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i

Navigating Life with

Amyotrophic Lateral Sclerosis
ii

Lisa M. Shulman, MD, FAAN

Editor-​in-​Chief, Neurology Now™ Books Series
Fellow of the American Academy of Neurology
Professor of Neurology
The Eugenia Brin Professor in Parkinson’s Disease and Movement Disorders
The Rosalyn Newman Distinguished Scholar in Parkinson’s Disease
Director, University of Maryland PD & Movement Disorders Center
University of Maryland School of Medicine
Baltimore, MD

Other Titles in the Neurology Now™ Books Series

Navigating Life with Parkinson’s Disease
Sortirios A. Parashos, MD, PhD; Rose Wichmann, PT; and Todd Melby

Navigating Life with a Brain Tumor

Lynne P. Taylor, MD, FAAN; Alyx B. Porter Umphrey, MD; and Diane Richard

Navigating the Complexities of Stroke

Louis R. Caplan, MD, FAAN

Navigating Life with Multiple Sclerosis

Kathleen Costello, MS, ANP-​BC, MSCN; Ben W. Thrower, MD;
and Barbara S. Giesser, MD

Navigating Life with Epilepsy

David C. Spencer, MD, FAAN
iii

Navigating Life with

Amyotrophic
Lateral Sclerosis
Mark B. Bromberg, MD, PhD, FAAN
Department of Neurology
University of Utah
Salt Lake City, Utah

Diane Banks Bromberg, JD

Fabian VanCott
Salt Lake City, Utah

1
iv

1
Oxford University Press is a department of the University of Oxford. It furthers
the University’s objective of excellence in research, scholarship, and education
by publishing worldwide. Oxford is a registered trade mark of Oxford University
Press in the UK and certain other countries.
Published in the United States of America by Oxford University Press
198 Madison Avenue, New York, NY 10016, United States of America.
© American Academy of Neurology 2017
All rights reserved. No part of this publication may be reproduced, stored in
a retrieval system, or transmitted, in any form or by any means, without the
prior permission in writing of Oxford University Press, or as expressly permitted
by law, by license, or under terms agreed with the appropriate reproduction
rights organization. Inquiries concerning reproduction outside the scope of the
above should be sent to the Rights Department, Oxford University Press, at the
address above.
You must not circulate this work in any other form
and you must impose this same condition on any acquirer.
Library of Congress Cataloging-in-Publication Data
Names: Bromberg, M. B. (Mark B.), author. | Banks-Bromberg, Diane, author.
Title: Navigating life with ALS / by Mark B. Bromberg, MD, FAAN, Department
of Neurology, University of Utah, Salt Lake City, UT,
Diane Banks-Bromberg, JD, Fabian Vancott, Salt Lake City, UT.
Description: New York, NY : American Academy of Neurology/Oxford University
Press, [2017] | Includes index.
Identifiers: LCCN 2016036224 | ISBN 9780190241629 (alk. paper)
Subjects: LCSH: Amyotrophic lateral sclerosis—Popular works.
Classification: LCC RC406.A24 B76 2017 | DDC 616.8/39—dc23
LC record available at https://lccn.loc.gov/2016036224
This material is not intended to be, and should not be considered, a substitute for
medical or other professional advice. Treatment for the conditions described in this
material is highly dependent on the individual circumstances. And, while this material
is designed to offer accurate information with respect to the subject matter covered
and to be current as of the time it was written, research and knowledge about medical
and health issues is constantly evolving and dose schedules for medications are being
revised continually, with new side effects recognized and accounted for regularly.
Readers must therefore always check the product information and clinical procedures
with the most up-​to-​date published product information and data sheets provided by
the manufacturers and the most recent codes of conduct and safety regulation. The
publisher and the authors make no representations or warranties to readers, express
or implied, as to the accuracy or completeness of this material. Without limiting the
foregoing, the publisher and the authors make no representations or warranties as to
the accuracy or efficacy of the drug dosages mentioned in the material. The authors
and the publisher do not accept, and expressly disclaim, any responsibility for any
liability, loss or risk that may be claimed or incurred as a consequence of the use and/​or
application of any of the contents of this material.
1 3 5 7 9 8 6 4 2
Printed by Sheridan Books, Inc., United States of America
v

We dedicate this book to Lois F. Hall and her husband, Ray A. Hall,
parents of Diane Banks Bromberg. Lois rose to the challenges of ALS
and lived her life enthusiastically until her passing. She participated
in a drug trial that she hoped would shed light on this disease for the
benefit of future patients. Ray unselfishly dedicated himself to helping
Lois through her illness in every way possible.
vi
vi

CONTENTS

About the AAN’s Neurology Now™ Books Series   |   xix

Lisa M. Shulman, MD, FAAN
Foreword   |   xxiii
Preface   |   xxv
Acknowledgments   |   xxvii

1. Introduction   |   1
What Are the Basic Features of ALS?   |   1
How It Starts: Patient Stories   |   4
How to Use This Book   |   10

2. ALS Origins   |   13
Why the Name ALS?   |   13
Is ALS Different from Motor Neuron Disease?   |   13
Is ALS a New or Old Disease?   |   14
How Common Is ALS?   |   15
Who Was Lou Gehrig?   |   15
Who Else Had ALS?   |   16

v ii
vi

v iii Contents

3. The Diagnosis of ALS   |   19

What Does the Neurologist Look for?   |   19
What Are the Symptoms and Signs of ALS?   |   20
Bulbar Difficulties   |   21
Upper Extremity Difficulties   |   21
Lower Extremity Difficulties   |   22
Progression of Symptoms   |   22
What Are the Most Important Tests?   |   23
What Are the El Escorial Criteria?   |   24
What Are “Rule Out” Tests?   |   25
Why Does Making the Diagnosis Take So Much Time?   |   26
Do I Really Have ALS? What Diseases Mimic ALS?   |   27
Multifocal Motor Neuropathy with Conduction Block   |   27
Inclusion Body Myositis   |   28
Kennedy Disease   |   28
Cervical Spondylitic Myelopathy   |   29
Lumbosacral Spondylitic Radiculopathy   |   29
Lyme Disease   |   30
Carpal Tunnel Syndrome   |   30
Ulnar Neuropathy at the Elbow   |   31
Should I Get a Second Opinion?   |   31
How Was the Diagnosis Given and Received?   |   31

4. Causes of ALS   |   33
What Causes ALS?   |   33
Why Do Neurons Die in ALS?   |   34
Glutamate Excitotoxicity   |   34
Oxidative Stress   |   35
ix

Contents ix

Mitochondrial Dysfunction   |   35
Protein Aggregation   |   35
Immune Dysfunction   |   36
Gene Mutations   |   36
Challenging Facts about ALS   |   36
Why Do I Have ALS?   |   38
Environmental Factors   |   38
Clusters   |   38
Military Service   |   39
What Is Familial ALS?   |   39
How Is Familial ALS Passed On?   |   41
Autosomal Dominant Inheritance   |   41
Autosomal Recessive Inheritance   |   41
X-​Linked Inheritance   |   42
How Many Genes Are Associated with Familial ALS?   |   45
Are There Genes Associated with Frontotemporal
Lobe Dementia?   |   45
Are Genetic Causes of ALS and Frontotemporal
Lobe Dementia Related?   |   45
How Do I Know If I Have Familial ALS?   |   46
Should I Have Genetic Testing for ALS?   |   47
Should Family Members of Individuals with Familial ALS
Have Genetic Testing?   |   47

5. Motor Progression in ALS   |   49

How Does ALS Progress?   |   49
How Is Progression Measured?   |   50
Muscle Strength   |   51
Functional Rating Scale   |   51
x

x Contents

How Fast Am I Progressing?   |   52

Does ALS Ever Get Better?   |   54
Are There Staging Scales for ALS?   |   54

6. Nonmotor Features of ALS   |   57

What Is Frontotemporal Lobe Dementia?   |   57
What Are the Features of Frontotemporal
Lobe Dementia?   |   58
How Is Frontotemporal Lobe Dementia Diagnosed?   |   58
Why Is It Important to Diagnose Frontotemporal
Lobe Dementia?   |   60
How Are Symptoms of Frontotemporal Lobe
Dementia Treated?   |   61
What Is Pseudobulbar Affect?   |   61
What Are the Features of Pseudobulbar Affect?   |   62
How Is Pseudobulbar Affect Diagnosed?   |   63
Why Is It Important to Diagnose Pseudobulbar
Affect?   |   63
How Is Pseudobulbar Affect Treated?   |   63
Can ALS Cause Changes in Bowel and Bladder
Function?   |   65
Why Do I Feel So Fatigued?   |   66
ALS Can Be Depressing. How Do I Know If
I Am Depressed?   |   67
Why Have I Noticed Changes to My Skin?   |   68
Why Do I Have Scaly Skin?   |   69
Why Do I Sweat So Much?   |   69
Why Do My Eyes Sting?   |   70
Why Do I Bite My Cheek?   |   70
Why Are My Hands or Feet Red, Swollen, or Cold?   |   70
xi

Contents xi

Do I Have to Worry about a Deep Venous Thrombosis

(Blood Clot)?   |   71
Do I Have to Worry about Skin Pressure Sores?   |   71

7. Management and Treatment of ALS   |   73

Do I Need a Primary Care Physician?   |   73
Should I Continue with My Current Medications?   |   73
Where Can I Get the Most Comprehensive Care?   |   74
What Happens in a Multidisciplinary ALS Clinic?   |   74
Neurologist   |   74
Nurse   |   75
Speech-​Language Pathologist   |   75
Occupational Therapist   |   75
Physical Therapist   |   75
Respiratory Therapist   |   76
Dietitian   |   76
Social Worker   |   76
Genetic Counselor   |   76
Palliative Care and Hospice   |   77
Pulmonologist   |   77
Gastroenterologist   |   77
Psychiatrist   |   77
Psychologist   |   78
Orthotist   |   78
How Often Should I Be Seen in the Clinic?   |   78
Should I Attend a Support Group?   |   78
What Is Available on the Internet?   |   80
What Should I Take for My ALS?   |   80
What about Riluzole (Rilutek)?   |   81
xi

x ii Contents

What Else Is Available?   |   82

What about Stem Cells for ALS?   |   83
What Can Stem Cells Do?   |   83
How Are Stem Cells Delivered in ALS?   |   84
Where Can I Get Stem Cell Treatment?   |   84
What about Dietary Supplements and Alternative
Therapies?   |   85
Will Protein Supplements Build Muscle?   |   86
Can Creatine Give Me More Strength?   |   86
Can Chelation Therapy Get Rid of Toxins?   |   86
Should I Replace My Amalgam (Silver) Dental Fillings?   |   87
What about Massage and Acupuncture?   |   87
Should I Exercise?   |   87
Can Exercise Improve Strength?   |   87
If I Don’t Exercise, Will I Lose Strength Faster?   |   88
Can Exercise Be Harmful?   |   88

8. Living with ALS   |   91

What Will My Quality Of Life Be?   |   91
What Can I Still Do?   |   93
What Should I Do in the Time I Have, and When Should
I Do It?   |   94
Can I Travel?   |   94
Can I Still Have Intimacy?   |   95
Can I Have a Child?   |   96
What Is My Legacy to My Family?   |   96

9. Nutrition and ALS   |   99

Why Is Nutrition Important in ALS?   |   99
Why Do I Lose Weight?   |   99
xi

Contents x iii

Which Foods Are Harder or Easier to Swallow?   |   100

How Much Weight Loss Is Too Much?   |   101
How Do I Stop Weight Loss?   |   102
High-​Calorie Foods   |   102
Supplements   |   103
What Is Patient-​Caregiver Stress over Food?   |   103
What If I Can’t Maintain My Weight despite
Supplements?   |   104
How Are Feeding Tubes Placed?   |   105
Percutaneous Endoscopic Gastrostomy   |   105
Radiologically Inserted Gastrostomy   |   106
Surgically Placed Gastrostomy   |   106
How Do I Use a Feeding Tube?   |   106
How Do I Take My Medications If I Have a Feeding
Tube?   |   108
What Can I Expect from a Feeding Tube?   |   108
Can I Still Eat If I Have a Feeding Tube?   |   109

10. Breathing and ALS   |   111

How Does ALS Affect Breathing?   |   111
How Does the Diaphragm Work?   |   111
How Will I Know If My Breathing Is Affected?   |   113
What Does High Carbon Dioxide Do?   |   114
How Is Breathing Measured in the Clinic?   |   114
What Can I Do to Help My Breathing on My Own?   |   116
What Is Air or Breath Stacking?   |   116
Should I Stop Smoking?   |   117
What Will Supplemental Oxygen Do?   |   117
What Breathing Numbers Concern Doctors?   |   117
xvi

x iv Contents

Why Is My Cough Weak?   |   118

What Is a CoughAssist Device?   |   119
What Causes My Throat to Tighten and Make It Hard
to Breathe?   |   119
What Happens When My Breathing Becomes
Weaker?   |   120
What Are Mechanical Aids to Breathing?   |   120
More about Noninvasive Ventilation   |   122
What Is Diaphragm Pacing?   |   123
More about Invasive Ventilation   |   124
How Do I Make the Choice for or against Full-​Time
Ventilation?   |   125
What Does “Locked in” Mean?   |   126
What If I No Longer Want to Continue Full-​Time
Ventilation?   |   127

11. Communication and ALS   |   129

How Does ALS Affect Speech?   |   129
How Can Speech Be Optimized?   |   130
What Are Low-​Tech Speech Aids?   |   130
Handwriting   |   131
Alphabet Board   |   131
What Are Medium-​Tech Speech Aids?   |   132
Speech Amplification   |   132
Mobile Telephones   |   132
Digital Tablets   |   133
What Are High-​Tech Speech Aids?   |   133
Head-​ and Eye-​Tracking Devices   |   133
Voice Banking   |   134
Brain-​Computer Interface Communication   |   134
xv

Contents xv

12. Mobility and ALS   |   137

How Can I Pursue Athletic Activities?   |   138
How Can I Manage Dressing?   |   138
How Can I Get on and off the Toilet?   |   139
How Can I Get in and out of the Shower and Tub?   |   139
Showers   |   139
Bathtubs   |   140
How Do I Manage Personal Hygiene?   |   140
How Do I Manage Brushing My Teeth with Weak
Hands?   |   141
How Can I Turn Over in Bed?   |   141
How Can I Support My Head and Back?   |   142
Why Do I Feel Unsteady and Fall?   |   143
What Can Be Done to Aid Walking?   |   145
Ankle-​Foot Orthosis   |   145
Cane or Walking Stick   |   145
Walker   |   146
Lift Chairs   |   146
Medications for Spasticity   |   147
What If I Fall and Cannot Get Up (Medical Alert Signaling
Systems)?   |   147
What about Wheelchairs?   |   147
Manual Wheelchairs   |   147
Power Wheelchairs   |   148
How Do I Get a Power Wheelchair in and out of the House
and Car?   |   148
Does Insurance Pay For a Wheelchair?   |   149
How Do I Order a Wheelchair?   |   149
What about a Scooter?   |   150
xvi

xvi Contents

Why Are Patients Reluctant to Use Mobility Aids?   |   150

How Can I Transfer Safely?   |   151
What Is a Lift?   |   152
Floor Lifts   |   152
Ceiling Lifts   |   152
What about Changes and Renovations to the Home?   |   152
Can I Still Drive?   |   153

13. Managing ALS Symptoms   |   155

How Can I Manage My Saliva?   |   155
How Can I Manage Thick Phlegm?   |   158
What Can I Do about Muscle Cramps?   |   158
How Can I Stop Muscle Twitches (Fasciculations)?   |   160
How Can I Reduce Leg Stiffness?   |   162
How Can I Manage Sudden Urges to Urinate?   |   165
How Do I Manage Constipation?   |   166
What Are Anticholinergic Side Effects of Medications?   |   168
How Do I Manage Pain?   |   169
How Can I Treat Depression?   |   171
How Can I Treat Anxiety?   |   173
How Can I Get Better Sleep?   |   173
What If I Can’t Swallow Pills?   |   175

14. The Caregiver and ALS   |   179

Who Provides Care?   |   179
How Does Providing Care Change with
ALS Progression?   |   180
What Is the Best Way to Offer Help?   |   181
How Do Patients And Caregivers Manage?   |   182
xvi

Contents x v ii

How Do Caregivers Manage Changes in Roles?   |   183

What Can Caregivers Do to Ease Their Care Burden?   |   183
Does Providing Care Affect Caregivers’ Health?   |   184
What Do Caregivers Really Feel?   |   185
Is Depression Common among Caregivers?   |   185
How Can a Caregiver Manage Stress?   |   185
What Are Respites?   |   186
What Is Bereavement While the Patient Is Alive?   |   187
Can a Caregiver Have Survivor’s Guilt?   |   187
How Should the Caregiver Prepare for Being Alone?   |   187

15. The End Stages of ALS   |   189

How Do People with ALS Die?   |   189
What If I Want to Continue Living?   |   191
What If I Can’t Make Up My Mind about Ventilation?   |   192
What If I Don’t Want to Continue Living?   |   193
Do Patients with ALS Take Their Own Lives?   |   193
What Are Palliative and Hospice Care?   |   194
When Should Hospice Care Be Considered?   |   195
How Can Spirituality Help?   |   196

16. Planning Ahead   |   197

Who Should I Tell?   |   197
How and What Should I Tell My Family?   |   198
What Should I Tell My Children?   |   198
What Are Medical Directives?   |   199
Living Will   |   200
Power of Attorney for Healthcare   |   200
Do-​Not-​Resuscitate Order   |   200
xvi

x v iii Contents

What Do I Need to Know about Estate Planning?   |   201

Will   |   202
Trust Agreement   |   202
Power Of Attorney for Financial Affairs   |   203
Lifetime Gift   |   203
What about Computer Passwords and Safety Deposit
Boxes?   |   204
What about Health Insurance?   |   204
Medicare   |   204
Military Veteran Benefits   |   205
Should I Keep Working?   |   206
What Is Family and Medical Leave?   |   206
What Are Other Financial Considerations?   |   207

17. Research in ALS   |   209

What Is Going On in ALS Research?   |   209
How Are Drugs Discovered and Tested?   |   210
Where Can I Get More Information about Trials?   |   212
Should I Participate in a Trial?   |   212
What Is Informed Consent?   |   213
Let Me Try It—​What Do I Have to Lose?   |   213

18. Concluding Thoughts   |   215

Neurologist’s Perspective   |   215
Caregiver’s Perspective   |   217

G L O S S A R Y     |     2 1 9
ABOU T T HE AMERIC AN AC ADEMY OF NEUROLOGY AND
A M E R I C A N B R A I N F O U N D AT I O N     |     2 3 1
I N D E X     |     2 3 3
xi

ABOUT THE AAN’S NEUROLOGY NOW™

BOOKS SERIES

Here is a question for you:

If you know more about your neurologic condition, will you do better
than if you know less?
Well, not simply optimism but hard data show that individuals
who are more knowledgeable about their medical conditions do have
better outcomes. So learning about your neurologic condition plays
an important role in doing the very best you can. The main pur-
pose of both the Neurology Now™ Books series and Neurology Now
magazine from the American Academy of Neurology (AAN) and the
American Brain Foundation (ABF) is to focus on the needs of peo-
ple with neurologic disorders. Our goal is to view neurologic issues
through the eyes of people with neurologic problems, in order to
understand and respond to their practical day-​to-​day needs.
So, you are probably saying, “Of course, knowledge is a good thing,
but how can it change the course of my disease?” Well, healthcare is
really a two-​way street. After you have had a stroke, you need to
find a knowledgeable and trusted neurologist; however, no phy-
sician can overcome the obstacle of working with inaccurate or
incomplete information. Your physician is working to navigate the
clues you provide in your own words combined with the clues from

xix
x

xx ABOUT NEUROLOGY NOW BOOKS

their neurologic examination, in order to arrive at an accurate diag-

nosis and respond to your individual needs. Many types of impor-
tant clues exist, such as your description of your symptoms or your
ability to identify how your neurologic condition affects your daily
activities.
Poor patient-​ physician communication inevitably results in
less-​than-​ideal outcomes. This problem is well described by the old
adage, “garbage in, garbage out.” The better you pin down and com-
municate your main problem(s), the more likely you are to walk out
of your doctor’s office with the plan that is right for you. Your neu-
rologist is the expert in your disorder, but you and your family are
the experts in “you.” Physician decision-​making is not a “one shoe
fits all” enterprise, yet when accurate, individualized information is
lacking, that’s what it becomes.
Whether you are startled by hearing a new diagnosis or you
come to this knowledge gradually, learning that you have a neu-
rologic problem is jarring. Many neurologic disorders are chronic;
you aren’t simply adjusting to something new—​you will need to
deal with this disorder for the foreseeable future. In certain ways,
life has changed. Now, there are two crucial “next steps”: the first
is finding good neurologic care for your problem, and the second
is successfully adjusting to living with your condition. This second
step depends on attaining knowledge of your condition, learning
new skills to manage the condition, and finding the flexibility and
resourcefulness to restore your quality of life. When successful, you
regain your equilibrium and restore a sense of confidence and con-
trol that is the cornerstone of well-​being.
When healthy adjustment does not occur following a new diag-
nosis, a sense of feeling out of control and overwhelmed often
persists, and no doctor’s prescription will adequately respond
to this problem. Individuals who acquire good self-​management
skills are often able to recognize and understand new symptoms
and take appropriate action. Conversely, those who are lacking
in confidence may respond to the same symptom with a growing
xxi

About Neurolog y Now Books xxi

sense of anxiety and urgency. In the first case, “watchful wait-

ing” or a call to the physician may result in resolution of the
problem. In the second case, the uncertainty and anxiety often
lead to multiple physician consultations, unnecessary new pre-
scriptions, social withdrawal, or unwarranted hospitalization.
Outcomes can be dramatically different depending on knowledge
and preparedness.
Managing a neurologic disorder is new territory, and you should
not be surprised that you need to be equipped with new informa-
tion and a new skill set to effectively manage your condition. You
will need to learn new words that describe both your symptoms and
their treatment to communicate effectively with the members of
your medical team. You will also need to learn how to gather accu-
rate information about your condition when you need it and to avoid
misinformation. Although all of your physicians document your
progress in their medical records, keeping a personal journal about
your neurologic condition will help you summarize and track all your
medical information in one place. When you bring this journal with
you as you go to see your physician, you will be able to provide more
accurate information about your history and previous treatment.
Your active and informed involvement in your care and decision-​
making results in a better quality of care and better outcomes.
Your neurologic condition is likely to pose new challenges in daily
activities, including interactions in your family, your workplace, and
your social and recreational activities. How can you best manage
your symptoms or your medication dosing schedule in the context
of your normal activities? When should you disclose your diagnosis
to others? Neurology Now Books provide you with the background
you need, including the experiences of others who have faced simi-
lar problems, to guide you through this unfamiliar terrain.
Our goal is to give you the resources you need to “take your
doctor with you” when you confront these new challenges. We
are committed to answering the questions and concerns of indi-
viduals living with neurologic disorders and their families in each
xxi

x x ii ABOUT NEUROLOGY NOW BOOKS

volume of the Neurology Now Books series. We want you to be as

prepared and confident as possible to participate with your doc-
tors in your medical care. Much care is taken to develop each book
with you in mind.
We include the most up-​ to-​
date, informative, and useful
answers to the questions that most concern you—​whether you
find yourself in the unexpected role of patient or caregiver. Real-​
life experiences of patients and families are found throughout the
text to illustrate important points. And feedback based on corre-
spondence from Neurology Now magazine readers informs topics
for new books and is integral to our quality improvement. These
features are found in all books in the Neurology Now Books series
so that you can expect the same quality and patient-​centered
approach in every volume.
I hope that you have arrived at a new understanding of why
“knowledge is empowering” when it comes to your medical care and
that Neurology Now Books will serve as an important foundation
for the new skills you need to be effective in managing a neurologic
condition.
Lisa M. Shulman, MD, FAAN
Editor-​in-​Chief, Neurology Now™ Books Series
Fellow of the American Academy of Neurology
Professor of Neurology
The Eugenia Brin Professor in Parkinson’s Disease
and Movement Disorders
The Rosalyn Newman Distinguished Scholar
in Parkinson’s Disease
Director, University of Maryland PD &
Movement Disorders Center
University of Maryland School of Medicine
xxi

FOREWORD

The past decade has seen a dramatic increase in the number of books
about amyotrophic lateral sclerosis. Is this one truly needed? Yes,
unequivocally. There is literature in which persons with ALS describe
their experiences, and other literature in which medical personnel
discuss ALS. The combined approach here is one that I am confident
will be particularly meaningful to those living with ALS and their
caregivers.
Physicians involved in the teaching and training of medical stu-
dents and residents have long known that trainees learn much more
effectively when presented with a combination of factual material
and patient encounters. This book uses that approach by providing
facts about ALS while concurrently permitting the reader to view
ALS through the eyes of those with the disease and their loved ones.
The factual sections of the book are broad-​ranging, covering
virtually all aspects of ALS pathogenesis, presentation, evaluation,
diagnosis, and management in a manner that is centered on what
patients and their caregivers want to know or should know. The
interweaving of patient and caregiver narratives throughout the
book facilitates exploration of topics that are central to living with
ALS but are rarely addressed. For example, the reader can feel the
frustration that so many individuals with ALS feel as test after test
is interpreted as “normal.” At the same time, that reader also can

x x iii
xvi

x x iv F oreword

find an explanation of the ALS evaluation that permits an under-

standing of why “normal” does not mean “no ALS.” The sections
on etiology and pathogenesis permit the authors to also tackle the
“Why me?” question with which so many affected individuals and
their caregivers struggle. I can easily imagine my patients and their
caregivers thinking, “That’s how I felt” or “That’s what happened
to me” as they read the narratives. Some sections are particularly
poignant. “Living with ALS,” for example, describes how individuals
with ALS may change their perspectives on what is important to
them for maintaining quality of life.
The authors bring together an uncommon combination of per-
spectives and skills. Dr. Mark Bromberg, a gifted and compassionate
physician whom I have known for more than 25 years, has dedicated
much of his professional career to the care of patients with ALS and
to research centered around this devastating disease. His coauthor,
Diane Banks Bromberg, has personally experienced the effects of
ALS through her role as a caregiver for her mother. Their experi-
ences frame the physician-​caregiver-​patient relationship so vital to
those who face this disease’s myriad physical, psychological, social,
and existential consequences.
As a physician who has spent the past 25 years in ALS clinical
care and research, I believe that Mark and Diane have done a tre-
mendous service for our patients and their caregivers by produc-
ing this remarkable book, which is consistently informative and at
various times inspiring, touching, heart-​rending, and heartwarm-
ing. Mark states in the book’s preface, “I try to put myself in the
positions of the patient and caregiver.” It is clear that he and Diane
have written this book from that perspective, and in doing so have
provided a much-​needed resource to the ALS community.
Zachary Simmons, MD, FAAN
Fellow of the American Academy of Neurology
Professor of Neurology and Humanities
The Pennsylvania State University
Director, ALS Center
Penn State Hershey Medical Center
xv

PREFACE

This is a book about ALS for patients with ALS, their caregivers, and
other family members. ALS is perhaps the most challenging disease
imaginable: researchers do not know what causes it or how to pre-
vent it, no markedly effective treatments are available, and the dis-
ease is inexorably progressive. Not only does it affect the patient,
but it has a life-​changing impact on the primary caregiver and other
members of the family.
I began working with patients with ALS early in my neurol-
ogy career, and it has been my main clinical and research empha-
sis over the past 30 years. I established a special ALS clinic at the
University of Michigan and then later established a second clinic
at the University of Utah. I have participated in research efforts on
using new techniques to make the diagnosis of ALS and am particu-
larly interested in issues related to quality of life for people with the
disease. I have also participated in many clinical drug trials related
to ALS in the hope of better understanding this fast-​paced disorder
and perhaps, one day, how to prevent it or slow its rate of progres-
sion. With every diagnosis of ALS that I make or confirm, and with
every clinic visit, I try to put myself in the positions of the patient
and caregiver. While this effort can only provide approximations of
what the patient and caregiver experience, I hope it helps me see

xxv
xvi

xxvi P reface

their issues and answer their questions in a realistic way. This book
reflects what I would want to know and how I would want to be
treated if I had ALS.
To further meet the needs of the patient, caregiver, and their
family, I asked my coauthor, who is also my wife, to help me tell this
story. We met when I treated her mother for ALS. Because she has
worn the shoes of the caregiver, she brings a unique perspective on
patient care and the patient viewpoint. That perspective has added
a new dimension to my view of clinical experiences. We hope our
unique combined voice will provide helpful guidance.
Mark B. Bromberg, MD, PhD
xxvi

ACKNOWLEDGMENTS

I would like to express my thanks first to the patients whose ques-

tions I have endeavored to answer in this book. In particular I want
to thank Creighton Rider, who has ALS, and his wife, Lisa, who
provides his care, for reviewing the manuscript and providing valu-
able suggestions. I also want to acknowledge and thank my mul-
tidisciplinary colleagues who have guided the answers to many of
the questions, including nurses (Dallas Forshew, Barbara Miano,
Bernadette Talon, Mary Jensen, Cassie Kuhn, Abby Smart), a dieti-
tian (Kari Lane), speech-​language pathologists (Michelle Taggart,
Kiera Berggren, Pamela Mathy), physical therapists (Nancy Ivy,
Heather Hayes), occupational therapists (Kasey Mitchell, Jenny
Ng), respiratory therapists (Natalie Bee, Aubrey Perman Isaacs), a
genetic counselor (Karin Dent), social workers (Sandra Iaderosa,
Kathy Day, Eva Tukuafu), clinic pharmacologists (Orly Vardeny,
Kristen Jefferies, Patricia Jerant, Sarah Dehoney), a pulmonologist
(Estelle Harris), a gastroenterologist (John Fang), and wheelchair
seating specialists (Ron Whiting, Travis Carlson, Scott Ingraham).
I am grateful to Craig Panner, editor at Oxford University Press, for
asking us to write the book and helping with the editorial process.
Mark B. Bromberg, MD, PhD, FAAN

x x v ii
xxivi

xxviii A cknowledgments

My contributions to this book reflect my personal rather than pro-

fessional expertise, experiences, and views. I was very close to my
parents throughout the course of my mother’s illness, and I appreci-
ate the opportunity to coauthor and add the caregiver perspective to
this book. I have tried to supplement the medical information with
the patient and caregiver points of view. I found that my approach
to dealing with ALS was most often more practical than the phy-
sician’s approach. Although mindful of the medical situation, my
focus was usually on the emotional or day-​to-​day difficulties rather
than strictly medical concerns. I believe the former aspects are as
important to both patient and family as the medical consensus, in
different ways. I also recognize that each patient and family situa-
tion is different, and that the practical impact on end-​of-​life treat-
ments and financial issues will vary dramatically for each person.
I thank my law partner, Jennifer Decker, for her input into the
estate planning information we have provided.
I would also like to thank my parents. It is sometimes difficult
to allow others to step in and help when a medical situation arises,
but my parents not only welcomed the help but allowed me to share
their fears, challenges, and frustrations during the course of my
mother’s illness. I hope that sharing the insights I gathered during
that time will help patients, caregivers, and family members who
are now dealing with ALS.
Diane Banks Bromberg, JD
Another random document with
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somewhere we made a mistake, there was something we failed to
see. I'd like to know what made him jump."
This time the American looked out to sea. He was silent.
The doctor took out an old briar pipe and began filling it from a
leather pouch. "Strange. His radio beacon is functioning normally.
There's no reason why his transmitter and receiver shouldn't be
working too. Yet we've been trying to contact him by means of voice
communication, and he doesn't answer. Maybe he's dead already.
There's no way to tell."
"Do you think he's worth saving?" the pilot asked after a minute.
"I'd like to know why he jumped."

In the briefing room, the American listened intently to the sounds

coming from the speaker. Dr. Valdez and the other members of the
Prospero's crew also listened. Dr. Valdez listened with his eyes
closed, drawing slowly on his pipe.
"Orbital ship Wabash Cannonball acknowledging Azores
transmission," the voice said. "Our condition is still AOK, repeat,
condition is still normal. We are still tracking survival beacon. Range,
10,000 kilometers and closing." There was another burst of radio
noise that momentarily drowned out the voice. The men in the
briefing room had been listening for nearly six hours now.
Occasionally one of them would go out for coffee or fresh air, but he
always returned within a few minutes. The American pilot had not
moved from his place since lift-off. Outside, it had begun to rain.
At last, the critical moment came.
"Range is now five hundred kilometers and closing," the voice said.
"I now have a visual sight. Repeat. I have a visual sight. I can see
him. Switching from computer to manual control." Several minutes of
silence. The pilot was jockeying closer to Duport, making delicate
adjustments in his ship's orbital path. He had a small target. A single
wrong judgement could cause him to drift hundreds of kilometers off
course, wasting a critical amount of fuel.
At last the report came, "Range is now five hundred meters. We are
suiting up and blowing cabin pressure. Stand by for further
transmission." Ten minutes passed. The crew was too busy to
broadcast now. The rain drummed softly on the roof of the briefing
room and ran in slow curtains down the windowpanes.
Finally the voice came on the air again.
"Orbital ship Wabash Cannonball resuming transmission. Rescue
operation is successful. Repeat, operation is successful. We have
him aboard. He's alive."
The American pilot looked up at the faces around him. Dr. Valdez
was rubbing his mouth thoughtfully. The other men stared at the
speaker with blank looks. The American noted that no one was
cheering.

Later, the pilot of the Cannonball described the rescue. When he had
first reported his visual sighting, he had been seeing the sunlight
reflected from the surface of Duport's suit. Duport was a white spark,
shining out among the stars like a meteor or nova. The sight had
given the rescue pilot a peculiar feeling, he mentioned later, seeing
this blue-white star slowly growing in the sky until it was brighter than
Venus, seeing this new star rise, a point of white fire, and knowing
the star was a man.
Then they had suited up and blown the cabin pressure. The co-pilot
had gone out the hatch while the pilot remained at his controls.
Watching through the periscope, he could see Duport spread-eagled
against the sky, the left side of his body a glare of sunlight, the right
side in shadow. Duport had not moved his arms or legs since they
had first seen him, neither did he acknowledge with his suit
transmitter. He was about five hundred meters from the ship and
drifting slowly closer. The co-pilot tethered himself to the hull, then
tossed out a line with a magnetic grapple on its end. He missed,
hauled in, and tossed again. On the third try the end of the line
passed within half a meter of Duport's body. Duport moved his arm,
took the end of the line, and hooked it to his belt. The co-pilot hauled
him in.

About a month later, the American pilot saw Rene Duport for the first
time since he had jumped from the Prospero. It was at the space
medicine laboratories at Walter Reed.
Dr. Valdez stood near the window, looking down at the sunlit lawn. In
the shade of a tall shrub a man was sitting in a lawn chair, his head
back, completely relaxed. He wore a blue denim hospital uniform.
His back was to the window.
"Physically he was in good condition when they brought him down,"
the doctor said, "except for a slight case of dehydration."
"Can I talk to him?" the pilot asked.
Dr. Valdez looked at him sharply, as if surprised by the request.
"You can talk to him if you like. But he won't answer you."
The pilot followed the doctor out of the room and down to the lawn.
They came up from behind the lawn chair and stood looking down at
the man sitting in it. His eyes were closed.
The pilot saw that Duport's jaw was slack. He could not tell whether
he was asleep. The flesh in his cheeks was sunken. He looked older.
Dr. Valdez said, "Catatonia, schizophrenia, it's like no condition I've
ever seen before. He is perfectly aware of what is going on around
him, you see. Bring him food and he eats. Stick him with a pin and
he jumps. All his responses are normal. He took the cable and
attached it himself, remember. But he will make no more than the
minimum necessary effort to survive." The doctor chewed his lip,
thinking. "If only he would say something."
"Have you decided why he jumped?" the pilot asked, not realizing
that he was whispering. "What made him panic?"
"No." The doctor shook his head. "Not panic, it wasn't fear alone, I
think. There was something else. We put him through equally critical
moments in training, and he didn't panic then. Fear was part of it, but
there was something else too."
"Well, what then?"
"I don't know the word. It's something new. Maybe Duport is a new
kind of human being. If not fear, call it—love, or desire. He jumped
into space because, I think, he wanted to."
"I don't understand that," the pilot said.
"I don't either—yet." Dr. Valdez moved a step closer to the man in
the chair. "Rene. Rene Duport."
Without moving his head, Duport opened his eyes.
"Stand up."
Duport got up and stood looking at some point half way between the
two men. His eyes no longer glistened.
"It's as if something has gone out of him," the doctor said.
"Do you know who I am?" the pilot asked. Rene Duport turned his
head until the pupils of his eyes were pointed at the American's face.
But his eyes did not seem to focus on him. Rather they were focused
at some point far beyond him.
"Why did you jump?" the pilot said. Moving a step closer, he looked
into the blank, dull eyes, that continued looking through him, focused
on some strange horizon. The eyes no longer seemed blue, but light
grey. The pilot tried to remember where he had seen eyes like that
before. Then he remembered one day, years before, when he had
looked down into the open eyes of a dead man. He shuddered and
turned away.
"If only he would talk," the doctor said.
The pilot had turned his back on Duport. "Why? If he could talk, what
would you ask him?"
It was two or three minutes before the doctor answered.
"I would ask him what it feels like to be a star."
And as the two men walked away, Rene Duport remained standing
where they left him. He was watching. The pupils of his eyes never
shifted, but he was always watching. The Earth, a swollen balloon,
floated past his field of vision. Slowly his right arm rose until his arm
was horizontal from his shoulder. Then the corners of his mouth lifted
in a faint smile, as his fingers touched the Clouds of Magellan.
THE END
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