of antibiotics to control the infection,bronchodilators to clear the airways,maintenance of general health throughrest and nutrition, and avoidance of fur-ther infections. Individuals may also learnspecial techniques ( bronchopulmonary
hygiene ) or receive treatment ( postural
drainage and chest physiotherapy ) to remove
respiratory secretions.
Damaged bronchi do not return to nor-
mal. If the inﬂammatory and destructiveprocess continues, surgical removal of thediseased part of the lung may be necessary,although the role of surgery in treatmentof bronchiectasis has declined.
Cystic Fibrosis
Cystic ﬁbrosis is a multisystem disorder
affecting the respiratory, digestive, skin,and reproductive systems (Dickinson-Herbst, 2001). It is a genetic condition inwhich mucus-secreting organs in thebody become obstructed by abnormal,thick mucus (Esmond, 2000). As a result,there is degeneration and scarring of theorgans involved.
Individuals with cystic ﬁbrosis once rare –
ly lived beyond childhood; however, withrecent improvements in the managementof complications, many individuals withcystic ﬁbrosis now survive well into adult-hood (Cystic Fibrosis Foundation, 2003).
Lung involvement is one of the most
frequent causes of disabling effects of cys-tic ﬁbrosis, and, when complications oc-cur, it can also result in death. Respiratoryinvolvement occurs because of the forma-tion of thick mucus in the small bronchi ,
which can lead to severe bronchitis and em-
physema . Atelectasis (collapse of the lung)
is not uncommon. Individuals may haveintermittent episodes of acute respiratoryinfections that persist. Coughing may bepronounced and may produce thick and348
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

purulent sputum. As the disease progress-es, there is usually a gradual decline in pul-monary function.
Although the lungs are involved, other
organs, such as the pancreas, are also af-fected. The pancreas may also become in-ﬂamed so that individuals develop pan-
creatitis . Because ducts of the pancreas are
plugged by the thick mucus production, en –
zymes produced by the pancreas that aid in
digestion are unable to function in this ca-pacity, leading to poor digestion of proteinand fat. As a result, individuals experiencechronic malnutrition and delayed growth.
Individuals with cystic ﬁbrosis also
have malfunctioning sweat glands, so thatexcessive loss of salt occurs, resulting inhyponatremia (decreased concentration
of salt in the blood).
Fertility problems are also common.
Males with cystic ﬁbrosis have high ratesof infertility (almost 99 percent; Knowles
& Durie, 2002), and women, although theyhave lower rates of infertility, have higher
rates of complications with pregnancy, such
as progression of lung disease (Hamlett,Murphy, Hayes, & Doershuk, 1996).
Diagnosis of Cystic Fibrosis
Because of the increased concentration
of sodium and chloride in the sweat ofindividuals with cystic ﬁbrosis, a sweat
electrolyte test is an important diagnostic
tool in identifying individuals with thecondition. The test is noninvasive andinvolves collecting and analyzing sweatfrom a small area of the individual’s arm.When the test is positive, additionalgenetic testing may be instituted.
Treatment and Management of
Cystic Fibrosis
Pulmonary symptoms require the ad-
ministration of antibiotics to prevent ortreat infection. Because mucus productionis increased, individuals with cystic ﬁbro-sis may have difﬁculty clearing secretions,which is important so that organisms donot have an environment in which theycan grow and thrive. To help clear secre-tions, individuals may be taught speciﬁcprocedures to facilitate coughing, or theymay be encouraged to increase ﬂuid intakein order to liquefy secretions. Other meas-ures include breathing warm humidiﬁedair or inhaling steam several times a day.Individuals may also be instructed in var-ious forms of postural drainage to be used
at home to drain mucus. For example,they may be instructed to lie over the sideof the bed with their head lower than therest of the body several times a day. Insome instances, individuals may be re-ferred for chest physiotherapy , in which pro-
cedures such as percussion are used.
Percussion is a form of massage in which
the chest is repeatedly tapped or vibratedto loosen the mucus and allow it to drain.This procedure may be done by a physical
therapist , or it may be done at home, either
with an electric percussor or manually by
a family member. Supplemental oxygenmay also be used on an as-needed basis tomaintain lifestyle and work activities.
Diet plays a direct role in the treatment
of pancreatitis. If pancreatic ducts becomeblocked, supplemental enzymes are taken at
mealtimes to aid in digestion and preventmalnutrition. Despite supplemental en-zymes, however, individuals with cysticﬁbrosis still frequently experience delayedgrowth and malnutrition, and they areoften underweight. Because of the in-creased salt loss characteristic of cysticﬁbrosis, dietary prescription is necessaryto ensure adequate salt intake. Adequatehydration is necessary to liquefy secre-tions, and other dietary prescriptionsmay be needed to prevent nutritional deﬁ-ciencies. Other Conditions Affecting Respiratory Function 349

When progression of the lung disease
associated with cystic ﬁbrosis reaches astage in which there is severe disability,double lung transplantation may be per-formed to halt disease progression andrestore function (Dickinson-Herbst, 2001;Lanunza, Lefaiver, & Farcas, 2000).
Psychosocial Issues in Cystic Fibrosis
Because cystic ﬁbrosis is a genetic cond-
ition, with symptoms usually present inchildhood, affected individuals grow up
with a chronic and potentially fatal di-sease
that can impact their successful pas-
sage through normal growth and develop-
ment. The attitudes of family, teachers,
and peers are instrumental in helpingchildren develop their self-concept andtheir view of their condition, and suchattitudes can also affect the individual’sfuture function.
The psychological impact of cystic
ﬁbrosis in adulthood varies (Burker, Carels,Thompson, Rodgers, & Egan, 2000; Crews,Jefferson, Broshek, Barth, & Robbins,2000). Adequate time, energy, and re-sources are needed to perform many of thehome therapy treatments, such as chest
physical therapy, dietary adjustment , moni-
toring for respiratory infection , enzyme
administration to aid in digestion, and rou-
tine use of other mediations such as bron-chodilators and antibiotics . Special skills are
required for monitoring symptoms, inter-preting changes, and making decisionsabout the need for altering treatment.Children who have been encouraged toassume more responsibility for their self-care will be more likely to grow into grad-ual independence (Esmond, 2000).
During adolescence, the behavioral re-
sponses to chronic disease may be mademore difﬁcult by the “need to ﬁt in” or bythe attitudes of rebellion and deﬁance thatare characteristic of that age. These factorsmay potentially result in health-compro-mising behaviors. Without appropriatesupport, individuals may be less able toadapt and cope with chronic illness; thisinability may result in isolation andaltered relationships.
Individuals with any chronic condition
may ﬁnd adherence to treatment difﬁcultbecause of its long-term and complexnature. Adhering to treatment is a dailyreminder of the condition. Some individ-uals may feel that, given the likelihood ofincreasing deterioration and potentialmortality, adherence is not worthwhile.
Fertility problems in individuals with
cystic ﬁbrosis may take an emotional toll.In addition to coping with the challengesof daily management of the condition,individuals also must incorporate issues ofinfertility into their relationship with sig-niﬁcant others.
Vocational Issues in Cystic Fibrosis
Cystic ﬁbrosis is an incurable chronic
condition that may produce increasingdisability over time. The rate of progres-sion of the condition varies signiﬁcantlywith different individuals. Because of theincurable and progressive nature of cysticﬁbrosis, employment of adults with thecondition has not been considered feasi-ble until recently (Mungle, Burker, &Yankaskas, 2002).
Individuals with cystic ﬁbrosis have
heightened susceptibility to respiratoryinfections and consequently should avoidenvironments in which exposure to res-piratory infections is likely. Flu and pneu-monia vaccinations are important toprevent respiratory complications fromoccurring. Individuals should also avoidexposure to dust or toxic fumes. Indi-viduals may show exercise intolerance ifthey are placed in an environment wherethey
are exposed to sudden temperature350 CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

changes or air pollutants. If supplemental
oxygen is needed for maintaining workactivities, oxygen precautions as discussedelse
where in this chapter should be in-
stituted.
Because excessive salt is lost through
sweat and because adequate hydration isnecessary to avoid excessive viscosity ofmucus, which can clog airways, hot,humid environmental conditions shouldbe avoided. Although many individualswith cystic ﬁbrosis are able to be employedfull-time, the time and energy required tocarry out daily treatment routines and thepotential disruptions because of periodichospitalizations must be incorporatedinto individuals’ vocational plans.
Apnea
The term apnea refers to cessation of
breathing. Apnea can be caused from a
variety of conditions. One of the more com –
mon conditions is sleep apnea , in which
there are repeated episodes of the cessa-tion of breathing during sleep. The condi-tion is more common in middle age. Indi-viduals with sleep apnea may be unawarethat they stop breathing during sleep, butthey may experience excessive daytimedrowsiness, difﬁculty with attention orconcentration, and irritability because ofdisruption of sleep. Although individualswith sleep apnea may be unaware of peri-ods of apnea during sleep, their sleep part-ners may complain of being awakened bytheir loud snoring or sudden body move-ments during an attack of apnea.
There are several types of sleep apnea.
The least common type is central sleep
apnea , which is caused by a disruption of
the signals from the central nervous sys-tem that stimulate respiration. This con-dition may be due to a variety of diseaseconditions of the central nervous system(see Chapter 2). Peripheral sleep apnea , alsocalled obstructive sleep apnea , is the most
common type and is caused by an upperairway obstruction. The obstruction canbe caused by narrowing of airways due toobesity, hypertrophy (enlargement) of
tonsils, or structural abnormalities thatpredispose the airway to narrowing or clo-sure during sleep (Flemons, 2002). Mixed
type sleep apnea is a combination of cen-
tral and peripheral sleep apnea. Individ-uals with sleep apnea, regardless of thetype, experience hypoxia (decrease of
oxygen), resulting in hypoxemia (de-
creased oxygen in the blood) and hyper-
capnia (buildup of carbon dioxide in the
blood).
The consequences of sleep apnea go
beyond sleep disruption and daytimedrowsiness. Individuals with sleep apneahave an increased risk of hypertension
(high blood pressure), heart failure, myo-
cardial infarction (heart attack), and
stroke. Because of sleep deprivation, peo-ple with sleep apnea are also at increasedrisk of accidents. The evaluation of sleepapnea often takes place in a sleep labora-tory, where breathing during sleep is mon-itored and recorded. There are alsoportable monitoring systems that can beused outside the hospital; however, read-ings may not be as accurate.
Treatment of Sleep Apnea
Sleep apnea may be treated behavioral-
ly, medically, or surgically. The type of treat-
ment chosen is dependent on individuals’symptoms and the function of the car-diopulmonary system. Treatment goals are
directed toward establishing normal breath-
ing and oxygenation of the blood and toeliminating disruption of sleep. Becausealcohol consumption reduces muscle toneof the upper airway and increases the fre-
quency of abnormal breathing during sleep,
treatment recommendations often includeOther Conditions Affecting Respiratory Function 351

limiting alcohol use. Individuals who areobese are encouraged to lose weight toreduce obstruction. Some individuals havemore difﬁculty with sleep apnea whenlying on their back, so in these instances,they are trained to sleep on their side.
The treatment of choice for some indi-
viduals with sleep apnea includes contin-
uous positive airway pressure delivered
through a mask. Machines used for thispurpose weigh only about ﬁve pounds.They are used at night and ﬁt on a bed-side table. Individuals may use a mask thatcovers only the nose, nasal prongs, or amask that covers both the nose and the
mouth. The amount of continuous positive
pressure applied is determined throughevaluation in a sleep laboratory. Ratherthan using the positive airway pressuremachine, some individuals choose oralappliances that are worn during sleep tohelp keep the airway open.
Surgical treatment can range from tra-
cheostomy (in which a surgical opening
is made through the neck into the tracheato enable the individual to breathe) to sur-gical correction of the structural abnor-malities of the palate or facial structurethat contribute to obstruction. Insufﬁcientawareness of sleep apnea among physi-cians and the public in general may resultin sleep apnea that goes undiagnosed andconsequently untreated.
Vocational Issues in Sleep Apnea
Sleep apnea can cause signiﬁcant voca-
tional impairment. Individuals with sleepapnea, in addition to daytime sleepiness,may also experience irritability, impa-tience, or even depressive manifestations,which can affect their relationship withothers at work. Individuals with sleepapnea may also experience cognitive im-pairments, including difﬁculty with atten-tion
and concentration, visual/motorabilities, and memory. Tasks involving
planning, verbal ﬂuency, or general intel-lectual performance may be impaired.Because of sleep deprivation, individualswith sleep
apnea may also be more prone
to accidents.
If sleep apnea is diagnosed and treated,
symptoms can be reversed. However, un-9fortunately, in many cases the conditionis not diagnosed and a decrease in job per-formance is attributed to other causes.
Chest Injuries
Fractured ribs are a common chest
injury. Although painful, they are usual-ly treated relatively easily by wrapping astrap or binder around the chest for sup-port. In some instances, however, a frac-tured rib punctures other organs, such asthe lungs or heart, and the consequencesare more serious.
An open wound to the chest, such as
a puncture wound, may allow air to enterthe thoracic cavity. This condition, calledpneumothorax , may cause the lung on
the affected side to collapse. Pneumo-thorax unrelated to trauma can be second-ary to a number of pulmonary conditions,such as COPD, asthma, or cystic ﬁbrosis.This is called spontaneous pneumothorax
and is caused by a tear or rupture of air sacs
in the lung, causing air to escape into thethoracic cavity. Pneumothorax is generallytreated by inserting a tube through thechest wall to facilitate expansion of thelung. Individuals who have experiencedpneumothorax should avoid smoking,high diving, or ﬂying in unpressurized air-craft, all of which can cause a recurrence.
Escape of blood into the thoracic cavi-
ty because of an injury to the chest thatdamaged vessels in the thoracic cavity iscalled hemothorax , which may also cause
collapse of a lung. In both pneumothoraxand hemothorax, the lung is compressed352
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

and breathing is hampered. A large pneu-mothorax or hemothorax requires emer-gency treatment to remove air or bloodand repair the injury. The removal of ﬂu-id from the thoracic cavity is called tho-
racentesis , a procedure in which a needle
is inserted into the thoracic cavity and ﬂu-id is aspirated out through the needle.
DIAGNOSTIC PROCEDURES FORRESPIRATORY CONDITIONSChest Roentgenography (X-ray)
Roentgenography (X-ray) of the chest
is a radiographic procedure that allows
bony structures (e.g., the ribs), the lungs,and other organs in the thoracic cavity tobe viewed as a still image on X-ray ﬁlm.It may be useful in diagnosing tuberculo-sis, in noting changes in the lungs due toCOPD, or in identifying structural abnor-malities or tumors.
Bronchoscopy
Visual examination of the bronchial
tubes through a long hollow tube insert-ed through the mouth and into the bron-
chus is called bronchoscopy . With the bron-
choscope , the physician can view the walls
of the bronchus and note any abnormal-ities. A pulmonologist (physician who
specializes in evaluation and treatment ofconditions of the lung) usually performsthe procedure, although other physicianswith special training in the procedure mayalso do so. Individuals undergoing bron-choscopy are usually given a sedative, butthey remain awake during the procedure.
Laryngoscopy
Laryngoscopy is visual examination
through a hollow tube called the laryngo-scope. The procedure enables the physician
to inspect the structures of the larynx visu-ally and to assess the function of the vocalcords. The procedure is usually performedunder a local anesthetic, although theindividual may be sedated.
Pulmonary Angiography
In a procedure called pulmonary angiog-
raphy , a catheter is inserted into a vessel
and a contrast agent (special dye that
enhances visualization of a structure) isinjected into the catheter to enable thephysician to visualize the pulmonary ves-sels. X-ray ﬁlms are then taken and the cir-culation of the lungs studied. Pulmonaryangiography may be used to assess the ex-tent to which emphysema has destroyedlung tissue or may be used prior to surgeryfor lung cancer to assess the potential ben-eﬁts of surgery.
Pulmonary Function Tests
Pulmonary function tests are used to
detect abnormalities in respiratory func-tion and to determine the degree ofimpairment of respiratory function. Phy-sicians use pulmonary function tests toassess the volume of air that an individ-ual can take in and expel from the lungs,as well as individuals’ ability to move airin and out of the lungs. Pulmonary func-tion tests may be used to determine thecause of dyspnea, the extent of lung dis-ease, or the effectiveness of treatment forlung disease. Generally done in a pul-
monary laboratory , the tests involve breath-
ing into a special machine called aspirometer , which measures several types of
pulmonary function. The results are thenprinted out in a graphic representationcalled a spirogram . The types of pul-
monary functions that are measured arethe following:Diagnostic Procedures for Respiratory Conditions 353

• Vital capacity: the maximum volume
of air that can be inspired andexpired.
• Forced expiratory volume (FEV): the
volume of air that the individual canforcibly exhale at 1-, 2-, and 3-secondintervals. Readings of the FEV arereported as FEV1, FEV2, and FEV3.
• Residual lung volume: the amount of
air left in the lungs after maximumexpiration.
• Maximum voluntary ventilation: the
maximum volume that an individualcan breathe in 12 seconds, breathingin and out as rapidly and forcefully aspossible.
• Tidal volume: the amount of air
breathed in and out at rest.
• Inspiratory capacity: the volume of air
taken in by maximal inspiration afternormal expiration.
• Functional residual capacity: the vol-
ume of air remaining in the lungsafter normal expiration.
Ventilation/Perfusion Scan (Lung Scan)
Ventilation is the process by which gas-
es are transported between the atmos-phere and the alveoli. Perfusion is the
process by which blood or other ﬂuid pass-es to a body part through a vascular bed.The ventilation/perfusion scan is a radi-ographic procedure that makes it possibleto measure the ventilation and/or perfu-sion of the lung. The test may be per-formed to determine whether a blood clothas traveled to the lung and lodged thereor to diagnose other disease conditions,such as emphysema. For the ventilationscan, the individual inhales radioactivegas; the image taken shows where venti-lation is occurring in the lung. For a per-fusion scan, a radioactive dye is injectedintravenously, enabling the radiologist tovisualize blood ﬂow to the lung.GENERAL TREATMENT FORRESPIRATORY CONDITIONS
Because many diseases of the respirato-
ry system are irreversible, treatment maybe directed toward controlling symptomsand preventing complications or furtherdeterioration. Pollutants and other irri-tants, especially cigarette smoke, shouldbe avoided, as they can aggravate respira-tory conditions. In areas where pollutionis severe or if allergies complicate the con-dition, special air ﬁlters or puriﬁers maybe needed. Even when pulmonary func-tion is compromised, physicians oftenadvise individuals with pulmonary condi-tions to engage in a daily walking andexercise program to keep in shape andbuild strength and endurance. A numberof medications may be used for respirato-ry conditions, depending on the nature ofthe respiratory dysfunction.
•Bronchodilators help to open the air-
ways so that more air can move inand out. Bronchodilators come in sev-eral forms, including pills, liquids,and sprays.
•Antibiotics may be taken for infec-
tions. They may be taken by mouthor injected.
•Diuretics , sometimes called water pills ,
rid the body of extra ﬂuid, such as theﬂuid that builds up because of rightventricular failure.
•Expectorants are oral medications that
make mucus thinner and easier toclear.
•Steroids are hormonal preparations
that help reduce swelling in the air-ways, consequently easing breathing.Steroids are usually taken orally butmay also be injected. Because of theirserious side effects, they are usuallyprescribed only for temporary relief ofsevere symptoms rather than for354
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

long-term use. Additional informa-tion about steroids can be found inChapter 14.
A variety of breathing aids may be used
in the treatment of respiratory disorders.Many devices are designed to help putmedicines, oxygen, or moist air deep intothe lungs and to help individuals cleartheir lungs of mucus. An intermittent pos-
itive pressure breathing machine is a device
used to deliver air under pressure to thelungs. It may have a nebulizer attached so
that it delivers medication and humidityto the lungs as well. Individuals with can-cer or severe chest injuries may requiresurgery. At times, the removal of the lung(pneumonectomy ) or a portion of the
lung is indicated. Most people are able tofunction normally even with a portion ofthe lung removed.
PSYCHOSOCIAL ISSUES INRESPIRATORY CONDITIONSPsychological Issues
Difﬁculty breathing can be a frighten-
ing and distressing experience. The asso-ciated fear and anxiety may lead to inac-tivity, which, in turn, may result in a vari-ety of additional physical problems.Prolonged breathing difﬁculty often caus-es feelings of helplessness and despair. Forthose individuals who have been activeand self-sufﬁcient, the inability to engagein even simple activities without breath-ing difﬁculty can be devastating. Depres-sion is common. Individuals may focus onthe activities in which they can no longerparticipate, at least not as vigorously,rather than attempting to attain theirhighest level of functional capacity.
When a respiratory condition reduces
oxygen concentrations in the blood andthe oxygen available to the brain is insuf-ﬁcient, associated cognitive changes mayresult. Clouding of consciousness orchanges in cognitive function can befrightening for individuals who experi-ence these changes, as well as for familymembers who observe them. Close mon-itoring of the oxygen and carbon dioxideconcentrations is important in the care ofindividuals with respiratory disorders sothat low oxygen or high carbon dioxideconcentrations can be identified andappropriate measures instituted to reestab-lish normal concentrations.
Emotional factors can compound the
physical symptoms of respiratory condi-tions. Anxiety or emotional upsets mayincrease the difﬁculty in breathing, caus-ing more anxiety and leading to more dif-ﬁculty in breathing. When it is possible toidentify situations that increase anxiety orstress, it is important to institute interven-tions to decrease anxiety so that the dif-ﬁculty in breathing does not escalate.
The responses of family and friends to
respiratory conditions may affect individ-uals’ ability to cope with their condition.
Family members may unintentionally place
individuals in an invalid role, reducing ex-pectations of them in the family structure
or removing responsibility from them, even
though they may be capable of engagingin a number of activities. Individuals mayrespond by using breathing difﬁculty toescape from life’s demands, to receiveemotional rewards, or to manipulate orcontrol the behavior of others. In otherinstances, family members may overesti-mate individuals’ abilities, not fully under-standing the seriousness of the conditionand its implications for function. Suchreactions may push individuals to go be-yond their functional capacity or to ignorephysicians’ speciﬁc recommendations forcontrolling the condition.
Circumstances that surround the devel-
opment of a respiratory condition mayPsychosocial Issues in Respiratory Conditions 355

elicit guilt on the part of the individualwith the condition, or anger on the partof family members. Because smoking islinked to a variety of respiratory condi-tions, individuals who have smoked heav-ily may feel guilty for their actions. Fam-ily members may express anger, blamingthe individual for smoking and possiblycontributing to the development of thedisease. When respiratory conditions arerelated to occupational factors, individu-als and their family members may beangry because of the exposure to unrecog-nized hazards or, if hazards were identi-ﬁed, the failure of the employer to takeproper precautions to protect employees.
Unless individuals have severe respira-
tory distress or use some type of breath-ing aid, respiratory conditions are notusually as easily recognizable as conditionsin which there are visual cues, such ascrutches or a wheelchair. Consequently,employers, coworkers, or casual acquain-tances may expect individuals to be ableto perform various activities that may notbe consistent with their functional capac-ity. Lack of visual cues may enable indi-viduals to deny their condition and avoidtreatment, which can prove hazardous,not only for the individual but also, inthe case of infectious disease (e.g., TB), forothers with whom the individual hascontact.
Lifestyle Issues
As with other chronic conditions, the
lifestyle changes required by respiratoryconditions depend on the condition’s seri-ousness and on the individual’s previousstate of health and functional capacity. Ingeneral, it is important for individualswith respiratory conditions to maintaingood nutritional status and a normalweight. Because of the increased load thatobesity or overeating can place on breath-ing capacity, individuals with respiratory
conditions should be urged to avoid both.
Cessation of smoking is a necessary
component of treatment, regardless of thetype of respiratory condition. Many indi-viduals consider this task the most difﬁ-cult part of their treatment. Even whenthey are aware that smoking exacerbatestheir respiratory condition, they mayﬁnd it difﬁcult to alter their behavior.Enrollment in specially designed smokingcessation programs may be necessary tohelp individuals stop smoking. Someindividuals resist participation even inthese interventions, however.
Although exertion can cause difﬁculty
breathing, individuals with respiratoryconditions are generally able to maintainactivity unless they have associated car-diac complications. Exercise programscan improve self-esteem and reduce symp-toms somewhat. If individuals experiencedyspnea partly because of ineffectivebreathing patterns and partly from lack ofconditioning, it is crucial that they workto increase exercise tolerance through dai-ly breathing or conditioning exercises, inaddition to participating in other exerciseroutines to increase exercise tolerance.
Unless the cause of dyspnea can be cor-
rected, individuals with respiratory condi-tions need to become accustomed tofeeling short of breath and to adapt to thesensation so that they can maintain theirmaximum level of activity without unduefear or anxiety. They may need extra timeto accomplish tasks so that they can takerest periods. They may need to dividesome activities into smaller tasks ratherthan trying to accomplish the completetask at one time.
Although an environment near sea lev-
el with a mild climate and minimal airpollution is ideal for individuals withchronic respiratory conditions, it is notalways possible to live in this type of envi-356
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

ronment. Individuals living in less mod-erate climates should avoid extremes intemperatures. Home and work tempera-tures should be kept cool. Radiant or base-board heaters may be better thanforced-air heating systems, as the latterhave ﬁlters that need to be cleaned orchanged regularly. If a humidiﬁer is used,it should be cleaned regularly to forestallmold growth. Fireplaces and wood-burn-ing or coal-burning stoves should beavoided as potential sources of air pollu-tion in the home.
Maintaining adequate hydration is im-
portant in respiratory conditions in whichthere is an overproduction of mucus. Theenvironment should be well humidiﬁed,and a nebulizer or aerosol may be used
periodically throughout the day to deliverhumidity directly to the lungs. High levels
of humidity may make breathing moredifﬁcult, however. Therefore, individualsliving in hot, humid environments shouldhave air conditioning to maintain thetemperature and the humidity at accept-able levels. Filters on air conditionersshould be changed on a regular basis.
If factors in the home contribute to the
respiratory disease, environmental modi-ﬁcations, such as removing the cause ofthe allergic reaction, may be required. Thisis especially distressing if the offendingfactor is a pet. The environment shouldalso be kept dust-free. It may be necessaryto install special ﬁlters to cut down onmolds and household dusts.
Individuals with respiratory conditions
may become very anxious about partici-pating in any type of physical activity thatincreases respiratory difﬁculty. Becauseboth the rate and the depth of respirationare increased during sexual excitement,the fear of suffocation may cause theseindividuals to be reluctant or restrainedwhen engaging in sexual activity. Al-though dyspnea may be uncomfortable,those who have a respiratory conditionwith no complications can generallymaintain sexual activity. They can oftenincrease their tolerance to sexual activitythrough conditioning, or their partnersmay assume a more active role.
Social Issues
Individuals with respiratory conditions
may avoid social contacts and social situ-ations that they once enjoyed if dyspnea,especially on exertion, is pronounced. Theresulting social isolation can contribute todepression and lowered self-esteem. Asmuch as possible, individuals should con-tinue to participate in social activity, mod-ifying the circumstances as necessary. Forexample, using a golf cart may reduce theexertion required in golf to the extent thatthose with respiratory conditions can stillenjoy the game as a form of recreation andtime to be spent with friends. Outdoor ac-tivities should be avoided, of course, whentemperatures are very hot or very cold, orif pollution levels are especially high.
Even though crowded, polluted envi-
ronments aggravate respiratory condi-tions, individuals with respiratory condi-tions need not refrain from participatingin activities in urban areas. It may be nec-essary to plan travel time to and from theevents so that traveling does not takeplace during the time of heaviest trafﬁc.Arriving early at events can help cut downon crowding and the potentially anxiety-
provoking rush. Establishments that do not
prohibit smoking altogether usually pro-
vide nonsmoking areas. Individuals should
check on smoking rules ahead of time andrequest seating in no-smoking sections.
Some respiratory conditions cause pro-
nounced coughing, which may be accom-panied by excessive, foul-smelling mucusproduction. Both the cough and excessivemucus can be embarrassing and interferePsychosocial Issues in Respiratory Conditions 357

with communication and social interac-tions. Individuals with respiratory condi-tions should be open and honest abouttheir condition with others, not makingexcuses or excessive apologies. Frequentmouth care can help to alleviate foul-smelling breath.
Because of the potential seriousness of
respiratory infections in individuals withlung disease, they should avoid contactwith persons who have upper respiratoryinfections or ﬂu as much as possible. Forexample, exposure to large groups of peo-ple in conﬁned environments at theheight of the ﬂu season should be discour-aged, although this is not always possiblewithout severely limiting social contacts.
VOCATIONAL ISSUES INRESPIRATORY CONDITIONS
The extent to which individuals with
respiratory conditions can continue regu-lar employment depends on the type ofwork, the work environment, and theseverity of the respiratory condition. Indi-viduals with severe respiratory incapacitymay still be able to function in the work-place if their job requires little physicalexertion. On the other hand, individualswith a small degree of respiratory incapac-ity may be unable to maintain employ-ment in an environment that requiresstrenuous activity. Work that requires ex-tensive use of upper extremities requireshigher levels of ventilation than doeswork that mostly involves the use of low-er extremities.
If factors in the work environment have
contributed to or aggravated the respira-tory condition, a change may be needed.In some instances, individuals may trans-
fer to another location in the facility where
the offending factors are not present.
The extent of activity that individuals
with respiratory conditions can tolerateshould be evaluated. If, for example, theycan walk the length of the hall but can-not walk up one ﬂight of stairs withoutsevere dyspnea, using an elevator may beindicated, or the individual’s workstationmay need to be moved to a different ﬂoor.The degree to which upper body move-ments are used in work and the impact ofthese movements on dyspnea should beconsidered. If the work demands liftingand carrying that increase dyspnea, alter-nate strategies may be devised so that thework can be performed with less exertion.Individuals, their employers, and theircoworkers should be helped to understandthat moderate dyspnea, although uncom-fortable, is not in itself life-threatening.Because cough and sputum productioncan be cosmetically displeasing, they mayinterfere with individuals’ effectiveness injobs that require close personal contact orcontinued conversation. The degree towhich the workplace demands this type ofinteraction and the impact of these symp-toms on job effectiveness should beassessed.
If any type of breathing device or aid is
used, the extent to which the aid will bea hazard in the work environment mustbe considered. Tubing in some portabledevices may be caught in machinery, for
example, or cause falls. Oxygen, because of
the danger of ﬁre or explosion, should notbe used in close proximity to open ﬂame.
In addition to stress in the work setting
that could increase anxiety and subse-quently add to breathing difﬁculty, stres-sors such as those involved in transpor-tation to and from work should also benoted. If commuting necessitates travel inpolluted, congested areas, it may be pos-sible to modify the work schedule to allowtravel at less busy times. Flexibility of thework schedule may also be important ifspeciﬁc treatments or rest periods arerequired during the day. 358
CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM

The legal implications of many lung
conditions, especially if they are occupa-tionally related, may be barriers to contin-ued employment. For individuals eligiblefor workers’ compensation or other ben-eﬁts, ﬁnancial considerations may affectmotivation and cooperation with treat-
ment. In other instances, the employer’s fear
of liability may limit job opportunities forindividuals with respiratory conditions.Medical rehabilitation programs for indi-viduals with respiratory conditions can beimportant to help them increase theiractivity level to their optimum capacity.
CASE STUDIESCase I
Ms. G. is 48 years old. She has a degree
in cosmetology and has worked as a self-employed hairdresser for the past 28years. She has also been a heavy smokerand was diagnosed as having emphysema5 years ago. Although she continues towork, she has found that keeping up withthe number of customers she had workedwith in the past is more difﬁcult. Herphysician has recently suggested that shemay be helped by having a portable oxy-gen tank.Questions
1. Is it feasible for Ms. G. to continue
working as a hairdresser?
2. What factors would you consider
when discussing with Ms. G. whethershe can remain in her current line ofemployment?
3. If Ms. G. elects to use a portable oxy-
gen tank, are there speciﬁc precau-tions that should be taken?
4. What is the general prognosis for Ms.
G.’s condition?
5. What lifestyle factors need to be con-
sidered in working with Ms. G. on herrehabilitation plan?
Case II
Mr. L., age 29, has been a counselor in
a drug treatment facility for the past 3years. The policy of the facility is to haveall employees tested for TB on an annualbasis. This year Mr. L. tested positive.
Questions
1. What vocational implications does a
positive TB test have for Mr. L.?
2. What additional steps need to be tak-
en if it is determined that Mr. L. hasactive TB?
3. What should Mr. L.’s coworkers be
told about their exposure?
4. What are the vocational implications
for Mr. L.?Case Studies 359

360 CHAPTER 12 C ONDITIONS OF THE RESPIRATORY (PULMONARY ) SYSTEM
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NORMAL STRUCTURE ANDFUNCTION OF THE URINARY TRACT
The urinary system enables the body to
rid itself of the byproducts of metabolismand to regulate body ﬂuids and their elec-trolyte content. It consists of two kidneys ,
bean-shaped organs lying on the posteri-
or(back portion) of the abdominal cavi-
ty on either side of the vertebral column;the urinary tract , consisting of two uretersor tubes (one from each kidney, leadingfrom the kidneys to the bladder); the blad-
der(the storage place for urine until it is
eliminated); and the urethra , a single tube
leading from the bladder to the outsideopening (urinary meatus) through whichurine is eliminated (see Figure 13–1). Theterm renal refers to the kidney, and the
term urinary tract refers to the collecting
system for urine (i.e., the ureters, bladder,and urethra).Urinary Tract and Renal ConditionsCHAPTER 13
363
Cortex
Renal Pyramids
Renal PelvisCalixMedulla
Ureter
BladderUrethraUrinary MeatusKidney
Figure 13–1 Kidneys and Urinary Tract.

The kidneys have multiple functions:
• They maintain the body’s internal
chemical balance ( homeostasis ) by
regulating its water content and theconcentrations of electrolytes (electri-
cally charged particles of substancesthat are important to many of thebody’s internal functions).
• They rid the body of metabolic waste
products (i.e., urea, uric acid , creatinine ).
• They remove foreign chemicals from
the body (i.e., drugs, pesticides; etc.).
• They secrete hormones:
–Renin inﬂuences blood pressure and
the sodium and potassium balancein the body. Renin stimulates a hor-mone (angiotensin) that stimulatesan endocrine gland, the adrenalcortex (see Chapter 9), to secrete ahormone called aldosterone, whichinﬂuences how the kidney regulatespotassium and sodium levels in thebody.
–Erythropoietin controls the produc-
tion of red blood cells.
–Vitamin D regulates calcium absorp-
tion from the intestine and inﬂu-ences calcium balance in the body.
A thin layer of white ﬁbrous tissue called
the renal capsule surrounds the kidney.The outer layer of the kidney is called thecortex ; the inner portion, the medulla .
Urine passes into the ureters through afunnel-shaped structure called the renal
pelvis . The medulla contains 10 to 15 tri-
angular structures called renal pyramids ,
which serve as a portion of the drainagesystem. Within the cortex and medulla lieunits called nephrons , which are the
functional units of the kidney. There are
approximately 1 million nephrons in each
kidney. Each nephron contains an initialﬁltering system called a renal corpuscle
(located within the cortex) and tiny tube-like structures extending from the renalcorpuscle called renal tubules (located
within the medulla). The renal corpuscleconsists of a glomerulus (a bunch of cap-
illary loops, or glomerular capillaries ) and
the Bowman’s capsule , which surrounds the
glomerulus. Extending from the glomeru-lus are the renal tubules, which end in acollecting duct called the calyx . Calyces
from each nephron merge to empty intothe renal pelvis.
The kidney ﬁlters a large volume of blood
each day. Approximately 20 percent of thebody’s blood ﬂow passes to the kidneythrough the renal arteries at a rate averag-ing about one liter of arterial blood per
minute. The remaining 80 percent of blood
remains in the general body circulation.Blood enters the kidney through the renal
artery and leaves from the kidney to enter
the general body circulation through therenal vein .
Blood entering the kidney ﬂows ﬁrst to
the glomerular capillaries, where it is ﬁl-tered, and then to a second capillary bedsurrounding the tubules (peritubular capil-laries), which form the veins throughwhich blood leaves the kidney. Theprocess by which the kidney removeswaste products from the blood is calledglomerular ﬁltration . Initial ﬁltration, which
occurs as the blood enters the glomerulus,removes some waste products. As theglomerular filtrate continues to move
through the tubules of the nephrons, sub-stances are either reabsorbed into thebloodstream or continue through thetubules. As the ﬁltrate moves into the col-lecting system, it eventually drains intothe calyx at the mouth of each pyramid
and empties into the renal pelvis as urine.
From the renal pelvis, urine drains intothe ureters to the bladder , where it is stored
until ready to be excreted through the ure-
thraand urinary meatus .
Metabolic end products and toxic sub-
stances are removed from the blood364
CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

Urinary Tract and Renal Conditions 365
through the ﬁltration process so they canbe eliminated from the body as urine.Other substances, such as sugar and amino
acids (the building blocks of protein), are
reabsorbed into the bloodstream. Electro-lytes (e.g., sodium and potassium) are alsoreturned to the bloodstream, along with99 percent of the water in the ﬁltrate.Potassium is important to muscle contrac-
tion and nerve and heart function, andsodium is important to heart and nerve
function as well as to water balance. Theamounts of water and electrolytes reab-sorbed vary and are regulated according tothe body’s speciﬁc needs; this internalchemical balance is crucial for the gener-al function of most other organs.
Because of the kidneys’ many functions,
disorders of the kidney affect many bodysystems.
URINARY TRACT ANDRENAL CONDITIONSCystitis (Lower Urinary Tract Infection)
The bladder and the urine contained
within it are usually sterile. Cystitis is a
condition in which bacteria have enteredthe bladder, causing it to become infect-ed and inﬂamed. Bacteria can invade thebladder through the external urinary mea-
tus, or infection of the bladder can be sec-
ondary to an infection in another locationof the urinary tract. Although cystitis itselfis generally not a disabling condition, itcan be a serious complication for individ-uals with chronic illness or disability.
Symptoms of cystitis may include fre-
quent urination even though the bladdermay not be full; dysuria (painful urina-
tion); pain in the lower abdomen or low-er back; and, at times, hematuria (blood
in the urine). Recurrent or inadequatelytreated cystitis can produce more seriousconsequences if infection travels up theurinary tract to the kidney, causing kidneyinfection ( pyelonephritis ). If the infection
continues and bacteria enter the blood(septicemia ), a systemic infection that is
potentially life-threatening can result.
Cystitis is diagnosed by the symptoms re –
ported and by examination of the urine for
evidence of bacteria, white blood cells, orother indication of infection or inﬂamma-tion. Treatment of uncomplicated infec-
tions includes the administration of medica-
tions, such as antibiotics . If cystitis is recur-
rent, treatment may include identifying
and removing or correcting factors that con-
tribute to the development of urinary tractinfections. Examples are structural condi-tions, such as a stricture or narrowing ofpart of the lower urinary tract that pre-vents adequate emptying of the bladder,thus predisposing individuals to infection.
Immobility, use of an external catheter,
or a generally weakened physical condi-tion can also predispose individuals to de-velop cystitis. Because of the potentiallyserious nature of this complication in indi-viduals with chronic illness or disability,
symptoms that suggest the presence of low-
er urinary tract infection should be treated
promptly. Prevention of cystitis is impor-tant to reduce the risk of serious complica-tions. Proper bladder care, adequate ﬂuidintake, maintenance of urine acidity, orperiodic urine analyses and cultures forearly detection of unsuspected infectioncan help individuals prone to cystitis pre-vent or lessen the chance of its occurrence.
Pyelonephritis
Pyelonephritis (infection of the kidney)
can be a complication of cystitis in whichbacteria have progressed to the kidneysfrom an infection of the lower urinarytract, or it can be caused from the spreadof infection elsewhere in the body to thekidney. It can also be caused by obstruc-

tion or stricture of a portion of the urinarytract that leads to stasis (stagnation) of
urine, which enhances the growth of bac-teria. Pyelonephritis may be acute orchronic. No long-term debilitation is usu-ally associated with acute pyelonephritis ,
although individuals may be acutely illwith fever and chills, ﬂank or abdominalpain, nausea, and vomiting. Prompt treat-ment with appropriate antibiotics can
eradicate the infection. If an obstructionor stricture is causing the infection, surgi-cal intervention to remove it can preventacute pyelonephritis from recurring. Ifacute pyelonephritis is not adequately
treated, or if treatment is not permanently
successful because of urinary tract obstruc-tion or stricture, chronic pyelonephritis can
develop. Chronic pyelonephritis can causeirreversible degenerative changes to kid-ney structure and function, leading torenal failure , described later in the chapter.
The diagnosis of pyelonephritis is based
on symptoms and on examination of urine
for the presence of bacteria and whiteblood cells.
Urinary or Renal Calculi (KidneyStones; Nephrolithiasis; Urolithiasis)
Ranging in size from a stone as tiny as a
grain of sand to a stone large enough to ﬁll
the inner portion of the kidney, kidney
stones ( renal calculi ) may occur anywhere
in the urinary tract. They cause severe painand can be a source of obstruction and sec-ondary infection. Some individuals appear
to be more prone to develop kidney stonesthan others. Structural or metabolicabnormalities, prolonged immobility orbedrest, and a variety of chronic illnessesand disabilities can predispose individualsto develop kidney stones.
Kidney stones may produce no symp-
toms, or they may cause excruciating painin the ﬂank or kidney area, along withnausea and vomiting, hematuria (blood
in the urine), or frequency of urination.Diagnosis is based on the symptoms, to-gether with an examination of the urinefor hematuria or bacteria. A radiologicexamination called an intravenous pyelo-
gram or retrograde pyelogram (described lat-
er in the chapter) may also be performedto detect the presence of stones and, ifthey are present, to evaluate the extent ofthe obstruction. If a stone severelyobstructs urine ﬂow, urine may back up tothe kidney ( hydronephrosis ), causing
kidney damage. Most calculi are passedthrough the urinary meatus spontaneous-
ly. If a stone does not pass spontaneous-ly, it may have to be removed surgically.The type of surgery depends on the sizeand the location of the stone. Where avail-able, a procedure called lithotripsy (de-
scribed below) may be used to break up the
stone through the use of ultrasound.
Treatment of Renal Calculi (Kidney Stones)
Lithotomy
When kidney stones do not pass out of
the body with the urine ﬂow, it may benecessary to remove them surgically. Thisprocedure is called lithotomy . Several sur-
gical techniques may be used. The surgeonmay make an incision in the lower part ofthe abdomen (a suprapubic incision ) and re-
move the stone through the incision. At
times, surgeons can remove stones from the
bladder by inserting a cystoscope into the
bladder through the urethra , passing a spe-
cial instrument through the cystoscope, and
then grasping and crushing the stone withthe instrument; this procedure is calledlitholapaxy . The crushed fragments of
stone are then passed in the urine.
Depending on the location of the stone,
other surgical procedures may also beused. A special instrument called a nephro-366
CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

scope may be inserted through the skin
directly into the kidney to remove thestone. If the kidney pelvis is entered, theprocedure is called pyelolithotomy . If the
renal calyx is entered, the procedure iscalled a nephrolithotomy . Surgical re-
moval of stones from the ureter is called
ureterolithotomy . In this procedure, an
incision is made through the lower ab-domen or ﬂank, the affected ureter is sur-gically opened, and the stone is removed.
Lithotripsy
Lithotripsy refers to a procedure used for
crushing kidney stones. This may be accom –
plished in several ways. A noninvasive pro-
cedure, extracorporeal shock wave lithotripsy ,
disintegrates kidney stones with shockwaves. Because it is not a surgical proce-
dure, it may be performed on an outpatient
basis or, if done in the hospital, with onlya minimal hospital stay. Individuals un-dergoing the procedure may be positionedin a padded chair that is lifted into a stain-less steel tub of warm water, or a ﬂuid bagmay be placed between the individual andthe source of the shock waves to serve asa buffer. Once the individual is positionedto receive maximum effect, shock wavesfrom a machine called a lithotriptor are
directed to the stones, which are visual-ized radiographically. The stones are bro-ken apart by the sound waves, and thefragments can then be passed in the urine.
Another lithotripsy technique uses a
laser instead of shock waves. The laser is
directed to the location of stone and dis-integrates the stone to small particles soit may be excreted.
Hydronephrosis
An obstruction can occur anywhere in
the urinary tract and may be caused by astone or a stricture that is related to aninfection, an injury, a congenital abnor-mality, or a tumor. Obstructions preventurine from ﬂowing through the urinarytract so that urine backﬂows into the kid-neys. Because the kidneys continue to pro-duce urine even though there is backup ofurine from the urinary tract, the kidneypelvis eventually becomes swollen anddistended. This distension is calledhydronephrosis . Obstruction of the uri-
nary tract and backﬂow of urine also pre-dispose individuals to infection of thekidney ( pyelonephritis ).
Individuals with hydronephrosis may
experience pain or may feel little discom-fort. Diagnosis of hydronephrosis is usu-ally made through X-rays. The kidneymust be drained and the obstruction rem-oved to prevent further damage to the kid-ney. The degree of disability experiencedbecause of hydronephrosis depends on thedegree of permanent damage to the kid-ney. In severe cases hydronephrosis canresult in renal failure.
Glomerulonephritis
Nephritis is an inﬂammation of the kid-
ney. Glomerulonephritis is a type of
nephritis characterized by inﬂammation
of the glomeruli of the kidney. Glomerulo-
nephritis does not result from an invasionof the glomeruli themselves by an infec-tious organism, but rather occurs as an im-munologic response to bacteria or
viruses.
The immunologic response often fol lows
an infection elsewhere in the body, suchas streptococcal pharyngitis (“strep throat”)
or bacterial endocarditis (see Chapter 11).
Glomerulonephritis may be acute or
chronic . With the acute form, symptoms
may be mild, going undetected. Whensymptoms are present, they often includehematuria (blood in the urine); protein-
uria (protein in the urine); some impair-
ment in kidney function with the reten-Urinary Tract and Renal Conditions 367

tion of salt and water, possibly leading toelevated blood pressure ( hypertension );
and edema (swelling), especially in the face
and hands. Generalized edema ( anasarca )
may also occur and may be accompaniedby other symptoms, such as dyspnea (dif-
ﬁculty breathing) on exertion, visual dis-turbances, and headache.
Although many individuals recover
completely, glomerulonephritis that is notadequately treated or that goes undetect-ed can result in irreversible, permanentstructural changes in the kidney leadingto end-stage renal disease (ESRD). The ex-
tent of kidney damage depends on thespeed and the effectiveness with whichthe process can be stopped through appro-priate treatment. Treatment of glomeru-lonephritis focuses on the symptoms andthe underlying cause.
Nephrosis (Nephrotic Syndrome)
Nephrosis is general term used to
describe conditions in which a kidney hasbeen damaged by something other thandirect infection of the kidney itself (suchas glomerulonephritis ). It is a collection of
signs and symptoms that can be caused bya variety of kidney conditions. It may bethe result of hypertension (see Chapter 11),
diabetes , glomerulonephritis (described above),
or the hyperproliferation (overgrowth) of
renal cells because of a tumor. It may also
be mediated by the immune system and ap-
pear secondary to a systemic disease, suchas rheumatoid arthritis (see Chapter 14).
The collection of symptoms experienced
in nephrosis is termed the nephrotic syn-
drome . This syndrome may include a vari-
ety of symptoms, but proteinuria (protein
in the urine) or albuminuria (albumin in
the urine) is its hallmark. When the kid-
neys are damaged, certain substances that
normally would be reabsorbed into thebloodstream during the ﬁltering processare passed through the membranes of theglomerular capillaries and excreted in the
urine. Protein is one of these substances.Thus, an important complication ofnephritic syndrome is severe protein mal-nutrition, which may require nutritionalsupplementation. Although the kidneysmay sometimes repair themselves, neph-rosis may also result in renal failure.
Polycystic Kidney Disease
Polycystic kidney disease is a hereditary
disease characterized by the presence ofmany cysts in the kidneys. The cysts en-large, compressing and exerting pressureon functioning kidney tissue. The diseaseprogresses slowly over many years. Con-sequently, individuals may be unawarethat they have the disease. Physicians maydiscover the condition by accident duringa routine examination, or, as cysts enlarge,individuals may begin to experiencesymptoms such as low back pain, hema-
turia (blood in the urine), or frequent uri-
nary tract infections.
The condition eventually progresses to
ESRD, but progression sometimes takes aslong as 20 years. Treatment of ESRD mayinclude dialysis and/or kidney transplan-tation (both discussed later in the chap-ter). Although transplantation is feasiblefor those with polycystic kidney disease,close family members may not be appro-priate donors because of the hereditarynature of the condition.
Nephrectomy
If trauma has severely injured the kid-
ney, if stones have caused severe damageor are too large to remove, or if the kid-ney is chronically infected or nonfunc-tional, the entire kidney may be removed.This surgical procedure is called nephrec-
tomy . Individuals can live normal lives368
CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

with one functioning kidney, but theyshould guard against infection or injurythat could compromise the function ofthe one remaining kidney.
Renal Failure
When the kidneys become so damaged,
or the functional capacity of the kidneyshas declined to the extent that it is insuf-ﬁcient to meet the body’s demands, indi-viduals are said to be in renal failure . Renal
failure can be acute or chronic , temporary or
permanent . Signs and symptoms of renal
failure, whether acute or chronic, dependon the:
• cause of renal failure• degree of dysfunction of the kidney• rate of renal failure
The symptoms experienced by individ-
uals whose kidneys have failed depend onthe stage of the condition. Individualsmay lose a signiﬁcant amount of kidneyfunction before any symptoms are noted.Both acute and chronic renal failurediminish the kidneys’ ability to ﬁlterblood adequately and remove water andwastes. As a result, wastes that once wouldhave been excreted by the kidney throughthe urine continue to circulate in thebody. This decreased function affects thebody’s delicate internal chemical bal-ance. Because the kidneys have multiplefunctions, kidney dysfunction has animpact on all other organ systems. Kidneydamage can eventually progress to end-
stage renal disease , in which the kidneys
essentially cease to function at all.
Acute Renal Failure
Acute renal failure is sudden and can
occur as a complication of other medicalconditions, surgery, or trauma. Dimin-ished blood volume ( hypovolemia ) dueto hemorrhage or severe dehydration, ex-treme low blood pressure ( hypotension ),
septicemia (bacteria in the blood), urinary
tract obstruction, and nephrotoxins (sub-
stances harmful to the kidney, such as cer-tain drugs, solvents, or metals) are allpotential causes of acute renal failure.
Treatment of acute renal failure is
directed toward removing the cause of thekidney failure when possible, preventingpermanent damage to the kidney andcomplications, and restoring the bodychemistry to its normal state. Dependingon the cause of acute renal failure, it canoften be reversed with no permanentdamage to the kidney. If the causative fac-tor can be corrected before irreversiblestructural changes occur in the kidney,acute renal failure may be temporary. Toprevent permanent kidney damage, treat-ment must begin immediately. Dialysismay be instituted temporarily to take overkidney function until the cause of the kid-ney failure can be corrected. Dialysis mayalso be used to remove toxic substancesfrom the body, such as in drug overdose.
Chronic Renal Failure
(End-Stage Renal Disease)
Chronic renal failure (ESRD) can result
from acute renal failure in which irre-versible damage occurs before the cause ofthe acute failure can be corrected, or it canresult from complications of a number ofother conditions related to the kidney,such as glomerulonephritis , pyelonephritis , or
polycystic kidney disease , all discussed ear-
lier in the chapter. ESRD can also resultfrom the complications of a number ofsystemic conditions, such as hypertension
(see Chapter 11, diabetes (see Chapter 9),
autoimmune diseases such as lupus ery-thematosus (see Chapter 14), or vascular
disease resulting in nephrosclerosis (con-
dition in which the arteries of the kidneyUrinary Tract and Renal Conditions 369

become thickened). It can also result fromexposure to nephrotoxins , drug toxicity, or
drug overdose .
Symptoms of End-Stage Renal Disease
Whereas acute renal failure occurs rap-
idly, chronic renal failure may progressgradually over time. ESRD is usually bro-ken into stages:
• Early-stage renal disease, with 40 to 75
percent loss of nephron function(renal impairment )
• Second-stage renal disease, with 75 to
80 percent of nephron function ( renal
insufﬁciency )
• ESRD, with less than 15 percent
nephron function ( renal failure )
(Sosa-Guerrero & Gomez, 1997)
In early-stage renal disease, symptoms
may be barely perceptible. Difficultyconcentrating or development of short-ened attention span may be the ﬁrstsymptom of renal failure. In the earlystages there may also be increased urineproduction
(polyuria ). Examination of the
urine ( urinalysis ) may reveal protein in
the urine as an early sign. Since protein is
usually re absorbed into the regular body
circulation after being ﬁltered through thekidney, the presence of protein in theurine is an indication of failure of thismechanism.
As kidney function declines, waste
products of metabolism ( urea and creati-
nine) build up in the blood, a condition
called uremia . As waste products contin-
ue to increase and circulate in the gener-al circulation, individuals may experienceoverall itching ( pruritus ).
The ﬁnal stage of renal disease is end-
stage renal disease . At this stage individu-
als must be treated through dialysis orreceive a kidney transplant if they are tosurvive. Individuals with ESRD are unableto regulate water balance in their body.Urine production is severely diminished(oliguria ) or nonexistent ( anuria ).
Consequently, water that
would have
been excreted as urine remains in the
body, creating ﬂuid overload. Althoughsome water is excreted through the gas-trointestinal and respiratory systems, aswell as through perspiration, the kidneysare the main source of ﬂuid excre
tion.
Overload of ﬂuid in the body causes stress
on the circulatory system, contributing tohypertension (high blood pressure) and
especially stressing the heart, potentiallycausing cardiac dysfunction and failure.Outward symptoms of ﬂuid overload con-sist of weight gain, edema , and difﬁculty
breathing (dyspnea ). The problem of ﬂu-
id overload is compounded if there is alsoincreased sodium in the blood, as dis-cussed in the following paragraph.
Sodium , an electrolyte, helps regulate
the ﬂuid content of the body’s tissues and,along with potassium and another electro-
lyte ( chloride ), regulates the body’s inter-
nal chemistry. In ESRD, the kidneys areunable to excrete sodium. Too much sodi-um in the body causes retention of ﬂuidand swelling ( edema ). As a result, individ-
uals may experience sudden weight gainand pufﬁness or swelling of the face, feet,ankles, legs, and at times the arms andabdomen. Individuals may have highblood pressure and complain of feelinguncomfortable and bloated. They mayalso experience difficulty breathingbecause of the ﬂuid overload on the heartand lungs.
Another electrolyte, potassium , is impor-
tant for the stimulation and relaxationthat allows muscles, including the heartmuscles and the muscles used for respira-tion, to contract. When there is too muchor too little potassium, muscle weakness
can occur as well as dangerous distur-bances in heart and respiratory function .370
CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

Normally most potassium taken inthrough the diet is excreted by the kidney;however, in ESRD the kidney is unable tosecrete potassium, so it is retained andbuilds up in the blood ( hyperkalemia ).
Excessive amounts of potassium in theblood can adversely affect organs such asthe heart, potentially causing cardiacarrest.
Calcium in the blood is decreased below
normal levels in ESRD, partially becausethe kidney is unable to produce sufﬁcientamounts of vitamin D , necessary for calci-
um absorption, and also because calciumabsorption in the intestine is decreased.ESRD also causes overactivity of theparathyroid glands (see Chapter 9), causing
calcium loss from the bone ( osteoporosis ),
further contributing to bone pain andfractures.
Individuals with ESRD
may become
malnourished because of anorexia (loss of
appetite), nausea and vomiting, or lack ofappetite due to depression. Anemia (re-
duction of circulating red blood cells)accompanied by iron deﬁciency is charac-teristic in renal failure, not only becauseof diet, but also because the kidneys areno longer able to produce erythropoietin , a
hormone responsible for initiating redblood cell production, and because indi-viduals with ESRD have decreased abilityto absorb iron from the intestine. As aresult, they experience weakness and lowexercise tolerance. It may be difﬁcult formany individuals with ESRD to walk veryfar without resting.
Walking may be further impeded by a
condition called peripheral neuropathy ,
which is common in individuals withESRD. Uremia has toxic effects on nerves,and especially the peripheral nerves of thehands and feet, resulting in peripheral
neuropathy , which causes weakness and
loss of sensation in the arms and legs. Thecentral nervous system may also be affect-ed in ESRD. Intellectual impairment maycoincide with worsening uremia , so that
individuals have increased difﬁculty con-centrating or demonstrate a shortenedattention span.
Because of both the physical and emo-
tional changes present in ESRD, individ-uals with ESRD may also experienceimpaired sexual function (Ifudu, 1998).Not only may there be diminished inter-est in engaging in sexual activity, but theremay also be diminished physical responseto sexual stimulation in both males andfemales.
Treatment and Management of
End-Stage Renal Disease
Because there is no cure for chronic
renal failure, treatment is directed at con-trol. In very early stages of chronic renalfailure, treatment may include restrictingwater to an amount equal to urine output,carefully monitoring body weight, andmanaging the diet to provide adequatenutrition without overtaxing the kidneywith metabolic waste products. The kid-ney can normally continue to functionwith as little as 10 percent of its function;however, loss of function beyond thispoint requires either dialysis or renaltransplant in order for individuals to sur-vive (Teichman, 2001).
Diet. When kidney function is signif-
icantly reduced, individuals’ diet and ﬂu-id intake must be regulated to conform tothe limited or absent function of the kid-neys. The intake of protein, sodium,potassium, ﬂuid, and calories must becarefully regulated and monitored to:
• minimize waste products in the body • maintain electrolyte levels in the
body within normal limits
• avoid either too much or too little
ﬂuid in the bodyUrinary Tract and Renal Conditions 371

Diets are prescribed individually. There
is no one diet that is appropriate for allindividuals with ESRD. Individuals withESRD work closely with dietitians (indi-
viduals who specialize in the science ofapplying nutritional information to theregulation of diet) to develop a dietplan right for them. The type of dietaryprescription is based on individual needsand the type of kidney disease the individ-ual has.
Since, in ESRD, the kidney is no longer
able to ﬁlter out the waste products of pro-tein metabolism, the dietary intake of pro-tein must be controlled. Consequently,dietary intake of foods especially high inprotein, such as meat, ﬁsh, eggs, poultry,and dairy products, is restricted.
Because in ESRD the kidneys are unable
to excrete potassium and because of theadverse, and potentially fatal, effects ofhigh levels of potassium on heart muscleas well as on other muscles in the body,potassium buildup in the blood ( hyper-
kalemia ) must be avoided. Dietary intake
of foods rich in potassium must, therefore,be restricted. Examples of foods high inpotassium content are listed in Table 13–1.The more severe the loss of kidney func-tion, the more carefully potassium levelsmust be regulated.
Because sodium is important in the reg-
ulation of ﬂuid in the body, as well as inmaintaining the body’s internal chemicalbalance, and because the kidneys in ESRDcannot excrete sodium effectively, individ-uals with ESRD must also regulate theirdietary intake of sodium. Sodium restric-tions affect the intake of not only salt butalso a number of other foods high in sodi-um. Foods containing high levels of sodi-um must be restricted. (See Table 13–2.)
Since individuals with ESRD are unable
to regulate ﬂuid balance, the amount ofﬂuid taken orally must be restricted. In-
cluded in ﬂuid intake are ice cubes, gelatindesserts, sherbet, or any other food thatliqueﬁes at room temperature. Physicianscalculate the amount of ﬂuid individualsmay have based on the amount of ﬂuidlost through perspiration and respiration.Adequate ﬂuid intake is based on thepatient’s weight gain at speciﬁc intervals.In some instances ﬂuid intake may be re-stricted to no more than one cup of ﬂuidper day.
Table 13–1 Foods High in Potassium
oranges, orange juice strawberriesgrapefruit, grapefruit juice raisinsbananas beetsapricots cabbagecantaloupe carrotskidney beans celerypears many breakfast
cereals
potatoes many breadsspinach many nutstomatoes salt subtitutespeaches
Table 13–2 Foods High in Sodium
corned and chipped beefbaconhamcold cuts such as bolognapork sausagecanned tuna, salmon, sardinespork sausagehot dogscheddar and Swiss cheesesnack foods such as pretzels, popcorn, potato
chips, some crackers
most canned vegetables olivessoft drinks372 CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

Because of food restrictions, individuals
may have difﬁculty maintaining sufﬁcient
caloric intake. Foods low in sodium, potas-sium, and protein may be needed through
–
out the day as supplements to add addi-tional calories. For in-between snacking,the dietitian may recommend specializedproducts that are high-calorie, low-pro-tein, and low-electrolyte foods.
Medications. Because of dietary restric-
tions in the intake of dairy products andother foods high in calcium, individualswith ESRD may experience calcium deple-tion. Consequently, calcium supplementsas well as supplements of vitamin D deriv-atives normally produced in the kidneyare also given by mouth. The physicianmay also prescribe supplementary vita-mins and certain minerals.
Oral iron supplements are frequently
given as part of the medical treatment ofESRD, as well as erythropoietin (a sub-
stance normally produced in the kidneythat stimulates production of red bloodcells). The medication is given subcuta-
neously (into the fatty tissue under the
skin) three times a week after each dialysistreatment (discussed later in the chapter).
Individuals with ESRD are often placed
on antihypertensive medications to help
control blood pressure. Excess ﬂuid isremoved through the process of dialysis,discussed below.
Dialysis. Individuals with renal failure,
whether acute or chronic, cannot surviveunless there is a method for compensat-ing for kidney function. Dialysis performsthe function of the kidney, that of remov-ing waste and ﬂuid from the body. It maybe used temporarily , as in the case of acute
renal failure , or it may be used to sustain
life when kidney damage is irreversibleand permanent, as in ESRD. For individ-uals with acute renal fail
ure, dialysis is alife-saving necessity. When individuals
reach ESRD, either dialysis or a transplantis necessary for survival.
If individuals with chronic renal failure
or ESRD are suitable candidates for renaltransplantation, dialysis may be used un-til an appropriate donor kidney is avail-able. Not all individuals with ESRD aresuitable
candidates for kidney transplant.
If a trans plant is not feasible, individuals
with ESRD must remain on dialysis therest of their lives.
There are two types of dialysis, peritoneal
dialysis and hemodialysis . Both types sim-
ulate kidney function in that:
• waste products of metabolism are
removed from the blood
• an appropriate balance in the body
chemistry is maintained
•
excess ﬂuid is removed from the blood
Both types of dialysis involve the use of
a semipermeable membrane , a porous mate-
rial that allows some substances to passthrough but keeps other substances in theblood. The blood of individuals undergo-ing dialysis is on one side of the mem-brane, and a specially prepared solutioncalled a dialysate is on the other side of
the membrane. Difference in concentra-tions of the blood and dialysate allow cer-tain particles, but not others, to pass fromthe blood, through the membrane, andinto the dialysate, where they can then beremoved through dialysis.
The development of hemodialysis for
ESRD has enabled many people who nor-mally might have died from their condi-tion to live useful and productive lives(Himmelfarb, 2002). However, dialysis isnot without risk. The mortality rate forindividuals with acute renal failure is high(Bonventre, 2002). Individuals with ESRDreceiving dialysis have a ﬁrst-year mortal-ity rate of approximately 25 percent (USRenal Data System, 2001). Since most peo-Urinary Tract and Renal Conditions 373

374 CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS
Peritoneal
cavityDialysate
Catheter
adapter
Catheter
Used dialysate
and waste
products
Figure 13–2 Peritoneal Dialysis.
ple with ESRD have other systemic condi-tions that have caused or contributed totheir renal failure, improved outcomes indialysis may depend on improved medicalcare in the early stages of chronic renal
disease (Kinchen, Sadler, Fink, et al., 2002).
Peritoneal Dialysis. In peritoneal dialysis
the semipermeable membrane needed for
dialysis consists of the peritoneum (the thin
membrane that lines the abdominal cav-ity). A tube or catheter is surgically placedwithin the abdominal cavity. During peri-toneal dialysis, dialysate from a bag is
drained through the catheter into theabdominal cavity (see Figure 13–2). Thecatheter is clamped, and the dialysate isleft in the abdominal cavity for a speciﬁedamount of time. During this time, wasteproducts and excess ﬂuid pass from theblood, through the peritoneal mem
brane,
and into the dialysate. At the end of the
speciﬁed period, the catheter is unclamped,
and the dialysate, which now contains thewaste products and excess ﬂuid, is drainedfrom the body through the catheter. The

tube is again clamped and remains inplace for the next dialysis treatment.
Peritoneal dialysis is performed at
home, manually or with a machine.There are several methods of peritonealdialysis:
•Continuous ambulatory peritoneal dial-ysis (CAPD )
•Intermittent peritoneal dialysis (IPD)
•Continuous cycling peritoneal dialysis(CCPD )
Regardless of the method used for peri-
toneal dialysis, the principles remain thesame. With CAPD, dialysate is instilled
in-
to the abdominal cavity manually, using
gravity. A bag of dialysate solution is con-nected to the catheter. The individualthen elevates the bag, causing the dialy-sate to ﬂow into the abdominal cavity. Thecatheter is clamped and the dialysate leftin place for 4 to 8 hours. The catheter isthen unclamped and the bag lowered sothat the dialysate drains from the abdom-inal cavity by gravity. When the bag is full,the individual detaches the bag from thecatheter, attaches a new bag of dialysate,and begins the process again. Individualschange the dialysate manually three toﬁve times a day. Individuals using thistype of peritoneal dialysis are able to con-tinue their regular daily activities, stop-ping only for periodic intervals to drainthe dialysate and attach a fresh bag.
IPD and CCPD both use a machine.
Intermittent peritoneal dialysis is performed
three or more times a week , with each
exchange lasting for 10 or more hours.With IPD, the catheter is connected to thecycling machine at night. The exchangetakes place while individuals sleep; conse-quently, they are free to engage in theirregular activities during the day. CCPDalso uses a cycling machine but is per-formed daily. In CCPD the catheter is con-nected to the cycling machine, whichperforms multiple solution exchangeswhile the individual sleeps. In the morn-ing, individuals disconnect the catheterfrom the machine, leaving the last solu-tion in the abdomen all day while theyengage in their regular activities.
Peritoneal dialysis may be chosen as the
dialysis method for individuals who have,in addition to kidney disease, other med-ical conditions that increase the risk ofcomplications associated with hemodial-ysis. In other instances, peritoneal dialy-sis may be chosen because of the relativeease of the procedure and the limited useof sophisticated equipment, factors thatenable individuals to use peritoneal dial-ysis in the home. Depending on the typeof procedure used, individuals may enjoymore mobility with peritoneal dialysisthan with hemodialysis. If severe vasculardisease interferes with the blood supply tothe peritoneum or there is an increasedvulnerability to infection, however, perito-neal dialysis may be contraindicated.
Although generally a safe procedure,
peritoneal dialysis can have a number ofassociated complications. The most com-mon is peritonitis (inﬂammation of the
peritoneum) caused when the peritoneumis contaminated with bacteria. If peritoni-tis develops, antibiotics may be used totreat the infection, or peritoneal dialysismay be discontinued and hemodialysisbegun. Other complications that mayoccur as a result of peritoneal dialysis areplugging or displacement of the catheter,development of hernias , or painduring dialy-
sis. Over time, infection or the dialysateconcentration itself may damage the peri-toneum. Peritoneal dialysis is usually alimited procedure because of the loss ofmembrane function.
Hemodialysis. The most common type of
dialysis used for individuals with ESRD ishemodialysis , in which an artiﬁcial kid-Urinary Tract and Renal Conditions 375

376 CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS
Artery
Artery
Artery FistulaVeinGraft
Shunt Vein
Vein
Figure 13–3 Access for Hemodialysis Through a Graft, a Shunt, or a Fistula.ney machine ( dialyzer ) circulates and ﬁl-
ters the blood outside the body to removewaste products and excess ﬂuid. Hemo
dial-
ysis requires that there is access to indi –
viduals’ circulation. Access routes throughwhich blood is removed from the individ-ual to be circulated through the dialysismachine and then returned to the individ-ual’s circulation may be surgically createdthrough a graft, an external arteriovenous
shunt (less commonly used today), an
internal arteriovenous ﬁstula (Figure 13–3),
or a subclavian cannula (see Figure 13–4).
Access routes are created surgically,
most often in the forearm. Grafts that con-
nect an artery and a vein may be made bysurgical placement of synthetic material orof a vein that has been removed fromanother part of the individual’s body. The
external arteriovenous shunt consists of sur-
gical placement of a tube ( cannula ) under
the skin to connect an artery to a vein.The internal arteriovenous ﬁstula is also cre-
ated surgically. In this procedure, an arteryis joined to a vein underneath the skin,establishing an opening called a ﬁstula be-
tween the two. Shunting arterial bloodinto the vein causes the vein to becomethickened and enlarged so it can be re-peatedly used as an access to the circula-tion for dialysis. It may take from 2 to 6weeks for the ﬁstula to become thickenedand enlarged enough so that it can beused for dialysis. In the meantime, tem-porary access may be maintained throughthe subclavian artery.

For hemodialysis, a large needle is
placed into the artery side of the accessroute. Another large needle is placed inthe vein side of the access. Tubes areattached to the needles and connected tothe dialysis machine. Blood moves fromthe ﬁrst tube to the dialysis machine,where it is cleansed and ﬁltered. Thecleansed blood is then returned to theindividual through the second needle inthe vein.
The artiﬁcial kidney has two compart-
ments, one for the individual’s blood andone for the dialysate solution. A synthet-ic semipermeable membrane separatesthese compartments within the artiﬁcialkidney. Blood cells and other importantsubstances are too large to pass throughthe pores of the membrane, so theyremain in their compartment. Most wasteproducts, however, are small enough topass through the membrane into thedialysate, and they are washed away. Thecleansed blood then returns through thetube to the individual.
Hemodialysis is usually performed for
3 to 6 hours a day three times a week(Owen, Pereira, & Savegh, 2000). It hasalso been performed successfully on a dai-ly basis for 2 hours a day (Schifﬂe, Lang,& Fischer, 2002). Hemodialysis can beperformed at a kidney dialysis center or,in some instances, at home. Becausehome hemodialysis requires a high degreeof individual control, self-destructivetendencies in individuals or unwillingnessof family members or caregivers to partic-ipate in the procedure is a contraindica-tion for home hemodialysis. Homedialysis requires someone (a family mem-ber, or someone who has been hired) whohas been trained to assist with the pro-cedure. The level of responsibility as wellas the need to be always present at speci-ﬁed times of dialysis can cause consider-able stress for the helper, especially if heUrinary Tract and Renal Conditions 377
Subclavianvein
Subclaviancannula
Figure 13–4 Access for Hemodialysis Through a Subclavian Cannula.

or she is a family member. Stress levelsassociated with home hemodialysis areusually evaluated and monitored beforesuch a home dialysis program is imple-mented.
The success of hemodialysis depends
on individuals’ level of motivation, thepresence of other medical conditions thatmay cause complications, and the devel-opment of complications from the hemo-
dialysis itself. The numerous potential com-
plications related to hemodialysis rangefrom technical problems with the accessroute to more generalized complicationsthat could result in death. Arteriovenousgrafts and subclavian catheters are espe-cially prone to infection that can result insepticemia (toxins in the blood), a poten-
tially life-threatening complication.Cardiac-related complications, such aspericarditis (inﬂammation of the outer
layer of membrane surrounding the heart),
myocardial infarction (heart attack),
arrhythmias (irregular heartbeat), or
hypertension (high blood pressure), may
also occur (Ifudu, 1998). Another possiblecomplication of hemodialysis is stroke orsome other thrombolytic event. Becauseof the risk of clot formation in the accessroute, individuals on hemodialysis mayreceive anticoagulant medication during the
procedure. Administration of this medica-tion, however, may also increase the riskof bleeding. Hemodialysis itself is painless,although some light discomfort mayoccur when needles are inserted for dial-ysis. Although during dialysis individualsare conﬁned to the dialysis unit, they canread, watch television, or sleep.
Rapid changes that occur in ﬂuid and
chemical balances in the body during dial-ysis cause some individuals to experiencenausea, vomiting, headaches, or musclecramps in association with hemodialysis.Some individuals may become anemic,and some experience sleep disturbances ormental cloudiness. Individuals on pro-longed hemodialysis may have changes innerves of the extremities resulting inperipheral neuropathy (loss of sensation
and weakness in the arms and legs).
Hemodialysis can relieve many of the
symptoms of renal disease, but not all.Individuals on hemodialysis may developsecondary conditions that increase the riskof bone fractures, or the procedure maynot adequately clear all wastes, leading tofeelings of weakness. The degree of func-tional capacity varies from individual toindividual. Many continue to lead near-normal lives, however, except for theirdialysis treatments.
Renal Transplantation
Renal transplantation involves surgical-
ly placing a kidney from another individ-ual inside the body of an individual withrenal disease. The diseased kidney is notremoved from the individual receiving therenal transplant. Transplants may be re-ceived from a family member ( living-relat-
ed donor ), an individual who is not related
to the individual ( living-unrelated donor ), or
an individual who has recently died(cadaver donor ). Regardless of the status of
the donor, the donor’s blood and tissuesmust closely match those of the recipientto decrease the chances of the body’s rejec-tion of the transplant (El Nahas, Harris, &Anderson, 2000).
Although renal transplant is common-
ly performed because of ESRD, it may alsobe performed if one kidney has beenremoved and the second kidney is injuredor ceases to function. Renal transplantfrees individuals from the
restrictions
associated with dialysis, diminishes manysymptoms of chronic renal fail
ure, and
improves overall quality of life.
Before being considered for renal trans-
plant, recipients undergo careful and378 CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

thorough medical evaluation to see if theyare suitable candidates. General health,including the presence of other diseasesthat may potentially affect the success ofthe transplant or make it impossible, areevaluated. Pre-transplant evaluation alsoincludes a thorough psychological evalu-ation. Discussion of the risk of rejectionand infection and the lifelong need to useimmunosuppressants with their corre-sponding risk (discussed below) is a partof the pre-transplant protocol.
Expectations for the transplant are dis-
cussed with both the recipient and the liv-ing donor. Also evaluated is the recipient’sability to adjust to the transplant as wellas his or her ability to adjust should thetransplant fail. The degree of family andsocial support is also assessed. The cost ofthe kidney transplant itself, as well as thecost associated with travel to the trans-plant center, lodging for family, and thelifelong medication needed after thetransplant, can be staggering. Conse-quently, the social service evaluation also
includes the ﬁnancial status of candidatesfor transplant and the identiﬁcation ofresources that can help to cover costs.
Scarcity of donors is the major factor that
inhibits renal transplantation. Whetherthe donor is a living donor or cadaverdonor, compatibility of tissue type andblood type and a variety of other factorsdetermine the degree of success of thetransplantation. Tissue typing is most im-portant in decreasing the possibility ofrejection. The most desirable sources ofkidneys for transplantation are closely re-lated, living donors, but surgeons in newprotocols are attempting to use living,unrelated, and blood type-incompatibledonors (Delmonico, 2004).
At the time of transplant, both donor
and recipient are hospitalized. The indi-vidual with ESRD has dialysis the daybefore the transplant. The donor has renalangiography (discussed elsewhere in thechapter) to determine which kidney willbe used for the transplant. The surgicalprocedure for the kidney transplant con-sists of removing the kidney from thedonor and placing it in a surgically con-structed pocket in the lower abdomen ofthe recipient.
Once the donor kidney has been trans-
planted, it may begin to function immedi-ately. Early functioning of the trans-planted kidney is a good prognostic signfor success of the transplant. When therecipient is discharged from the hospital,he or she must be careful to avoid infec-tion due to the immunosuppressant drugsused to prevent rejection of the new kid-ney. Risk of infection is greatest the ﬁrst6 months after surgery (Soulillou, 2001).During this time, the individual needs tobe particularly careful to avoid contactwith persons with communicable disease.After transplant, prophylactic antibioticsare to be administered prior to any den-tal work. After transplant, the individualreceiving the kidney may return to workwithin 3 weeks to 1 month. Because of theextensive surgical procedure necessary forremoving the kidney from the donor,recovery time for the donor is consider-ably longer.
The major complication of kidney
transplantation is rejection, which candestroy the transplanted kidney (Pascual,Theruvath, Kawai, Tolkoff-Rubin, &Cosimi, 2002). Although the rejection ratein the ﬁrst year after renal transplant hasdramatically decreased over the lastdecade (Hariharan et al., 2000), long-termsuccess is less dependable, with about halfof individuals receiving a cadaver kidneylosing it after 10 years (Marsden, 2003).The body’s defense system, or immunesystem, naturally attacks foreign sub-stances in the body. Unfortunately, theimmune system does not distinguishUrinary Tract and Renal Conditions 379

between a life-saving transplanted kidneyand harmful substances. To prevent rejec-tion, medications called immunosuppres-
sants are prescribed after transplant. These
medications block the body’s normalimmune response. Unfortunately, im-munosuppressants can cause a number ofcomplications, including an increasedrate of malignancy (cancer), susceptibili-
ty to infection , formation of cataracts , and
degeneration of bone . If rejection takes place
immediately, it may be necessary toremove the transplanted kidney to avoida generalized body reaction that could befatal. Rejection most commonly occurswithin the ﬁrst 6 weeks after the trans-plantation; however, chronic rejectionmay occur months or years later.
Psychological Issues in
End-Stage Renal Disease
The initial shock and realization of kid-
ney failure and its ramiﬁcations may beimmobilizing. Reactions vary in degreefrom severe depression to total denial.Denial can be helpful in reducing stresslevels, but it can be life-threatening if itleads to noncompliance with the recom-mended treatment.
Individuals who begin dialysis may also
have a period of adjustment. When ﬁrstbeginning dialysis, individuals may behopeful and conﬁdent because of theimmediate physical improvements theyexperience after dialysis. In the early ses-sions of dialysis, individuals may experi-ence apprehension or uneasiness aboutthe possibility that the machine may mal-function. As they become more comfort-able with dialysis, these fears generallysubside.
As the individuals continue in dialysis,
they may become discouraged and disen-chanted when they come to the realiza-tion that there is no hope that thekidneys will miraculously begin to func-tion again. They may experience loss ofself-esteem and feelings of helplessnessand inadequacy as they come to recognizethat they are dependent on the dialysismachine for their existence. Fears ofdeath may then conﬂict with fears of con-tinuing to live a life sustained by dialysiswith its subsequent restrictions. Manyindividuals reach a stage of acceptance inwhich the limitations and complicationsof dialysis are incorporated into their lives.Even when individuals reach this stage ofadjustment, however, there may be alter-nating periods of depression.
Anger and hostility are frequent mani-
festations of conﬂicts between dependen-cy and independence—even conflictsbetween living and dying. Feelings of hos-tility may be expressed openly, but theymay also be internalized as individuals ondialysis realize their degree of dependencenot only on a machine, but also on thosewho provide their care. These internalizedfeelings can be self-destructive if individ-uals rebel against the necessary care andtreatment. Feelings of sadness, hopeless-ness, and despair may become so severethat individuals with ESRD consider sui-cide as a way to resolve the problems sur-rounding the condition. Suicide attemptsmay be subtle and covert, such as notadhering to the diet, taking in too muchﬂuid, or in other ways failing to cooper-ate with treatment.
Transplantation involves a number of
psychological issues as well. Individualsidentiﬁed as eligible recipients of a trans-planted kidney are often elated about theanticipated improvement in their qualityof life after the transplantation. Conse-quently, rejection of the transplanted kid-ney can be devastating. Even if rejectiondoes not occur and quality of life is signif-icantly improved, individuals with atransplanted kidney may express disap-380
CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

pointment that long-term care and eval-uation are still necessary. In addition, theymay be disappointed if transplantationhas not restored the state of health thatwas theirs before the onset of ESRD. Evenafter the initial postoperative period, thechance of later rejection or the risk thatinfection will damage the transplantedkidney may be sources of anxiety.
Individuals may also go through several
stages of adjustment after transplantation.Despite signiﬁcant psychological prepara-tion usually prior to transplantation,postsurgical psychological reactions maystill occur. If the donor is a family mem-ber or friend of the recipient, the relation-ship may be altered. At times the relation-ship is strengthened, but it may alsobecome weakened. Recipients may expe-rience guilt, anxiety, or depression becauseof the donation of the kidney theyreceived. If the donor felt pressure, eitherreal or imagined, to donate a kidney, heor she may experience stress, conﬂict, orguilt, especially if he or she decided todecline, or resentment if he or she donat-ed the kidney under duress. If the kidneywas received from a cadaver donor, recip-ients may have fantasies about embody-ing the spirit of someone who is dead.Whether the transplant was from a livingor cadaver donor, recipients may still feelthat the kidney is not a part of them.
Lifestyle Issues in End-Stage Renal Disease
Individuals with renal failure face pro-
found changes in the activities of daily liv-ing. When kidney function is impaired ornonexistent, intake of foods and ﬂuidsmust be carefully monitored. Such restric-
tions are necessary to minimize the amount
of waste products and to avoid the pres-ence of too much or too little ﬂuid in thebody. Because in chronic renal failure orESRD the kidney is no longer able to ex-crete adequately, total ﬂuid intake be-tween dialysis sessions must also be mon-itored. Fluid intake includes not onlybeverages, but also water contained infoods. Individuals are weighed beforeand after each dialysis treatment to mon-itor ﬂuid gain so that the dialysis proce-dure may be adjusted accordingly.
Although there are no limitations or
restrictions regarding sexual activity, sex-ual function is impaired in many individ-uals on dialysis. Some men with ESRDexperience impotence or have a dimin-ished interest in sexual activities. Womenwith ESRD often report general disinterestor a diminished interest in sexual activi-ties, or a decreased response to sexualstimulation. Sexual dysfunction amongindividuals on dialysis probably resultsfrom a combination of generally poorhealth and emotional reactions to a life-threatening illness. The reproductivecapacity of both men and women on dial-ysis is severely diminished. Sexual func-tion may improve after renal transplant,and conception is possible. If the trans-plant is rejected or the individual is heav-ily medicated, sexual function may beimpaired.
Social Issues in End-Stage Renal Disease
Renal conditions may not affect individ-
uals’ social activities until the kidneys be-come dysfunctional, causing restrictionsor alterations in regular activities. Al-though family, friends, and associates playan important supportive role in individ-uals’ adjustment to kidney failure, anoverindulgent attitude can impede indi-viduals’ return to the earlier level of inde-pendence. Individuals on dialysis mayhave to alter activities, both because oftheir physical condition and because ofthe dialysis schedule. Those with shuntsshould avoid any activity that could ex-Urinary Tract and Renal Conditions 381

pose the shunt area to potential injury.Because heat intolerance is often associat-ed with ESRD, activities requiring expo-sure to heat should be avoided.
Previously existing relationship prob-
lems may be ampliﬁed after a diagnosis ofESRD. Additional stress brought on bydialysis or the wait for transplantationmay intensify discord if it exists. If dialy-sis is conducted at home, the family mem-ber assisting with dialysis may feelburdened and strained by the addedresponsibility and regimen of the dialysisprogram, since activities must be pro-grammed around the dialysis schedule.Individuals’ physical complaints, fatigue,and loss of interest in sexual activities maycompound the problem. Although ﬁnan-cial assistance for dialysis is usually availa-
ble through government or private agencies,
the overall ﬁnancial burden of medical bills,
dialysis, and lost income if the individualwith kidney disease is not able to contin-ue working exerts additional stress on rela-
tionships. Even if these individuals feel well
enough to participate in social activities,many activities may be altered because ofdietary and ﬂuid restrictions. Individualsmay be reluctant to accept the dinner invi-tations of friends because of dietary re-strictions, or they may themselves give upentertaining because of the limitations oftheir condition. Increasing social isolationcan increase loss of self-esteem, feelings ofdepression, and hopelessness.
Vacations are still possible but require
careful planning for individuals who areon hemodialysis. Dialysis units near thevacation spot must be located, and ar-rangements must be made for dialysis atthe center prior to departure. Peritonealdialysis, although offering more ﬂexibili-ty, also requires that individuals plan fortravel. Depending on the amount oftime away and the method of travel, theymay need to prearrange shipment ofdialysate or the cycling machine to theirdestination.
Although transplantation can free indi-
viduals from some limitations, otherissues may arise. If the donor is a familymember or friend, a strong bond maydevelop between the donor and the recip-ient; in some cases, however, problemsoccur in the relationship. The donor mayresent the attention paid to the recipientafter the transplant or may feel aban-doned. The recipient, on the other hand,may have feelings of guilt because of thepotential jeopardy to the donor, who isleft with only one kidney.
Vocational Issues in
End-Stage Renal Disease
As ESRD progresses and symptoms
become more pronounced, the impact onvocational function increases. Fatiguemay necessitate a shortened workday orrest periods during the day. Problems ofimpaired judgment, difﬁculty with mem-ory, or irritability may interfere withadequate job performance. Peripheralneuropathy may make it difficult orimpossible to perform tasks such as liftingor to complete tasks that require manualdexterity.
Individuals on dialysis may need a ﬂex-
ible work schedule to accommodate thedialysis schedule. Many dialysis centers areoperational 24 hours each day, enablingindividuals to arrange dialysis in off-hours.Blood access routes, such as shunts, re-quire protection; occupations that pose apotential threat of damage to the shuntshould be avoided. Fatigue or the de-creased ability to walk caused by periph-eral neuropathy may necessitate a changeto a more sedentary line of work. Environ-mental issues should also be considered.Work that requires exposure to high tem-peratures should be avoided because of the382
CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

heat intolerance associated with kidneydiseases.
DIAGNOSTIC PROCEDURES FORRENAL AND URINARY TRACTCONDITIONSUrinalysis
Urine may be examined by direct visu-
alization, under a microscope, or throughother laboratory tests. The urinalysis re-port contains information about the con-centration, acidity, and appearance of theurine, as well as the presence of any oth-er components such as protein, sugar,blood, bacteria, or various types of cells inthe urine. A urinalysis not only providesa gross estimate of kidney function, but italso permits identiﬁcation of other poten-tial problems (e.g., infection) or systemicconditions (e.g., diabetes) that may exist.
A urinalysis is often a screening test to
help determine what, if any, other tests areneeded. Collection of urine can be exter-nal or, if a sterile specimen (one that isuncontaminated by organisms outside theurinary tract) is needed, through a tube orcatheter passed through the externalopening (urinary meatus) into the bladder.
Urine Culture
Laboratory examination of a sterile
urine specimen, a urine culture , helps to
determine whether there is an infectionwithin the urinary tract and, if so, whichorganisms are causing the infection. Speci-mens for a urine culture are usuallyobtained through catheterization.
Blood Urea Nitrogen
A blood test in which the level of urea
nitrogen (a waste product of protein metab-
olism) in the blood is measured can behelpful in evaluation of kidney function.The kidneys normally excrete urea nitro-gen. The presence of urea nitrogen in theblood indicates potential kidney impair-ment. The urea level may also be elevat-ed in conditions other than renal disease.Conditions such as starvation, dehydra-
tion, or conditions in which the blood sup-
ply to the kidneys is poor may also causeelevated urea nitrogen levels.
Serum Creatinine
Creatinine is a waste product of a high-
energy compound (creatine phosphate)found in skeletal muscle tissue, and it isusually ﬁltered out of the blood throughthe kidney. Elevation in creatinine levelsin the blood indicates damage to a largenumber of nephrons . Determination of the
creatinine level is a more sensitive test
than that of the blood urea nitrogen level
and is a better reﬂection of kidney function.
Creatinine Clearance Test
The creatinine clearance test is used to
determine the kidneys’ glomerular ﬁltration
rate. It involves a comparison of the amount
of creatinine in the blood serum ( serum
creatinine ) with the amount of creatinine
excreted in the urine over a speciﬁed peri-od of time. For the test, individuals collectand save all their urine during a speciﬁedperiod of time. Blood tests are performedat various points during that time period,and the amounts of creatinine in theblood serum and in the urine are com-pared. A decreased creatinine clearancerate indicates decreased glomerular func-tion and, thus, kidney dysfunction. Thecreatinine clearance rate is a better indi-cator of renal dysfunction than is themeasurement of serum creatinine alone.Creatinine clearance tests may be used todiagnose kidney dysfunction or to evalu-ate the progress of renal disease.Diagnostic Procedures for Renal and Urinary Tract Conditions 383

Kidney, Ureter, and BladderRoentgenography (KUB)
A simple X-ray of the kidney, ureters,
and bladder is called a KUB. The X-ray ﬁlm
outlines the size, shape, and location ofthese structures, but it does not indicatekidney function.
Intravenous Pyelogram
Radiologic examination of the kidneys,
ureters, and bladder through an intra-
venous pyelogram may be done on an out-
patient basis. During the test, a special dyeis injected into a vein in the individual’sarm. The dye is ﬁltered by the kidney andexcreted through the urinary tract, duringwhich time X-ray ﬁlms are taken at inter-vals for approximately 1 hour. The intra-venous pyelogram helps to identify notonly any structural abnormalities of thekidney, but also any problems withpassage of the dye through the urinarysystem. Because some individuals arehypersensitive to components of the dyeand may have severe allergic reactions,questions about known allergies and skintests are usually routine prior to testing.
Cystoscopy
A urologist (a physician who specializes
in the diagnosis and treatment of condi-tions of the urinary tract) may visualizethe urethra and bladder directly through a
special tube, called a cystoscope , inserted
through the urinary meatus and urethra
into the bladder . This procedure is called
cystoscopy and can be used either as a diag-
nostic procedure or as a part of treatment.It may be performed on an outpatient orinpatient basis and may be performedunder local or general anesthesia. The cys-toscopic examination makes it possibleto identify any abnormalities in the inter-nal structure of the bladder, as well asto remove foreign objects or calculi fromthe bladder, to remove tumors or otherabnormal tissue from the bladder, or toperform a retrograde pyelogram , described
below.
Retrograde Pyelography
Retrograde pyelograms are performed to
assess the function of the kidneys and
ureters and to detect possible abnormali-
ties or obstructions in the collecting system.
During a retrograde pyelogram, a smallcatheter is inserted through a tube ( cysto-
scope ), which is then directed into the
ureters to the pelvis of the kidney. A spe-
cial dye is injected through the catheterand X-ray ﬁlms are taken to visualize thecollecting system. The procedure may bedone on an outpatient or inpatient basis.
Renal Biopsy
In some cases, it is necessary to remove
a small piece of kidney tissue for the diag-nosis of kidney disease. This procedure iscalled a renal biopsy and may be done in
several ways. One method involves a sur-gical incision over the kidney so that thephysician can directly view the kidneyand remove the specimen. Because thisprocedure is done under a general anes-thetic in a hospital setting, it has a pro-longed recuperation period. The second,more commonly used method involvesthe insertion of a specially designed nee-dle through the skin over the kidney. Theneedle is then inserted into the kidney,and a small amount of kidney tissue isremoved. This technique is called a percu-
taneous renal biopsy . It is generally per-
formed under a local anesthetic in ahospital setting. Because it requires noincision or general anesthesia, only lim-ited recuperation time is needed.384
CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

Renal Angiography
In order to examine the vascular func-
tion of the kidney, a diagnostic procedurecalled renal angiography may be done. The
procedure is performed by inserting a nee-dle into the femoral artery (located in the
groin). A small catheter is passed throughthe needle into the artery and advanceduntil it reaches the renal arteries. Dye isthen injected through the catheter, and X-ray ﬁlms are taken at 2- to 3-second inter-vals to examine the functioning of therenal artery.
PSYCHOSOCIAL ISSUES IN RENALAND URINARY TRACT CONDITIONSPsychological Issues
Not all kidney conditions are life-
threatening, and they do not all imposemajor changes in functional capacity.Although they may cause some pain anddiscomfort, conditions such as cystitis fre-
quently leave no functional or psycholog-ical sequelae. ESRD, however, has aprofound impact on all areas of individu-als’ lives, causing signiﬁcant psychologi-cal stress.
Psychological changes are associated
both with emotional reactions to a life-threatening disease and with the physio-logical changes that occur with ESRD.Emotional reactions to end-stage kidneyfailure vary. Mourning over the loss ofbody function or loss of control, feelingsof disconnectedness, and anger are allemotional reactions commonly reported(Moua, 2000). Elevated levels of toxicwaste in the blood can produce cognitivechanges, such as impaired judgment,drowsiness, and difﬁculty with concentra-tion. Other possible cognitive changesinclude memory loss, speech im-pairments, and irritability. The physicaldiscomfort associated with dialysis, suchas interrupted sleep patterns, nausea,lethargy, and shortness of breath, mayincrease the individuals’ psychological dis-tress. Individuals eligible for kidney trans-plant may also experience the stress ofwaiting for a transplant as well as fear ofrejection if the transplant takes place.
Uncertainty is also an issue in ESRD.
Treatment choices are not always ﬁnal.Individuals who choose peritoneal dialy-sis rather than hemodialysis may need toswitch if complications from peritonealdialysis occur. Individuals using hemodial-ysis may later consider a kidney transplantshould one become available. Even afterindividuals receive a kidney transplant,uncertainty remains because there isalways the chance that rejection of thetransplant may occur.
Lifestyle Issues
Many renal and urinary tract disorders re-
quire lifestyle changes during the acute
phase of the condition; however, after treat-ment, few limitations may exist. The excep
–
tion is those individuals with ESRD , for
whom lifestyle impli cations are profound.
Not only are there strin gent dietary restric-
tions, but the require ment of regular dial-
ysis treatments restricts individuals’
freedom of time. Even when individuals
receive a successful transplant, the medical
regimen is demanding both before andafter the transplant.
Exercise can increase strength and en-
durance and reduce stress. Individualswith ESRD may be unable to tolerate asmuch physical activity as before; howev-er, exercise programs individually tailoredto and prescribed for individuals’ speciﬁcneeds and abilities may be possible.Although many of their daily activitiescan be continued, individuals shouldapproach activities with ﬂexibility, sincePsychosocial Issues in Renal and Urinary Tract Conditions 385

their physical tolerance of various activi-ties from day to day may be unpredictable.
The desire for sexual activity may change
for individuals with ESRD, both becauseof side effects of medications and becauseof the physical manifestations of ESRDitself. Sexual desire may also change foremotional reasons. If individuals experi-ence depression or anxiety, or if relation-ship problems exist, sexual desire and/orfunction may also be altered.
Traveling is possible whether individu-
als are using hemodialysis or peritonealdialysis if arrangements are made well inadvance. Individuals on hemodialysismust locate a dialysis unit in the area tobe visited so that dialysis sessions can bescheduled well in advance. Individualswith peritoneal dialysis need to makeplans for backup medical care as well asfor the availability of dialysate and acycling machine if used.
Social Issues
Many conditions of the kidney and uri-
nary tract have little impact on socialfunctioning. There may be signiﬁcant im-pact, however, on individuals with ESRD,a serious chronic condition that ﬂuctuatesand requires lifelong management andtreatment for survival. Dialysis regimes,dietary restrictions, and medications maylimit activities and socialization to somedegree.
Preexisting social or relationship prob-
lems are frequently made worse by prob-lems associated with ESRD.
Physiologic changes, treatment demands,
and the chronic nature of ESRD affect notonly the individuals with the conditionbut also the whole family unit. Familymembers’ reaction and stability can helpor hinder individuals’ acceptance of theircondition. Family members can be over-solicitous or rejecting, making it more dif-ﬁcult for individuals to reestablish theirown emotional balance and their rolewithin the family. Individuals and theirfamilies may focus on ESRD as the centerof family functioning. They may requireassistance in developing a life that incor-porates ESRD into family structure, ratherthan overwhelming it.
Individuals with ESRD may become
withdrawn from family and friendsbecause of feelings of inadequacy. Theymay struggle to resolve their need fordependence on dialysis and on others forassistance with their treatment. Individ-uals with unresolved dependency conﬂictsmay become uncooperative and ill-tem-pered. Rather than confronting the indi-vidual, family and friends may excusetheir behavior, reinforcing the sick role.
VOCATIONAL ISSUES IN RENAL ANDURINARY TRACT CONDITIONS
Many renal and urinary tract disorders
have no long-term impact on individuals’ability to work. Renal failure, however, hasan impact on psychological, social, andvocational function, and a variety of alter-ations in individuals’ daily life may benecessary. The degree to which kidney dis-ease affects employment depends on indi-viduals’ occupation, previous workhistory, medical condition and treatment,and status of any secondary disabilities.Individuals with beginning renal failurecan generally continue their previous job,especially if it is sedentary and does notrequire strenuous activity. Individualswith ESRD may experience reduced worktolerance due to impaired concentrationand/or fatigue. If they are no longer capa-ble of the physical activity that the workrequires, a job modiﬁcation or changemay be necessary.
Work schedule ﬂexibility may be neces-
sary to accommodate recurring medical386
CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS

problems, periods of hospitalizations,dialysis treatment, and time away due tonormal medical checkups. Other possiblebarriers to employment are ﬁnancial dis-incentives or employer concern about lostwork time.
Individuals with ESRD who experience
decreased attention span or inability toconcentrate may require jobs that accom-modate these limitations. Excess heat inthe work environment should be avoidedsince individuals with ESRD are unable toadequately regulate body heat. Individualswith peripheral neuropathy as a result ofrenal failure may have difﬁcult with man-ual dexterity or walking. In addition, indi-viduals with a shunt or graft forhemodialysis may have limited use of thearm containing the access route.
CASE STUDIESCase I
Mr. H. is 42 years old and has worked
as a metalworking machine operator forthe past 22 years. As part of his job heoperates powerful, high-speed machinesthat require strict adherence to safety rulesto avoid accidents. He normally works 40hours a week, but at times of increasedproduction he is asked to work overtime.The metalworking shop in which heworks operates two shifts daily that work-ers are required to alternate every othermonth. Mr. H. has polycystic kidney dis-ease. He is now in ESRD and requires dial-ysis. He is now on hemodialysis but hopesto obtain a kidney transplant.Questions
1. Given Mr. H.’s type of work, what
speciﬁc factors related to his medicalcondition would you consider whenestablishing a rehabilitation plan?
2. What factors regarding hemodialysis
would you consider?
3. Are there special accommodations
that will need to be made if he is tocontinue in his current line ofemployment while still on dialysis?
4. What issues related to a kidney trans-
plant might be a factor in Mr. H.’srehabilitation plan?
Case II
Ms. L. is 36 years old and experienced
severe kidney damage several years ago asa result of a drug overdose that was partof a suicide attempt. She is now on CAPD.She has a high school education and hadexperience as a cab driver in a large cityin the past. Ms. L. has expressed interestin returning to cab driving. She now livesin a moderately sized midwestern city.
Questions
1. What speciﬁc factors might you con-
sider in evaluating Ms. L.’s rehabili-tation potential?
2. Is her goal of returning to cab driv-
ing in her current situation realistic?Why or why not?
3. What speciﬁc issues related to her
peritoneal dialysis would you consid-er when helping Ms. L. establish arehabilitation plan?Case Studies 387

388 CHAPTER 13 U RINARY TRACT AND RENAL CONDITIONS
REFERENCESBonventre, J. V. (2002). Daily hemodialysis: Will
treatment each day improve the outcome inpatients with acute renal failure? New England
Journal of Medicine, 346 (5), 362–364.
Delmonico, F. L. (2004). Exchanging kidneys:
Advances in living-donor transplantation. New
England Journal of Medicine, 350 (18), 1812–1814.
El Nahas, A. M., Harris, K., & Anderson, S. (Eds.).
(2000). Mechanisms and clinical management of
chronic renal failure (2nd ed.). New York: Oxford
University Press.
Hariharan, S., Johnson, C. P., Bresnahan, B. A.,
Taranto, S. E., McIntosh, M. J., & Stablein, D.(2000). Improved graft survival after renal trans-plantation in the United States, 1988–1996. New
England Journal of Medicine, 342 , 605–612.
Himmelfarb, J. (2002). Success and challenge in dial-
ysis therapy. New England Journal of Medicine,
347(25), 2068–2070.
Ifudu, O. (1998). Care of patients undergoing
hemodialysis. New England Journal of Medicine,
339(15), 1054–1062.
Kinchen, K. S., Sadler, J., Fink, N., et al. (2002). The
timing of specialist evaluation in chronic kidneydisease and mortality. Annals of Internal Medicine,
137, 479–486.
Marsden, P. A. (2003). Predicting outcomes after renal
transplantation: New tools and old tools. New
England Journal of Medicine, 349 (2), 182–184.Moua, M. N. (2000). End-stage. Rehabilitation
Counseling Bulletin, 45 (1), 53–55.
Owen, W. F., Pereira, B. J. G., & Savegh, M. H. (Eds).
(2000). Dialysis and transplantation: A compan-ion to Brenner and Rector’s The kidney .
Philadelphia: W.B. Saunders.
Pascual, M., Theruvath, T., Kawai, T., Tolkoff-Rubin,
N., & Cosimi, A. B. (2002). Strategies to improvelong-term outcomes after renal transplantation.New England Journal of Medicine, 346 (8), 580–588.
Schifﬂe, H., Lang, S. M., & Fischer, R. (2002). Daily
hemodialysis and the outcome of acute renal fail-ure. New England Journal of Medicine, 346 (5),
305–310.
Sosa-Guerrero, S., & Gomez, N. J. (1997). Dealing
with end-stage renal disease. American Journal of
Nursing, 97 (10), 44–51.
Soulillou, J. P. (2001). Immune monitoring for rejec-
tion of kidney transplants. New England Journal of
Medicine, 344 (13), 1006–1007.
Teichman, J. M. H. (Ed). (2001). Twenty common prob-
lems in urology . New York: McGraw-Hill, Health
Professions Division.
U.S. Renal Data System. (2001). Excerpts from the
United States Renal Data system 2001 annual datareport: Atlas of end-stage renal disease in theUnited States. American Journal of Kidney Disease,
38(Suppl. 3), S1–S247.

NORMAL STRUCTURE ANDFUNCTION OF THEMUSCULOSKELETAL SYSTEMThe Skeletal System
Bones make up the general framework
of the body. The skeletal system, which ismade up of 206 bones, supports the sur-rounding tissues and assists in movementby providing leverage and attachment formuscles (see Figure 14–1). It also protectsvital organs, such as the heart and brain.The tough outer covering of bone is calledthe periosteum . Bones also have a network
of sensory nerves and a network of tinyvessels to supply blood. They have manyfunctions other than support, movement,and protection. Red blood cells are man-ufactured in the red bone marrow bymeans of a process called hematopoiesis .
Bone also stores calcium and other min-eral salts. New bone is constantly beingproduced and old bone replaced, creatinga dynamic relationship between calciumin the bone and calcium in the blood.
Types of Bone
Bones are classiﬁed according to shape.
Long bones are found in the arms and legs
(e.g., the humerus and the femur ). ShortConditions of the
Musculoskeletal SystemCHAPTER 14
389
Cranium
Clavicle
Coracoid Process
Xiphoid Process
Humerus
Radius
Ulna
Carpals
Metacarpals
Phalanges
Femur
PatellaGreater
TrochanterCoccyxSacrumIliumSternumScapula
Tibia
Fibula
Tarsals
Metatarsals
Figure 14–1 Anterior View of the Skeleton.
Source : Reprinted with permission from S. M.
Jacob and W. J. Lossow, Structure and Function
in Man , 4th ed., p. 93, © 1978, W. B. Sanders
Company.

bones are found in the hands and feet (e.g.,
the carpals and the tarsals ). Flat bones are
those like the skull ( cranium ) and ribs,
and irregular bones have differing shapes,
such as the vertebrae and mandible (jaw
bone).
The vertebrae (irregular bones that sur –
round the spinal cord) support the headand trunk of the body, protect the spinalcord, and enable bending and ﬂexing. Theseven vertebrae at the neck and upperback are called cervical vertebrae . The 12
that extend from the upper to lower backare called thoracic vertebrae . In the low-
er back there are ﬁve lumbar vertebrae ;
a bony prominence called the sacrum ,
which consists of fused bone; and the coc-
cyxor small residual “tail bone,” which
extends from the end of the sacrum.
Connective Tissue
Connective tissue supports and, as its
name implies, connects other tissues andtissue parts. Not only bones but also liga-
ments , tendons, and cartilage are connec-
tive tissue. Ligaments are tough bands of
ﬁber that connect bones at the joint siteand provide stability during movement.Tendons are bands of tissue that connect
muscle to bone, enabling muscle move-ment. Cartilage is a dense type of connec-
tive tissue that creates form, maintainsstructure, and can withstand considerabletension. There are several different typesof cartilage. For example, there is cartilagebetween the vertebral disks of the spineand in the joint of the knee to absorbshock and prevent friction, while the car-tilage in the external ear and nose provideform.
Between each two vertebrae that sur-
round the spinal cord are disks of cartilagecalled intervertebral disks . They act as
cushions against shock. The tough, ﬁbrousouter portion of the disk is called theannulus , and the spongy inner portion is
called the nucleus pulposus . Vertebrae are
connected by ligaments and are sur-rounded in part by a joint capsule contain-ing synovial ﬂuid like other synovial joints .
Joints
A joint is the place where two or more
bones are bound together. The comingtogether of two bones at a joint is calledarticulation . Some joints, such as those in
the skull, are ﬁbrous, or ﬁxed, meaningthat they provide no movement. Otherjoints, such as the pubis symphysis (pubic
bone) in the pelvis, are cartilaginous (con-
tain cartilage) and provide slight move-ment. Synovial joints are freely movable,
enabling both motion and change of posi-tion (Figure 14–2). They are enclosed in asac called the bursa , which is lined with a390
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM
SynovialMembraneSynovialFluidArticularCartilage
Figure 14–2 Synovial Joint.

Normal Structure and Function of the Musculoskeletal System 391
synovial membrane . This membrane
secretes synovial ﬂuid , which aids joint
movement by acting as a lubricant.Synovial ﬂuid also helps cushion the jointagainst the shock produced by jointmovement. Articular cartilage lines the end
of each bone, helping to absorb shock. Itreceives its nourishment from the synovialﬂuid.
Synovial joints are capable of various
different types of movements (see Figure14–3):
•Circumduction: circular movement
•Eversion: movement in which a body
part is turned outward
•Inversion: movement in which a
body part is turned inward
•Flexion: bending movement
•Extension: straightening movement
•Abduction: movement of a body part
away from the midline of the body
•Adduction: movement of a body part
toward the midline of the body•Ulnar deviation: lateral movement of
the hand away from the body
•Radial deviation: lateral movement
of the hand inward, toward the body
•Pronation: turning movement of a
body part downward
•Supination: turning movement of a
body part upward
•Dorsiﬂexion: backward movement of
a body part
The type of motion of a particular syn-
ovial joint depends on the type of joint:
•Circular motion is provided by ball-
and-socket joints, such as those foundin the hip and shoulder.
•Back-and-forth motion is provided by
hinge joints, such as those in theelbow and knee.
•Gliding motion is provided by joints of
the vertebrae.
•Pivotal motion is provided by vertebrae
that connect the head and the spine.
Flexion of wrist
Flexion of
elbow
Extension
of hip Abduction
of hipAdduction
of shoulder
Flexion of
fingers
Figure 14–3 Movement of Synovial Joints. Source : Copyright © 1999 Rachel Clarke.

The Muscular System
There are several types of muscles in the
body. Some are involuntary muscles that
work automatically , such as the cardiac
muscle (myocardium ) of the heart and the
smooth muscle found in the digestive
tract. In contrast, striated muscle (skeletal
muscle ), which makes up 40 to 50 percent
of an individual’s body weight, is undervoluntary control .
A muscle sheath (a hard band of connec-
tive tissue) contains blood vessels and nerve
ﬁbers and surrounds every muscle. Eachof the two ends of the muscle is attachedto a different bone. The muscle attach-ment closer to the midline of the body iscalled the origin of the muscle, and the
attachment of the end farther from themidline of the body is called the insertion .
Muscles produce movement by the con-
traction of opposite muscle groups. Theyare classiﬁed by their function. Musclesthat bend a limb are called ﬂexors ; those
that straighten a limb are extensors .
Muscles that move a limb laterally , away
from the body, are called abductors , where-
as muscles that move a limb closer to the
body are called adductors . Muscles that
bend a body part backward are called dor-
siﬂexors . Because of continuous nerve
stimulation to muscle, muscles maintain a
partial state of contraction (tone) even
when they are at rest and aren’t being used.
CONDITIONS OF THEMUSCULOSKELETAL SYSTEMTrauma
Fractures
Any break or disruption in the continu-
ity of bone is a fracture. There are severaltypes of fractures with different levels ofseverity (see Figure 14–4):• A closed or simple fracture is an
uncomplicated break in a bone withno breaking of skin.
• An open or compound fracture is a
break in a bone in which the skin isbroken so that the bone protrudesthrough it.
• A complete fracture is a break in a
bone that extends through the bonefrom one side to the other, includingthe periosteum, or outer cover.
• An incomplete or partial fracture is a
break that does not extend all the waythrough the bone.
• A transverse fracture is a fracture that
extends straight across the bone.
• An oblique fracture is a fracture across
the bone at a slant.
• A spiral fracture occurs in a spiral
around the bone and is usually causedby a twisting injury.
• An impacted fracture is a break in
which one portion of the bone isimpacted, or forcibly driven, intoanother portion of the bone.
• A comminuted fracture is a break in
which the bone has been shattered,leaving fragments of bone at the siteof the break.
• A displaced fracture is a break in a
bone in which the two ends of thebone are separated.
• A complicated fracture is a break in a
bone in which the tissue surroundingthe bone, such as blood vessels andnerves, has also been injured.
• A compression fracture is a break in
which the ends of the bones arepressed against each other.Compression fractures often occur inthe vertebrae.
• A pathologic fracture is a break in a
bone due to disease of the bone itself,rather than to an injury.
• A Colles’ fracture is a break in a bone
near the wrist.392
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

Conditions of the Musculoskeletal System 393
Closed/SimpleFractureOpen/CompoundFracture
ComminutedFractureCompressionFracture
DisplacedFractureIncompleteFracture
Figure 14–4 Common Types of Fractures. Source: Copyright © 1999 Rachel Clarke.

• A stress fracture is a small break in a
bone that occurs as the result of pro-longed or unaccustomed activity.
Some fractures may be treated by closed
reduction , a procedure in which bone frag-
ments are realigned manually, withoutsurgery, and immobilized with a plastercast. Other fractures must be treated byopen reduction , a procedure in which bone
fragments are realigned and stabilized sur-gically. Traction may be used in combina-
tion with either closed or open reduction.
Many fractures heal well and result in
no disability. In other instances, howev-
er, complications can occur that can cause
signiﬁcant disability. Bone edges or frag-ments of compound, displaced, or com-minuted fractures may injury tissue ornerves in the surrounding area, causingpermanent damage. In fractures of largebones, such as the femur, blood loss canbe signiﬁcant. Open or compound frac-tures can become infected, leading toosteomyelitis (infection of the bone).When individuals with another chronicillness or disability fracture a bone, com-plications related to the fracture, in addi-tion to complications related to immo-bility can develop and can pose a signiﬁ-cant threat not only to function but alsoto general health and well-being.
Dislocations
Displacement or separation of a bone
from its normal joint position is called adislocation . If the bone is not totally sep-
arated from the joint, the condition iscalled a subluxation . In addition to caus-
ing extreme pain, a dislocation causes apartial loss of movement at the joint andcan impede the blood supply to the sur-rounding tissue.
Dislocations can result from trauma or
from a congenital weakness or abnormal-ity of a joint that predisposes individualsto dislocation when the joint is moved acertain way. The shoulder and the hip arecommon sites of dislocation, althoughany joint can become dislocated.
Prompt treatment of joint dislocation is
important in order to prevent complica-tions, such as nerve damage or injury dueto the decreased blood supply. The bonescan usually be slipped back into placemanually. If there has been no damage tothe nerves, blood vessels, or surroundingtissue, there is usually no permanent dis-ability. If dislocations recur in the samejoint, however, individuals may need toavoid movements that appear to con-tribute to the dislocation. When disloca-tion recurs frequently, surgical ﬁxation ofthe joint may be necessary.
Contusions
Musculoskeletal injuries may not always
involve bone; sometimes the injuryinvolves underlying structures, such as thesoft tissue under the skin. A contusion is
a soft tissue injury that results from ablunt, diffuse blow. Although the skin isnot usually broken and no bones are bro-ken, local hemorrhage with associatedbruising, swelling, and damage to the deepsoft tissue under the skin occurs. Bleedingunder the skin is responsible for the pur-plish discoloration at the site of injury—the bruise ( ecchymosis ). When a major
vessel or a muscle is injured, a hematoma
(sac ﬁlled with accumulated blood) maydevelop under the skin.
Strains and Sprains
Although the terms strain and sprain are
often used interchangeably, they refer totwo different types of injuries. A strain is
an overstretching or overuse of tendonsand muscles, whereas a sprain is an injury394
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

or overstress of a ligament and its attach-ment site.
Strains may be acute, resulting from a
sudden twisting or wrenching movement,
or they may occur with unaccustomedvigorous exercise. Chronic strain may bethe result of repetitive muscle overuse.Individuals with strains may be treatedwith analgesics, muscle relaxants, or anti-inﬂammatory drugs. One goal of therapyis to increase muscle strengthening. Con-sequently, immobilization is usually notrecommended.
Sprains are categorized as mild, moder-
ate, or severe (ﬁrst-, second-, or third-degree
sprains). First- and second-degree sprainsare usually treated with analgesics, anti-inﬂammatory agents, or muscle relaxants.Treatment of second-degree sprains mayalso include immobilization of the injuredjoint and therapeutic exercises and phys-ical therapy to promote early return tomotion. A severe sprain tears a ligamentcompletely from its attachment and mayrequire surgical repair.
Lacerations
An injury that has torn or cut the skin
and underlying tissues is referred to as alaceration . Puncture and penetration
injuries generally have a small entrancewound but cause extensive damage to tis-sues under the skin. Stabbing wounds andgunshot wounds are puncture and pene-tration wounds, respectively.
The degree of disability experienced
with lacerations, puncture wounds, orpenetration wounds depends on the loca-tion; the amount of damage to the under-lying tissues, such as nerves, blood vessels,and internal organs; and associated com-plications, such as infection. The risk ofinfection is dependent on the source andcircumstances of the injury.Overuse and Repetitive Motion Injuries
Bursitis
Inﬂammation of the bursa (the sac that
contains the synovial ﬂuid in the synovialjoints) is called bursitis and may be acute
or chronic. It may result from chronicoveruse of a joint, trauma to the joint, orthe invasion of the bursa by infectiousorganisms. Although it may affect anysynovial joint, bursitis commonly occursin the shoulder, elbow, or knee.
Bursitis is characterized by pain and ten-
derness over the joint and by limitationof joint motion. Acute attacks may last fordays to weeks, and they may recur.Splinting and rest of the joint are gener-ally recommended. Bursitis may becomechronic, causing varying degrees of dis-ability.
Tendonitis and Tendosynovitis
The term tendonitis describes a condi-
tion in which there is an inﬂammation ofa tendon . The term tendosynovitis de-
scribes a condition in which there is aninﬂammation of the sheath of tissue thatsurrounds the tendon. The two conditionsusually occur simultaneously. Althoughthe exact cause is unknown, tendonitismay be associated with trauma, strain, or
unaccustomed exercise. The primary symp-
tom is pain on motion at the site ofinﬂammation. The condition usually sub-sides with appropriate treatment, al-though surgery may be indicated on rareoccasions.
Carpal Tunnel Syndrome
Carpal tunnel syndrome is a condition
in which there is compression of themedian nerve in the wrist, causing painand paresthesia (tingling, pricking sensa-Conditions of the Musculoskeletal System 395

tion) in the hand. This condition is clas-siﬁed as a compression or entrapmentneuropathy. It can be associated withrheumatoid arthritis (discussed later inthis chapter) or diabetes (see Chapter 9),or it can result from strenuous or repeti-tive use of the hand (sometimes
called a
repetitive motion injury) (Sequeira, 1999).
Muscle strength in the hand may be weak-ened to the extent that individuals havedifﬁculty opening jars or twisting lids.They may experience pain that can be dulland aching or that can radiate into theforearm. Individuals may complain ofwaking at night because the affectedhand is numb, or they may complain ofnumbness in the hands in the morning,feeling they have to shake their hands toget the circulation back. Symptoms maybe mild and of short duration, or theymay become chronic.
Treatment of Carpal Tunnel Syndrome
Irreparable nerve damage may occur if
carpal tunnel syndrome is left untreated.In mild cases, a wrist splint at night maybe sufﬁcient to help the symptoms, alongwith medications such as nonsteroidalanti-inﬂammatory drugs. If the splint doesnot interfere with activity, the individualmay also wear the splint or wrist supportduring the day. Corticosteroids are some-times injected into the area if the splintis unsuccessful at relieving symptoms;however, improvement may be tempo-rary. When the hand becomes weakened,or when the symptoms become intolera-ble, surgery to relieve pressure on thenerve may be indicated.
Vocational Issues in
Carpal Tunnel Syndrome
Individuals whose work places biome-
chanical stresses on the hands and wrists,especially those whose work involvesrepetitive motion of the hands, are espe-cially vulnerable to carpal tunnel syn-drome. In some instances the conditionbecomes severe enough to limit individ-uals’ ability to work. It can be of particu-lar concern to individuals who rely onsign language as a major form of commu-nication (Smith, Kress, & William, 2000).With continued exposure to risk factorswithout adequate care or rest, permanentdamage to the soft tissue and nerves canresult.
When carpal tunnel syndrome is relat-
ed to activity, both the nature of the workand the amount of time spent on a taskcontribute to the potential for injury. Ifcarpal tunnel syndrome is related to a spe-ciﬁc activity, ergonomic modiﬁcationssuch as providing forearm support whentyping, adjusting the height of the key-board or work area, or positioning thehands differently may be indicated. Forindividuals with carpal tunnel syndromethat is aggravated by work-related activi-ty, the factors in the work environmentmay need to be modiﬁed. Ofﬁce workersand those using computer keyboards mayneed to take periodic rest breaks through-out the day. When using a desk and chair,different body positions may help reducemuscle fatigue and prevent exacerbationof carpal tunnel syndrome symptoms.Individuals should avoid bent, extended,or twisted hand positions for long periods.When possible, they should alternatehands for work tasks and avoid awkwardhand positions.
Degenerative Conditions
Osteoporosis
Osteoporosis is a condition in which the
bone mass (the amount of bone) is re-duced, causing bones to become weak-396
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

ened, fragile, and easily broken (Prestwood& Raisz, 2002). In some instances, al-though there has been no bone fracture,individuals may experience aching in var-ious bones and often have chronic back-ache. Vertebral fractures, a seriousconsequence of osteoporosis, can lead toacute and chronic back pain as well asspinal deformity (Meunier et al., 2004).
Individuals with osteoporosis common-
ly have no symptoms until a bone is bro-ken as a result of little or no trauma.Frequent sites of bone fractures are thehips, especially in older or frail individu-als, and the wrist ( Colles’ fracture ). Crush
or compression fractures may occur in the
vertebrae.
Risks for Osteoporosis
Although osteoporosis commonly oc-
curs in individuals after middle age, sec-ondary osteoporosis may occur as a resultof other medical conditions or as a sideeffect of the overuse or long-term use ofsteroid medication. Individuals with a dis-ability may be at higher risk of osteoporo-sis. Individuals with physical disabilitymay be nonambulatory and have boneloss due to immobility. Individuals on
medications, such as anticonvulsants, may
also have increased risk. Women with phys –
ical and cognitive disabilities are at espe-cially high risk for osteoporosis and osteo-porosis-related fractures (Schrager, 2004).
Treatment of Osteoporosis
Osteoporosis is progressive, but appro-
priate treatment may slow the diseaseprocess. Fractures related to osteoporosiscan result in substantial morbidity andmortality (Solomon, Finkelstein, Katz,Mogun, & Avorn, 2003). Consequently,prevention is a key component of treat-ment. The best treatment for osteoporo-sis is prevention through the daily intakeof adequate amounts of dietary calcium;engagement in weight-bearing exercisethroughout life; avoidance of the long-term use of steroid medications, whichpromote bone loss; and prevention of falls(Boskey, 2001; Marcus, 2000).
When osteoporosis does occur, anal-
gesics, heat, or rest may relieve the pain.In some instances, braces or splints maybe indicated. Exercise that strengthensmuscles, thus providing additional sup-port, may be beneﬁcial. Although gener-al activity is encouraged, heavy lifting orany activity that increases the risk of fallsshould be avoided.
Calcium supplements are usually pre-
scribed for both men and women withosteoporosis. Women with osteoporosismay be given hormones to decrease boneloss and to increase absorption of calcium.
When calcium absorption is impaired, sup –
plemental vitamin D may also be given.
Osteoarthritis (Degenerative Joint Disease)
Associated with “wear and tear” of the
joints, osteoarthritis is a local joint dis-
ease, nota systemic disease. Although any
joint may be affected, joints in the kneesare frequently affected. Risk factors forosteoarthritis of the knee are previous sur-gery or injury of the knee, occupationalkneeling and squatting, and obesity (Rajan& Kerr, 2000).
Since osteoarthritis is not systemic,
symptoms are located around the affect-ed joint. Bone spurs ( osteophytes ) develop
on the surface of the joints, eroding thecartilage so that it can no longer serve asa cushion or shock absorber. Consequent-ly, the ends of the bones at the joint rubon each other, causing pain and inﬂam-mation. Weight-bearing joints, such as theknees, hips, and spine, are frequentlyaffected. Finger joints may also be affect-Conditions of the Musculoskeletal System 397

ed. When osteoarthritis affects the kneesor hip, it may be considerably disabling,interfering with mobility.
Joints affected by osteoarthritis may
have been previously injured or exposedto long-term strain. Obesity places extrastrain on joints and is thought to be onepredisposing factor for osteoarthritis. Thereason that some individuals who have noknown predisposing factor developosteoarthritis is unknown, although thecondition may be associated with aging.
Osteoarthritis is generally unremitting.
Overuse of the affected joints, cold anddamp weather, or other factors may inten-sify symptoms. The amount of disabilityexperienced depends on the type andmagnitude of joint damage, the numberof joints involved, the particular jointsinvolved, and the daily activity of theindividual.
Treatment of Osteoarthritis
Treatment of osteoarthritis is directed
toward increasing function and prevent-ing further dysfunction. Speciﬁc exer-cises, including range-of-motion andstrengthening exercises, are often part ofthe treatment prescribed to meet this goal.It may be necessary to balance rest of thejoint with its use.
The use of assistive devices, such as canes
or crutches, may prevent undue weight
bearing on joints. If individuals with osteo-
arthritis are obese, weight re duction may
be advisable to remove undue pressure on
the joints. Oral administration of aspirin
or nonsteroidal anti-inﬂammatory agents , as
well as injection of steroids into the joint,
may also be helpful. In cases of severejoint damage, total joint arthroplasty(surgery to replace damaged joints withartiﬁcial joints) can restore individuals topain-free functional independence inmany cases (Ritz & Mann, 2000).Vocational Issues in Osteoarthritis
Osteoarthritis can be a cause of long-
term disability (Hawker et al., 2000). The
limitations experienced depend on the spe-
ciﬁc joints affected. Individuals with osteo –
arthritis of the knees, for instance, may beunable to walk long distances or stand forlong periods of time. They also may havedifﬁculty with bending or stooping. Indi-viduals with osteoarthritis of the upperextremities or vertebrae of the spine mayhave difﬁculty lifting, turning, and reach-ing. When ﬁngers are affected, individu-als may be unable to perform tasks that re-quire signiﬁcant ﬁnger motion.
Back Pain
Back pain can be caused by a variety of
conditions and can produce a number ofsymptoms in addition to pain, dependingon its location. Some back pain may bepsychogenic in origin, meaning that no or-
ganic cause of the pain can be found. Be-cause of the subjective nature of pain andthe different meaning of pain to differentindividuals, diagnosis and treatment ofback pain in these individuals may be dif-ferent. Even if back pain is psychogenic innature, however, the pain that individu-als experience is real and no less debilitat-ing. It is important that the cause of backpain be established so that appropriatetreatment can be implemented.
Back pain is classiﬁed as mild, moderate ,
or severe . Since pain is a subjective meas-
ure, the extent of impaired function asso-ciated with back pain is often an indicatorof the severity.
Types of Back Pain
Low Back Pain
Low back pain is one of the most com-
mon conditions experienced. It is deﬁned398 CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

as pain in the lumbar or sacral region ofthe lower back. It may be experienced inthe erect, nonmoving spine ( static pain ) or
during movement ( kinetic pain ). Low back
pain may be caused by:
• mechanical problems due to poor pos-
ture, such as lordosis (swayback pos-
ture)
• poor body mechanics at work, caus-
ing sprain or strain
• injury due to falls, motor vehicle acci-
dents, or sports
•spondylolisthesis (forward slippage
of a vertebra)
•spondylolysis (breakdown or degen-
eration of a vertebra)
• arthritis or osteoporosis• infection of the bones of the spine or
tissue between vertebrae
• tumors in the spine, or metastasis of
cancer from another part of the body• herniation of an intervertebral disk•referred pain from other organs of the
body, such as kidneys or uterus
Back pain may be accompanied by sci-
atica or may occur alone. Sciatica is a syn-
drome of pain that radiates from the lowerback into the hip and down the leg. It may
be accompanied by numbness, tingling, and
muscle weakness. Sciatica can accompanya number of disorders of the lower back,herniated disk being the most common.
Herniated or Ruptured Disk (Herniated
Nucleus Pulposus)
Rupture of the soft, inner portion of the
intervertebral disk ( nucleus pulposus )
through a tear in the tougher outer por-tion of the disk ( annulus ) is called a herni-
ation (see Figure 14–5). A sprain or strain
of the back or a disease that weakens theannulus may cause herniation of a disk.Conditions of the Musculoskeletal System 399
Herniatednucleuspulposus
Lateral herniation ofintervertebral discCordCord
Central herniation ofintervertebral disc
Figure 14–5 Forms of Vertebral Herniation. Source : Reprinted with permission from J. Luckman
and K. C. Sorenson. Medical Surgical Nursing: A Psychological Approach , 1st ed., p. 407, © 1974,
W. B. Saunders Company.

It results in back pain, often accompaniedby spasms of the back muscles. Protrusionof the herniated disk exerts pressure onthe nerves that surround the area. Pressureon the nerves can cause a partial loss ofsensation and/or weakness in lower ex-tremities. In severe cases, pressure on thenerves can also cause problems with bow-el or bladder function.
The pain experienced with a herniated
disk is frequently exacerbated by straining,coughing, or lifting. Symptoms may be in-
termittent at ﬁrst but later may progress to
continuous pain or loss of sensation. Treat-
ment, consisting of physical therapy and
the use of anti-inﬂammatory medication ,
usually eliminates pain; however, a herni-ated disk is the most common reason forback surgery (Deyo, 1998). When surgicaltreatment of herniated disk is necessary,diskectomy (removal of the disk) may be
performed (Deyo, Nachemson, & Mirza,2004).
Degenerative Disk Disease
Degenerative spondylolisthesis is character-
ized by slippage of the vertebral body intothe one below. It is associated with degen-eration and narrowing of the involveddisk. The major symptom is back pain,especially with bending, lifting, or twist-ing. Individuals may also complain of legpain or may have neurologic signs.
Flexion exercises are often prescribed as
one form of treatment. Some individualsﬁnd corset support to be helpful. Individ-uals may also be treated with nonsteroidal
anti-inﬂammatory drugs (NSAIDs). Most
important are lifestyle changes such as
avoidance of repetitive bending, heavylifting, or twisting of the trunk of thebody. Spinal fusion surgery (spinal arthrode-
sis), in which two disks are fused, may alsobe necessary for treatment of instabilityand deformity of the disk.Scoliosis
Scoliosis is a lateral, S-shaped curvature
of the spine that can be congenital (pres-
ent at birth) or can be a complication ofamputation or other medical conditionthat alters posture, such as poliomyelitisor cerebral palsy. Scoliosis can be correct-ed with early recognition and proper treat-ment. If the spinal deformity becomes
ﬁxed, however, the condition is difﬁcult to
reverse. In severe cases, scoliosis can inter-fere with respiratory capacity and can
cause pressure on organs in the thoracic or
abdominal cavity, interfering with organfunction.
Diagnosis of Back Pain
Back pain can sometimes be diagnosed
by a history of symptoms and observation
of individuals in various body postures
and activities, as well as by physical exam-ination. X-rays can be helpful in identify-ing bony abnormalities of the spine;however, conditions such as ruptured diskcannot be seen on a regular X-ray. Otherdiagnostic tests may involve electromyo-graphy, which provides information aboutnerve function and nerve damage.Computerized tomography (CT scan) or
magnetic resonance imaging may also be
used to identify disk degeneration or aruptured disk.
Treatment of Back Pain
Exercise is an important part of preven-
tion as well as treatment of either acute orchronic back pain. Although in the pastbedrest was thought to be the treatmentof choice, little evidence has shown thatindividuals who maintain bedrest haveany different outcome than those whomaintain regular activities during theacute period of back pain (Deyo, 1998;400
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

Deyo & Weinstein, 2001). Maintainingactivity is, of course, dependent on thedemands of individuals’ regular activities,such as the need for heavy lifting.
Back pain may be treated with muscle
relaxants, or antidepressants may be usedfor some individuals if they show symp-toms of depression (Deyo & Weinstein,2001). Physical therapy or acupuncturemay provide symptomatic relief.
Preventive measures, such as condition-
ing of the muscles of the back or the useof proper body mechanics, are helpful inavoiding recurrences; however, adheringto speciﬁc exercise routines after symp-toms have subsided may be difﬁcult. Al-though most back pain symptoms subsidespontaneously, a complication of backpain can be a overreaction to the condi-tion that leads to drug-seeking behavior,which can precipitate a more serious dis-ability of substance dependency.
Low back pain persisting for 6 months
or more is considered chronic back pain.Chronic back pain may be an extension of
symptoms due to injury or may be due toosteoporosis, degenerative spondylolis-thesis, or a narrowing ( stenosis ) of the
spinal canal. It may be more difﬁcult toresolve chronic back pain, depending onthe cause, and extensive, long-term ther-apy may be necessary. Chronic back painmay be treated medically or surgically.Medical treatment may consist of exercise,biofeedback, stress management, andmedication such as muscle relaxants orsteroids to reduce inﬂammation and non-narcotic analgesics to reduce pain. Spinalsurgery has a limited role in alleviatingchronic back pain and is usually reservedfor those individuals who also have neu-rologic symptoms, such as loss of urinaryor bowel control or footdrop. Surgicalinterventions are varied, depending onthe cause of the pain. Some surgical inter-ventions include simple diskectomy. Vocational Issues in Back Pain
Acute back pain caused by strain or
sprain may signiﬁcantly impair functionfrom days to weeks. Pain is precipitated byrepeated twisting or lifting, prolonged sit-ting, or operation of vibrating equipment.Individuals may have difﬁculty standingerect and may need to change position fre-quently. Most individuals with acute backpain return to work within 6 weeks; how-ever, recurrences are common, and disabil-ity caused by back pain has steadily risen(Deyo, 1998). Although low back pain israrely permanently disabling, return towork after an episode of low back pain isinﬂuenced by clinical, social, and econom-ic factors (Deyo & Weinstein, 2001).
Prevention is the best way to reduce
back injury. Education about good bodymechanics and conditioning can help toreduce further injury. Individuals who arenot physically active are more likely tohave acute lower back injury; conse-quently, exercises that increase strengthand muscle tone can help prevent injury.Modiﬁcation of the workplace to reducemechanical stresses may also be importantto decrease both the frequency and costof lower back injuries.
Individuals whose job requires lifting or
heavy physical work may need mechanical
assistance devices or tables to allow liftingfrom the waist. Regular rest breaks may be
needed as well as instruction regarding good
lifting techniques. An ergonomics review
of the workstation and equipment can help
to ensure that individuals are sitting andmoving in ways that reduce the risk ofstrain. As much as possible, equipmentthat causes whole-body vibration shouldbe modiﬁed to reduce vibration as muchas possible. Individuals using equipmentwith signiﬁcant vibration should schedulefrequent rest periods or should rotateworkstations to a less strenuous task. Conditions of the Musculoskeletal System 401

Chronic Pain
Pain is a complex human experience
that can dramatically affect the quality oflife. It is a multidimensional concept that
includes physical, psychological, spiritual,
and social functioning (Glajchen, 2001).The purpose of pain is mainly protective;pain is a signal or warning that an area ofthe body needs attention. Pain and pain-related problems may be associated witha number of body systems and diseaseconditions. Chronic pain can be experi-enced because of a number of conditions,ranging from cancer to pain experiencedwith chronic headaches. It may also beassociated with a number of conditions ofthe musculoskeletal system, from traumato rheumatoid arthritis.
Pain is subjective, is difﬁcult to quanti-
fy, and has different meanings to differentindividuals. Pain perception may be inﬂu-
enced by anxiety, fear, and depression aswell as by cultural, ethnic, and other lifeinﬂuences. Individuals also have differentpain thresholds (points at which sensation
is perceived as pain) as well as differentlevels of pain tolerance (points at which the
individual ﬁnds the pain unbearable).Individuals with heightened pain toler-ance may minimize the importance of
symptoms and delay seeking medical atten-
tion until the pain is severe. Individualswith low pain tolerance may tend to have
an exaggerated reaction to pain. Therefore,
pain intensity is difﬁcult to determine if it
is based on the reactions of the individ-ual, and it is not always a reliable indexof the seriousness of the condition. Indi-viduals’ response to pain ( pain expression )
is inﬂuenced by a number of cognitive,
emotional, behavioral, and cultural factors.
Some cultures encourage a stoic responseto pain, whereas other cultures permit afree expression of feelings in response topain. Different individuals may not re-spond to the same pain stimuli in thesame way. Similarly, the same person mayreact differently to pain in different cir-cumstances. Anxiety and fear tend toenhance the perception of pain and theintensity of the pain response, whereasdistractions tend to lessen the perceptionof and response to pain.
Acute Versus Chronic Pain
Pain can be classiﬁed as acute or chron-
ic. Acute pain is deﬁned as pain that oc-curs with the onset of illness or injury andis generally of short duration. It usuallyhas an identiﬁable cause. As healing oc-curs or the cause of pain is corrected orremoved, the pain usually decreases with-in an established course of time. The con-trol of most acute pain is based ontreatment of the underlying cause.
When pain becomes chronic, however,
it is often treated as a condition in itself.
Chronic pain is deﬁned as pain that con-
tinues more than 3 months. As it persistsover time, chronic pain loses its biologicfunction of signaling injury or di
sease and
imposes psychological and phys ical stress
on individuals who experience it. Individ-uals experiencing chronic pain may devel-op chronic pain syndrome , a condition
characterized by physical, social, and be-havioral dysfunction. These individualsoften have marked alteration of behavior,
including depression or anxiety, restriction
in daily activities, excessive use of medica-
tions, and frequent use of medical services.
Pain becomes a central issue in their lives.
Types of Chronic Pain
Chronic pain may be of three types:
• Pain that persists beyond the normal
healing time (e.g., pain at the site ofa fractured bone after the bone has402
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

healed; phantom pain in an amputat-ed limb)
•
Pain related to a chronic, degenerative,
or malignant disease (e.g., rheumatoidarthritis, osteoporosis, cancer)
• Pain that persists for months or years
but has no readily identiﬁable organ-ic cause (psychogenic pain), such asback pain or headache with no iden-tiﬁable physical reason for the pain
For some people, pain is unrelenting
and persists at intolerable levels despiteanalgesic medication and futile attemptsfor other medical cures. Some individualswith chronic pain engage in cycles of falsehope, frustration, and guilt. They may beunable to work and begin to withdrawfrom family and social activities. Personalrelationships may deteriorate. They maybelieve no one else has ever endured the
type of pain they are enduring and may be-
come demoralized, depressed, and angry.
Treatment of Chronic Pain
Treatment of pain that cannot be con-
trolled or eradicated is directed toward help –
ing individuals learn to cope with the pain.Physicians may ﬁnd treatment of chronic
pain difﬁcult because various treatments for
chronic pain are ineffective and becauseindividuals may become addicted to med-ications used in treatment (Foley, 2003).Consequently, they may refer individualswith chronic pain to a clinic that usesmultiple, simultaneous therapeutic ap-proaches to chronic pain. Alternate formsof pain control may be used alone or incombination with analgesic medication.
Medications
Generally, medications are used more
often in the treatment of acute pain thanin the treatment of chronic pain. Whenmedications are used as part of pain man-agement, the type chosen depends on thecause and the type of pain. Muscle relax-
antsmay be prescribed to relax tight mus-
cles or muscle spasms. Analgesics , which
range from medication such as aspirin tonarcotics such as Demerol or morphine,
may also be used, but professionals oftenlimit their use of narcotic analgesics forthe long-term treatment of chronic pain(unless pain is part of a terminal illness)because of their fear of physical and/orpsychological dependence or addiction,even though studies have shown thatthere is little risk of addiction in individ-uals who have had no history of substanceabuse (Portenoy, 1994). Because individ-uals with chronic pain frequently experi-ence depression as well, antidepressant
medications may occasionally be prescribed.
Noninvasive Procedures
Physical Therapy. Physical therapy may be
prescribed to help individuals with chron-ic pain gradually increase their exercisetolerance and activity level. It is directedtoward stretching and strengthening spe-ciﬁc muscles and joints. It may also beused to improve functional activity andreduce muscle spasm. Splints or bracesmay be used to support painful body partsor may be prescribed to help individualsincrease their activity.Transcutaneous Electrical Nerve Stimulation(TENS). Transcutaneous electrical nerve stim-
ulation may help to relieve pain. In this
technique, electrodes of a small, battery-operated device are placed over the pain-ful area. When the unit is on, it stimulatesnerve ﬁbers electrically, providing a coun-terirritation that, in turn, blocks painimpulses. The treatment itself is painless.The length of time individuals wear theunit varies, ranging from all day to only1 or 2 hours a day. Conditions of the Musculoskeletal System 403

The success of TENS depends to some
degree on individuals’ understanding of
the technique and motivation to use it. The
degree and duration of pain relief withTENS units are variable. For some individ-
uals, the effects wear off after a few months.
Others may use the TENS unit successful-ly for a longer period of time. Because ofthe expense of TENS units, long-term orpermanent use is usually not feasible.Stress Management. Other speciﬁc tech-
niques may be used to help individualsreduce the stress response. When individ-uals are tense, the heart beats faster, bloodpressure rises, and muscles tighten. Theseresponses can make pain more intense.Stress management includes speciﬁc proce-
dures designed to reduce stress and pro-mote relaxation.
Stress management may be useful in the
treatment of a variety of disorders, but it
may be particularly useful in the treatment
of chronic pain. Because tension and anx-iety tend to accentuate pain perception,
removing tension and anxiety can also serve
to reduce the perception of pain. There aremany types of stress management. Someindividuals may be helped to identify thesources of stress and learn ways to controltheir reaction to it. Other individuals maylearn speciﬁc relaxation techniques.Guided Imagery. Another technique, guid-
ed imagery , uses audiotaped instruction or
descriptive narrative provided by a trainedtherapist. This technique is directedtoward helping the individual relax andvisualize positive outcomes. The tech-nique is based on the assumption thatimages can directly or indirectly inﬂuencephysiologic processes in the body (Rakel& Shapiro, 2002).Relaxation Therapy. Relaxation therapy is a
technique aimed at helping individualslearn the relaxation response, which de-creases blood pressure, heart rate, oxygenconsumption, and alpha wave (type ofbrain wave) activity on electroencephalo-
gram. The technique can be learned through
practice and may include breathing tech-niques as well as progressive relaxation.
Individuals who undergo progressive
relaxation training are taught to tightenand relax different muscle groups gradu-ally. The procedure promotes relaxation,
decreases anxiety, and lessens muscle ten –
sion. When used daily, these techniquescan help the individual cope with stress. Meditation. Meditation is a technique that
helps individuals obtain a relaxed state byfocusing attention on a repetitious event,usually a word or phrase recited repeated-ly. This new focus removes individuals’focus on the painful sensation. Individualsconcentrate on a variety of other focuses,such as breathing, chanting, or forming avisual image. Hypnosis. Hypnosis is a procedure by
which individuals are induced into a trance
state, during which suggestion is used toalter attitudes, perceptions, or behavior.The hypnotic state is obtained by focus-ing on a soothing image or situation, pur-posefully relaxing voluntary muscles, andcontrolling one’s breathing. For individu-als with chronic pain, hypnosis may beused to alter the reaction to painful stim-uli or the perception of pain. Skilled hyp-notherapists may help individuals distracttheir thinking of pain through posthyp-
notic suggestion. Hypnosis should be con-
ducted only by trained, certiﬁed indi-viduals. It has varying degrees of successin the management of chronic pain.Biofeedback . Some individuals ﬁnd bio-
feedback helpful in controlling pain, es-pecially if pain is due in part to muscle404
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

tension. Biofeedback is a technique based
on operant conditioning and feedbacklearning of a physiologic response. Inprinciple, biofeedback is a technique ofmeasuring a naturally occurring bodyfunction and amplifying it so that individ-uals can acquire the ability to control thatfunction. Through biofeedback, individu-als learn to elicit the relaxation responseand control the physiologic mechanismsthat produce the stress-related symptoms.
Individuals receive feedback from a spe-
ciﬁc physiologic measurement such asheart rate, muscle tension, or skin temper-ature. Electrical equipment is used to pro-vide this feedback. Electrodes are placedon the skin over localized muscles. Wiresfrom the electrodes are attached to anelectromyogram machine, which meas-ures the electrical activity of the muscles.Individuals receive information through atone or lights. After learning differentmethods to reduce the amount of muscletension, individuals can monitor the ef-
fectiveness of these methods in controlling
muscular activity through the feedback
system. With practice, individuals can learn
to reduce the stress response at will with-out the beneﬁt of physiologic monitoring. Operant Conditioning. A behavioral tech-
nique designed to decrease the functional
impairment associated with chronic pain,operant conditioning , does not cure or re-
duce the pain itself but rather alters theindividual’s behavioral response to thepain. The technique is based on theoriesof learning and conditioning. The painexperience often results in a series ofbehaviors that communicate discomfort(e.g., grimacing, guarding, or limping) andusually elicit responses from others in theform of sympathy, decreased expectationsfor performance or success, or even mon-etary compensation. Behavioral respons-es to pain may also be reinforced by thefact that they may help individuals avoidactivities that they ﬁnd unpleasant. Suchreinforcement of these pain behaviors
may condition individuals to display them
and, as a result, may increase the function-al impairment associated with the painbehaviors. Operant conditioning involves
withdrawing reinforcement for “pain” be-
haviors and reinforcing “well” behaviors.Pain Groups. Joining a Chronic Pain
Anonymous (CPA) group is another way
individuals can learn to live with theirpain when no other technique or medicalintervention has helped. The CPA modelborrows from Alcoholics Anonymous (AA)and is based on the concept that similarpsychological and emotional disturbancesare seen in alcoholism and intractablebenign pain in that both disrupt person-al and work relationships; both cause lossof control, obsession, and isolation; andboth are chronic conditions. CPA uses thesame 12 steps as AA, substituting the wordpain for the word alcohol .
Invasive Procedures
Acupuncture. Acupuncture has gained
increased acceptance in the treatment ofchronic pain. It is an ancient Chineseform of analgesia in which long, ﬁne nee-dles are inserted into selected points ( trig-
ger points ) of an individual’s body to
eliminate the pain sensation.
There is no simple explanation for the
mechanisms that underlie the analgesiceffects of acupuncture. Although it is con-sidered an invasive technique, it has few,if any, complications when it is done un-der sterile conditions by those who havebeen trained and certiﬁed in acupuncturetechniques. Nerve Blocks. Nerve blocks eliminate pain
locally. They are commonly used for sur-Conditions of the Musculoskeletal System 405

gical procedures, as well as in the treat-ment of chronic pain. Local anestheticsare injected close to nerves, thus blockingtheir ability to conduct the painful stim-uli. Generally, nerve blocks are given forthe temporary relief of pain; however, incases of severe pain, such as in terminalcancer, nerve blocks may be performed sothat the effects are irreversible. Neurosurgical Procedures. Neurosurgical pro-
cedures in which surgeons sever the sen-
sory nerves supplying the painful area may
be used when severe pain cannot be ame-
liorated or controlled by other means. Cut –
ting the nerves removes not only the sen-sation of pain, but also the sensations ofpressure, heat, and cold. Consequently, in-dividuals who have undergone these pro-cedures must be aware of the necessity ofprotecting the area from injury. The typeof neurosurgical procedure used dependson the type and location of the pain. Forexample, sympathectomy involves the auto-
nomic nervous system; neurectomy , either
the cranial or the peripheral nerves; andrhizotomy and chordotomy , the nerves
close to the spinal cord (see Chapter 3).
Amputation
The general term for loss of all or a por-
tion of a body part is called amputation .
Amputation can result from injury ( trau-
matic amputation ), such as loss of an
extremity in an explosion or in a motorvehicle accident (Blank-Reid, 2003; Proehl,2004). Amputation may be performed sur-gically to treat disease, such as amputationof a breast ( mastectomy ) because of can-
cer or amputation of a leg because of gan-grene. Amputation may also be congenital ,
such as when individuals are born with-out a limb.
Upper-extremity amputations are often
associated with accidents, burns, explo-sions, or other types of traumatic injury,whereas lower-extremity amputations aremore frequently associated with disease,such as peripheral vascular disease (seeChapter 11).
Levels of Extremity Amputation
Amputation of an extremity may be per-
formed at different levels. In order to pro-vide for maximal length of the stump ofthe extremity and, thus, maximal func-tion with a prosthesis, surgeons usuallyperform an amputation as distal (farthest
from the center of the body) as possible.The levels of upper-extremity amputationare as follows:
• Forequarter or interscapular-thoracic
amputation: the most severe upper-extremity amputation, in which theentire arm, clavicle, and scapula areremoved
• Shoulder disarticulation: removal of
the arm at the shoulder joint
• Above the elbow: removal of the arm
anywhere between the shoulder andthe elbow joints
• Elbow disarticulation: removal of the
arm at the elbow joint
• Below elbow: removal of the arm any-
where between the elbow and thewrist
• Wrist disarticulation: removal of the
hand at the wrist
• Partial hand: amputation of one or
more ﬁngers or the loss of a portionof the hand
The levels of lower-extremity amputa-
tion are as follows:
• Hemipelvectomy, or hindquarter
amputation: the most severe lower-extremity amputation, in which theentire lower limb and half of thepelvis are removed406
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

• Hip disarticulation: removal of the leg
at the hip joint
• Above the knee: removal of the leg
anywhere between the hip and theknee joints
• Knee disarticulation: removal of the
lower leg at the knee
• Below the knee: removal of the low-
er leg anywhere between the knee andthe ankle
• Syme’s amputation: removal of the
foot at the ankle
• Transmetatarsal or partial foot:
removal of a portion of the foot
It is especially important to retain the
maximal length of the stump in lower-extremity amputations, because theamount of energy required to use an arti-ﬁcial limb increases with the height of theamputation. Individuals with a below-the-knee amputation, for example, require ap-proximately 10 to 37 percent more energy
for movement than do individuals without
an amputation (Wilson, 1998).
Treatment of Amputation
Surgery
When an underlying disease such as can-
cer or arteriosclerosis necessitates amputa-tion, the type of surgical amputation per-formed, the postoperative course, and thetype of rehabilitation depend to a greatextent on the circumstances surrounding
the amputation and the individual’s gener –
al condition. Individuals with an amputa-
tion due to an underlying disease may beat risk for additional amputations becauseof the disease process itself or because ofcomplications that result from the disease.
Reimplantation
An extremity, or portion of an extrem-
ity, that has been totally severed by aninjury can sometimes be surgicallyreattached to the body with partialrestoration of function. This process iscalled reimplantation . Reimplantation
may be especially important after am-putation of an upper extremity, sinceupper-limb prostheses provide limitedfunction (Daigeler, Fansa, & Schneider,2003).
With the evolution of surgical tech-
niques and scientiﬁc technology, reim-plantation has become more successful.The degree of success depends on the gen-eral condition of the individual, the avail-ability of rapid transportation to a reim-plantation center, and appropriate care ofthe severed body part prior to reimplan-tation. The goal of reimplantation is topreserve quality of life by improving func-tion and appearance (Brown & Wu, 2003).Studies have demonstrated that reimplan-tation can, in many cases, achieve 50 per-cent function and 50 percent sensation inthe replanted part (Wilhelmi, Lee,Pagensteert, & May, 2003), although insome cases return of motor function isgreater than return of sensory function(Wiberg et al., 2003). The success of thissurgery in increasing quality of life andfunction is partly determined, however,on individuals’ appropriate and realisticexpectations of the appearance and func-tion of the body part after reimplantation(Wilhelmi et al., 2003).
Prostheses
A prosthesis is a fabricated substitute for
a missing body part, such as an artiﬁciallimb that replaces an amputated limb.Prosthetic devices may either enable indi-viduals to regain independent function ormay be prescribed only for cosmetic pur-poses. The type of prosthesis, its purpose,and its maximal use are dependent on thereason for the amputation, the type andConditions of the Musculoskeletal System 407

level of the amputation, the presence ofany underlying disease, the developmentof any complications, and, most impor-tant, needs of the individuals and theirmotivation to use the prosthesis (Bussell,2000). The physician who performed thesurgery, usually the orthopedic surgeon
(physician who specializes in surgicaltreatment of bones), also prescribes theprosthesis on the basis of the individual’sdaily activities, occupation, and cosmeticneeds. A certiﬁed prosthetist , an individual
who specializes in making prosthetic de-vices, then fabricates the prosthesis.
In some instances, the surgeon may
place a temporary prosthesis on the stump
of a lower extremity immediately after sur-gery. In this case, a rigid total contactdressing is applied to the stump in theoperating room, and a pylon or adjustablerigid support structure is attached. Anankle-foot assembly is then attached to the
lower end of the pylon. The immediateplacement of a temporary prosthesis mayhave a psychological beneﬁt for individ-uals, fostering a sense of independenceand optimism as soon as they wake fromsurgery. It also promotes ambulation,thus reducing the risk of complicationsassociated with immobility. Immediateplacement of a temporary prosthesis iscontraindicated, however, when there issevere underlying disease, such as diabetesor infection, or if there has been extensivedamage as a result of injury. It is also con-traindicated for individuals with limitedmental capabilities, who may be unable tounderstand instructions or to regulate theweight placed on the stump in the earlypostoperative period.
When immediate prosthetic ﬁtting is
not advisable, individuals receive a tem-porary prosthesis 2 to 3 weeks after sur-gery. Placement of a temporary prosthesisis necessary, whether the ﬁtting is imme-diate or delayed, so that edema (swelling)can subside and the stump can shrinkbefore the permanent prosthesis is ﬁtted.A permanent prosthesis can usually be
placed within 3 months.
As much as possible, lower-extremity
prostheses are designed to enable ambu-lation. Generally, the lower the level ofamputation, the easier the use of the pros-thesis. The ankle-foot attachment may be
immovable or movable. A commonly usedankle-foot mechanism is the solid ankle-cushion heel foot. In above-the-kneeamputations, the prosthesis must alsoreplace knee function, providing a jointthat is stable for both standing and walk-ing. The socket of the prosthetic devicemust be aligned well and ﬁt well for opti-mal balance and support. Because properalignment varies with the heel heights ofshoes, individuals who wish to wearshoes with different heel heights on occa-sion may need several removable prosthet-ic feet designed to accommodate thevarying heel heights.
Prosthetic devices for use after hemipel-
vectomy are more difﬁcult to use. The
increased energy needed for ambulationoften makes the use of a prosthesis afterhemipelvectomy unrealistic for other thancosmetic use, although such a prosthesiscan be functional. For example, a prosthe-sis may be worn after hemipelvectomy tohelp individuals maintain proper posturewhile sitting in a wheelchair. When usingthe prosthesis for ambulation, individualswith hemipelvectomy can usually onlywalk at a slow pace and only on levelground. Most individuals who have un-dergone hemipelvectomy choose to usecrutches or a wheelchair for their dailyactivities, reserving the prosthesis for spe-cial events when cosmetic appearance ismore important than ambulation (Ampu-tee Coalition of America, 2000).
Upper-extremity prostheses vary in type
and purpose and are custom-made accord-408
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

ing to individual need. The complex func-tion of the hand cannot be replaced, butfunctions such as lifting, grasping, andpinching can often be restored with aprosthesis. A terminal device is a prosthe-
sis that substitutes for a hand. The levelof amputation and individuals’ needsdetermine the type of terminal deviceused. In general, the greater the cosmeticappearance of the device, the less its func-tional capacity. Individuals who needgrasping, holding, or lifting actions mayﬁnd a hook more beneﬁcial as a prosthe-sis. For others, prosthetic hands thathave grasp or pinch function for light ob-jects may be most useful. The cosmeticappearance of the prosthetic hand may bemore important to some individuals thanits functional capacity.
A prosthesis is activated by using the
muscles in the remaining portion of thelimb. For this reason, a prosthesis for ahigh-upper-extremity amputation mayhave limited function. A prosthesis placedafter a shoulder disarticulation or an inter-
scapular-thoracic amputation , for example,
may be mostly cosmetic with little, if any,functional capacity. Myoelectrical prosthe-
ses, which are activated by electrical po-
tentials produced by muscles, may beconsidered in some instances. In this typeof prosthetic device, electrodes are placedover the skin of the muscles to be used.The electrodes pick up electrical impulsesfrom the muscles, transferring them to amotor in the prosthesis that then activatesthe hand to open and close. The functionof the myoelectric prosthesis does not,however, approximate the function ofnormal hand movement and dexterity.Myoelectrical prostheses are best suited forindividuals with below-the-elbow prosthe-ses. They are heavier because of the bat-tery and motor that they contain, andthey are more expensive than regular pros-thetic devices. Complications of Amputation
Complications may develop after either
upper- or lower-extremity amputation. Itis extremely important that the prosthe-sis ﬁts well and that there is no unduepressure or rubbing that could lead toulceration. All individuals, but especiallythose whose amputation was necessarybecause of underlying peripheral vasculardisease, must be careful to avoid skinulceration that could become infected andnecessitate a higher-level amputation.
Swelling ( edema ) of the stump after the
permanent prosthesis has been placed notonly can interfere with the proper ﬁt ofthe prosthesis, but it can also increasepressure and restrict the blood ﬂow to thestump, contributing to the likelihood ofulceration. An improper ﬁt of the prosthe-sis, rubbing, or swelling of the stumpshould be immediately brought to theattention of the physician and prosthetistso that appropriate adjustments of theprosthesis can be made.
Individuals undergoing amputation
must also concentrate on preserving therange of motion in the remaining jointsof the amputated limb. Contractures
(deformities in which permanent contrac-tion of a muscle makes a joint immobile)may occur because of the improper posi-tioning or limited activity of the remain-ing joints. Contractures impede or preventeffective use of the prosthetic device. They
are easier to prevent through regular range-
of-motion exercises of joints than they areto cure. When contractures develop, theymay be corrected with extensive physical
therapy and, occasionally, surgery.
Other complications of amputation
include bone spurs, scoliosis, and phan-tom pain. Bone spurs or bone overgrowthmay develop at the end of the stump,changing its shape and causing pain.Scoliosis (S-shaped lateral curvature of theConditions of the Musculoskeletal System 409

spine) may occur after a lower-extremityamputation because of the improperalignment or because of improper use ofthe prosthesis. After a higher-upper-extremity amputation, scoliosis may de-velop if the prosthetic device unbalancesthe trunk. In both instances, scoliosis canbe prevented by making sure that theprosthesis is in good alignment. Indi-viduals who have had an upper-extremi-ty amputation may also perform exercisesto strengthen the muscles that support theprosthesis.
Although all individuals who have had
an amputation experience some degree ofphantom sensation (sensation that the
amputated extremity is still present), thesensation usually diminishes over time.Some individuals, however, experiencechronic, severe pain sensation in theamputated extremity, called phantom
limb pain (Kooijman, Dijkstra, Geertzen,
Elzinga, & van der Schans, 2000). Phan-tom limb pain may gradually diminishover time, but it sometimes becomes dis-abling. In some instances, treatment toblock the nerves that serve the amputat-ed extremity may alleviate the pain. Attimes, neuromas (bundles of nerve ﬁbers)
imbedded in the scar tissue of the stumpmay cause pain and can be removed; how-ever, this may not totally alleviate phan-tom limb pain. Individuals with chronicdisabling phantom pain may need chron-ic pain management.
Psychosocial Issues in Amputation
Individuals with amputation have to
make permanent behavioral, social, andemotional adjustments to cope with themultiple problems that can exist withamputation (Gallagher & MacLachlan,1999). Amputation forces individuals tomake a major adjustment not only to achange in body image, but also to achange in functional capacity. When in-dividuals are ﬁtted with a prosthetic limb,they are confronted with the irrevocablefact that not only have they lost a limb,but they must now learn how to incorpo-rate the false limb into daily function.
Traumatic amputation produces psy-
chological and social impacts that can beoverwhelming if not openly and candid-ly addressed. The earlier psychosocialintervention can be implemented, themore likely it is that psychological factorswill not impede functional outcome(Meyer, 2003). Individuals whose ampu-tation was due to chronic disease may ﬁndit less difﬁcult to adjust, especially if thebody part amputated was a source of painor immobility prior to the amputation.Individuals who have lost a body part sud-denly, such as with amputation of thebreast because of breast cancer or loss ofa limb due to traumatic injury, may havemore difﬁculty with adjustment becausethey have had inadequate time to preparefor the loss.
Regardless of the reason for the ampu-
tation, it is important to understand indi-viduals’ interpretation of the loss. Indi-viduals’ ability to adapt to amputationdepends on the circumstances surround-ing the amputation, the usefulness of theprosthetic device, and individuals’ percep-tion of the disability. Some individualswho have lost a limb no longer considerthemselves whole. They may fear thatthey will never again be able to functionas they did prior to the amputation. Forthese individuals, a prosthesis is a re-minder of perceived inadequacy ratherthan a restoration of function. In someinstances, loss of a limb is comparable tothe loss of a loved one. Individuals mayneed sufﬁcient time to grieve and adjustto their loss.
Individuals who have undergone ampu-
tation, especially loss of a lower extremi-410
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

ty because of disease, must guard againstinjury and infection to the stump. Con-sequently, skin care is a vital part of reha-bilitation. Bathing in the evening ratherthan in the morning is advisable, sincedamp skin may swell and stick to a pros-thesis, causing irritation and rubbing.
Many limitations of activity depend on
the level of the amputation. Most individ-uals who have had a lower extremityamputated can bicycle, swim, dance, andparticipate in many athletic activitieswith adaptive equipment. Driving a car isusually not a problem, although automat-ic transmissions may allow individuals todrive more easily. Activities such as climb-ing, squatting, and kneeling may be moredifﬁcult; however, even these tasks aremastered by some individuals. Individualswith upper-extremity amputation mayrequire a number of assistive devices, inaddition to the prosthesis, in order to per-form some activities of daily living.
Although the amputation of an extrem-
ity has no direct effect on sexual activity,psychological factors and/or the reactionof sexual partners to the amputation mayalter sexual function. Partners can be sup-portive or unsupportive. The stability ofthe relationship prior to amputation, aswell as communication and understand-ing, are important components to adjust-ment and subsequently to the quality ofthe relationship.
Vocational Issues in Amputation
Individuals with amputation who wear
a prosthesis may need to avoid hot, humidenvironments, which can cause skinbreakdown or contribute to the deteriora-tion of the prosthesis. Dust or grit can beabrasive to the skin, exacerbating skinproblems, and can also interfere with thefunctioning of the movable parts of theprosthesis.In the case of lower-extremity amputa-
tion, the physical demands of the job,such as walking, climbing, or pushing,should be evaluated and altered, if neces-sary. The increased energy expenditurerequired in the use of a prosthesis shouldalso be considered part of the physicaldemands of the job. Individuals in profes-sional or managerial careers may havefewer limitations following the amputa-tion of either an upper or lower extremi-ty. Those with upper-extremity amputa-tion may have a greater cosmetic need fora prosthesis, however, than do those work-ers whose jobs require the prosthesis fortasks such as lifting.
Rheumatoid and AutoimmuneConditions
There are over 105 disorders classiﬁed as
rheumatic conditions, many of which arealso considered autoimmune conditions .
Autoimmune conditions are thought to bemediated by an autoimmune response inwhich the body’s immune system fails torecognize body tissue and attacks the tis-sues as if they were foreign objects. Thereason for this autoimmune response isunknown.
The term rheumatic disease describes
conditions that produce symptoms thataffect the joints , connective tissues , and mus-
cle. Rheumatoid conditions are character-
ized by pain, inﬂammation, fatigue , and loss
of motion in joints . The term arthritis is a
general term used to describe inﬂamma-tion of the joints. The term myositis refers
to inﬂammation of the muscle.
The effects of rheumatoid conditions are
diverse, with symptoms ranging frommild to severe. Most commonly, howev-er, symptoms are unpredictable, so thatindividuals with rheumatoid conditionscan never predict when pain, stiffness, ordeformity may occur.Conditions of the Musculoskeletal System 411

Rheumatoid Arthritis
Rheumatoid arthritis is a chronic, pro-
gressive, systemic disorder that causes sig-niﬁcant pain, joint destruction, and disa-bility (Kvien, 2004). It is characterized byinﬂammation and swelling of the synovial
joints , resulting in pain, stiffness, and de-
formity. It is one of the most common of
the rheumatoid conditions. The course of the
disease is unpredictable and ﬂuctuating.
Rheumatoid arthritis is thought to
result, in part, from an autoimmuneresponse in which the body’s normalmechanisms of defense produce an in-ﬂammatory type of reaction against itself,leading to cell destruction. Althoughmuch of the focus of rheumatoid arthri-tis is on the joints, rheumatoid arthritis isa systemic disease affecting other body sys-
tems as well. Individuals experience symp-toms such as fatigue, weight loss, or feveras well as joint pain and deformity. The
inﬂammatory process may also affect oth-er
body organs (e.g., the eyes, heart, lungs,
or spleen), causing changes that alterorgan function.
Rheumatoid arthritis is a progressive
condition, but not all individuals are af-fected to the same degree. The conditionmay be severe in some individuals, caus-ing moderate joint deformity in a relative-ly short amount of time. In others, it mayprogress more slowly or may never be-come severely debilitating.
Rheumatoid arthritis may be character-
ized by a series of remissions, in whichsymptoms subside for a period of weeks toyears, and exacerbation, in which symp-toms become worse. During exacerbation,joints may sustain increased damage sothat they never return to their normalstate, even during remissions.
During the exacerbations of rheumatoid
arthritis, the synovial membrane becomesinflamed; the joints become warm,swollen, and painful; and the synovialmembrane thickens. As the condition pro-gresses, a layer of scar tissue forms over thesynovial membrane. This tissue, calledpannus , interferes with provision of nutri-
ents to the cartilage of the joint, therebyleading to erosion and joint destruction.Scar tissue becomes so tough and ﬁbrousthat ankylosis (stiffness and ﬁxation of
the joint) occurs, impeding movement.
Rheumatoid arthritis may not affect all
joints, or it may affect different joints atdifferent times. The most common jointsto be involved are:
• wrists• ankles• knees• elbows• joints of the ﬁngers and toes
Occasionally, shoulder, hip, and neck
joints are also involved. Joints are usual-ly affected symmetrically. For example,both knees, rather than one knee, areaffected. Joint pain and stiffness are gen-erally worse in the morning, subsidesomewhat during the day, and againbecome painful at night.
The prognosis for individuals with
rheumatoid arthritis is variable. Someindividuals may experience rapid progres-sion of debilitating symptoms, whereasothers remain in a state of remission foryears, continuing their normal employ-ment and full activity.
Medical Treatment of Rheumatoid Arthritis
Physicians who specialize in the treat-
ment of rheumatoid conditions, includingrheumatoid arthritis, are called rheuma-
tologists . Since there is no cure for
rheumatoid arthritis, the goals of treat-ment are to induce and maintain remis-sion, which include decreasing jointdestruction, maintaining joint function,412
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

and preventing deformity (O’Dell, 2004).The cornerstones of therapy are restand
exercise .
Exercise. Exercise is almost always direct-
ed toward strengthening and increasingthe ﬂexibility of muscles without placingadditional wear and tear on the joints.Although activity alone is not necessarilytherapeutic, prescribed exercise is impor-tant in the treatment of rheumatoidarthritis to restore muscle strength, tomaintain joint mobility, and to preventcontractures. Individuals should followthe speciﬁc exercise plan recommended bytheir physician.
Exercises usually consist of specific
range-of-motion exercises for joints. Theyare performed regularly and daily to pre-vent deformity. Other exercises are used tohelp strengthen muscles to prevent ﬂex-ion deformity. Rest. Complete bedrest may be recom-
mended for short periods during the acutephases of the condition. In other in-stances, rest periods throughout the daymay be prescribed.
Splinting of speciﬁc joints may occa-
sionally be prescribed to reduce local in-ﬂammation. When there is acute in-ﬂammation, active exercises may not bepossible and splints may be prescribed forvarious joints. Splints, if used, are usual-ly used only at night and at rest and aredesigned to maintain the joints in exten-sion. When used, they are prescribed onlyas a temporary measure and are alwaysprescribed under the direction of a physi-cian. Long-term use of splints can causedeformity because of the lack of mobilityin the affected joints. Thermal Treatment. Thermal treatment (ap-
plications of either hot or cold) may beused to relieve pain. Treatments may in-clude hydrotherapy , such as a whirlpool
bath, or treatments using parafﬁn baths , in
which the affected body part is placed ina bath of hot parafﬁn. The body part isthen removed from the parafﬁn bath. Asthe parafﬁn cools externally, warmth tothe body part is held in, reducing bothpain and inﬂammation. Occupational and Physical Therapy. Occu-
pational therapists and physical therapists
may be consulted to help individuals in-crease their functional capacity. Occupa-tional therapists generally focus on tasksof daily living, whereas physical therapistsfocus on mobility issues.Medications. Medications are also a main-
stay of treatment in rheumatoid arthritis(Olsen & Stein, 2004). Medications areused to reduce inﬂammation, thereby alsoreducing pain. They are divided into threemain classes: NSAIDs, corticosteroids, anddisease-modifying antirheumatic drugs(DMARDs) (O’Dell, 2004). Often thesemedications are used in combination.
NSAIDs provide partial relief of pain and
stiffness. However, they have not beenshown to slow the progression of rheuma-toid arthritis, and consequently are usu-ally used in combination with DMARDs(American College of Rheumatology Sub-
committee on Rheumatoid Arthritis, 2002).
Long-term administration of these med-ications can also result in stomach irrita-tion, gastrointestinal ulcer, and in someinstances
perforation and hemorrhage
(O’Dell, 2004).
In some instances salicylates (e.g.,
aspirin) may still be prescribed in largedoses, to nearly the toxic level, to obtainthe desired therapeutic effect. This highconcentration of salicylates in the bloodmay exceed the liver’s ability to metabo-lize it, causing toxic effects, such as ring-ing in the ears ( tinnitus ). Because of theConditions of the Musculoskeletal System 413

large doses required, individuals mayexperience other side effects as well, suchas stomach discomfort and/or bleedingdue to irritation of the stomach lining.
Corticosteroids are added to the treat-
ment regimen to suppress the inﬂamma-tory response. When and how they areused in the treatment of rheumatoidarthritis remains controversial (Moreland& O’Dell, 2002). Corticosteroids have seri-ous side effects, such as thinning of skin,cataracts, osteoporosis, and hypertension.For this reason, these drugs are not usu-ally used on a long-term basis and aremore commonly used when individualsare having a major exacerbation of the dis-ease. To prevent serious complications,individuals taking steroids must not stopmedication suddenly but must graduallywithdraw from the medication under aphysician’s supervision.
DMARDs are used to retard or halt the
progression of rheumatoid arthritis bysuppressing inﬂammation. One exampleof a DMARD is methotrexate, which isoften selected for initial therapy (Mikuls& O’Dell, 2000). Complementary and Alternative Therapies.Complementary and alternative medicinetherapies are usually diverse products orpractices outside the mainstream ofWestern medical practice for promotinghealth and preventing or treating disease(Harpham, 2001). Several studies havedemonstrated that over 40 percent of indi-viduals with rheumatoid conditions usesome form of complementary or alterna-tive therapy (Boisset & Fitzcharles, 1994;Resch, Hill, & Ernst, 1997; Vecchio, 1994).
The types of alternative therapies used
in the United States vary. Some individu-als use topical ointments ranging from sil-icon lubricant to alcohol extract frommarijuana leaves; others use copper brace-lets or other special jewelry to relievesymptoms; others use dietary supple-ments such as glucosamine, vitamins, orherb preparations, as well as acupuncture(Kolasinski, 2001).
Many complementary and alternative
therapies have few side effects; however,
since individuals with rheumatoid arthritis
also often continue to receive traditionalmedical treatment, interactions betweentraditional therapies and alternative ther-apies may be of concern. Consequently,individuals should make both traditionalhealth care providers and alternativehealth care providers aware of all methodsof treatment they are currently using.
Surgical Treatment of Rheumatoid Arthritis
Most individuals with rheumatoid
arthritis are treated with a combination ofmedication, exercise, and rest. At times,however, because of severe joint inﬂam-mation or joint deformity, surgical proce-dures are necessary. Common surgicalprocedures for rheumatoid arthritis aresynovectomy and arthroplasty with joint
replacement. Synovectomy is surgical
removal of the synovial membrane sur-rounding a joint. Synovectomy preventsrecurrent inﬂammation, thus reducingjoint pain and further joint destruction.
Arthroplasty (surgical replacement, for-
mation, or reformation of a joint) may benecessary when the joint has become non-functional because of destruction or whenmovement of the joint becomes so painfulthat activity is severely hampered.
Psychosocial Issues in
Rheumatoid Arthritis
The debilitating nature of rheumatoid
arthritis, with its associated pain, can af-fect individuals’ ability to work or fulﬁllresponsibilities at home and can also cur-
tail their social life and ability to engage in414 CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

recreational activities (Walsh, Blanchard,Kremer, & Blanchard, 1999). Living withpain is a factor to which individuals withrheumatoid arthritis must adjust. Aware-ness of the fact that there is no cure for
the condition and that it is progressive may
lead to feelings of hopelessness. Individ-uals with rheumatoid arthritis may alsoexperience sleep disturbances, which in-crease fatigue and contribute to depressionand irritability.
In the early stages, before there is de-
formity, rheumatoid arthritis may beessentially an invisible disease. This invis-ibility may lead to misunderstanding byfamily and friends, who perceive individ-uals as merely seeking attention with theircomplaints of pain or attempting to avoidwork or other activities. The unpredictablenature of rheumatoid arthritis not onlycontributes to this misunderstanding butcan also cause stress for affected individ-uals, who are unsure on a day-to-day basiswhether they will be able to participate invarious activities.
Individuals may develop learned help-
lessness from the unpredictable, chronic,
and incurable nature of the condition. Help –
ing individuals gain a feeling of control byincreasing their self-management of arth-
ritic pain and ability to cope with the vague –
ness of the disease can improve their socialfunctioning and overall quality of life.
Independence is a critical issue for in-
dividuals with rheumatoid arthritis. Lossof the ability to perform certain tasks orassociated role changes may require signif-icant adjustment. Homemakers withrheumatoid arthritis may have to ask theirpartner to assume some of the housekeep-ing duties, and adjustment may be difﬁ-cult for both. Individuals with rheumatoidarthritis who once prided themselves asbeing self-sufﬁcient and strong may viewhaving someone else perform what theyconsider simple tasks a sign of weakness. If individuals have had to leave their job
because of rheumatoid arthritis, theirsocial identity may be threatened. Familyroles may also be changed, with otherfamily members taking over tasks onceperformed by the individual. As rheuma-toid arthritis progresses and individualsbecome more dependent on others or onassistive devices, they may feel a loss ofcontrol, which can lead to poor self-esteem.
Also contributing to poor self-esteem is
altered body image resulting from thejoint deformity or assistive devices thataccompany joint changes. However, theuse of devices can help individuals gainindependence and overcome feelings ofhelplessness. For grooming needs, individ-uals may use devices such as adaptive han-dles for combs and brushes or tooth-brushes. They may use a long-handledsponge that has a compartment to hold abar of soap. Devices such as a zipper pullor a button aid may be of help with dress-ing. Some individuals may be resistant tousing an assistive device, viewing it as“giving up” or fearing that if they use thedevice rather than their joint, they willlose their ability to perform the task.Others may be concerned about appear-ances or fear that using the assistive devicecalls attention to their condition. Thus theuse of assistive devices can be an emotion-ally charged issue. The degree of supportfrom family and friends can make a dif-ference in the individual’s willingness touse the device.
Vocational Issues in
Rheumatoid Arthritis
Individuals with rheumatoid arthritis
experience a number of work barriers,ranging from physical barriers, such ashandling, writing, and energy-relatedbarriers to psychosocial barriers, such asConditions of the Musculoskeletal System 415

hostility of others in the workplace(Allaire, Li, & LaValley, 2003). Because notall individuals with rheumatoid arthr-itis are affected in the same way, voca-tional implications will vary with theseverity of the disease and its progression.Not all individuals with rheumatoidarthritis will become totally disabled;however, most individuals will experiencereductions across a broad spectrum ofactivities.
A major limitation of the condition is
its unpredictability and not knowingwhen the condition will change and addi-tional disability will occur. Occupationsthat have signiﬁcant physical demandsmay be more difﬁcult for individuals tomaintain than those that are sedentary orrequire light activity. Even when indi-viduals are still able to perform moderatephysical work, the progressive nature ofthe condition and its potential for affect-ing mobility should be considered. Painon motion, limited motion, and muscleweakness may also affect individuals’ability to perform tasks. Tasks requiringmanual dexterity or pinch grip mayalso be difﬁcult, if not impossible, if de-formity of the hands has occurred. Workthat places stress or strain on joints mayexacerbate the disease and should beavoided.
If joints in the lower extremities are
involved, standing for long periods oftime or walking long distances may beaffected. Individuals may have difﬁcultywith climbing, stooping, bending, reach-ing, and kneeling. They may ﬁnd ituncomfortable or difﬁcult to remain inone position for long periods of time andmay need to change position frequently,since arthritic joints should not stayimmobile for long periods. If they spendlong periods of time traveling, they shouldtake frequent stops, and if on trains orplanes, walk around frequently. Individuals should attempt to organize
and plan tasks as much as possible to con-serve energy, protect joints, and minimizefatigue. They may attempt to do as muchas they can while seated. If cervical jointsare affected, individuals should avoidworking with their neck bent over. Theymay use a slanted or elevated table or deskto avoid neck ﬂexion. Individuals shouldset priorities, giving up activities of leastimportance. They may consider alternat-ing more difﬁcult tasks with those requir-ing less energy. In all cases, they shouldlearn to pace themselves, stopping to restoccasionally rather than persisting withthe task until they are exhausted.
If individual symptoms are increased by
temperature and humidity, an indoor, cli-mate-controlled environment may bepreferable. In most instances, sudden, fre-quent changes in environmental condi-tions are more bothersome than the exactlevel of temperature or humidity itself.Consequently, going in and out of exces-sively cold or warm environments shouldbe avoided.
Rheumatoid arthritis affects all activities
of daily living, not just work. Conse-quently, individuals may require extratime to get ready for work or to performtasks at home, and this can, in turn, affecttheir work schedule. The need for pre-scribed periods of rest and exercise mustalso be considered, both at home and inthe work environment. A variety of envi-ronmental alterations may assist individ-uals with rheumatoid arthritis tomaximize their functional capacity,whether at work or at home. The need forreaching and bending can be reduced withmodifications such as storing heavyobjects on lower shelves and using pull-out shelving or baskets to retrieve them.Pullout shelves can also be used to mini-mize bending and stretching for hard-to-reach items. Counters can be raised or416
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

lowered, permanently or with adjustablecomponents.
Assistive devices can help individuals
manage their work environment andtheir essential daily activities more easilyand should be used as appropriate. Devicesmay be of use for impairments in musclestrength, endurance, range of motion,manual dexterity, and mobility. Assistivedevices such as long-handled reachersmay be used to open cabinets. Knobs canbe replaced by levers so that the wholehand can be used. This may be helpful ifmanual dexterity or hand strength isaffected. Individuals with moderate tosevere rheumatoid arthritis may use amotorized wheelchair to increase mobili-ty and to conserve energy.
Although there is no impairment in
intellectual functioning or cognitive abil-ity with rheumatoid arthritis, the effect ofpain on individuals’ ability to concentrateshould be considered. The combination ofpain and the disabling consequences ofrheumatoid arthritis may be associatedwith depression, which in turn can affectfunctional capacity. Treatment of depres-sion when identiﬁed, as well as interven-tions to enable individuals to decreasetheir pain and cope more effectively, maybe beneﬁcial in maintaining their voca-tional status.
Systemic Lupus Erythematosus
The potential variety and severity of its
manifestations and the unpredictablecourse of systemic lupus erythematosuspresent signiﬁcant challenges to indi-viduals living with the condition (Sohng,2003). An autoimmune disease of un-known cause, systemic lupus erythe-
matosus can affect the skin, joints, kid-
ney, heart, lungs, nervous system, blood,and other organs of the body (Giffords,2003). It is most common in youngwomen and does not usually develop inindividuals past middle age (Trethewey,2004). The condition produces inﬂamma-tion and structural changes in manybody organs and has many neurologicaland psychiatric manifestations, includingcerebrovascular disease, movement dis-orders, seizure disorders, cognitive dys-function, and anxiety disorder (ACR AdHoc Committee on NeuropsychiatricLupus Nomenclature, 1999). It mayprogress rapidly or slowly, or it canbecome chronic with associated remis-sions and exacerbations.
Symptoms vary from individual to in-
dividual, but they may include a charac-teristic “butterﬂy rash” on the face, in-creased sensitivity to sunlight, loss ofappetite, and weight loss. As the conditionprogresses, it may have more seriouseffects, such as kidney damage; accumu-lation of ﬂuid around the heart or lungs;and mental changes, including forgetful-ness, confusion, and, in some instances,seizures.
The prognosis for individuals with sys-
temic lupus erythematosus depends onwhich organs are involved and the degreeof autoimmune reaction experienced. Thecondition is not curable and requires long-term management. For many individuals,appropriate treatment controls or sup-presses the symptoms; however, it mayalso result in death (Ruiz-Irastorza,Khamashta, Castellino, & Hughes, 1999).Some individuals experience years ofremission in which they are almost symp-tom-free, whereas others rapidly developkidney damage. Women may experienceﬂareups of disease activity at certain peri-ods of the menstrual cycle and during orafter pregnancy. Complications such ascardiac symptoms or renal damage aretreated as appropriate. Persons with severekidney involvement may require dialysis(see Chapter 13).Conditions of the Musculoskeletal System 417

Treatment of Systemic Lupus
Erythematosus
Mild cases of systemic lupus erythe-
matosus may require little or no therapy.When therapy is necessary, the type andlocation of the condition determine thetherapy prescribed. If major organs suchas the heart or kidney are involved, treat-ment is directed toward preserving func-tion and preventing organ failure thatcould result in disability or death. In mildcases, salicylates or NSAIDs may be used.
In more severe cases, steroids may be in-
dicated.
Individuals with systemic lupus erythe-
matosus may need more than the normalamount of rest. The more active the dis-ease, the more rest they need. Exercise tothe point of exhaustion and stressful sit-uations should be avoided, because bothcan cause exacerbation of the disease.
Psychosocial Issues in
Systemic Lupus Erythematosus
Although the diagnosis of systemic
lupus erythematosus may cause emo-tional reactions and psychological issues,some psychological symptoms may bemanifestations of the condition itself.Individuals with systemic lupus ery-thematosus may need considerable emo-tional support. Not only is lupus apotentially fatal disease, but most individ-uals are affected in young adulthood,when the psychosocial and vocationalimpact of the condition can have a pro-found effect.
Individuals with systemic lupus erythe-
matosus may need considerable rest,which may be difﬁcult because of otherresponsibilities. Since the stress of preg-nancy and childbirth may exacerbatesymptoms, the decision of whether or notto have children may be a difﬁcult one forwomen with the condition. The degree ofpsychosocial distress resulting from thecondition depends, to some degree, on theseverity of the symptoms.
Vocational Issues in
Systemic Lupus Erythematosus
Individuals with systemic lupus erythe-
matosus are often sensitive to sunlight,which may trigger symptoms. They mayneed to give up outdoor activities or atleast activities that entail exposure to thesun. When they cannot avoid sunlight,they may need to wear extra clothing orhats or use umbrellas to protect them-selves from the sun. Other musculoskele-tal conditions may become worse in cold,damp environments. As stated previous-ly, individuals with systemic lupus erythe-matosus may need more than the normalamount of rest. The more active the dis-ease, the more rest they need. Exercise tothe point of exhaustion and stressful sit-uations should be avoided, because bothcan cause exacerbation of the disease.While in remission, and if there is no asso-ciated, permanent organ damage, individ-uals may have few physical or emotionaldisabilities.
Gout
Gout is an arthritic disease that is char-
acterized by hyperuricemia (buildup in
the body of a substance called uric acid ).
Uric acid is a waste product of the metab-olism of substances called purines , which
are found in a variety of foods. It is nor-mally carried in the blood until it is ex-creted by the kidneys. With gout, uric acidlevels in the blood increase, either becausethe kidneys are not excreting uric acidfast enough or because the body is mak-ing too much uric acid. Excess uric acidchanges into crystals, called urate crystals ,418
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

which settle in the joints, causing swellingand excruciating pain. Individuals withgout are more likely than others to devel-op kidney stones ( urolithiasis ) (see
Chapter 13), which occasionally cause ob-struction and severe kidney damage(Terkeltaub, 2003).
Symptoms of Gout
Gout can be inherited, or it can be a
complication of another condition. Symp-toms, which appear suddenly, may be pre-cipitated by the intake of foods and bev-erages rich in purines, such as organ meats(e.g., liver, sweetbreads), gravies, and alco-hol. Symptoms may also be precipitatedby minor injury, stress, or fatigue. Attacksmay last only a few days at ﬁrst, but if thecondition is not adequately controlled,later attacks may last weeks.
If untreated, gout may result in chron-
ic joint symptoms with permanent jointdeformity and limitation of motion.Although gout cannot be cured, prophy-lactic treatment can control its symptoms.
Treatment of Gout
The objectives in the treatment of gout
are to terminate the acute attack and toprevent recurrent attacks. Treatment in-volves not only treating acute arthriticinﬂammation and urinary tract stones(urolithiasis), but also lowering urate lev-els in an attempt to prevent recurrentattacks and progression of the disease(Schlesinger & Schumacher, 2001;Wortmann, 2002).
During acute attacks, the affected joint
is placed at rest and anti-inﬂammatory
agents are prescribed. Prevention of further
attacks may require daily use of medica-tions for lowering the level of uric acid inthe blood or for increasing its excretion bythe kidneys. Occasionally, surgery is nec-essary to remove tophi (deposits of crystals
in the joints).
Individuals with gout should increase
ﬂuid intake to decrease the risk of kidneystones; follow a diet that excludes foodshigh in purines and fats, such as sardines,anchovies, organ meats, veal, or bacon;and avoid excessive alcohol intake.
Vocational Issues in Gout
Since gout can cause signiﬁcant pain in
affected joints, individuals may be unableto work during a gout attack. The extentof absences from work is dependent onthe frequency and severity of the attacks.Not all joints are affected. Consequently,the degree of functional impairment thatoccurs if there is resulting joint deformi-ty or loss of motion is dependent on thejoint involved and the degree to whichthe joint is crucial to job performance.Because stress can precipitate gout attacks,individuals should avoid stressful situa-tions or should be helped with stress man-agement.
Ankylosing Spondylitis
Ankylosing spondylitis is a rheumatic
condition that affects the joints and liga-ments of the spine. At times, it may alsoaffect the hips, ankles, or elbows. It is aprogressive condition that frequentlyleads to deterioration in spinal posture(Swinkels & Dolan, 2004). The inﬂamma-tory process around these joints causespain and can result in a fusing of thejoints, with subsequent loss and/or restric-tion of motion. Back pain of varyingintensity is the most common initial com-plaint. It is often worse at night. Othercomplaints may include morning stiff-ness that is relieved by activity and sys-temic symptoms, such as fatigue, weightloss, loss of appetite, and anemia. PosturalConditions of the Musculoskeletal System 419

abnormalities may develop with result-ing spinal deformities. If the condition isuntreated, a permanent postural deform-ity called kyphosis (hump back) may
occur.
The course of ankylosing spondylitis
and its severity are highly variable (Cush& Lipsky, 2000). With proper treatment,many individuals with ankylosing spon-dylitis have little permanent disability.There may be occasional ﬂareups whenthe symptoms become worse, but theremay also be long periods with no symp-toms (Cornell, 2004).
Treatment of Ankylosing Spondylitis
Management of ankylosing spondylitis
involves relieving pain and inﬂammationand maximizing function through phys-ical therapy and exercise. NSAIDs may beadministered to decrease inﬂammationand pain, thereby facilitating exercise.
Exercises are designed to strengthen
supporting muscles and to maintain goodposture and function. Good posture is es-sential to prevent spinal deformity. Physi-cal therapy should begin early to keep thespine as straight as possible and thus topreserve the chest’s ability to expand. Inthe case of severe deformity, surgical inter-vention may be indicated.
Vocational Issues in Ankylosing Spondylitis
Individuals with ankylosing spondylitis
experience no limitation with regard tocognitive skills, vision, or motor coordina-tion. Because of the stiffness and poten-tial fusing of joints of the spine, however,twisting and turning motions as well aslifting may be limited. If individualsdevelops kyphosis, altered self-image maybe accompanied by embarrassment and areluctance to work in situations in whichthe public is encountered.Other Conditions of theMusculoskeletal System
Osteomyelitis
Osteomyelitis (infection of the bone)
can be a debilitating condition with a longcourse of treatment (Calhoun, Laughlin,Mader, & Maher, 1998).
Causes of Osteomyelitis
There are several ways in which osteo-
myelitis can occur. Pathologic organisms
can enter the bone directly through an in-jury, such as an open or compound frac-ture in which the broken bone fragmenthas penetrated through the skin. Osteo-myelitis may also result from infection ofsurrounding tissue that then extends tothe bone. For example, ulceration of tis-sue in a lower extremity that occurs be-cause of vascular insufﬁciency (inadequate
blood and oxygen to a body part) in con-ditions such as arteriosclerosis or diabetesmay become infected, and infection maythen extend to the bone. In otherinstances, pathologic organisms that arepresent in the blood may settle and local-ize in the bone.
Treatment of Osteomyelitis
Osteomyelitis is often difﬁcult to cure.
If initial treatment is ineffective, osteo-myelitis can lead to chronic infection oramputation. Treatment may be medicalor surgical. Medical treatment consists ofthe administration of antibiotics to treatthe infection and bedrest until the infec-tion has been eradicated. Surgical inter-vention may be indicated to removeinfected tissue, replace a portion of bonewith a graft, or replace an infected pros-thetic joint. In some instances, amputa-tion may be required.420
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

Fibromyalgia
Fibromyalgia is a rheumatologic con-
dition characterized by widespread pain,aching, and stiffness in muscles and/orjoints, with associated sleep disturbance,fatigue, and extensively distributed areas
of tenderness known as tender points (Millea
& Holloway, 2000). Symptoms may rangefrom mild to moderate to severe.
The pain and discomfort associated with
ﬁbromyalgia are diffuse, involving theneck, shoulders, lower back, and hips, aswell as other sites. Fibromyalgia is not aprogressively degenerative disease anddoes not cause damage to bones or joints;consequently, there are no objective ﬁnd-ings or a deﬁnitive diagnostic test that canlegitimize the condition. As a result, thecondition is often perplexing for individ-uals with the symptoms, as well as fortheir physicians, who are unable to estab-lish a clear-cut diagnosis (Clauw, 2000).
Since there are no deﬁnitive laboratory
or radiographic tests used in the diagno-sis of ﬁbromyalgia, diagnosis is based onindividuals’ self-report of symptoms andhistory, with the identiﬁable tender pointsbeing a prime diagnostic marker. Indi-viduals with ﬁbromyalgia can also experi-ence symptoms other than musculo-skeletal pain, including headache or man-ifestations of irritable bowel syndrome (seeChapter 10). Complaints of sleep distur-bances and fatigue are common. Any sig-nificant life stress can exacerbatesymptoms.
Fibromyalgia can occur concomitantly
with other serious rheumatic disorderssuch as lupus and rheumatoid arthritis.Psychological symptoms of anxiety anddepression frequently accompany thecondition. Fibromyalgia can interfere withindividuals’ quality of life and may causeinterpersonal difﬁculties because of thesymptoms. Individuals often ﬁnd it help-ful to be reassured that the condition is“real.” Legitimizing individuals’ com-plaints of symptoms can help reestablishself-control and self-esteem, enablingthem to cope with their symptoms.
Treatment of Fibromyalgia
Fibromyalgia is a chronic condition in
which only relative improvement can beexpected. Individuals may ﬁnd neck sup-port during sleep or abdominal exercisesto relieve stress on the lower back helpful.Some individuals may ﬁnd aerobic exer-cise such as walking or swimming effec-tive in reducing pain and tenderness aswell as in helping with sleep disturbances.Recent studies have suggested that hyper-baric oxygen therapy (discussed later inthe chapter) may also be useful in treat-ment of ﬁbromyalgia (Yildiz et al., 2004).
Although simple analgesics such as acet-
aminophen or NSAIDs may be recom-mended by physicians, medications suchas systemic corticosteroids or stronger pain
relievers are not recommended. Low-dosetricyclic antidepressants (see Chapter 6) may
also be beneﬁcial to assist individuals withsleep. If individuals are experiencing stressor have other underlying psychologicalfactors that exacerbate sleep disturbancesor pain perception, stress management,relaxation, or counseling may be neededto help them cope with the condition.Some individuals also ﬁnd support groupsuseful. It is important that individualsremain physically and socially active andthat they identify and eliminate thestresses or environmental disturbancesthat may exacerbate the symptoms.
Vocational Issues in Fibromyalgia
Individuals with ﬁbromyalgia may have
repeated absenteeism at work because ofpain, fatigue, or both. The direct effect onConditions of the Musculoskeletal System 421

individuals’ ability to work depends on anumber of factors, including the nature ofthe job, the motivation to follow suggest-ed lifestyle changes, and the presence ofany underlying psychological factors.
Many individuals with ﬁbromyalgia
may need to learn how to pace them-selves, since certain physical activities maytake longer than before. Very active indi-viduals may have to cut back on activities.Individuals should be encouraged toremain active but not to push themselvesbeyond their limit.
Flexibility in scheduling may be bene-
ﬁcial. Some job modiﬁcation and restruc-turing may be necessary to preventoveruse or overexertion of muscle groups.Physical stressors that are identifiedshould also be avoided. Because sleep dis-turbance is an accompanying symptom,individuals may have difﬁculty concen-trating while at work. Because of the vaguenature of the symptoms and the fact thatthere presently is no deﬁnitive test to diag-nose ﬁbromyalgia, individuals may be sub-ject to the scrutiny of coworkers or theiremployer, who may question the legitima-cy of their diagnosis and their symptoms.Individuals may be labeled as malingerers,and resentment from coworkers mayresult. Education of employers andcoworkers can help to dispel myths andmisinformation.
DIAGNOSTIC PROCEDURES FORCONDITIONS OF THEMUSCULOSKELETAL SYSTEMRoentgenography (Radiography, X-rays)
The most widely used diagnostic tool for
musculoskeletal disorders is roentgenogra-phy (X-ray). The painless procedureinvolves positioning the body part to bestudied against photographic ﬁlm andexposing the ﬁlm by irradiation. A radi-ographic technician generally takes the X-ray ﬁlms, and a radiologist (physician
who specializes in radiation and the useof radioactive materials for the diagnosisand treatment of disease) interprets them.For musculoskeletal conditions, X-ray isuseful for identifying deformity or injuryof bones.
Arthrography
To perform an arthrogram (radiograph-
ic study of a joint), the radiologist ﬁrstinjects the joint to be examined with alocal anesthetic and then injects a specialmaterial or contrast medium and/or airinto the joint cavity. The joint is thenmoved through its range of motion and aseries of X-ray ﬁlms are taken. This diag-nostic procedure is done to identify injuryto the joint or supporting ligaments.
Diskography and Myelography
Although diskography and myelogra-
phy are similar to arthrography, theyinvolve the study of different areas of thebody. Diskography is a radiographic
study of the cervical or lumbar disks , and
myelography is a radiographic study of
the spinal cord .
Arthroscopy
Arthroscopy is the visualization of a
joint through a small instrument called anarthroscope that is inserted into the joint
to be studied. Videotaped pictures may betaken of the internal joint structures.
Arthrocentesis
Arthrocentesis is a procedure in which
the physician aspirates synovial ﬂuid with
a needle that has been inserted into the422 CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

joint cavity. The synovial ﬂuid is then
examined for abnormalities, such as blood,
crystals, or infection. In some instances,arthrocentesis may be used to remove ﬂu-id from the joint to relieve pain. If thejoint has been injured or is infected, exam-ination of blood or pus in the synovial ﬂu-id can help in determining the type ordegree of injury or infection.
Bone Scan
A bone scan is a procedure in which
radioactive substances, called radioiso-
topes , are injected intravenously. The
radioisotopes concentrate in the bone,and the amount of concentration is meas-ured by a special machine, called a scan-
ner. The scanner produces a picture of the
bone ( scan), enabling physicians to iden-
tify any abnormalities.
Magnetic Resonance Imaging (MRI)
A painless, noninvasive procedure, mag-
netic resonance imaging produces rapid de-
tailed pictures of body tissue. It is widelyused to assist in the diagnosis of a num-ber of musculoskeletal disorders, as well asdisorders of other body systems. The pro-cedure requires no radiation. It may beperformed without contrast (substance
that is injected and enables visualization)or with contrast, meaning that individu-als are injected with a contrast substanceintravenously.
For magnetic resonance imaging, indi-
viduals are placed in a horizontal cylinder,where they are exposed to a magnetic ﬁeldmuch greater than the earth’s. As a result,hydrogen atoms within the body line upparallel to the magnetic ﬁeld. Low-ener-gy radio waves are then directed into theindividual’s body, causing protons in thebody to move out of alignment. Thisprocess is called resonance . When the radiowaves are discontinued, the protonsrealign. The machine picks up the amountof energy released by the protons as theyswing back into alignment and convertsit into an image of the body part beingstudied.
Because of the strength of the magnet-
ic ﬁeld used for the procedure, individu-als with metal in their body should notundergo magnetic resonance imaging.Thus, the procedure is contraindicated forindividuals with a cardiac pacemaker, met-al clips that have been placed in the bodyas part of a prior surgical procedure, orsmall pieces of metal embedded by injury(e.g., shrapnel).
Magnetic resonance imaging is used for
diagnosing diseases of many body sys-tems. In the musculoskeletal system, mag-netic resonance imaging is helpful indiagnosing diseases of the joints, conﬁrm-ing infection of the bone ( osteomyelitis ),
discovering small fractures of the bonethat may not be detectable by othermeans, and identifying soft tissue andbone tumors.
Computed Tomography (ComputedAxial Tomography, CAT Scan, CT Scan)
Computed tomography (CT scan ) is a non-
invasive radiographic procedure that maybe performed with or without contrast (asdescribed above). It may be used to diag-nose a number of types of conditions inmany different body systems. In the mus-culoskeletal system, it may be helpful inidentifying fractures, tumors, bone defor-mities, or soft tissue damage. During theprocedure, individuals are placed withina hollow tube where X-rays are passedthrough the body part at many differ-ent angles, resulting in the CT image.Because each tissue contains a differentdensity, each density is given a numericalvalue, which is computed and displayedDiagnostic Procedures for Conditions of the Musculoskeletal System 423

on a screen. The image is then recordedon ﬁlm. The CT scan is performed by theradiologist.
Blood Tests
In and of themselves, blood tests are not
diagnostic of speciﬁc disorders of the mus-culoskeletal system; however, some bloodtests indicate inflammation or tissueinjury and, thus, may be used as part ofthe diagnostic process. For example, adetermination of the erythrocyte sedi-
mentation rate may be part of the diagnos-
tic workup for conditions such asrheumatoid arthritis. Another test, C-
reactive protein , may also be used to iden-
tify inflammatory processes or tissuedestruction.
A blood test commonly used in the
diagnosis of rheumatoid arthritis is therheumatoid factor (RF). These latex ﬁxation
or agglutination tests for RF are not deﬁn-
itive tests for rheumatoid arthritis butmerely provide supportive evidence whenindividuals have corresponding symp-toms. The blood test determines whetherthere is abnormal protein in the serum.Many individuals with rheumatoid arth-ritis have such protein, although thosewith many other conditions (e.g., tuber-culosis and bacterial endocarditis) mayalso have it.
Another blood test that may be used,
especially in the diagnosis of systemiclupus erythematosus, is the antinuclear
antibodies test . Antinuclear antibodies are
proteins found in the blood of some indi-viduals with autoimmune diseases. Theantinuclear antibody test is a blood testthat identiﬁes the presence of these pro-teins. However, since the test may be pos-itive in many different autoimmunediseases or may be positive as a resultof some medications, the test is not de-ﬁnitive.GENERAL TREATMENTS FORCONDITIONS OF THEMUSCULOSKELETAL SYSTEMMedications
Pain and inﬂammation are common
manifestations of many musculoskeletaland connective tissue disorders. Salicylates
(e.g., aspirin) are commonly the ﬁrstchoice of medication to reduce pain andinﬂammation. For some conditions, suchas rheumatoid arthritis, it may be neces-sary to prescribe as many as 15 or moretablets of salicylate a day to reach a ther-apeutic dosage. This high concentration ofaspirin in the blood may exceed the liv-er’s ability to metabolize it. The side effectsof a high salicylate dosage include gastricirritation and ringing or noise in the ears(tinnitus ).
NSAIDs may also be used to reduce the
pain and inﬂammation associated withmusculoskeletal conditions. Like salicy-lates, these drugs can irritate the stomachlining, causing pain and, in some in-stances, bleeding. Corticosteroids can pro-
duce dramatic short-term anti-inﬂamma-tory effects, but they do not prevent pro-gression of joint destruction and, becauseof their potency and subsequent sideeffects, can be used only on a short-termbasis. Some side effects of prolonged use
include cataracts, demineralization of bone,
delayed wound healing, poor resistance toinfection, and symptoms similar to thoseof Cushing’s syndrome (see Chapter 9).More serious systemic effects may involvesevere adrenal insufﬁciency following with-
drawal (see Chapter 9). Steroid use shouldalways be carefully monitored by a physi-cian, and steroids should never be discon-tinued suddenly. Although steroids formusculoskeletal conditions are generallytaken orally, they are sometimes injecteddirectly into an inﬂamed joint for the tem-porary suppression of the inﬂammation.424
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

Hyperbaric Oxygen Therapy
Hyperbaric oxygen therapy is a treat-
ment in which 100 percent oxygen is given
at two to three times the atmospheric pres –
sure at sea level. It is used for a number of
conditions in addition to musculoskeletalconditions, including carbon monoxidepoisoning, decompression sickness, radia-tion-induced tissue injury, and severe skininjury. Speciﬁc conditions of the musculo-skeletal system for which hyperbaric oxy-gen therapy is used include chronic osteo-myelitis (Sugihara et al., 2004); crush in-juries or other severe traumas to the ex-tremities that cause insufﬁcient blood ﬂowto the extremity resulting in necrosis (tis-
sue death) (Chen, Ko, Fu, & Wang, 2004);thermal burns and skin grafts or ﬂaps (see
Chapter 15) that have inadequate blood ﬂow
or oxygen and are thus not healing prop-
erly; and foot and leg ulcers associated with
diabetes (see Chapter 9) (Kranke, Bennett,Roeckl-Wiedmann, & Debus, 2004).
Hyperbaric oxygen therapy works by
restoring the body’s defense against infec-tion and increasing the rate at which thebody is able to kill common bacteria.Individuals inhale hyperbaric oxygen inthe atmosphere of a special cylindrical sin-gle-occupant chamber in which theyhave been placed or through masks,hoods, or special tubes that are insertedinto their trachea (windpipe).
The length and amount of treatment
depend on the reason for the treatment.Single treatments can range from 45 min-utes for carbon monoxide poisoning toalmost 5 hours for severe decompressiondisorders. For musculoskeletal disorders,treatments may average 90 minutes for 20to 30 treatments. Although hyperbarictreatment can be costly, when comparingthe cost with hospitalization and disabil-ity for conditions that are not successful-ly treated, the savings can be extensive.Physical Therapy
Many types of musculoskeletal disorders
can be improved through physical thera-py. These techniques are usually per-formed by a physical therapist or a physical
therapy assistant . A physical therapist is an
individual with a bachelor’s degree inphysical therapy who provides servicesthat help develop, restore, or preservephysical function. A physical therapy assis-
tant is generally an individual with an
associate’s degree who works under thedirection of a physical therapist.
The type of physical therapy admin-
istered depends on the particular mus-culo
skeletal condition. Physical therapy
may be directed toward increasing or
maintaining a joint’s range of motion,
increasing muscle strength, relieving pain
or muscle spasms, or teaching techniquesfor ambulation.
Some techniques used in physical ther-
apy involve therapeutic exercise, whichmay be passive or active. In passive exer-
cise, the therapist or a mechanical device
exercises the body part. In active exercise ,
individuals independently perform aspeciﬁed exercise regimen under the direc-tion or supervision of the physical thera-pist or physical therapy assistant. Exercisemay be designed to increase or maintainrange of motion, prevent atrophy (shrink-
ing of the muscles), prevent deformitydue to contractures, or increase musclestrength.
Other physical therapy techniques may
involve applying heat or cold or massag-ing the muscles for relaxation or relief ofpain. Heat may be applied through hotpacks, hot soaks, infrared radiation, orwhirlpool baths. Another procedure forapplying heat is diathermy, a process inwhich the temperature of the body part israised through high-frequency ultrasonicwaves. Because cold has a numbing effect,General Treatments for Conditions of the Musculoskeletal System 425

it may also be used to relieve pain. Coldpacks or chemical packs may be appliedto the painful area. Massage, the manip-ulation of muscles through rubbing orkneading, may be used to relax muscles,improve muscle tone, relieve musclespasm, or increase blood ﬂow to the area.
Casts
A variety of musculoskeletal conditions
are treated with casts. Although casts maybe synthetic, they are more commonlymade of plaster of Paris. Casts provideimmobilization and support for a bodypart while it is healing. They may also beused to prevent or correct various muscu-loskeletal deformities. The type and size ofcast depend on the condition and the pur-pose of the casting. In addition to castsused on extremities, there are spica casts,which extend the entire length of the low-er extremity from the middle of the trunkof the body. In some instances, a full body
castis necessary.
Assistive Devices
Individuals with musculoskeletal disor-
ders may use assistive devices to aid inambulation, to prevent undue strain on abody part, or to restore or enhance func-tional capacity. Assistive devices may beused therapeutically in the healing periodafter musculoskeletal injury or on a con-tinuing basis. Some examples of assistivedevices that aid in ambulation or preventexcessive weight bearing on a lowerextremity are canes , crutches , and walkers .
Other examples are those special devicesused for individuals with upper-extremi-ty deformity who need help in perform-ing activities of daily living, such aslong-handled gripper s, zipper pulls , or jar-
opening devices (see Chapter 17 for a fuller
discussion of assistive devices).Orthoses
Orthoses are devices used to straighten
or correct a deformity; they are mechan-ical devices applied to the body to controlthe motion of joints and the force orweight distribution to a body part. Abrace, for example, is an orthotic deviceused to provide support or to prevent orcorrect a deformity. The type of deviceused depends on the purpose of the brac-ing and the condition itself. An orthotist
is an individual who constructs orthoticdevices to meet individual needs.
Orthoses may be prescribed for any
musculoskeletal area, depending on thenature of the problem. For example, low-
er limb orthoses are orthopedic shoes or
orthoses for the foot, ankle, knee, or hip.Spinal orthoses may be used to relieve com-
pression forces on the spine, to restrictmovement of the spine, or to modify thealignment of the spine. There are at least50 types of spinal orthoses, classiﬁedaccording to the level of application.Cervical orthoses may be prescribed for a
wide variety of problems, ranging fromwhiplash to fracture of the cervical spine.The Taylor , the Jewett Hyperextension TLSO ,
and the CASH are spinal orthoses pre-
scribed to restrict trunk ﬂexion and rota-tion or to provide hyperextension andreduce ﬂexion in the thoracic-lumbarspine. Lumbar-sacral spinal orthoses, such
as the Knight Chairback and the Williams ,
are prescribed primarily for low back painand may consist of ﬂexible or semirigidcorsets that provide support and protec-tion. The Milwaukee brace is an orthotic
device designed for treatment of scoliosis
(lateral S-shaped curvature of the spine).
Orthotic devices may also be used for the
upper extremities. In these instances, they
are most frequently prescribed because of
injury. Upper-extremity orthoses may be ap-
plied to the shoulder, elbow, or wrist/426 CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

hand. Newer orthotic devices, called frac-
ture orthoses , are designed to allow early
ambulation on fractures of the lowerextremity. These devices permit function-al use of the extremity much earlier thandoes conventional casting. Recently, someupper-extremity fractures have also beentreated with fracture orthoses.
Traction
Individuals with a variety of musculo-
skeletal conditions may beneﬁt from trac-tion, a therapeutic method in which amechanical or manual pull is used to re-store or maintain the alignment of bonesor to relieve pain and muscle spasm (seeFigure 14–6). It may be applied in several
ways. When traction exerts a constant pull,
it is said to be continuous . If the pull is
relieved periodically, the traction is said tobe intermittent . Traction may be applied
externally or internally .
Skin traction is applied by fastening
straps, belts, or other external devicesaround the body and then to a source ofcountertraction. In contrast, skeletal trac-tion is applied internally; metal wires
(Kirschner wires), pins(Steinmann pins),
or tongs (Crutchﬁeld tongs) are inserted
through the bone surgically and attachedto a source of countertraction outside thebody. Kirschner wires and Steinmann pinsare typically used to reduce (align) fractures
of the long bones of the extremities inorder to promote bone healing or to sta-bilize the fracture until surgical treatmentcan be undertaken to correct it. Crutch-ﬁeld tongs are inserted into the skull forinjuries of the cervical spine.
The use of traction may prevent surgical
intervention in some cases, and it offersmore freedom of movement than does acast. However, it usually requires pro-
longed hospitalization. Furthermore, in the
case of skeletal traction, there is a risk ofcomplications, such as osteomyelitis, thatcan contribute to permanent disability.
Surgical Treatment
Individuals with musculoskeletal condi-
tions may require surgical treatment to cor-
rect, remove, or replace injured or diseasedGeneral Treatments for Conditions of the Musculoskeletal System 427
Figure 14–6 Skeletal Traction. Source: Copyright © 1999 Rachel Clarke.

structures. Surgery may be performed onan emergency basis in the case of traumat-ic injury or on an elective basis in the case
of disease, deformity, or an old injury. There
are several types of surgical interventions:
•Open reduction: surgical alignment
of the fractured bone.
•Internal fixation: placement of
screws, pins, wires, rods, or other de-vices through the bone to hold thebone fragments together.
•Arthroplasty: replacement of all or
part of a joint with a prosthetic deviceto relieve pain or to restore function.Common sites of arthroplasty are the
hip, shoulder, knee, and elbow. Reasons
for arthroplasty include a broken hip(total hip replacement ) or arthritis.
•Arthrodesis: surgical fusing (joining)
of two joint surfaces, making thempermanently immobile. The proce-dure was once commonly performedto relieve joint pain. With improvedarthroplasty procedures, however,arthrodesis is now less common.
•Synovectomy: surgical removal of
the synovial membrane surrounding a
joint. Synovectomy prevents recurrent
inﬂammation, thus reducing jointpain and further joint destruction.
•Laminectomy: surgical removal of a
portion of a vertebra, exposing thespinal cord. It is usually performed tofacilitate the removal of any source ofpressure on the spinal cord (e.g., toremove bone fragments from spinalcord injury; to remove tumor fromthe spinal cord).
•Spinal fusion: grafting of bone from
another area of the body into the diskinterspace after a surgical procedureon the spine (e.g., laminectomy).After spinal fusion, mobility at thepoint of the fusion is lost. •Carpal tunnel repair: a surgical pro-
cedure in which the median nerve isdecompressed by the transection ofsurrounding ligaments. It is per-formed when the symptoms of carpaltunnel syndrome are severe andinclude progressive sensory loss in theﬁngers and hand.
PSYCHOSOCIAL ISSUES INCONDITIONS OF THEMUSCULOSKELETAL SYSTEMPsychological Issues
The emotional needs of individuals with
musculoskeletal conditions often relate toprolonged dependence on others, thelong-term nature of the condition, anduncertainty about the ability to resumenormal responsibilities and activities.Restrictions on mobility and naturalmovements because of casts, braces, ortraction or because of pain, deformity, orabsence of a limb are, for many individu-als, unbearable. Depending on the extentof immobility, they may have a sense ofpowerlessness leading to anger, hostility,and, later, depression. If the muscu-loskeletal condition necessitates giving upsome valued activity permanently, depres-sion may deepen. Some individuals whohave a strong athletic identity may fail todisclose their condition, continuing theactivity even though it may cause addi-tional damage.
The prolonged pain associated with
many musculoskeletal system conditionsconsumes energy and may contribute toincreased self-centeredness and depend-ence as pain becomes the central issue inindividuals’ lives. The discomfort, as wellas the limitations on mobility, can con-tribute to irritability, discouragement,and depression. Pain perception is relatednot only to various personality factors, but428
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

also to factors such as workers’ compen-sation, litigation, or other beneﬁts thatmay decrease individuals’ motivation toreduce pain or restore function.
Individuals planning to undergo musculo-
skeletal surgery may experience a mixtureof fear and anticipation regarding the ex-tent to which the surgery will restore lostfunction. Those having repeated surgery,such as a second joint replacement forarthritis or continuing treatment for os-teomyelitis, may lose patience and hope.
Individuals with chronic conditions of
the musculoskeletal system may forcethemselves to do more than they comfort-ably can because of fear of being a burdento others. Inability to maintain previousactivity levels may cause continued frus-tration. The unpredictability of conditionsthat have remissions and exacerbation(e.g., rheumatoid arthritis) may also be asource of tension.
The deformity associated with many
musculoskeletal conditions, such as rheu-matoid arthritis, ankylosing spondylitis,osteoporosis, or amputation, alters bodyimage. Most individuals react to anybody image change with anxiety and fear
of rejection. Concern about appearance can
lead to continual worry about acceptanceby family, friends, and acquaintances.
Lifestyle Issues
Although many conditions of the mus-
culoskeletal system require only short-term treatment and impose only tempor-ary disability, some conditions require life-long adaptation and signiﬁcant lifestylechanges. Restrictions of body movementresulting from a loss of muscle strength,deformity of joints, or pain alter the activ-ities of daily living, as well as social andrecreational activities. Individuals mayneed to learn new ambulation and trans-fer techniques and ﬁnd alternatives toactivities that place undue stress on joints.It may be necessary to install grab bars andsafety rails in the home to provide stabil-ity and prevent falls.
Conditions affecting the ankles or the
feet may require that individuals wear spe-cial shoes for comfort and protection ofthe joints. Sitting while performing manytasks, such as meal preparation, may savewear and tear on weight-bearing joints.Conditions affecting joints of the hands,such as rheumatoid arthritis, may requirethe use of assistive devices, such as hooks,zipper pulls, special openers, or other self-help aids, if individuals are to performactivities of daily living independently.Adaptive handles for combs and brushesmay be of help for grooming. Soft leadpencils and felt-tipped pens may be use-ful for decreasing pressure on ﬁnger jointswhen writing.
At home, work centers may be estab-
lished where all the items needed for aspeciﬁc task are kept within easy reach. Itmay be necessary to lower tables and cab-inets so that individuals with a muscu-loskeletal disorder may be seated whilethey work and to raise beds, toilet seats,and chairs so that sitting and arising areeasier. Organizing and planning dailytasks can help to reduce strain and fatigue.
Dietary modiﬁcations may also be nec-
essary in some conditions of the muscu-loskeletal system. Obesity places extrastrain on joints; consequently, if obesityis an issue, a weight reduction diet may beprescribed. Speciﬁc conditions, such asgout, also require dietary modiﬁcations.Individuals with pain or deformity in thehands or those who have undergoneupper-extremity amputation may needspecial adaptive eating utensils for activ-ities such as cutting meat.
Many conditions of the musculoskele-
tal system require some form of therapeu-tic exercise to maintain joint function,Psychosocial Issues in Conditions of the Musculoskeletal System 429

restore strength and/or joint motion, orprevent deformity. Such exercise pro-grams must be incorporated into the dai-ly routine. Conditions such as rheumatoidarthritis may require speciﬁed rest periodsduring the day.
Most conditions of the musculoskeletal
system do not hamper sexual activity; how-
ever, pain, deformity, decreased range ofjoint motion, or alteration in body imagemay affect sexual function. Positioningmay be difﬁcult or painful, as in the caseof rheumatoid arthritis or low back pain.In some instances, the medications usedfor the treatment of musculoskeletal dis-orders can affect sexual function. Steroidsprescribed for a number of musculoskele-tal conditions may decrease libido, andpain may inhibit sexual desire.
Social Issues
Because conditions of the musculoskele-
tal system can impair mobility andbecause in some cases individuals mustdepend on others for assistance, the sup-port and understanding of family andfriends are paramount. Reassurance thatphysical changes or deformities are unim-portant can be valuable to individuals’self-esteem and conﬁdence; however, suchreassurance by family and friends is notalways forthcoming.
Depending on the extent of discomfort,
limitation of motion, and deformity, indi-viduals may be unable to perform all oftheir previous tasks, making it necessaryfor other family members to share house-
hold chores and duties. Their willingness or
lack of willingness to accept necessary alter-
ations in home life can affect how indi-viduals adjust to their condition. Whenwork or social activities are signiﬁcantlyaltered by the individual’s musculoskele-tal condition, his or her social identitymay be altered. These role changes may bea source of stress. If friendships have devel-oped around speciﬁc activities that nowmust be altered because of the condition,individuals may feel a sense of social iso-lation and of no longer ﬁtting in.
When mobility is altered because of a
musculoskeletal condition, it may be nec-essary to plan vacations around the con-dition. Other manifestations of themusculoskeletal condition may also needto be considered when planning vaca-tions. Individuals with systemic lupus ery-thematosus may have to avoid hot, sunnybeaches, whereas individuals with othermusculoskeletal conditions may need toavoid colder climates. Individuals who areexperiencing severe pain may be reluctantto venture on vacation at all.
Family and friends may have difﬁculty
in coping with the feelings of hostility,frustration, or irritability expressed byindividuals because of pain or increaseddependency. Others may view these indi-viduals as demanding, manipulative, anddifﬁcult. Depending on the premorbidfunctioning of the family and the degreeand quality of communication betweenfamily members, family dynamics can bean increased source of tension.
Individuals with musculoskeletal disor-
ders, especially those with ongoing pain,are especially vulnerable to unorthodox,unproven “miracle cures.” Although alter-native and complementary practices havea place in the treatment of musculoskele-tal conditions (Pelletier, Astin, & Haskell,1999), some unscrupulous individualscan take advantage of individuals’ vulner-ability by marketing and selling methodsthat are dubious. In addition to theexpense, many fraudulent measures havedangerous side effects that, in someinstances, can be fatal. Even if there are noside effects, individuals may use thesemethods in place of recommended treat-ment, thus losing the therapeutic effects430
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

of legitimate treatment. When comple-mentary and alternative methods areused by individuals to treat musculoskele-tal conditions, the safety of the methodand the legitimacy of the individuals pro-viding it should always be determinedbefore use.
VOCATIONAL ISSUES IN CONDITIONSOF THE MUSCULOSKELETAL SYSTEM
The disability associated with work-
related musculoskeletal disorders is anincreasing problem. Although many indi-viduals with work-related injury return totheir job, a substantial number of individ-uals do not (Turner et al., 2004). Theimpact of musculoskeletal disorders onvocational function depends on the typeof job previously held and on individualfactors related to job history and motiva-tion, as well as on the type of muscu-loskeletal disorder (Rahman, Ambler,Underwood, & Shipley, 2004). Theamount of sitting, bending, stooping, orlifting that the job requires must be con-sidered. Modiﬁcation of the work environ-ment, such as by raising or loweringworktables or chairs, may be necessary.Generally, it is important that individualsreturn to work and daily activities as soonas possible to maintain good work habits.
Injured worker programs and work-
hardening programs have gained popular-ity for helping individuals return to work.These programs use a systemic approachof case management, evaluation, andtreatment that prepares workers for suc-cessful and safe reentry into the workforceafter injury (Schonstein, Kenny, Keating,& Koes, 2003; Weir & Nielson, 2001).
In injured worker programs, individuals’
physical capacity or level of function isevaluated as they are progressed throughgraded levels of job simulation tasks.Evaluation provides objective data regard-ing their physical and functional capaci-ty so that goals and a treatment plan canbe established. Services are provided on anoutpatient basis. Work tolerance screeningfocuses on individuals’ musculoskeletalstrength, endurance, speed, and ﬂexibili-ty. Functional capacity evaluation docu-ments individuals’ ability to return towork from a physical, behavioral, andergonomic perspective. Work condition-ing (work hardening) prepares individualsto return to competitive employment(Johnson, Archer-Heese, Caron-Powles, &Dowson, 2001). The goal is to increasework tolerance, increase work rate, helpindividuals learn to control symptoms,increase conﬁdence and proﬁciency, andhelp individuals learn to use work adap-tations and assistive devices. The programis highly structured. Along with simulat-ed or real-work tasks, this structure instillsexpectations for the real-world environ-ment, such as promptness, attendance,and appropriate dress.
Assistive devices such as crutches, walk-
ers, or canes may make ambulation slow-er and more difﬁcult. In addition, the useof such devices makes it difﬁcult, if notimpossible, to carry objects from onepoint to another.
Rheumatoid arthritis, ankylosing spon-
dylitis, systemic lupus erythematosus,and a number of other connective tissuedisorders are progressive in nature andoften unpredictable. Although not all in-dividuals with these conditions becomeseverely disabled, ongoing medical careand evaluation are necessary. When thecondition has remissions and exacerba-tions, individuals may have unexpectedperiods of exacerbation in which work ismissed. Individuals may need to avoidoverexertion, stress, and fatigue, whichmay in turn necessitate altered or short-ened work schedules to accommodateperiods of rest. Vocational Issues in Conditions of the Musculoskeletal System 431

Overuse of damaged joints should be
avoided. For example, individuals withosteoarthritis of the knees should avoidexcessive walking; those with carpal tun-nel syndrome should avoid repetitiveactivities with the hands. Deformity ofjoints not only may interfere with occu-pational function, but also may be poten-tially embarrassing to the individual inpersonal interactions at work. Occasion-ally, barriers such as ﬁnancial disincen-tives, the status of legal claims, and otherdisability and compensation protocols caninterfere with effective rehabilitation ofindividuals with musculoskeletal disor-ders. Individuals may be hesitant to learnnew skills or use devices that help themto maintain independence if, in so doing,they imperil possible ﬁnancial beneﬁts orare expected to return to a work environ-ment they did not or will not enjoy.
CASE STUDIES Case I
Mr. P., a 52-year-old self-employed farm-
er, was mowing his pasture when his trac-tor hit a hole, throwing him from thetractor and subsequently running over hisright leg, crushing it. He was rushed to thehospital; however, the injury was sosevere that his leg was amputated abovethe knee. Mr. P. received a lower-extrem-ity prosthesis but experienced consider-able depression after his injury, and he wasunmotivated to learn to walk with hisprosthesis. His wife, a homemaker, hascontinued to be supportive, although shehas expressed concern about his lack ofresponsiveness to the support she andfriends have attempted to provide to him.Mr. P. grew up on a farm and has had noother type of employment. He has a highschool education. Questions
1. What factors in addition to his injury
will contribute to Mr. P.’s effectiverehabilitation?
2. What other sources of referral might
be helpful to Mr. P. in his rehabili-tation?
3. How would you approach Mr. P.
about his rehabilitation?
4. What special equipment or adaptive
devices might be helpful to Mr. P. ifhe chooses to go back to farming?
5. What special equipment or adaptive
devices might be helpful to Mr. P.in terms of his functional capacityon a day-to-day basis unrelated tofarming?
6. What environmental factors might
you consider when helping Mr. P.with a rehabilitation plan?
Case II
Mrs. R., a 45-year-old medical transcrip-
tionist, has worked in her current occupa-tion for the past 20 years. Her job involvesusing a transcribing machine with aheadset and foot pedal to transcribe dic-tated reports. She began noting morningstiffness in her hands several years ago.The stiffness has become worse and nowalso involves her elbows and shoulders.She has been diagnosed as havingrheumatoid arthritis. Currently herrheumatoid arthritis is in remission withthe help of large quantities of aspirin eachday, which are causing her to experiencetinnitus.
Questions
1. What medical factors about Mrs. R.’s
condition should be considered whenassessing her rehabilitation potential?432
CHAPTER 14 C ONDITIONS OF THE MUSCULOSKELETAL SYSTEM

2. Is Mrs. R.’s current job a good choice
for employment given her current
medical condition? Why or why not?
3. Are there assistive devices or job mod-
iﬁcations that could help Mrs. R.maintain her current employment asa medical transcriptionist?
4. Is there additional medical informa-
tion that could be helpful in estab-lishing Mrs. R.’s rehabilitation plan?References 433
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Skin Conditions and BurnsCHAPTER 15
437NORMAL STRUCTURE ANDFUNCTION OF THE SKIN
The skin is the largest organ of the body.
It has a number of functions:
• Protection of the body’s inner struc-
tures from microorganisms, drying,and trauma
• Regulation of body temperature
through evaporation of perspirationfor cooling and constriction of super-ﬁcial blood vessels to conserve heat
• Excretion of water and electrolytes
through perspiration
• Sensory perception of touch, pressure,
and pain
The skin consists of two layers, the epi-
dermis and the dermis (see Figure 15–1).
The outer layer of the skin ( epidermis )
protects the deeper tissues from drying,from invasion by organisms, and fromtrauma. The epidermis has several layers.The deepest layer of the epidermis con-stantly produces new cells, which arepushed to the surface of the skin; therethey die, are shed, and are replaced by newcells. Cells called melanocytes contain the
skin pigment melanin , which is responsi-
ble for skin color. Dark-skinned peoplehave more melanin than do light-skinnedpeople.The inner layer of skin ( dermis ) lies
beneath the epidermis. It contains bloodvessels, nerves, lymphatics, hair follicles,and sebaceous and sweat glands, as wellas various types of cells that promotewound healing. The dermis also containsmajor sensory ﬁbers responsible for distin-guishing pain, touch, heat, and cold.
With the exception of the palms of the
hands and the soles of the feet, hair folli-cles are located in the dermis throughoutthe body, although they are more numer-ous is some areas, such as the scalp, axil-lae, and pubic area. Hairs are continuallyfalling out and being replaced by newones. When this process is excessive, thin-ning or baldness results.
Sebaceous glands , contained within the
dermis and surrounding hair follicles, pro-duce an oily substance called sebum that
protects the skin from excessive dryness.Sweat glands , also located in the dermis,
are present all over the body but are con-centrated in the axillae, forehead, palmsof the hands, and soles of the feet. Theyproduce perspiration, which aids in tem-perature regulation of the body as well asexcretion of water and electrolytes. Whenthe environment is warm, evaporation ofperspiration cools the body. When theenvironment is cool, constriction of super-
ﬁcial blood vessels conserves body warmth.

Interfacing with the dermis at its lower
level is a subcutaneous (under the skin)
layer of fat, or adipose tissue . This subcu-
taneous fat not only provides insulationfor the body, but it also gives shape andcontour over bone.
PSYCHOLOGICAL, SOCIAL, ANDVOCATIONAL IMPACT OF SKINCONDITIONS
The skin is readily visible, and its con-
dition determines to a great extent howindividuals appear to the world. Therefore,skin conditions, although not generallylife-threatening, can have an impact onquality of life, restricting work, social, fam-ily, leisure, and sexual activities (Lamberg,1997; Morgan, McCreedy, Simpson, &Hay, 1997). Healthy skin is correlated withself-esteem and self-image. A skin disorderthat may seem trivial to others can havea major psychological impact on the indi-vidual who experiences it. Society placesgreat emphasis on appearance, and ap-pearance often helps to determine how in-dividuals interact with society. Not onlydoes society place great value on clear,healthy skin, but skin disorders are some-times perceived as being associated withuncleanliness or contagion.
Even though few skin conditions are
actually contagious, people may avoidcontact with individuals with skin condi-tions because of fear that they are conta-gious. Disﬁguring skin conditions have anegative effect not only on individuals’self-image but also on interpersonal rela-tionships, often leading to stigmatizationby society. Individuals with obvious skindisease or scarring due to burns or traumamay experience stares, expressions ofrevulsion, or avoidance by others so thatthey become social outcasts. As a result,they may experience signiﬁcant secondarypsychological symptoms, including de-438
CHAPTER 15 S KINCONDITIONS AND BURNS
Hair
Subaceous
GlandsEpidermis
Dermis
Sweat GlandSubcutaneous
Fat
Figure 15–1 Section of Normal Skin.

Skin Conditions 439
pression, social phobia, or paranoia. Thepsychological implications of skin condi-tions may extend to all domains of indi-viduals’ lives, including the workplace.
SKIN CONDITIONS
Because the skin is in constant contact
with the environment, it is vulnerable toinjury and irritation. It is also vulnerableto changes in the internal body environ-ment, and it may reﬂect systemic condi-tions, such as lupus erythematosus (see
Chapter 14). Emotional factors can alsoprecipitate or contribute to disorders ofthe skin. Skin disorders may be localizedor may involve the entire body. They maycause mild discomfort or severe pain anddisﬁgurement.
Dermatitis
The general term dermatitis describes a
superﬁcial inﬂammation of the skin. Atopic
dermatitis , also called eczema , is a type of
dermatitis characterized by redness ( ery-
thema ), swelling ( edema ), and itching
(pruritus ). It is more common in child-
hood, but it can be a lifelong condition.Depending on its location, it can affectappearance, especially if on the face.Constant scratching of the skin can causetenderness and bleeding. If the skin’s pro-tective outer layers crack, individuals arealso at risk for infection.
Treatment of atopic dermatitis includes
avoiding prolonged contact with hotwater (i.e., taking lukewarm showersrather than long, hot baths), avoiding dry-ing soaps, and using moisturizers on theskin. Medications such as antihistaminesor steroid creams and ointments may beused to control itching; however, lengthyuse of steroid medications is contraindi-cated because of their potential sideeffects. New medications called topical im-munomodulators are also now used. Insevere cases, phototherapy (light therapy)or photochemotherapy (combination ofultraviolet light and special medication)may be used.
Contact dermatitis is a localized skin
inﬂammation that results from contactwith a speciﬁc substance. The symptomsoccur at the site of contact. The substancemay produce a localized allergic response(allergic contact dermatitis ) as a result of a
previous exposure, or the substance maybe a primary irritant that causes a nonal-lergic skin reaction ( irritant contact dermati-
tis) following exposure. Common causes
of localized allergic contact dermatitis arechemicals, dyes, cosmetics, and industri-al agents. Alkalis, acids, metals, salts, sol-vents, and various dusts can cause irritantdermatitis. Usually, only the skin thatcomes into contact with the substance isinvolved, so the area of skin affected israther clearly demarcated. Symptoms gen-erally disappear when contact with theoffending agent is avoided. In addition tolocalized allergic reactions, individuals canexperience generalized allergic reactions,as described below.
Allergic Reactions
An allergy is a hypersensitivity to a spe-
ciﬁc substance or substances. Some indi-viduals experience allergic reactions afterexposure to certain substances that causean immune response within the body.Their sensitization to the substance maytake days or weeks, but, once the responsehas been established, the next contactwith the substance produces allergicsymptoms.
Allergic responses may be external or
systemic. External allergic reactions con-sist of symptoms such as hives ( urticaria ),
redness, swelling, itching, or rash. Sys-temic allergic responses, usually caused by

allergic reactions to medication or certainfoods, may include skin manifestations inaddition to generalized body symptoms,some of which can seriously compromiserespiratory function. The treatment ofallergy is usually directed toward avoidingcontact with the offending agent, reduc-ing sensitivity to the substance if contactcannot be avoided, or reducing or elimi-nating the symptoms associated with theallergic response.
Psoriasis
Psoriasis is a chronic, inﬂammatory dis-
ease in which there is greatly acceleratedepidermal cell turnover. As a result of therapid formation of these cells, individualsdevelop noticeable skin lesions. There aredifferent variations of psoriasis. It is cate-gorized as localized or generalized depend-ing on the severity of the condition andits overall impact on the individual’s qual-ity of life and well-being (Pardasani,Feldman, & Clark, 2000). Plaque psoriasis
is characterized by plaques of erythema
(redness), covered with silvery scales,which tend to shed. Patches or plaquesmay occur on localized areas, such as theelbows and knees, lower back, and thescalp, or they can cover the entire body.In some instances, individuals developpustular psoriasis , in which small pustules
are spread over the body; these can some-times lead to systemic infection. Someindividuals with psoriasis develop psoriat-
ic arthritis , which causes aching and dis-
ﬁgurement of joints.
Although the primary cause of psoria-
sis remains unknown, it is considered animmune-mediated disorder (Pardasani etal., 2000). Psoriasis may be triggered by acombination of genetic, systemic, or envi-ronmental factors. There are periods ofremission (when symptoms become bet-
ter) and periods of exacerbation (whensymptoms become worse) of varying fre-quency and duration. The course of thepsoriasis is often unpredictable and canimprove or get worse for no obvious rea-son. Emotional stress and anxiety mayaggravate the condition. Climate changeor warm temperatures also tend to makethe condition worse.
Psychosocial Impact of Psoriasis
Psoriasis ranges from being cosmetical-
ly annoying to being physically disablingand disﬁguring. It does not affect the indi-vidual’s general health, but the psycholog-ical and social stigma associated with anobviously unsightly skin disease maycause frustration and despair. It can causedifﬁculty with work performance, prob-lems with social rejection, sexual dysfunc-tion, and depression. Itching may be mildor severe and can cause loss of sleep andgeneral fatigue, which can contribute toirritability. The condition can be a burdenon ﬁnancial and time resources, interferewith work, and disrupt the individual’slifestyle. Although psoriasis is not infec-tious, it may be a source of stares, embar-rassing questions or comments, oroutright avoidance of the individual byothers. The prognosis depends on theextent and the severity of the disease. Ingeneral, the earlier the disease begins, themore severe are the manifestations.
Treatment of Psoriasis
There is no known cure for psoriasis, so
treatment is directed toward controllingthe condition. The goal of treatment is tosuppress the immune-mediated response,which causes the symptoms. Any aggrava-ting factors should be identiﬁed and re-moved if possible. Since injury to the skincan trigger ﬂareups, trauma to the skinshould be avoided as much as possible. 440
CHAPTER 15 S KINCONDITIONS AND BURNS

Treatment depends on the severity of
the condition. Psoriasis can be treatedwith topical agents, or, if symptoms donot respond to topical treatment, it maybe treated with a systemic agent. If thecondition is limited, most dermatologists
(physicians who specialize in diagnosisand treatment of conditions of the skin)initially treat psoriasis with a topical
steroid . There are seven potency levels of
topical steroids. The strength prescribeddepends on the area to be treated and thethickness of the plaques. For example, aweaker strength of steroid would beapplied to the face. Topical steroids areapplied one to three times a day with anaim to decrease immune activity locally.Emollients are often used in combinationwith topical steroids to increase their efﬁ-cacy. Although they are relatively safe andhave few, rare systemic side effects, topi-cal steroids cannot be used indeﬁnitely. Ifapplications are applied too frequently orif too strong an agent is used, atrophy
(shrinkage) or thinning of the skin canoccur. Long-term use of steroids can leadto a phenomenon called tachyphylaxis ,
a condition in which the body becomesimmune to the effects of the medicationbecause of repeated use.
Topical steroids can also be used in com-
bination with other agents, such as vita-
min D analogs or topical retinoids. Combi-
nation therapy allows lower doses of indi-vidual agents to be used, helping to min-imize side effects and maximize efﬁcacy.
Older, but still effective topical treat-
ments include the use of coal tar prepara-tions or anthralin , a cream most often used
to treat scalp psoriasis. Coal tar can be for-mulated into shampoos, gels, or solutionsfor soaking. Although the mechanism ofaction is not clear, coal tar seems to reduceinﬂammation. Coal tar preparations areinexpensive but can be messy to use,staining both skin and clothing. Anthralincan also stain skin, clothing, and bedding.Because of some reports that link coal tarto cancer, some countries and states pro-hibit its sale.
Individuals with moderate to severe pso-
riasis may have any of the above treat-ments used in conjunction with photo-
therapy , which can lead to remission. Both
ultraviolet B and ultraviolet A are used. Inphototherapy, individuals come to thephysicians’ ofﬁce and spend several min-utes in a light booth, where they receivea regulated dose of ultraviolet light. Thelight decreases the activity of the immunesystem. Ultraviolet B therapy can beeither broadband or, more recently, nar-row band. The dose of light is based onindividuals’ skin type and the minimaldose that produces redness ( erythema ).
When combined with other topical or sys-temic medications, care must be taken notto increase individuals’ photosensitivity
(sensitivity to light), which could result inburns.
Some individuals do not respond to
phototherapy, or they may be unable toreceive it because of the traveling distancebetween the treatment facility and theirhome, or because of their work schedule.In these instances, systemic medicationsmay be used instead. Medications such asmethotrexate, aceitretin, or cyclosporinemay be used to treat moderate to severepsoriasis. Although all three are effective,they have side effects that require frequent
blood monitoring (Lebwohl, 2000), includ –
ing liver damage, renal damage, increasedblood lipids, and bone marrow suppres-sion. Other side effects include hyperten-sion and dryness of the skin and mucousmembranes. In addition, the medicationscan be teratogenic (causing fetal abnor-
malities) and so should not be used whenthere is a chance of pregnancy.
The newest treatment options for pso-
riasis are biological drugs . This class of med-Skin Conditions 441

ication consists of substances derivedfrom living material that are injected orgiven intravenously. They act at the cel-lular level and affect various targets in theimmune system that are involved in thepathophysiology of psoriasis. These med-ications have been shown to have equalor better efﬁcacy than older systemic treat-ments of psoriasis, and they appear tohave few side effects. A major limitationto their use is, however, expense, sincethey cost up to $1,000 per month.
Infections of the Skin
A number of organisms, including bac-
teria, fungi, parasites, and viruses, mayinfect the skin. Infection may be the pri-mary cause of a skin disorder, or it may bea secondary condition associated withanother skin disorder. The degree andlength of disability associated with infec-tions of the skin depend on the type andseverity of the infection. Effective treat-ment requires that one accurately identi-fy the causative organisms and institutetreatment that is appropriate to those par-ticular organisms.
Acne
Acne is the most commonly encoun-
tered skin condition. It results from inter-action between bacteria in the skin, excessoil production, and hormones. The face,neck, and trunk of body are most fre-quently affected. Although acne is mostcommon in adolescence, some individu-als, especially women, have acne that con-tinues into young adulthood. Acne initself is not frequently thought of as a dis-abling condition; however, it can have adevastating effect on the individual’s self-image and self-esteem. The goal of treat-ment is to prevent the clogging of hairfollicles, reduce inﬂammation, cut downon infection, and minimize scarring.Treatment usually consists of topicalapplication of medication and, occasion-ally, systemic medication in severe or pro-longed cases. In some instances individ-uals with severe scarring from acne maychoose to have cosmetic procedures suchas resurfacing or dermabrasion (a proce-
dure in which scars, wrinkles, or otherskin blemishes are worn away) to dimin-ish the scarring once their acne is nolonger active.
Herpes Zoster (Shingles)
Herpes zoster , or shingles , is a reactivation
of the virus that caused chickenpox inindividuals at a younger age. After theindividual has had chickenpox, the virus
lies dormant in the nervous system. When
an individual’s immune system becomesweakened because of aging or because ofmedical conditions such as organ trans-plantation, cancer, or HIV infections, thevirus can become reactivated. Vesicles
(ﬂuid-ﬁlled blisters) erupt along a periph-eral sensory nerve route. The blisters,which form a band along the nerve, areusually located on the trunk of the body,causing pain, itching, burning, and ten-derness along the nerve route. Althoughvesicles usually appear on the trunk of thebody, they may also affect the face andeye. Pain in the affected area may besevere. The condition may last up to amonth. Since the reactivated virus is con-tagious, individuals who have never hadchickenpox or who have never beenimmunized against chickenpox shouldavoid contact with individuals with her-pes zoster.
Treatment of Herpes Zoster
The goal of treatment is to relieve pain,
reduce potential complications, and short-442 CHAPTER 15 S KINCONDITIONS AND BURNS

en the duration of the outbreak. Antiviralmedication, administered either orallyor intravenously, is often required. Ster-oids or anti-inflammatory medicationmay also be used. The pain accompany-ing herpes zoster is often treated withanalgesics.
Herpes zoster usually has no residual
effects; however, complications of thecondition can include prolonged pain atthe site of the skin lesion even after lesionshave subsided. Complications can alsoinclude scarring, which may be quite dis-ﬁguring if the scars involve the facial area.If the eye is affected, another complicationmay consist of ulceration, which couldresult in blindness.
Skin Cancers
Cancer of the skin occurs more frequent-
ly than does cancer of any other organ.Because basal cell carcinoma is directly
visible, it can be diagnosed earlier and,therefore, has a high cure rate. Malignant
melanoma , a cancer originating in the
melanocytes (cells containing skin pig-
ment), is a more dangerous and potential-ly fatal type of skin cancer because itspreads rapidly into deeper skin layers andmetastasizes to other body organs (seeChapter 16). Because of the seriousness of
the condition, surgical removal of the mela-
noma itself, as well as large portions of sur-rounding tissue, may be necessary to erad-icate the cancer; this can cause signiﬁcantdeformity, depending on the location.
GENERAL DIAGNOSTIC PROCEDURESFOR CONDITIONS OF THE SKINBiopsy
Biopsy consists of removing a speciﬁc
tissue specimen for microscopic examina-tion. Biopsies are performed to diagnosea variety of conditions, including skin can-cer and many other types of skin lesions.It is a relatively simple procedure that canbe performed on an outpatient basis.
Scrapings, Cultures, and Smears
Scales of a skin lesion may be gently
scraped from the surface of the skin and
examined under a microscope. If there isan exudate (ﬂuid or matter from tissue),
a sample is removed with a swab andimplanted in a culture medium , where it is
later examined for growth of organisms.In other instances, the exudate is placedon a slide and examined immediatelyunder the microscope; this procedure isknown as a smear .
Patch Tests
In order to identify the substances that
are responsible for allergic reactions, patch
testsmay be performed. Small amounts of
various substances that are suspected ofcausing the reaction are applied to theskin, and the area is later examined forpossible reactions.
GENERAL TREATMENT OFCONDITIONS OF THE SKINMedications
Many skin disorders are treated with
topical medications that are applied di-rectly to the skin surface ( topical applica-
tion) in the form of lotions, creams,
ointments, or powders. The type of medi-cation chosen is dependent on the causeof the skin disorder. For example, antifun-
galsare used for fungal infections, antibi-
otics or antibacterials for bacterial
infections, and antivirals for viral infec-
tions. Topical antipruritics may be appliedGeneral Treatment of Conditions of the Skin 443

to reduce the discomfort due to itching.Topical corticosteroids are sometimes pre-
scribed to reduce local inflammatoryresponses.
Because topical medications can have
side effects, prolonged use or overuse ofmedications such as corticosteroids shouldbe avoided. Some skin conditions may betreated with systemic medication (medica-
tions that are injected or taken orally tobe carried throughout the body), such asantibiotics and corticosteroids. Althoughcorticosteroids can produce dramatic im-provement, they also have serious poten-tial side effects. Consequently, the use ofcorticosteroids requires careful monitoringby a physician.
Dressings and Therapeutic Bathsor Soaks
Treatment of skin conditions in which
there is excessive skin scaling or in whichcrusts have formed over lesions may in-clude wet soaks or therapeutic baths to re-duce the drying effects of air, relieve dis-comfort, or enhance the removal of scalesand crusts so that healing may take place.In some instances, dressings are applied toskin lesions to protect the skin from injuryand infection from the environment.
Light Treatment (Phototherapy)
Artiﬁcial light sources may be used for
localized or generalized treatments of var-ious skin conditions. Light therapies arefrequently accompanied by therapeuticbaths or soaks, or they may be used incombination with topical medication.
Dermabrasion
Dermabrasion consists of bufﬁng, or
abrading, the top surface of the skin inorder to reduce scarring.Chemical Face Peeling
This procedure produces a controlled
chemical burn and destruction of theupper layer of the skin. It is generally donefor cosmetic purposes to remove ﬁne linesor blemishes, but it can also be helpful inthe treatment of acne and precancerousgrowths. Individuals with chemical facepeeling should avoid the sun and be awarethat the skin will not tan evenly.
Plastic and Reconstructive Surgery
Plastic and reconstructive surgery is a
branch of surgery involving the correctionof deformity, the restoration of functionof parts of the body, or the enhancementof physical appearance. It plays an impor-tant part in rehabilitation, not only toenhance healing and establish or reestab-lish function, but also to enhance individ-uals’ self-image and minimize limitations.
Although plastic surgery is important to
conditions of the skin, plastic surgeons donot limit surgery to only one body part,but rather use concepts and techniques ofplastic surgery on many parts of the body.For example, it may be used to minimizeor correct congenital anomalies such ascleft lip or cleft palate. It may be used torestore function lost due to contractures
(tightening or shortening of tissue arounda joint that limits range of motion). It maybe used to correct deformity and restorefunction after hand injury or to promotehealing and correct deformity causedfrom complications such as decubitus ul-
cers(also known as pressure sores, caused
by immobility and lack of blood supply totissue so that tissue death occurs). Plasticand reconstructive surgery may also beused to correct deformities secondary toa variety of medical conditions or theirtreatment, such as cancer, in which a largeportion of tissue has been removed.444
CHAPTER 15 S KINCONDITIONS AND BURNS

Plastic and reconstructive surgery can
help to restore function and minimize dis-figurement, thus helping individualsadjust to their condition and reenter theworkplace and the community. The extentto which reentry is possible varies fromindividual to individual and depends onthe part of the body affected and theextent of the remaining limitations, aswell as on individuals’ own psychologicalcharacteristics.
BURNS
The most traumatic of all skin injuries
is that caused by burns (Balasubramani,Kumar, & Babu, 2001). Any tissue injuryresulting from direct heat, chemicals,radiation, or electrical current is a burn.The treatment and prognosis of individ-uals with burns are dependent on thecause or type of burn, the depth of burn,and the amount of body surface that hasbeen burned.
Types of Burn Injury
Thermal
The most common burns, thermal
burns , are caused by ﬁre, hot liquids, or
direct contact with a hot surface. In addi-tion to causing direct injury to the skin,thermal burns can cause severe damage tounderlying structures if the heat has beenintensive or if the exposure has been pro-longed.
Chemical
Chemical burns result from direct con-
tact with strong acids (e.g., sulfuric acid)or alkaline agents (e.g., lye), gases (e.g.,mustard gas), or other chemicals, such assulfuric acid, that cause tissue death. Theextent of injury from chemical burnsdepends on the duration of the contact,
the concentration or strength of the chem –
ical, and the amount of tissue exposed tothe chemical source. Some chemicalscause burns directly through the produc-tion of physiologic changes in the tissuewith which they come into contact,whereas other chemicals cause burnsindirectly through the heat produced bytheir chemical reaction with the skin.
Radiation
The degree of damage caused by a radi-
ation burn depends on the dose of radia-tion received. Sources of radiation burnsmay include ultraviolet radiation , such as
the sun, as well as ionizing radiation , such
as nuclear materials and X-rays. Localizedskin reactions to low doses of radiationmay cause discomfort but usually healspontaneously. Larger doses of local radi-ation may damage underlying tissues andorgans, however, requiring more extensivetreatment.
Electrical
Electrical burns result from direct con-
tact with electrical current or lightning .
Injuries from electrical burns range
from local tissue damage to sudden deathbecause of cardiac arrest. The effects ofelectricity on tissue depend on the cur-rent, the voltage, the type of current (e.g.,direct or alternating), and the duration ofcontact. Because the entry point of theelectric current may be relatively small,electrical burns may appear to havecaused little external damage. However,usually extensive internal damage resultsbecause as the current travels through thebody tissues it damages nerves, blood ves-sels, and other major organs. The electri-cal current may also interfere with theelectrical activity of the heart, causing theBurns 445

heart to stop ( cardiac arrest ). Electrical
burns are generally full-thickness burns and
are associated with severe postburn dis-
abilities that may include multiple amputa –
tions because of damage to blood vessels,nerves, bones, or muscles. If clothing ofthe individual caught on ﬁre as a result ofexposure to the electricity source, thermalburns may also be present. Individualsmay also experience secondary injuriessuch as fractures , dislocations , or spinal cord
injury because of falls associated with the
injury. They may also have some sen-sorineural hearing loss, which generallyimproves over time.
Lightning injuries may be classiﬁed as
mild, moderate, or severe. Being struckby lightning can, of course, be fatal; how-ever, a number of people survive. In mildcases, individuals may appear dazed andconfused, having only mild physicalinjury. In more severe cases, individualsmay experience sensory organ damage,such as rupture of the tympanic membrane
in the ear or cataract formation in the eye,
which may not show up for weeks ormonths after the incident. If cardiacarrest occurred and the individual ex-perienced hypoxia (decreased oxygen)
before resuscitation could occur, he or shemay have brain damage or develop aseizure disorder. People who survivelightning injury may also have residualeffects of insomnia or other sleep distur-bances, anxiety, or reduced ﬁne intellec-tual function.
Inhalation
Inhalation injury to the respiratory
tract is caused by inhalation of steam,toxic gases, or vapors. Individuals withinhalation injury experience cough, in-creasing hoarseness, shortness of breath,anxiety, and wheezing. Inhalation of nox-ious gases alone may lead to brain injuryor death. Direct injury to the respiratorytract may also cause swelling, compromis-ing the patency of the airways. Treatmentusually involves administrating 100 per-cent oxygen and maintaining open air-ways (Nelson & Thompson, 1998).
Burn Depth
The degree of tissue damage caused by a
burn varies with the source of the burn, but
several other factors also affect burn sever-
ity. One such factor is the burn depth. Burn
depth depends on the temperature of theburning agent and the length of exposure.
Burn injuries may consist of only one burn
depth, or there may be a combination ofdifferent burn depths. Burn depth is typ-ically divided into four categories.
•Superﬁcial (ﬁrst-degree) burn: a burn
that affects only the epidermis (outer
layer of the skin). The skin becomesreddened and painful, but no under-lying structures are damaged.
•Partial-thickness (second-degree) burn:
a burn that affects both the epidermis
and the dermis . The skin is reddened
and blisters erupt, providing a portalof entry for organisms that can causeinfection at the burn site. In addition,second-degree burns are very painfulbecause of the stimulation of sensitivenerve endings in this layer of the skin.
•Full-thickness (third-degree) burn: a
burn that destroys the dermis and epi-dermis, as well as skin appendages,such as hair follicles, sebaceousglands, and sweat glands. There is lit-tle pain, because nerve endings havebeen destroyed. Full-thickness burnscannot heal spontaneously and aremore susceptible to infection.
•Fourth-degree burn: a burn that extends
to the underlying subcutaneous fat,muscle, or bone.446
CHAPTER 15 S KINCONDITIONS AND BURNS

Burns 447
Figure 15–2 Rule of Nines.In addition to the source of the burn
and the burn depth, the percentage of body
surface affected determines the severity ofthe burn. A common method of calculat-ing the amount of body surface injured isthe Rule of Nines , by which the body is
graphically divided into areas that repre-sent different percentages of the totalbody surface (see Figure 15–2). A moreaccurate method of estimating the totalbody surface burn is the Lund and Browder
method . Since body proportions are differ-
ent in children and adults, this methodcalculates the surface area of differentbody parts according to age. The chart listsvarious body sections and the percentageof body surface each section representsfrom 1 year of age to adult. Each burnedarea is thus given percentage points basedon the age of the individual. Points arethen added to estimate the total area ofthe body surface burned.Burn Severity
The location of the burn also affects
burn severity. For example, those withburns to the upper body, especially thehead and neck, may be prone to respira-tory complications because of possiblesmoke inhalation, heat damage to the res-piratory structures, exposure to the toxicbyproducts of combustion of materialsuch as the synthetic material used inhome furnishings, or restriction of air pas-sages due to swelling caused by theinjury. For electrical burns, the points ofcontact and the pathway that the currentfollowed through the body are importantconsiderations in determining the severi-ty of tissue damage. Individuals’ age andmedical history are also important consid-erations. Individuals who are very youngor very old are most vulnerable to theeffects and complications of burns. Pre-
Head 9%
Arm 9%
Leg 18%Perineal area
1%Front 18%
Back 18%

existing debilitating systemic conditions,such as heart disease, diabetes, lung dis-ease, or chronic abuse of drugs or alcohol,can further complicate recovery andseverely affect recovery and prognosis.
Individuals experience a systemic res-
ponse after major burns. Severe burns dis-rupt the body’s internal balance. Becauseof tissue injury, plasma seeps from bloodvessels into surrounding tissues, causingswelling and decreasing the amount of ﬂu-id in the general circulation. As a result,the body’s general homeostasis (equilib-
rium) is lost, which can affect all body sys-tems. A second danger affecting the prog-nosis for individuals with burns is infec-tion, especially for those with partial-thick-
ness or full-thickness burns . After burns
have healed, individuals with burns mayexperience severe pruritus (itching) for a
year or longer after the injury because oftissue regeneration.
Depending on the extent and location
of the burn, individuals may experience avariety of disabilities. For example, burnsinvolving the hand may result in contrac-ture of the ﬁngers, limiting joint motion.Severe burns of a leg may necessitate am-putation. Burns around the head and facemay involve loss of vision or loss of nose,ears, or hair. Other causes of disﬁgurementmay be hypertrophic scars , large ropelike
conﬁgurations of scar tissue that form onthe skin surface. Scar development oftenbecomes worse over time.
Burn Treatment
The type of treatment used for burns
depends on the severity of the injury andthe treatment philosophy of the burn cen-ter treating the individual. Not all institu-tions engage in the same type of treatmentprotocols.
Burns may also be combined with oth-
er injuries if they were associated with avehicle accident or explosion. Although
individuals with minor burns and no com-
plications may be treated at home, thosewith moderate or severe burns require hos-pitalization. Most individuals who havebeen moderately or severely burned aretransferred to a hospital that has a special-ized burn center. Burn centers are special-
ly equipped to provide multifaceted care
for individuals with moderate to severeburn injury. The staff are specialists inburn care and are trained to use a multi-disciplinary approach. Professionals work-ing in the burn unit may include physi-
cians , nurses , psychologists , psychiatrists ,
physical therapists , occupational therapists ,
dietitians , and rehabilitation counselors .
Although the amount of time individualswith burns spend in the hospital varieswith the amount, degree, and location ofthe burn and their general condition pri-or to the burn injury, a general rule ofthumb is one day of hospitalization forevery percentage of body burned. Duringthe acute phase, treatment of moderate orsevere burns is directed toward stabilizingindividuals’ general condition, restoringﬂuid balance, and preventing complica-tions. The major task for the individualduring this phase is survival. The greaterthe surface area of the body burned andthe greater the degree of the burn, thegreater the risk of complications.
A major complication of burn injury is
infection, which, unless controlled, canresult in widespread infection throughoutthe body ( sepsis ). Therefore, during the
acute phase, the eschar (charred, dead tis-
sue) must be removed ( debrided ) to re-
duce the risk of infection and to promotewound healing. Debridement involves clip-
ping away dead, charred tissue to preventgrowth of bacteria under the burn’s sur-face. Debridement is a very stressful andpainful procedure that is often performedon a daily basis until all necrotic (dead)448
CHAPTER 15 S KINCONDITIONS AND BURNS

tissue has been removed. Individuals maybe taken to surgery for surgical debride-ment of dead tissue; in the past, debride-ment was often performed while theindividual was in a whirlpool bath.
Since individuals who have been severe-
ly burned are vulnerable to infection, pre-cautions must be taken to prevent themfrom being exposed to harmful organisms.Most danger comes from organisms with-in the individual’s own body; however, insome burn units individuals with severeburns are placed in a room with a specialair ﬁltration system that screens out harm-
ful organisms.
In some burn units persons who provide
care may also wear caps, gowns, gloves,and masks to protect individuals withburns from infection. Visitors may be re-stricted, or when allowed to visit, may alsobe asked to wear masks and gowns. As aresult, individuals with burns may alsoexperience an increased sense of social iso-lation. Because of the stress these restric-tions impose and the growing evidencethat the risk of infection from outsidesources is minimal, some burn units nowrestrict the environment much less, sothat caps and gowns are not worn by per-sonnel or visitors and the time frame forvisiting is more liberal.
The nutritional needs of individuals
with severe burns are great. In the earlypostburn period, individuals may lose upto one pound or more per day. Thus, ahigh caloric intake is essential to meet theincreased energy requirements during thepostburn period. To supply extra calories,it may be necessary to administer specialﬂuids intravenously, as well as to providea high-calorie diet. In many instancesindividuals are given feedings through atube that has been placed down theesophagus rather than through intra-venous feedings, to diminish the chancesof infection through the injection site. Burn wounds are treated in different
ways. At times, burns are treated with anexposure method in which no dressing orcovering is applied to the wound. In theseinstances, more sterile conditions areessential to prevent infection. In otherinstances, the wound may not be covered,but topical medication, such as silver sul-
fadiazine to inhibit bacterial growth, may
be applied. In some cases, burn woundsare covered with dressings that arechanged daily. The method of treatmentdepends on the type and extent of theburn wound, as well as on the general phi-losophy of the burn unit in which indi-viduals are being treated. Individuals withsevere burns require daily hygiene to deterinfection. Some burn centers use “ tub-
bing,” which involves placing the individ-
ual in a whirlpool bath ﬁlled with a solu-tion of water and a chemical that helps toﬁght infection. Other centers have theindividual bathe daily with antimicrobialsoap, just as they might at home. Duringbathing the burn area may also bescrubbed to remove dead cells. Both ofthese treatments are extremely painful.
Certain parts of the body require special
care when burned. When hands, arms,legs, or the neck have been burned, spe-cial care is necessary to prevent the loss offunction due to scarring or contracture
(ﬁxation of a joint in a position of non-function). In some cases, affected jointsmay be splinted in a position of functionto prevent the formation of contractures.Facial burns not only can cause disﬁgure-ment, but also can damage the ears oreyes. Every effort must be made to preventcomplications that could further interferewith function.
After the acute phase of treatment, graft-
ing procedures usually begin. A graft is tis-
sue that is transplanted to a part of thebody to repair an injury or defect. Attimes, biologic dressings (also known asBurns 449

grafts as listed below) are used to cover aburn wound temporarily and prepare it forgrafting. The types of biologic dressingsinclude the following:
•Xenograft (heterograft): a graft taken
from another species. Porcine (pigskin) grafts are often used for burnwounds.
•Homograft (allograft): a graft taken
from the same species, but not thesame person. Homografts may be tak-en from a living donor or from acadaver skin bank. Another type ofhomograft is amnion , which consists
of placental membrane.
•Biosynthetic graft : a graft that has
been chemically manufactured. Syn-thetic skin substitutes are alternativesas temporary wound covering. Thematerial is semitransparent and ster-ile. It adheres to the wound and pre-vents infection, and it can help indebridement. It is left in place for 3 to4 weeks and gradually separates fromthe wound as new skin is formed.
It is often necessary to change biologic
dressings every several days. Biologicdressings can decrease the amount of painindividuals experience by covering nerveendings. They also help to prevent infec-tion until permanent grafting occurs, oruntil the wound heals. Most biologicdressings are changed every 2 to 5 days toprevent the body from rejecting the graft.
When the burn wound appears healthy,
a skin graft is applied. An autograft is a
section of the individual’s own skin thathas been removed from an uninvolvedsite. Depending on the size of the graftneeded, the same donor site may be usedrepeatedly. A split-thickness graft is the
epidermis and part of the dermis and con-sists of two types. The ﬁrst type, a sheetgraft, is a single layer. A mesh graft is a
graft in which many little slits have beenmade to allow it to expand and cover alarger area. A full-thickness graft , which
includes the epidermis and the dermisfrom the donor site, may be used for re-construction. The graft area may be band-aged or not, depending again on the areainvolved as well as the philosophy of theburn unit. The grafted part of the bodyshould be kept immobilized. If the graftis on an extremity, a splint may be appliedto prevent movement, which could dis-rupt the graft. When lower extremities areinvolved, the legs may be kept elevated toreduce swelling and subsequent rupture ofsmall blood vessels. Elastic hose may alsobe worn when the individual is up. If theindividual’s face has received a graft, stren-uous exercise, which could disrupt thegraft, should be avoided.
When larger quantities of tissue are
needed, a ﬂapmay be used. A ﬂap is a tis-
sue in which one area remains attached tothe donor site and consequently has itsown blood supply. The free end of the ﬂapis then placed over the injury, sutured intoplace, and allowed to heal. Because ﬂapsmaintain their own blood supply, theymay produce better cosmetic results thangrafts, which may not maintain naturalskin color.
The healing burn area may be com-
pressed with elastic dressings to prevent ordecrease the formation of hypertrophicscars. Special elasticized garments, such asgloves, vests, face masks, or neck gar-ments, are available to be worn continu-ally over the body part for a year or moreto prevent this type of scar formation (seeFigure 15–3). The garments are customizedto ﬁt the speciﬁc body part involved.
Individuals may be required to wear
compression garments for 1 to 2 yearsafter the initial injury. The garmentsmust be worn 23 hours per day. Becausethey are unattractive and are hot and un-comfortable, individuals may have a dif-450
CHAPTER 15 S KINCONDITIONS AND BURNS

ﬁcult time emotionally adjusting to thisphase of treatment. If contractures haveoccurred as a result of the burn, physicaltherapy may be necessary to return mobil-ity to a joint. When the measures areunsuccessful or if the contracture is severe,surgical intervention may be necessary.
Many individuals with severe burns re-
quire reconstructive or plastic surgery afterthe wound has healed, especially if therehas been severe deformity or disﬁgure-ment. Such surgical interventions may beperformed to reconstruct a body part, suchas the nose or the ear, or to remove hyper-trophic scar tissue. Surgery may be per-formed for cosmetic purposes, to restoreor improve function, or both. Many ofthese procedures take place over a num-ber of years after the initial burn injury.Corrective cosmetics (camouﬂage therapy)can also be used for skin discoloration orto minimize scars or suture lines. Correc-tive cosmetics differ from standard make-up in that they provide heavier coverageand adhere better to the skin (LeRoy,2000). If individuals with severe burnsexperience major hair loss, wigs or toupeesmay also be worn.
Psychosocial Issues in Burn Injury
Burn injury is often devastating, with
long-term physical and psychosocial ef-fects. It threatens the integrity of both thephysical and psychological identity of theaffected individual (McQuaid, Barton, &Campbell, 2000). Burn scars are cosmeti-cally disﬁguring and force individuals todeal with alterations in body appearance.In addition to the traumatic nature of theburn accident, individuals also undergopainful treatment, which may induce ad-ditional emotional and psychological re-sponses. Responses to burn injury vary;Burns 451
Figure 15–3 Pressure Garments. Source: Copyright © 1999 Rachel Clarke.

the individual’s premorbid personalitytraits, the characteristics of the burninjury, and the psychological meaning ofthe injury to the individual all play a part(Gilboa, 2001).
Individuals who have been scarred or
disﬁgured by burns must make psycholog-ical and physiologic adjustments, not onlyto their disﬁgurement but also to the im-
mediate injury and to the long-term course
of hospitalization and treatment. The sud-denness of the injury itself produces a pri-mary emotional stress. The impact of theinjury is heightened by a variety of othersituational factors, such as the separationfrom family, friends, and other sources ofgratiﬁcation; the experience of pain; thedisruption of future life plans; and the
threat to the sense of desirability and attrac –
tiveness to others. Recovery for individuals
with severe burns is long and sometimesdehumanizing. In the initial stage of burnrecovery, individuals frequently experi-
ence anxiety, partly because of the trauma t-
ic nature of the injury and the loss of inde-pendence, but also because of the painfultreatments and the fear of death. Lookingat the injury for the ﬁrst time can be atraumatic event for which individualsneed reassurance and optimism as well asa realistic view of their injury and their po-tential for recovery (Birdsall & Weinberg,2001). During this stage of recovery, indi-viduals may become agitated and hostile.They may experience sleep disruption anddeprivation, which increase their irritabil-ity. As recovery progresses and individu-als become more aware of their circum-stances, they may regress, becoming over-ly dependent. Loss of independence, fearof disﬁgurement, and exposure to contin-uing painful treatments and proceduresare a constant source of stress.
Treatment of burns often involves iso-
lation, pain, multiple operations, and pro-cedures over an extended period of time.Depression is prevalent in individuals whohave been burned (Van Loey & Van Son,2003). Depression frequently results fromfeelings of helplessness and grief over lossof function or appearance. Individuals ina burn unit are subjected to numerouspainful treatments and procedures, andthey may also feel isolated. They may feelthey have little power over what is doneto them and for them, which can befrightening and frustrating. They may feela loss of both personal and social satisfac-tion, as well as an alteration in their rela-tionships with others. As already men-tioned, anxiety is also a common psycho-logical response to burns. Individualswith burns may be apprehensive, and real-istically so, because of the painful proce-dures that they must endure. For some,the pain associated with the treatmentprocedures is a reminder of the initialinjury, thus increasing the anxiety andintensifying the pain. Reactions maybecome generalized so that, even after thetreatment period, individuals may contin-ue to experience anxiety about unknownor unrecognized dangers.
Individuals with burns are at high risk
for the development of posttraumaticstress, not only because of the trauma ofthe initial injury, but also because of theongoing pain associated with treatment,which serves as an additional source ofanxiety and as a continuing reminder ofthe incident (Yu & Dimsdale, 1999).Because burns are frequently associatedwith accidents, individuals may experi-ence anger, guilt, regret, or resentment,depending on the circumstances. If theaccident was caused by the negligence oractions of others, individuals may experi-ence hostility and anger. If the accidentwas caused by their own actions or if oth-ers were also injured as a result, self-blameand guilt may intensify individuals’ reac-tion to their injury.452
CHAPTER 15 S KINCONDITIONS AND BURNS

As individuals begin to think about the
future after the immediate burn treatmentperiod, psychological responses may becharacterized by false hopes and magicaloptimism, particularly when skin graftingand reconstruction begin. They may haveunrealistic expectations about the resultsof surgery or deny that there will be a per-manent deformity. When continuing dis-ﬁgurement and/or limitations becomeapparent, they may again sink into a stateof depression and withdrawal before grad-ually adjusting to their condition.
Discharge from the hospital does not
mark the end of stress for individuals withburns. Discharge often marks a new set ofstresses when individuals integrate intothe larger social setting after having beensheltered in the burn unit. It is difﬁcultpsychologically for individuals who havebeen disﬁgured by burns to reenter thecommunity, where they may be subject-ed to the pity and curiosity of strangers aswell as to stares and rude remarks. Indi-viduals may test family and friends withunusual requests or with behaviors des-igned to get attention. Adjusting to thereactions of others, dealing with socialstigma, and realistically accepting limita-tions are important psychological issueswith which the individual with burnsmust deal.
The lifestyle issues that arise in relation
to burns depend on the extent, nature, and
location of the burn. For example, severeburns of the hands can result in contrac-ture of the hands and ﬁngers, necessitat-ing the use of assistive devices for theactivities of daily living. Burns to the facethat result in loss of vision may also makeit necessary to use adaptive devices.
The sexuality of individuals with burns
is often a neglected part of treatment andrehabilitation. Sexual concerns are impor-tant during acute treatment in the burnunit and also during discharge and ongo-ing rehabilitation. Sexuality encompasses
much more than sexual activity or sexualfunction; it encompasses the whole person
and is an important part of identity, self-image, and self-concept. The disﬁguringnature of burn injury can challenge indi-viduals’ view of themselves as sexualbeings and can affect their adjustment andadaptation.
Individuals who have been burned
severely often require a series of recon-structive operations over several years.Thus, frequent hospitalizations and/orclinic visits interrupt work and homeactivities. Relationships may be altered be-cause of the absences from the social envi-ronment necessitated by these repeatedhospitalizations. Increased dependenceand length of hospitalization due toburns can disrupt relationships within thefamily, as well as other social relation-ships. Friends and family may be shockedat the sudden change in an individual’sappearance after a burn injury. Dependingon the circumstances of the accident, fam-ily members and friends may feel anger,guilt, or resentment, which can be mani-fested in a variety of ways. In an attemptto make sense of the tragedy and its after-math, family members, friends, andcoworkers may focus on the question ofresponsibility for the accident. Thosewho were present at the time of the injurymay feel they should have done more orthat they were to blame. Others may won-der why they escaped the same type ofinjury. These feelings may affect their reac-tions to the individual and their furthersocial interactions with him or her. Fami-lies may grieve for the image they oncehad of the individual, or they may grievefor the potential they feel the individualhad that now will not be realized. Familyreactions can range from oversolicitude toemotional withdrawal. Concerns aboutﬁnancial considerations and altered socialBurns 453

roles may cause additional family stress.Support groups can help individuals andtheir families share common concerns.Groups such as the National Phoenix
Society help individuals and their families
cope with the ongoing difﬁculties ofreturning to society.
Vocational Issues in Burn Injury
The ability of individuals who have
been burned to return to their formeroccupation is dependent not only on theoccupation itself, but also on the extentand location of the burn. At times, themain factor in determining how success-ful individuals can be in returning to theirformer position is the attitudes of othersin the workplace. Acceptance of the burnsurvivor in the workplace by fellowemployees may be difﬁcult for a variety ofreasons. If the injury was work related,depending on the circumstances, cowork-ers may feel guilty and treat the individ-ual differently. Others may feel uncom-fortable because of the individual’s appear-ance and avoid contact with him or her.In some instances, even though individ-uals who have been severely burned maynot consider themselves disabled, theymay be perceived as such by others be-cause of their appearance.
The emotional stress on the part of
coworkers can prevent the individualfrom effectively reentering the workplace.Employers may not have conﬁdence inindividuals’ ability to return to the formerjob or may be concerned about others’reaction if there has been disﬁgurement.Considerable work with coworkers andemployers is sometimes necessary to pro-vide a smoother transition for individualsreturning to work after burn injury. Thosewith severe burns that necessitate exten-sive reconstructive surgery may requireintermittent hospitalizations over a 1- to2-year period after the initial injury. Thedisruption to work activity associated withthese hospitalizations should be consid-ered before individuals return to regular
employment. Those who have other disabil-
ities resulting from burns, such as the loss
of a limb or the loss of vision, have other
vocational limitations as well (see relatedchapters). Contracture as a result of burnsmay also limit mobility and, if the handsare involved, manual dexterity.
Individuals who must wear compression
garments to prevent hypertrophic scarringmay need to avoid extremely warm workenvironments because of the excessivewarmth of the garment. Those who wearcompression gloves also have decreasedmanual dexterity. A facial mask may be acosmetic disability if dealing with the pub-lic is a requirement of the occupation. Thedegree to which cosmetic appearance dueto the burn is a factor in employmentdepends on the individual, the occupa-tion, and the employer.
Skin that has been grafted may be more
sensitive than normal skin is. Consequent-ly, grafts should not be exposed to ex-tremes of temperature. Because of this skinsensitivity, individuals should take sunprecautions and avoid mechanical traumathat could injure the skin. In addition,there may not be as much fat insulationin burned areas as there is in healthy nor-mal tissue, which affects the individual’sability to tolerate extremes of temperature.Extremely dry climates may exacerbatethe itching that may be associated withthe new skin growth of skin grafts; thus amore humid environment may be desir-able. Other residual problems from burnsmay also affect the appropriateness of thework environment. For example, individ-uals who have experienced altered lungfunction as a result of inhalation injuryshould avoid work settings in which
there is air pollution or exposure to smoke454 CHAPTER 15 S KINCONDITIONS AND BURNS

and dust. Individuals with burns to thelower extremities may have difﬁcultystanding for prolonged periods and mayneed more sedentary employment.
PSYCHOSOCIAL AND VOCATIONALISSUES IN CONDITIONS OFTHE SKINPsychological Issues
The skin, exposed and readily observ-
able, determines to a great extent individ-uals’ appearance to others, and it isthrough personal appearance that othersbuild an image about someone. Individ-uals, in turn, observe the reaction of oth-ers and incorporate it into their own self-image. Consequently, conditions affectingthe skin can have considerable impact onindividuals’ perception and attitudes. Di-sease or injury affecting the face may beparticularly devastating. More than anyother body part, the face is tied to person-al identity. Although clothing can coverother body parts, the face is left exposedso that disﬁgurement is readily observable.Our society places considerable emphasison a clear, radiant appearance. When dis-ease or injury mars this image, it is notsurprising that the psychological impacton the affected individuals is considerable.
Disease of and injury to the skin may
isolate individuals perhaps more than anyother condition. Some people, becausethey associate skin diseases with unclean-liness and contagiousness, may avoid indi-viduals with skin disorders even thoughthese associations are unfounded. Becauseof the reactions of others, individuals withskin disease or injury may become verysensitive. Having experienced stares orother negative reactions, they may devel-op an accentuated state of awareness andassume that others are focusing totally ontheir appearance. They may become ex-tremely self-conscious and withdraw fromsocial contact.
Although conditions of the skin may
not affect sexual function directly, socie-ty places considerable importance onphysical attractiveness, especially whenrelated to issues of sexuality. Consequent-ly, skin disease or disﬁgurement, particu-larly of the face, as well as the reactionsof others, may alter individuals’ feelingsof desirability. The anxiety or depressionthat accompanies skin conditions may fur-ther disrupt sexual function.
Lifestyle Issues
Changes in lifestyle resulting from skin
conditions are dependent on the severityof the condition and on the extent andcircumstances of the disability. Skin con-ditions resulting from exposure to or con-tact with certain substances within theenvironment make it necessary to avoidthose substances. The discomfort associ-ated with some skin conditions, such asitching, may affect daily activities to somedegree. If special baths or dressings arerequired, these treatments must be provid-ed for within the daily routine. Acute skinconditions may be treated and prevented.Chronic skin conditions require ongoingtreatment or intervention. Stress affectssome skin conditions, and individualswith these conditions may need to learnways to reduce stress in their environmentor ways to alter their reaction to stress.
Social Issues
Visible disabilities provoke greater dis-
crimination and social stigma than doinvisible disabilities. Physical attractive-ness is highly valued in society, whereattractiveness is viewed as a salable com-modity. Skin conditions, especially if theyinvolve the face, evoke even more pro-Psychosocial and Vocational Issues in Conditions of the Skin 455

found responses from others. People mayfeel uneasy in the presence of individualswith disﬁgurement or deformity and un-certain as to what to do or say. In socialsettings, individuals with deformity or dis-ﬁgurement due to a skin condition orinjury may encounter staring, feelings ofpity, or repulsion. These reactions maycause individuals to limit or avoid socialactivities or to restrict their social interac-tions with others.
Vocational Issues
Most individuals with skin conditions
continue in their regular line of employ-ment, although individuals whose skincondition is precipitated or exacerbated byexposure to substances in the work envi-ronment may require special considera-tions. In these instances, alterations in thework site or precautions in the perform-ance of certain work-related activitiesmay be necessary. If stress precipitates orexacerbates the skin condition, measuresto decrease stress at the work site or toimprove the individual’s reaction to stressshould also be taken. In some instances,it may be necessary to alter the work site.
Because skin cancer appears to be relat-
ed to exposure to the sun, those who workoutside should take precautions to avoidexcessive exposure, such as by wearingprotective clothing or sun shields. Thosewho have had skin cancer or who have apropensity toward it should take addition-al precautions to avoid direct exposure tothe sun as much as possible. Likewise,individuals who are being treated withmedications that cause photosensitivity,or individuals who have new skin grafts,may also need to avoid the sun.
The attitudes of employers and cowork-
ers may create barriers to employment forindividuals with skin conditions, especial-ly when the condition alters their appear-ance considerably. Coworkers may fearcontagion or may be uncomfortable be-cause of the individual’s appearance. Con-sequently, education and strategies toalleviate misperceptions may be importantfactors in facilitating the individual’s suc-cessful reentry or continuation in thework setting.
CASE STUDIESCase I
Ms. N. was a passenger in a small com-
muter plane when it crashed during asevere storm 2 years ago. Although all oth-er passengers were killed, Ms. N. survived;however, she experienced second- andthird-degree burns over 60 percent of herbody. As a result of her injury she receivedsevere facial scars and the amputation ofher left hand. She has had a number ofreconstructive surgeries since her injury.She uses a terminal device for her lefthand that is cosmetic. Other than herfacial scarring and amputation of herhand, she has no other physical limita-tions. Ms. N. has a bachelor of sciencedegree in ﬁnance. Before her injury shewas a teller at a bank. She is 29 years old.She asks you to help her explore her reha-bilitation potential.
Questions
1. What speciﬁc issues regarding the
scarring would you consider?
2. What other issues might be impor-
tant to consider in Ms. N.’s case?
3. What vocational options might be
feasible for Ms. N. given her situa-tion?
4. What additional medical information
would you like to obtain in workingwith Ms. N. to develop her rehabili-tation plan?456
CHAPTER 15 S KINCONDITIONS AND BURNS

Case II
Mr. A. and four other men were burned
at a construction job because of a gasexplosion from a gas leak that was ignit-ed when he lit a cigarette. The accidentoccurred 2 years ago. Mr. A. is now 30years old. Mr. A.’s burns were localizedover his chest and upper extremities andwere second- and third-degree burns. Asa result of the burns he also lost one earand both of his thumbs. He has under-gone a number of surgeries since hisinjury. The construction company is will-ing to have him return to work, but hedoes not believe he is psychologically ableto return. He has a high school educationand no other skills other than those helearned on the job. He had worked for theconstruction company for 5 years. Pre-viously he had been a gas station atten-dant. He has been divorced for 5 years buthas no children. He had been dating awoman before his injury, but since hisinjury she has told him she no longerwants to see him.
Questions
1. What issues related to the accident
might be important to address?
2. How will the speciﬁc injuries Mr. A.
received in the accident affect hisrehabilitation potential?
3. What additional issues might be
important to address?
4. What types of information would
you want to obtain in working withMr. A. to develop a rehabilitationplan?References 457
REFERENCESBalasubramani, M., Kumar, T. R., & Babu, M. (2001).
Skin substitutes: A review. Burns, 27 (5), 534–544.
Birdsall, C., & Weinberg, K. (2001). Adult patients
looking at their burn injuries for the ﬁrst time.Journal of Burn Care Rehabilitation, 22 (5), 360–364.
Gilboa, D. (2001). Long-term psychosocial adjust-
ment after burn injury. Burns, 27 (4), 335–341.
Lamberg, L. (1997). Dermatologic disorders dimin-
ish quality of life. Journal of the American Medical
Association, 277 (21), 1663.
Lebwohl, M. (2000). Psoriasis treatment options con-
tinue to grow. Dermatology Times, 21 (11), 14–16.
LeRoy, L. (2000). Camouﬂage therapy. Dermatology
Nursing, 12 (6), 415–419.
McQuaid, D., Barton, J., & Campbell, E. A. (2000).
Body image issues for children and adolescentswith burns. Journal of Burn Care Rehabilitation,
21(3), 194–198. Morgan, M., McCreedy, R., Simpson, J., & Hay, R. J.
(1997). Dermatology quality of life scales: A meas-ure of the impact of skin diseases. British Journal
of Dermatology, 136 , 202–206.
Nelson, L. A., & Thompson, D. D. (1998). Burn
injury. Plastic Surgical Nursing, 18 (3), 159–169.
Pardasani, A. G., Feldman, S. R., & Clark, A. R. (2000).
Treatment of psoriasis: An algorithm-basedapproach for primary care physicians. American
Family Physician, 61 (3), 725.
Van Loey, N. E., & Van Son, M. J. (2003).
Psychopathology and psychological problems inpatients with burn scars: Epidemiology and man-agement. American Journal of Clinical Dermatology,
4(4), 245–272.
Yu, B. H., & Dimsdale, J. E. (1999). Posttraumatic
stress disorder in patients with burn injuries.Journal of Burn Care Rehabilitation, 20 (5), 426–433.

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CancersCHAPTER 16
459NORMAL STRUCTURE ANDFUNCTION OF THE CELL
The basic unit of all living things is the
cell. The human body contains approxi-mately 75 trillion cells. Although differenttypes of cells perform different functions,all cells have certain basic characteristicsin common. All cells require nutrition andoxygen in order to live, and almost allcells have the ability to reproduce. Re-production of cells is a controlled processso that cells die and form at an approxi-mately equal rate in adults, maintaininga balance in the number of cells presentat any time. The precise way in which cellgrowth and reproduction are regulatedwithin the human body is unknown.Some cells, such as those that make up thelayers of the skin or the lining of the in-testine, grow and reproduce frequently.Other cells, such as those that make up themusculature of the gastrointestinal tract,may not reproduce for years. Cells thatmake up neurons, the functional unit ofthe nervous system, do not reproduce atall. Similarly, little is known about themechanism that controls the number ofeach speciﬁc cell type that is produced.
Different types of cells make up differ-
ent parts of body tissue. Cells are namedfor their different characteristics. For ex-ample, epithelial cells are cells found in the
skin, the lining of body organs (e.g., thelining of the intestine), and glandular tis-sue (e.g., the breast or prostate). Blood ves-
sels, lymph vessels, and other lymph tis sue
are composed of endothelial cells . Different
types of cells are also found in muscle,nerve, bone, and other tissues in the body.
Every cell contains DNA (genetic mate-
rial that is the blueprint for all the body’s
structures). Genes , composed of DNA, car-
ry hereditary information about all char-
acteristics of the organism. Although each
cell contains all the genes for a particularorganism, it uses only particular genes.This discrimination in the use of genes isthe basis for different cell types. Genes de-termine the growth characteristics ofcells, as well as when or whether the cellsdivide to form new cells. Before cells canreproduce, however, genes must reproducethemselves. After genes reproduce, the celldivides, forming another cell identical toitself. It is through this systematic, organ-ized reproduction of cells that continuityof life is maintained.
DEVELOPMENT OF CANCER
Cancer is not one disease, but many dis-
eases. There are well over 100 types of can-
cers. Cancers can arise from any type of
cell and are classiﬁed according to the cell
of origin . Most frequently, the term tumor
is assumed to be synonymous with cancer ;
however, not all tumors are cancerous. A

tumor , also called a neoplasm , is a new and
abnormal growth of cells that serves nouseful function and may interfere withhealthy tissue function. The reason for theproliferation of cells is often unknown.Tumors may be benign (noncancerous).
Although benign tumors may disturbbody function by exerting pressure on sur-rounding tissues and, thus, preventingsurrounding organs from obtaining a suf-ﬁcient blood supply, they usually growslowly, do not invade surrounding tissue,remain localized, and do not recur onceremoved. Generally, cells in benign tu-mors closely resemble normal cells in thetissue from which they multiplied.Malignant tumors are those that are ca-
pable of destructive growth and have theability to invade surrounding tissues andmove to other parts of the body. Malig-nant tumors are cancerous tumors .
Cancer develops when there has been
an alteration ( mutation ) in the DNA with-
in the normal cell. As a result, the controlmechanism that regulates cell reproduc-tion is lost. Because the reproduction ofcancer cells is uncontrolled, they repro-duce at a rate that exceeds the rate atwhich the normal cells in the tissue aredying. Some of the more virulent cancercells are often described as anaplastic ,
meaning that their appearance takes onabnormal characteristics so that they areless differentiated than are the normalcells from which they are derived.
The original site of cancer cell reproduc-
tion is called the primary site , sometimes
referred to as the primary tumor . Cancer
cells do not remain conﬁned to the origi-
nal site, but extend and invade surrounding
tissues as they reproduce. In addition, can-cer cells are less adhesive than are normalcells. Selected cancer cells may break offfrom the original cluster, enter the blood-stream or the lymph system, and travel toother parts of the body, where they beginanother abnormal pattern of reproduction.
The movement of cancer cells from theoriginal site to another part of the bodyis called metastasis . Cancer cell reproduc-
tion at this additional site is called a sec-
ondary tumor , meaning that metastasis has
occurred and that the secondary tumor isnot the original site of cancer growth.
Cancer cells compete with normal cells
for nutrients. The reproduction of cancercells is not well regulated, and some can-cer cells reproduce at a more rapid ratethan do normal cells. Eventually, availablenutrients are taken from the normal cellsto nourish the cancer cells.
CAUSES OF CANCER
The exact cause of cancer is unknown.
There are probably many causes, and itmay be necessary for a variety of factorsto be present for cancer to develop. Al-though speciﬁc causes are unknown, sev-eral factors are known to increase the riskof cancer.
• Radiation• Some chemicals and pollutants• Smoking and tobacco use• Some viruses• Chronic physical irritation to a
body part
• Ultraviolet rays (sun)• Hereditary predisposition
Chemicals or other substances that are
thought to cause cancer are called car-
cinogens . Some carcinogens may be pres-
ent in the environment but not readilyevident. Individuals may be exposed tocarcinogens within the environment orworkplace for a number of years beforecancer develops. Some substances may notbe carcinogenic in themselves but mayserve as co-carcinogens, promoting tumorformation in combination with other car-cinogenic agents. Other factors, such as460
CHAPTER 16 C ANCERS

Staging and Grading of Cancer 461
hormonal secretion, diet, and stress, havebeen implicated as potential factors in thedevelopment of or propensity for cancer,but the speciﬁc mechanisms that con-tribute to this relationship are unknown.
TYPES OF CANCER
Any type of cell in the body may be the
source of cancer. Cancers are named forthe type of tissue from which they originat-
ed. Some common types of cancers andthe corresponding tissue from which theyarise are the following.
•Carcinoma: cancer of the epithelial
cells
•Sarcoma: cancer of the bone, muscle,
or other connective tissue
•Lymphoma: cancer of the lymphat-
ic system
•Leukemia: cancer of blood cells or
blood precursor cells
•Melanoma: cancer of the pigment-
producing cells, usually of the skin
Because the speciﬁc behavior of cancer
cells depends on the type of cell from which
they originated, there can be no general-izations made about cancer. Each type ofcancer may progress at a different rate andmay respond to different types of treat-ment in different ways. Consequently, theclassiﬁcation of cancer is important in de-termining both treatment and prognosis.
STAGING AND GRADING OF CANCER
When cancer is diagnosed, it is impor-
tant to determine not only the cancer typebut also the extent to which cancer cellshave spread. This process is called staging .
Staging of all cancers not only helps physi-cians determine the prognosis (prediction
of the course and outcome of the diseaseprocess), but also helps to determine the
form of treatment that is most appropriate.The most common system for staging
today is the TNM system , which classiﬁes
cancer according to tumor size, node in-volvement, and metastasis. The letter Tstands for tumor; N , for node; and M, for
metastasis . When there is no evidence of
a primary tumor, the stage is deﬁned asT0. If cancer cells are present but have not
invaded surrounding lymph nodes, thestage is deﬁned as Tis(previously called in
situ). As the tumor increases in size, it may
be staged from T1 to T4, depending on thetumor size and involvement. When thereis no lymph node involvement, the Nstaging is N0. If cancer cells extend be-
yond the initial tissue site and involve thelymph nodes in the surrounding area,however, the stage is N1, N2, or N3 (pre-
viously called regional involvement ), de-
pending on the degree of involvementand the abnormality of the nodes. If thecancer cells remain at the original siteeven though the surrounding tissues andlymph nodes are involved, the M stagingis M0. When cancer cells have metasta-
sized to another area of the body, howev-er, staging is M1, M2, or M3, depending on
the extent of the metastasis.
Histologic studies and grading are labora-
tory procedures in which the type andstructure of cancer cells are determinedmicroscopically. Histological grading isbased on the appearance of cells and thedegree of differentiation. Cells are gradedas follows:
• Grade I: mild dysplagia (cells are
slightly different from normal)
• Grade II: moderate dysplagia (cells are
more abnormal)
• Grade III: severe dysplasia (cells
are very abnormal and poorly differ-entiated)
• Grade IV: anaplasia (cells are imma-
ture and undifferentiated; cells of ori-gin are difﬁcult to determine)

A pathologist (a physician who special-
izes in the diagnosis of abnormal changesin tissues) examines the cells under a mi-croscope to determine their type and theextent to which they differ from their nor-mal precursors. The histologic type of celland the grading of the cell are importantin the determination of the treatment im-plemented andthe prognosis. Individuals
with tumor cells that are well differentiat-
ed(more similar to the cell of origin, with
a more organized structure) may have abetter prognosis, for example, than doesan individual with tumor cells that areconsidered anaplastic (containing more ab-
normalities in structure).
GENERAL DIAGNOSTIC PROCEDURESIN CANCER
In general, the earlier the diagnosis of
cancer, the better the prognosis. Somecancers grow and invade surroundingtissue without causing physical symp-toms. These cancers are called occult ma-
lignancies . Tests and procedures used to
detect abnormalities before symptomsdevelop are called cancer-screening proce-
dures . When symptoms occur or when
screening procedures have positive or sus-picious results, additional diagnostic test-ing is necessary.
Radiographic Procedures (X-ray)
In addition to conventional X-rays, com-
puted axial tomography , magnetic resonance
imaging , ultrasound , and, occasionally, ar-
teriography may be helpful in identifying
an abnormality in normal anatomic struc-ture or the presence of a tumor. Mammog-
raphy is a soft tissue radiographic exam-
ination of the breast that is frequentlyused as a screening and diagnostic proce-dure because it can reveal cancerous le-sions before they can be detected by directexamination of the breast. Although thesetests are important in identifying abnor-malities, they rarely are used alone in thediagnosis of cancer. A positive diagnosisrequires microscopic examination of thetumor cells ( histologic testing ).
Diagnostic Surgery
In some instances, surgery may be
done to conﬁrm or rule out the presenceof cancer. Depending on the size andlocation of the tumor, the surgical proce-dure may be relatively minor, such as theremoval of an external wart or polyp, ora major intervention, such as an explora-
tory laparotomy (the surgical opening of
the abdomen for the purpose of investi-gation).
Regardless of the type of diagnostic sur-
gery performed, an accurate diagnosis ofcancer can be made only after a micro-scopic examination of the tissue. For suchan examination, a biopsy is performed to
remove a small portion of tissue from thebody. Biopsies may be done by insertinga needle into the tumor and removingsome cells through the needle ( needle biop-
sy). Biopsies may also be done by making
an incision and removing a portion of thetumor ( incisional biopsy ). The type of biop-
sy done depends on the size and locationof the tumor.
Cytology
The study of cells that have been
scraped from tissue surrounding the areaof interest is a cytologic study . Perhaps the
best-known example of diagnostic cytol-ogy is the Papanicolaou smear (Papsmear ).
Cells from sputum specimens that havebeen coughed up from the lungs and oth-er types of ﬂuids may also be examinedthrough diagnostic cytology.462
CHAPTER 16 C ANCERS

Endoscopy
An endoscopic examination involves the
insertion of a tubular device into a holloworgan or cavity to visualize the inside ofthe structure directly. The procedure maybe done through a natural body openingor through a small incision. Examples ofendoscopic examinations are bronchoscopy
(see Chapter 12), sigmoidoscopy , gastroscopy
and esophagoscopy (see Chapter 10), and
laryngoscopy (examination of the larynx or
vocal cords). Endoscopy is also a method
of obtaining a tissue sample from the inter-
nal structure for a histologic examination.
Nuclear Medicine
In nuclear medicine, small amounts of
radioactive materials are used for diagnos-
tic procedures, and somewhat largeramounts are used for the treatment of dis-ease. In the diagnosis of cancer, nuclearmedicine procedures may be used for thedetection and staging of cancers in thethyroid glands , liver, and bone. They may
also be used to detect the presence ofmetastatic disease.
Laboratory Tests
Although laboratory tests per se may
not be diagnostic of cancer, the results oflaboratory tests may indicate impairedphysiologic function as a result of the can-cer, such as the anemia or altered whiteblood cell count associated with leukemia.In some instances, laboratory tests areused for screening purposes. For example,both alpha-fetoprotein and carcinoembry-onic antigens are normally found in em-bryonic and fetal tissues but disappearafter birth. In later life, however, tumorsmay produce these substances. Conse-quently, elevated levels of either in adultsmay be an indication of certain types ofcancers, as well as of other diseases. GENERAL TREATMENT OF CANCER
Many modalities are available to pre-
vent, control, or cure cancer. Treatmentmodalities include:
• surgery• chemotherapy• radiation (external or internal)• biological therapy (immunotherapy,
hormone therapy, gene therapy)
• bone marrow transplantation
The above treatments may be used
alone or in combination. Many therapiesinvolve multiple approaches rather thanone. When treatment uses several differ-ent types of therapy, treatment is said tobe multimodal .
A number of factors are considered in
determining which procedures are best forthe treatment of a particular cancer. A ma-jor consideration is the typeof cancer. Be-
cause different cancers grow at differentrates, metastasize to different spots, andreact differently to various forms of treat-ment, the histologic type of cancer is a ma-
jor determinant in treatment decisions.The stage of cancer is also considered. The
extent to which cancer has invaded sur-rounding tissues and the presence of anymetastases determine the aggressiveness,as well as the type, of treatment. The lo-cation of the tumor and its relationship toother vital organs determine the accessi-bility of the tumor for removal or treat-ment. The goal of intervention also affectsthe type of treatment.
Goals for treatment of cancer can in-
clude:
• cure• extension of life • prevention of metastasis• palliation
In terms of cancer treatment, cureis usu-
ally deﬁned as no evidence of cancer for 5General Treatment of Cancer 463

years after treatment , indicating a normal
life expectancy for the individual. Treat-ment for the prevention of metastasis, alsocalled adjuvant therapy , is directed toward
eliminating cancer that, although not de-tectable and not symptomatic, may bepresent and may cause a recurrence of dis-ease. Palliative therapy is directed toward
the relief of symptoms or complications ofcancer, such as obstruction or severepain, rather than toward cure.
Factors related to the individual with
cancer must also be taken into consider-ation. Debilitation because of disease, the
cancer itself , or age may compromise indi-
viduals’ ability to withstand certain treat-ments. In some cases, individuals may feelthat the beneﬁts of some forms of cancertherapy are not worth the risks and sideeffects; consequently, they may refuse therecommended treatment.
Cancer may be treated systemically or lo-
cally. Often, treatment of cancer consists
of a combination of the two. Cancer maybe treated surgically , chemically (chemo-
therapy), with radiation , or with other
means—separately or in combination.
Surgical Procedures
Usually directed toward the local treat-
ment of cancer, surgical procedures maybe preventive, curative, palliative, or re-constructive. Preventive surgery may be per-
formed when precancerous or suspiciouslesions are found. For example, a mole orpolyp that, although not malignant, hasa high probability of becoming malignantin the future may be removed. Curative
surgery is generally more extensive. It may
involve not only the tumor but also an or-gan or surrounding tissue. Depending onthe size and location of the tumor, cura-tive surgery can affect subsequent func-tion only minimally, can impair functionseverely, or can cause permanent disﬁgure-ment. Palliative surgery is directed toward
reducing the size or retarding the growthof the tumor, or relieving severe discom-fort associated with the presence of the tu-mor. In all instances, the goal of palliativesurgery is to prolong or increase the qual-ity of life rather than to cure the disease.Reconstructive surgery is directed toward
restoring maximal function or correctingdisﬁgurement.
The surgical procedures used in treat-
ment of cancer may be considered simple
or radical . Simple surgical procedures usu-
ally involve removing the tumor whileleaving surrounding structures and organsintact. Radical surgical procedures are more
extensive. In radical surgery not only thetumor is removed, but also some under-lying tissue (e.g., muscle or organ). Radicalsurgery often results in alteration of func-tion or appearance to some degree.
With advances in medical techniques,
less radical procedures are now being per-formed. Surgery now might include laser
surgery or cryosurgery (using low tempera-
tures to devitalize or destroy cells).
Chemotherapy
Chemotherapy alone is often curative in
many cancers, and in others adjuvantchemotherapy used in conjunction withother therapies can augment the survivalbeneﬁt of the other therapies (Green,2004). Antineoplastic medications (chemical
agents that destroy cancer cells) are usedin the systemic treatment of cancer. Theseagents may be used alone or in conjunc-tion with other forms of therapy, such assurgery and radiation. This type of thera-py, called chemotherapy , can be used for
cure, prevention, or palliation. In gener-al, chemotherapeutic agents affect thegrowth and reproduction of cancer cells.
There are a number of antineoplastic
medications used in the treatment of can-464
CHAPTER 16 C ANCERS

cer. Although these medications are differ-ent and may be administered differently,most affect rapidly dividing cells. Unfor-tunately, in addition to destroying anddamaging cancer cells, these medicationscan also damage normal cells that growrapidly, such as the cells of the hair folli-cles, skin, the lining of the gastrointesti-nal tract, and bone marrow. As a result,
the toxic side effects of chemotherapy may
include hair loss ( alopecia ), loss of ap-
petite, nausea, vomiting, diarrhea, fatigue,and suppression of bone marrow function.The altered bone marrow function may in-terfere with the production of variouscomponents of blood. Therefore, individ-uals undergoing chemotherapy may de-velop anemia, may bruise easily becauseof decreased blood clotting ability, andmay be highly susceptible to infection be-cause they have fewer white blood cells.
Chemotherapeutic agents may be given
intravenously, intramuscularly, subcuta-neously, orally, or topically. In other in-stances, high concentrations of chemo-therapeutic agents may be injected direct-ly into a body cavity, such as the bladderor the peritoneal cavity, to treat localizedtumors. Treatment may be conducted oneither an outpatient or an inpatient basis.
Chemotherapeutic regimens vary with
the agent and the disease. Some treat-ments are given daily; others are given for1 day every 3 to 8 weeks. Some individu-als may use a portable device ( infusion
pump ) that pumps small amounts of the
chemotherapeutic agent constantly into avein ( infusion therapy ). In some instances,
because the chemotherapeutic agent is ad-
ministered in small doses over time, toxic
side effects may be reduced. This type oftreatment delivers maximal dosage of themedication to the tumor site and can alsoreduce systemic side effects. New agentscalled chemoprotectants (drugs that pro-tect the body against cancer medications)ameliorate the toxic effects of drugs usedin chemotherapy at higher doses.
Not all individuals who receive chemo-
therapy experience side effects. Those whodo not have severe side effects can, for themost part, continue their daily activities.No special precautions are necessary, withthe exception of avoiding exposure to in-dividuals with colds or ﬂu because resist-
ance may be lowered during chemotherapy.
Radiation Therapy
With radiation therapy, high-energy
rays are used to damage cancer cells andprevent them from growing and reproduc-ing. This technique may be used to curecancer, to relieve symptoms, or to keepcancer under control.
Radiation therapy may be delivered ex-
ternally or internally. During external ra-diation therapy, a machine beams high-energy rays to the cancer so that the max-imum effect of radiation takes place in thetumor itself within the body. Even thoughthe radiation penetrates the skin and un-derlying tissue, it does minimal damage tothese structures. Internal radiation thera-py involves inserting small amounts of ra-dioactive material into the body. This iscalled brachytherapy .
There are various types of internal radi-
ation. With intracavity therapy, a radioac-tive substance is placed in a body cavityfor a period of approximately 24 to 72hours and is then removed; for example,a radioactive implant may be placed intothe vagina for the treatment of cervicalcancer. With interstitial therapy, a radioac-tive substance is placed into needles,beads, or seeds and implanted directlyinto the tumor. The interstitial implantmay be removed after a speciﬁc period oftime, or it may be left in place permanent-ly, depending upon the half-life of the ra-dioactive source.General Treatment of Cancer 465

Like chemotherapy, radiation therapy
can affect the growth and reproduction ofnormal cells, resulting in potentially tox-ic side effects. The number of normal cellsexposed to the radiation, the dosage of ra-diation, the part of the body receiving ra-diation therapy, and individual variabilitydetermine the side effects experienced.These side effects may appear immediate-ly or weeks or months after the radiationtherapy was administered. Some individ-uals experience generalized symptomssimilar to those of radiation sickness: nau-sea, vomiting, loss of appetite, fatigue, andheadache. Other individuals may experi-ence side effects speciﬁc to the area irra-diated, such as sore throat if the head orneck has been irradiated, or localized skinreactions, such as radiation burn. Likechemotherapy, radiation therapy mayalso cause bone marrow depression, result-ing in anemia, lowered resistance to infec-tion, and possible hemorrhage.
Biological Therapies
Immunotherapy
A newer approach to the treatment of
cancer is immunotherapy. Since humancancer cells express cancer-associated anti-gens, the goal of immunotherapy is tostrengthen the individual’s own immunesystem so that it recognizes cancer cells asforeign objects and destroys them(Rosenberg, 2004). Thus, the body’s ownimmune system is enhanced to ﬁght can-cer cells.
Immunotherapeutic agents can also
help to increase the susceptibility of can-cer cells to the cytotoxic agents (chemicals
that are detrimental to or destroy cells).Many immunologic approaches to cancertreatment are already being used. Exam-ples of immunotherapy are the interferons
and interleukin-2 . Another example is theuse of bacille Calmette-Guérin (BCG) in the
treatment of superﬁcial bladder cancer.
Hormone Therapy
Adjuvant hormone therapy can be used to
increase the beneﬁts of chemotherapy incancers that are hormone dependent(such as certain breast cancers that are es-trogen dependent and prostate cancersthat are androgen dependent). Hormonesare not used to kill cancer cells, but ratherto keep the cancer cells from growing fur-ther so that individuals are in remissionfor extended periods of time. Hormonepreparations work by blocking hormonereceptors to cells so that estrogen or an-drogen cannot be used by the cancer cells.
Gene Therapy
Gene therapy in the treatment of can-
cer is still in its infancy. Gene therapyworks by actually modifying the geneticstructure of the cancer cell to suppress orinhibit tumor growth.
Bone Marrow Transplantation
Bone marrow transplantation is performed
when the escalation of chemotherapy(chemical substances or drugs used to treatdisease) may result in a cure of the can-cer, but the dosage would be lethal to theindividual’s bone marrow. In addition,bone marrow transplants also seem tohave an antitumor effect themselves,aside from their use with chemotherapy.Bone marrow transplants are used for a va-riety of cancers, including leukemias ,
Hodgkin’s and non-Hodgkin’s lymphomas ,
multiplemyeloma , and breast cancer .
The goal of bone marrow transplanta-
tion is to provide healthy cells that candifferentiate into blood cells to replace de-ﬁcient or pathologic cells. The transplant-ed cells have the ability to completelyreplace and produce all red blood cells,466
CHAPTER 16 C ANCERS

platelets, T lymphocytes, and B lympho-cytes (see Chapter 8) as well as other mar-row stem cells (cells that can reproduce and
differentiate into other types of cells).
In preparation for bone marrow trans-
plantation, individuals receive large dos-es of radiation and/or chemotherapy thateradicate any viable marrow, as well as killtumor cells and suppress the immune sys-tem to reduce the chance of rejection ofthe transplant. As a result of immune sys-tem suppression, individuals receiving thetransplant are susceptible to infection. Theindividual receives an infusion of cellsfrom the donor, and the bone marrow re-generates using the new cells.
Taking bone marrow from the donor is
a surgical procedure in which marrow isremoved from the iliac crests (hipbone)
while the donor is under spinal or gener-al anesthesia. Allogenic transplants (taken
from another individual) have the advan-tage of not risking contamination withcancer cells, but the disadvantage of hav-ing a higher incidence of transplant rejec-tion, or graft versus host disease (GVHD), in
which the transplanted cells attack thecells of the individual who received them.To minimize the chance of rejection orGVHD, the more closely matched thedonor is to the individual the better, iden-tical twins being the most compatibledonors.
Individuals can also receive autologous
transplants (cells taken from their own
body). With autologous transplants, cellsare removed from the individual prior toirradiation or chemotherapy and frozen.They are then reinfused. The advantage ofautologous infusions is that rejection orGVHD is avoided. The disadvantage is thatthere is risk of contamination with tumorcells from the individual’s body. Also, au-tologous transplants lack the additionalantitumor effect that is seen with allo-genic transplants. In addition to being in the bone mar-
row, stem cells also circulate in the periph-eral blood and may also be used for trans-plant. Peripheral stem cell transplantationis a procedure by which cells are removedfrom the peripheral blood, thus avoidinga surgical procedure. For autologous trans-
plants , this procedure may be used if the
individual is too debilitated to withstanda surgical procedure. The disadvantage in
an autologous transplant is, again, the pos –
sible contamination with other cancer cells.
For allogenic transplant , although the
donor is able to avoid a surgical procedure,the recipient may run a greater risk ofrejection of the transplant or GVHD be-cause of receiving a greater number of Tcells from the donor. The most critical pe-riod is 2 to 4 weeks after the bone marrowtransplantation. Because of the immuno-suppression prior to surgery, individualsmay have an increased susceptibility toinfections for up to 3 months after thetransplant. In addition, because immuno-suppressive therapy drastically reduces thecomponents in the blood that controlbleeding, complications such as hemor-rhage may result.
COMMON CANCERS ANDSPECIFIC TREATMENTS
The diagnostic procedures, treatment,
and functional limitations associated withcancer differ depending on the anatomicsite involved. In many instances, a com-bination of treatments, including surgery,chemotherapy, and irradiation, is used. Inthe treatment of cancer in its very earlystages, surgery alone may be sufﬁcient.
Cancer of the Gastrointestinal Tract
Treatment of cancer of the gastrointesti-
nal tract or accessory organs often consistsof the removal or major resection of theCommon Cancers and Speciﬁc Treatments 467

organs involved. Because the symptoms ofcancers of the esophagus, stomach, liver,and pancreas frequently occur late in thedisease, treatment may be directed towardpalliation rather than cure.
Surgical treatment for cancer of the
mouth may include removal of the tumor,
as well as removal of the nearby lymphglands to determine whether cancer hasspread. If cancer has spread to the neck orother tissues, more radical surgery may beindicated, which can result in facial defor-mity or disﬁgurement because of theamount of tissue removed. If the tonguehas been partially removed, speech maybe affected. Reconstructive surgery may berequired later to minimize these effects.
Cancer of the esophagus has been
linked to smoking or gastroesophageal re-ﬂux disease (Brown, Hoover, Silverman, etal., 2001; Terry, Lagergren, Ye, Nyren, &Wolk, 2000; Wu, Wan, & Bernstein, 2001).Other risks include obesity (Lagergren,Bergstrom, Adami, & Nyren, 2000) andBarrett’s esophagus (Shaheen & Ransohoff,
2002), which is abnormal tissue extendingfrom the opening of the stomach into theesophagus. Treatment of cancer of the
esophagus may consist of radiotherapy
with or without chemotherapy or surgery.When esophageal cancer is localized, theaffected part of the esophagus may be re-moved and reattached to the remainingpart of the esophagus (Enzinger & Mayer,2003). When the cancer is more severe,esophagectomy (removal of the esopha-
gus) may be necessary. If the individualhas the esophagus removed, an artiﬁcialopening must be made into the stomachand a tube inserted through which liquidfeedings can be taken. After the feeding,the opening is then “plugged” to preventleakage. After removal of the esophagus,individuals lose the ability to eat or drinkthrough the mouth. The ramiﬁcations ofthis type of surgery may seriously inﬂu-ence individuals’ willingness to have sur-gical versus other forms of treatment fortheir cancer.
Treatment of cancer of the large bowel
(colon and rectum) usually involves bothsurgical removal of the tumor and someresection of the colon itself, through anincision in the abdomen (Pappas & Jacobs,
2004). In many instances, the diseased part
of the bowel can be removed and the tworemaining ends joined together, enablingthe individual to retain normal bowel
function. When this is not possible, a colos-
tomy may be performed (see Chapter 10).
Cancer of the Larynx
Although many other structures in the
head and neck can be a site of cancer, oneof the most common cancers of the headand neck is cancer of the larynx (voice-
box). Smoking and alcohol are two lead-ing risk factors for laryngeal cancer and aresynergistic in their effects (Wu et al.,2001). Some occupations also appear tohave increased risk due to secondary tox-ic exposures.
Symptoms of Cancer of the Larynx
The larynx contains the vocal cords.
The most common symptom in cancer ofthe larynx is alteration in voice quality orhoarseness. Other symptoms may in-clude dysphagia (difﬁculty in swallowing)
and cough.
Diagnosis of Cancer of the Larynx
The diagnostic procedures used to iden-
tify problems of the larynx often includea procedure called a laryngoscopy , in
which a hollow tube is inserted into thelarynx so that the physician can inspectthe structures of the larynx and assess thefunction of the vocal cords.468
CHAPTER 16 C ANCERS

Common Cancers and Speciﬁc Treatments 469
Treatment of Cancer of the Larynx
Although the treatment of cancer of the
larynx is dependent on a number of fac-
tors, it usually involves irradiation, surgery,
or a combination of the two. Although inthe past treatment of advanced cancer ofthe larynx usually involved total removalof the larynx, nonsurgical approaches in-volving chemotherapy and radiation arenow frequently used instead of surgery inmany cases (Forastiere et al., 2003). Whenthe tumor is small (stage T1 or T2, N0 andM0), radiation alone may be used rather
than surgery to eradicate the tumor (Vokes
& Stenson, 2003). Laser treatment, whichdestroys the tumor by intense light beams,may also be used to treat cancer of the lar-ynx in its early stages. If the tumor is dis-covered early, before there has beenextensive involvement of the surroundingtissues, it may be necessary to remove onlypart of the larynx. This procedure is calleda subtotal (partial) laryngectomy . Both
subtotal laryngectomy and laser treatmentcan preserve the capacity for normalspeech, although they may affect voicequality to some degree.
When the cancer is more advanced, it
may be necessary to remove the larynxcompletely. This procedure is called a la-
ryngectomy . Usually, individuals who
have undergone this type of surgery areunable to breathe or speak by normalmechanisms. After the larynx has been re-moved, the trachea is no longer connect-ed either to the nasopharynx or to thenasal passages (see Chapter 12). The sur-geon creates a permanent opening calleda tracheostomy in the individual’s neck
and trachea, and the individual breathesthrough this opening ( laryngostoma ) rather
than through the nose and mouth.Although they are able to eat and drinknormally, individuals must breathe,cough, and sneeze through the tracheosto-my. The sense of smell, and in turn taste,is diminished because air ﬂows throughthe opening in the neck instead ofthrough the nose.
Psychosocial Issues in Cancer of
the Larynx
The psychosocial and vocational effects
of laryngectomy can be profound. Ahealthy voice is critical for effectiveness atwork as well as in personal and social in-teractions (Zeitels & Healy, 2003). Individ-uals immediately lose the ability to make
vocal sounds for speech as well as the audi –
ble sounds of laughter or crying. Conse-quently, when at all possible, physiciansattempt to preserve as much of the larynxas possible. When this is not possible,individuals must learn new techniques forspeaking.
Attempts to improve the ability to
speak after treatment of laryngeal cancerhave been successful. Surgical techniqueshave evolved so that much of the larynx
can be spared and it doesn’t have to be total –
ly removed. In addition, improved meth-ods of voice rehabilitation after totallaryngectomy have been devised. Thereare three basic types of voice rehabilitationtechniques after total laryngectomy:
• Tracheo-esophageal speech (shunt
speech)
• Esophageal speech• Electrolaryngeal speech
In tracheo-esophageal techniques a ﬁs-
tula (a passageway from one structure to
another) is surgically constructed be-tween the trachea and esophagus with asmall prosthesis being placed in the ﬁstu-la. Closing the tracheostomy with thehand or ﬁngers moves air from the tracheato the esophagus, creating a pseudovoice .
As a result, individuals are able to producelung-powered speech of better quality

than was previously accomplished withother methods such as esophageal speech .
The prosthesis in the ﬁstula prevents foodand liquid from entering the airway whenindividuals are eating. A limitation of theﬁstula is the need for periodic removal theprosthesis for cleaning and replacement,and the need to use one hand to occludethe tracheostomy during speech. Specialvalves that ﬁt into the opening are, how-ever, available to eliminate the need formanual coverage of the opening. Duringnormal breathing the valve remains open;however, when individuals begin to speak,because of increased expiratory pressure,the valve closes.
Esophageal speech is a technique of
speaking that involves trapping air in theesophagus and gradually releasing it at thetop of the esophagus to produce a pseudo-
voice. If sounds produced by esophageal
speech are too soft to be heard, a person-
al ampliﬁer-speaker may be used to increase
sound volume. Since the air capacity of the
upper esophagus is considerably less than
that of the lungs, esophageal speech is typi –
cally limited in rate, volume, and duration.
Electrolaryngeal speech is another speech
alternative that may be used by individu-als with laryngectomy. It utilizes a battery-powered vibratory device called anartiﬁcial larynx . There are several types
available; however, most are electronic,battery-operated devices that are heldagainst the throat to produce sound.Although the artiﬁcial larynx is relativelyeasy to use, the speech produced has a me-chanical, monotone sound that some in-dividuals ﬁnd objectionable.
Regardless of the type of speech alterna-
tive individuals use, speech-language pathol-
ogists are usually consulted for evaluation
and possible treatment based on individ-uals’ speciﬁc voice issues. Speech-languagepathologists assess factors that have an im-pact on voice production, identify anyproblem behaviors, and plan treatment torectify the problem.
When individuals have a total laryngec-
tomy, they must also adjust to the visibleopening in the neck, the laryngeostoma.Any disﬁgurement, especially when relat-ed to the face, may damage individuals’self-concept and self-image. For cosmeticpurposes, individuals may wear a scarf orother covering loosely around the neck.This covering can also keep dust and dirtout of the opening. Another type of cov-ering available is a foam ﬁlter, which keepsmoisture loss to a minimum and also pre-vents hair, shaving cream, or other parti-cles from falling into the trachea duringroutine daily hygiene. Since the openingleads directly into the trachea and lungs,individuals must avoid activities such asswimming and water sports in which wa-ter could enter the opening. For shower-ing, special laryngectomy shower collarsthat prevent water from running into theairway are available. With a laryn-geostoma, individuals no longer have thebeneﬁt of having air humidiﬁed as it pass-es through the upper airway passages.Consequently, they may need to run a hu-midiﬁer, especially at night, to keep thetrachea moist.
Because the quality of speech is also al-
tered, individuals may avoid social situa-tions in which they have to speak, becausethey perceive their altered speech as dis-tasteful and embarrassing. Although indi-viduals with laryngectomy can carry outmost activities of daily living normally,some individuals may notice a decreasedability to lift heavy objects because theycannot close the tracheostomy to build upinternal pressure, as those who breathenormally can do, by compressing their lips
and holding their breath. Individuals who
have had a total laryngectomy should al-ways carry an identiﬁcation card or weara medical identiﬁcation bracelet to inform470
CHAPTER 16 C ANCERS

emergency personnel that they are a to-tal neck breather.
Vocational Issues in Cancer of the Larynx
Only a few jobs may prove difﬁcult for
individuals after a laryngectomy. Thosejobs performed in environments with ex-treme heat or cold, or those that exposeindividuals to extreme dust or fumesshould probably be avoided. Although thephysical aspects of laryngectomy may notaffect individuals’ ability to work, the im-pact that the use of alternate modes ofspeech may have on employment can bestriking, especially if individuals’ use ofvoice is a necessary component of work.Employers and coworkers may also viewindividuals as being less socially accept-able because of their speech and thereforeavoid interactions. Social support fromfriends and family or participation in peersupport groups like the Lost Chord Club
can help signiﬁcantly in the adjustment.
Cancer of the Lung
Lung cancer is one of the leading causes
of cancer death in the United States andtobacco products cause over 80 percent ofthe lung cancers diagnosed (Miller, 2000).Occupational ex
posure to carcinogens ac-
counts for approx imately 15 percent of
lung cancer cases (Cleary, Gorenstein, &
Omenn, 1996); however, when exposure is
associated with tobacco use, the risk of lungcancer increases dramatically (Miller, 2000).
Lung cancer can be found in a variety
of cell types with varied rates of growth,with some types being slow-growingwhile others are aggressive and fast-grow-ing. Symptoms are often not present un-til lung cancer has reached an advancedstage. Lung cancer is usually diagnosedthrough chest X-ray, CT scan, broncho-scopy, or biopsy.Treatment of lung cancer may be surgi-
cal, with removal or resection of the lung,
or may consist of radiation therapy, chemo –
therapy, or a combination. When used inthe treatment of lung cancer, radiationtherapy is usually for palliation, rarely forcure. For individuals with a type of lungcancer called small-cell carcinoma , chemo-
therapy is generally the treatment ofchoice. If surgical intervention is used forlung cancer, the primary aim is to removethe total tumor. The extent of the surgerydepends on the cancer and its location inthe lung. Removal of an entire lung iscalled a pneumonectomy ; the removal of
only one lobe of the lung is called a lobec-
tomy . A segmental resection is a surgical
procedure in which a segment of the lungis removed. After having a portion of thelung removed, individuals may need tolimit their physical activity to some de-gree, depending on the amount of lung re-moved and the functional capacityremaining.
Since cigarette smoking is frequently
linked to lung cancer, emphysema mayalso coexist, further limiting respiratorycapacity and, consequently, also limitingphysical activity.
Cancer of the Musculoskeletal System
Musculoskeletal cancers frequently re-
sult in the amputation of an extremity (seeChapter 14). For some types of bone can-cers, however, it may be possible to re-move only a section of bone and to avoidamputating the whole extremity. In someinstances, bone cancers may be reducedby chemotherapy and then controlled byradiotherapy.
Cancer of the Urinary System
Cancer can develop in any organ of the
urinary system, but the most frequent siteCommon Cancers and Speciﬁc Treatments 471

is the bladder. There is a high relationshipbetween bladder cancer and cigarettesmoking (Droller, 1998).
The most common symptom of bladder
cancer is hematuria (blood in the urine).
When bladder cancer is suspected, the in-dividual generally undergoes a procedurecalled cystoscopy in which a tube called
a cystoscope is inserted into the bladder, en-
abling the physician to visualize the innersurface of the bladder and to take a biop-sy for laboratory examination.
Bladder cancer is generally classiﬁed as
superﬁcial (in which the cancer cells are
conﬁned to the lining of the bladder) orinvasive (in which the cancer cells have
penetrated other tissues). Although cancerof the bladder may be treated in a varietyof ways, depending on the stage and typeof cancer involved, the most commontreatment for invasive cancer is a proce-dure called radical cystectomy , in which the
total bladder is surgically removed. Re-moval of the whole bladder necessitatessurgical reconstruction to provide a meansfor urinary drainage, a procedure calledurinary diversion . Although removal of the
bladder once affected individuals’ qualityof life, now, because of major advances inurinary diversion, radical cystectomy is amore acceptable option. Continent urinary
diversions allow individuals to avoid exter-
nal collection devices and have minimalchange in body image with only a smallstoma (opening) in the abdomen.
When the total bladder is removed, an
artiﬁcial reservoir for collection of urinemust be substituted. Several types ofreservoirs may be used. If the entire low-er urinary system is removed, includingthe urethra (tube structure through which
urine is excreted from the bladder to theoutside of the body through the urinarymeatus), an internal reservoir is construct-ed with an opening through which acatheter can be inserted for urinary drain-age to the outside of the body. If the blad-der alone is removed, leaving the urethraintact, a reservoir may still be construct-ed, but the individual will be able tocontinue to excrete urine through the ure-thra and to have near normal urinationfunction.
At times, only a portion of the bladder
may be removed. The removal of a portionof the bladder may greatly diminish thecapacity of the bladder, necessitatingmore frequent urination. Another proce-dure for urinary diversion is cutaneous
ureterostomy , in which the ureters are
brought through the abdomen to the out-side of the body, where they drain direct-ly into a bag attached to the outside of theabdomen. A special bag called an urosto-
my bag is worn over the opening to col-
lect urine. Still another urinary diversionprocedure is called an ileal conduit , which
involves removing a segment of small in-testine (the ileum ) and reconnecting the
two remaining ends of bowel. Ureters arethen connected to one end of the loop ofsmall intestine that has been removed,and the other end of the loop of small in-testine is brought to the outside of the ab-domen to form an opening throughwhich urine can drain. There is no volun-tary control over the drainage of urinethrough the opening of either the cuta-neous ureterostomy or the ileal conduit.
Less common types of urinary diversion
include ureterosigmoidostomy , in which the
ureters (tubes that drain urine from the
kidneys to the bladder) are connected tothe colon so that urine is excreted throughthe rectum. Because urine mixes with thecontents of the colon, bowel movementsare liquid, and frequent evacuation ofstool is necessary. Because of the potentialcontamination of the urinary system byorganisms of the colon, a major compli-cation of this type of urinary diversion ischronic pyelonephritis (see Chapter 13).472
CHAPTER 16 C ANCERS

When cancer of the bladder is superﬁcial ,
the cancer may be treated with bacille
Calmette-Guérin (BCG), a form of im-
munotherapy in which the body’s own im-
mune system is stimulated to respond toand ﬁght the cancer cells. BCG therapyconsists of instilling the vaccine of BCGinto the bladder. When superﬁcial cancerof the bladder is more advanced, chemo-therapy may also be used.
Cancer of the kidney may necessitate re-
moval of the kidney ( nephrectomy ). When
both kidneys are involved, a portion ofone kidney may be left intact to maintainrenal function. If both kidneys must becompletely removed, individuals must beplaced on regular dialysis (see Chapter 13).
Cancer of the Brain or Spinal Cord
When malignant tumors of the brain
are small and accessible and have not in-vaded surrounding tissue, they may besurgically removed and the individualtreated with chemotherapy or radiation.If there are no complications from surgery,individuals may be able to return to activelife. Some individuals experience someneurologic deﬁcits after surgery (see Chap-ter 2). At other times, the tumor may beembedded in the brain or may be locatedin a part of the brain that is inaccessible,
so that surgery is not possible without con-
siderable risk to the individual. In these in-stances, chemotherapy or radiation ther-apy alone may be instituted as a means ofcontrol or palliation. The degree or typeof limitation that results from a malignantbrain tumor depends on the type of can-cer, its size, and its location within thebrain, as well as on any residues thatmight be experienced from surgery.
Cancers develop less often in the spinal
cord than in the brain. Symptoms of aspinal cord tumor may be similar to thoseexperienced with a spinal cord injury, in-cluding paralysis (see Chapter 3). Spinalcord tumors are usually treated surgically,with irradiation and chemotherapy as ad-junct therapies.
Lymphomas
The lymphatic system is a connection
of lymph nodes and vessels in which aclear ﬂuid called lymph circulates throughthe body. The lymphatic system acts toﬁght infection and contributes to thebody’s immune system (see Chapter 8).Cancers of the lymphatic system arecalled lymphomas .
There are two classiﬁcations of lym-
phomas:
• Hodgkin’s disease • Non-Hodgkin’s lymphoma
Hodgkin’s Disease
Hodgkin’s disease is a chronic, progres-
sive disease in which abnormal cells grad-ually replace the normal elements withinthe lymph nodes. The cause of Hodgkin’sdisease is unknown. Many individualswith Hodgkin’s disease are asymptomatic
(have no symptoms) or have only periph-eral lymphadenopathy (enlargement of
lymph nodes).
Hodgkin’s disease is usually diagnosed
through an excisional biopsy of an affect-
ed lymph node. Bone marrow is rarely af-fected; however, a bone marrow biopsymay also be performed.
Although many individuals’ disease is
advanced at the time of diagnosis, ad-vances in treatment have made Hodgkin’sdisease mostly curable (DeVita, 2003).Treatment of Hodgkin’s disease varies withthe stage at which it is diagnosed. Earlystages are usually treated with radiationtherapy, and later stages, with chemother-apeutic agents. In the late stage of the con-Common Cancers and Speciﬁc Treatments 473

dition, a variety of chemotherapeuticagents may be used in combination totreat the disease. Because of the severe tox-ic effects of this treatment regimen, indi-viduals may have symptoms of nauseaand vomiting, bone marrow suppression,and peripheral neuropathy (changes of
sensation in the extremities). When treat-ed in the early stages, Hodgkin’s diseasehas a high rate of remission, and individ-uals with this condition have an excellentprognosis.
Non-Hodgkin’s Lymphoma
Non-Hodgkin’s lymphomas consist of
a proliferation of lymph cells that usual-ly disseminate throughout the body. Diag-nosis is made through examination oftissue that has been removed. Often bonemarrow biopsy is also done because bonemarrow involvement is likely.
Unlike with Hodgkin’s disease, most in-
dividuals with non-Hodgkin’s lymphomaare in advanced stages of the disease be-fore diagnosis is made. The most commonsymptom is generalized adenopathy (en-
largement of the lymph nodes).The con-dition may be low grade, meaning that itprogresses slowly, or aggressive high grade,meaning that it progresses rapidly and can
be fatal in months. Individuals with high-
grade non-Hodgkin’s lymphoma general-ly have symptoms of unexplained weightloss or unexplained fever. Non-Hodgkin’slymphomas are usually treated with radi-ation therapy in the early stages, andchemotherapy in conjunction with radi-ation therapy in the later stages, with pos-sible bone marrow transplant.
Multiple Myeloma
Multiple myeloma is a slowly progres-
sive cancer in which there is the uncon-trolled reproduction of abnormal plasmacells leading to the destruction of the bonemarrow and extending into the bone.Bone marrow produces red blood cells,white blood cells, and platelets, whichcontrol blood clotting. As the bone mar-row is destroyed, individuals with multi-ple myeloma may experience anemia andabnormal bleeding. The ﬁrst symptom ofmultiple myeloma is often bone pain,which may be concentrated in the back.Bone destruction can also lead to patholog-
ic fractures (fractures that occur because of
the disease of the bone rather than frominjury) and spinal cord compression.
The diagnosis of multiple myeloma may
be based on blood tests, radiologic exam-ination of the skeletal system to identifybone destruction, or biopsy of the bonemarrow itself. Chemotherapy and, attimes, radiation therapy are major formsof treatment.
Because inactivity results in additional
breakdown of bone, emphasis is placed onhelping individuals remain active. Theprognosis is dependent on the stage of thedisease when diagnosed; however, multi-ple myeloma is not currently curable.
Leukemia
Cancers of tissues in which blood is
formed are called leukemias. There are var-ious types of leukemias. Leukemia can beclassiﬁed as acute or chronic .
Acute Leukemia
In most instances there is no known
cause of acute leukemia; however, factorssuch as exposure to radiation, occupation-
al exposure to certain chemicals, and virus –
es and genetic links have all been cited aspossible contributing factors (Appelbaum,2000). In acute leukemia, there is prolif-eration of malignantly transformed stem
cells (cells from which other cells origi-474
CHAPTER 16 C ANCERS

nate) in the bone marrow that suppressthe growth and differentiation of normalblood cells. Many abnormal, immaturewhite blood cells are released into the cir-culatory system. As a result, individualswith acute leukemia frequently experienceanemia, neutropenia (small numbers of
mature white blood cells), and thrombo-
cytopenia (abnormal number of plate-
lets). They may also experience fatigue,headache, susceptibility to infection, andbruising or hemorrhage.
Since acute leukemia is a rapidly pro-
gressing disease, treatment is usually insti-tuted immediately. The goal of treatmentis to induce complete remission. Treat-ment usually consists of chemotherapyand in some instances bone marrowtransplantation.
Chronic Leukemia
Chronic leukemia consists of a broad
spectrum of disorders and involves over-production of white blood cells, causingsplenomegaly (enlargement of the
spleen). Individuals are often withoutsymptoms initially, so that the conditionis ﬁrst discovered through blood tests dur-ing a routine physical, or from medicalconsultation because of another problem.When symptoms are present, individualsoften have fatigue or weight loss.
Chronic leukemia is an unpredictable
disease. In some individuals the conditionprogresses slowly so that they live with itfor decades, often dying because of othercauses, whereas in others the condition re-quires frequent and multiple forms oftherapy and can result in death within afew years (Rai & Chiorazzi, 2003). Al-though there are few or no symptoms inthe early stages of the disease, if the dis-ease progresses individuals may experi-ence headaches, bone pain, joint pain, orfever. Diagnosis is based on results ofblood tests as well as the presence of anenlarged spleen.
Immediate treatment of chronic leuke-
mia is usually not necessary unless thereare complications. Initial treatment mayconsist of oral medication to control theabnormal blood cell proliferation. Latertreatment may consist of medicationssuch as interferon (discussed earlier in thechapter) or other types of chemotherapy.
Cancer of the Breast
As with other types of cancer, early di-
agnosis of breast cancer is most predictiveof prognosis and cure (Fletcher & Elmore,2003). The use of breast self-examinationand mammography can lead to early de-tection and, thus, permit early treatment.The primary treatment of breast cancer isbased on the stage of the disease at thetime of diagnosis. The treatment may belocal, regional, or systemic. Local/region-al control usually involves surgery.
Treatment of breast cancer previously
involved removal of the entire breastthrough either simple mastectomy or
radical mastectomy , in which the entire
breast as well as its underlying tissue, in-cluding muscle and lymph nodes, was re-moved. Studies have shown, however,that modiﬁed procedures in many casesare just as effective in preventing metas-tasis or improving survival (Fisher, Bauer,Margolese et al., 1985). These alternativesurgical techniques may include:
•lumpectomy (removal of the cancer-
ous lesion itself and a small amountof surrounding breast tissue)
•partial or segmental mastectomy
(removal of a quadrant of the breast)
The appropriateness of using more con-
servative surgical techniques that preserveas much of the breast tissue as possible de-pends on the size and location of the tu-Common Cancers and Speciﬁc Treatments 475

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