Amyotrophic Lateral Sclerosis (American Academy of Neurology)

257 Pages·2004·1.36 MB·English

by  Robert G. Miller

Amyotrophic
Lateral Sclerosis
Robert G.Miller,MD
Deborah Gelinas,MD
Patricia O’Connor,RN
New York
Demos Medical Publishing, 386 Park Avenue South, New York, NY 10016
www.demosmedpub.com
© 2005 by Demos Medical Publishing. All rights reserved. This book is protected by
copyright. No part of it may be reproduced, stored in a retrieval system, or transmit-
ted in any form or by any means, electronic, mechanical, photocopying, recording, or
otherwise, without the prior written permission of the Publisher.
Library of Congress Cataloging-in-Publication Data
Miller, Robert G. (Robert Gordon), 1942–
Amyotrophic lateral sclerosis / Robert G.Miller, Deborah Gelinas, Patricia
O’Connor.
p. cm.
Includes index.
ISBN 1-932603-06-9 (pbk. : alk. paper)
1. Amyotrophic lateral sclerosis—Popular works. I. Gelinas, Deborah F.
(Deborah Faith), 1956– II. O’Connor, Patricia, 1954– III. Title.
RC406.A24M556 2005
616.8'3—dc22
2004018921
Contents
Foreword . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . v
Lewis P. Rowland, MD
Acknowledgments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vi
Preface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . vii
Authors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ix
Section 1:Introduction
1. What is Amyotrophic Lateral Sclerosis? . . . . . . . . . . . . . . . . . . . . . 3
2. The Diagnosis of ALS. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15
3. Clinical Trials and Finding New Drugs for ALS. . . . . . . . . . . . . . 33
Section 2:Managing the Symptom of ALS
4. A Multidisciplinary Approach to Care . . . . . . . . . . . . . . . . . . . . . 67
5. Quality of Life and Psychosocial Issues. . . . . . . . . . . . . . . . . . . . . 81
6. Treating the Symptoms of ALS. . . . . . . . . . . . . . . . . . . . . . . . . . . 91
7. Nutrition and Swallowing. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103
Alycia Chu, MS, RD
8. Speech, Communication, and Computer Access. . . . . . . . . . . . . 119
Amy Roman, MS, CCC-SLP
9. Staying Mobile . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137
10. Breathing and Sleeping . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 149
iii
Contents
11. Thinking and Behavioral Changes . . . . . . . . . . . . . . . . . . . . . . . 157
Susan Woolley Levine, PhD
12. Palliative Care. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 167
Section 3:Resources
13. Finding a Cure and Improving Living with ALS:
How the ALS Association Helps Patients and Families. . . . . . . . 179
Mary Lyon
14. The Muscular Dystrophy Association (MDA), ALS Division . . . 197
Ronald J. Schenkenberger
15. Negotiating the Insurance Maze. . . . . . . . . . . . . . . . . . . . . . . . . 211
With Dorothy E. Northrop, MSW, ACSW
Section 4:Appendix
Resources . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231
Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 235
iv
Foreword
This book is one of the first in a series sponsored by the American Academy
of Neurology designed to assist people with neurologic diseases and their
families. This guide to amyotrophic lateral sclerosis, also known as ALS or
Lou Gehrig’s disease, appears at a propitious time for those confronted with
newly diagnosed ALS, a particularly heart-rending disorder.
Hopes for prevention or cure have been raised by the accelerating
pace of research in the past decade, starting with the discovery of a gene
for familial ALS in 1993, followed within a year by the creation of an ani-
mal model in mice. We have gained more information about the cellular
abnormalities in ALS from these mice than we did during the preceding
100 years. More scientists are working on the disease now than ever
before. We know that before long, a cure or mode of prevention will pro-
tect us from this scourge. But we do not know how long it will take before
that goal will be achieved, and patients need assistance now, not later.
The authors have wisely included informative chapters about the dis-
ease itself, specific symptoms and how they can be ameliorated, how mul-
tidisciplinary centers work, how voluntary health agencies help, how com-
puters can help, how the Internet can be used, and even how to deal with
the helter-skelter system of health insurance companies. These topics can
guide patients to the best possible arrangement to meet their needs. The
authors are all experts in the management of ALS, and this book will be
much appreciated by patients and professionals alike.
Lewis P. Rowland, MD
Neurological Institute
Columbia University Medical Center
New York, NY
v
Acknowledgments
We wish to thank our patients and their families, whose courage in
battling ALS inspired us to write this book.
We also wish to acknowledge the following members of the extraor-
dinary multidisciplinary team of ALS specialists at the Forbes Norris
MDA/ALS Research Center in San Francisco: Jodi Bales, Roberta
Greenberg, Felicia Harte, Catherine Madison, Jason Mass, Sandy
McDade, and Margie Petrakis. Each member of this team brings excep-
tional commitment, devotion, and clinical excellence to the care of our
patients, and they have taught us much.
We are grateful to our own families who supported us during the
writing of this book and were patient with us during our time taken away
from family.
We wish to thank Ann Haugh for her considerable help with the
graphic design of the figures and with the selection of the book cover, as
well as help with the completion of the manuscripts. We also wish to thank
Lois Esteban for her substantial help in completing the manuscripts.
We are most grateful for the superb chapters written by our contrib-
utors.
We wish to acknowledge the expertise and help of Dr. Diana M.
Schneider, President of Demos Medical Publishing, for her review of the
entire manuscript and support in producing this book.
We wish to acknowledge the tremendous contribution of Dr. Forbes
Norris and Dee Holden Norris, RN, who worked tirelessly to raise the
standard of care for patients with ALS. They too have inspired us to write
this book.
vi
Preface
If you are reading this book, we assume that you or a close family mem-
ber or friend has been diagnosed with ALS, or that the diagnosis is under
consideration. We have written this book to help you understand the dis-
ease and what can be done to manage it effectively.
ALS is a treatable disease, although it is far from being curable. We
now have treatments that can make a major difference in enhancing the
quality of life and prolonging life for those with this diagnosis. In addition,
we have treatments for many of the symptoms of ALS that can help ease
the burden of the disease. We have begun to use evidence-based medicine
to select state-of-the-art management strategies for each and every patient
with ALS. Multidisciplinary teams in specialized ALS centers provide top
quality care and comprehensive rehabilitation for persons with ALS. The
impact of various treatments for patients with ALS is now being measured
in a continuing way using a North American patient database known as
the ALS CARE program. ALS CARE is one of the first databases to track
the outcome of this neurologic disease, thereby improving the standard of
care for persons with ALS.
In spite of the progressive nature of ALS and its clear tendency to
shorten life, there are a number of reasons for cautious optimism. One
drug has been approved by regulatory agencies to slow the disease, albeit
to a modest degree. As mentioned above, numerous other treatments are
now available to help manage symptoms. The momentum of ALS research
is expanding dramatically. Three new genes have been discovered in the
last three years and numerous clinical trials are underway, testing promis-
ing new therapies. Both gene therapy and stem cell treatment have shown
extremely promising results in animal models, and clinical trials in humans
will hopefully start in the not too distant future. Our understanding of the
vii
Preface
basic causes of ALS is expanding gradually. The substantial resources of
patient advocacy groups, such as the Muscular Dystrophy Association
(MDA) and the Amyotrophic Lateral Sclerosis Association (ALSA), pro-
vide tremendous help and support for patients and families.
In spite of the adjustment required after learning that they have ALS,
most patients tell us that life goes on. In fact, the vast majority of patients
begin living life again fully and with joy and courage after working
through the initial transition and adjustment to the diagnosis. Many of our
patients become extraordinary heroes and discover new courage from
within to battle the disease and live life with vigor and enthusiasm. It is our
sincere hope that the information in this book will prove useful to you in
proactively managing your ALS.
The book begins with a discussion of the nature of the disease process
in ALS, how symptoms develop, how the disease is diagnosed, and the
therapies available to slow its progress. We then discuss treating the symp-
toms of ALS and the benefits of receiving care in a specialized ALS center,
emphasizing the importance of preserving quality of life and addressing
family and social issues. Chapters on nutrition, communication, getting
around and staying active, sleeping and breathing issues, and the changes
in personality and thinking that affect some people with ALS will help
readers to better cope with these aspects of the disease process. A consid-
eration of end-of-life issues emphasizes approaching the advanced stages
of ALS with dignity. Finally, a summary of helpful resources is provided to
help ease the burden of the disease.
Robert G. Miller, MD
Deborah Gelinas, MD
Patricia O’Connor, RN
viii
Authors
Robert G. Miller, M.D.
Director
Forbes Norris MDA/ALS Research Center
Chairman, Dept. of Neurosciences
California Pacific Medical Center
San Francisco, California
Deborah F. Gelinas, MD
Clinical Director
Forbes Norris MDA/ALS Research Center
California Pacific Medical Center
San Francisco, California
Patricia O’Connor, RN
Nurse Case Manager
Forbes Norris MDA/ALS Research Center
California Pacific Medical Center
San Francisco, California
With contributions by:
Alycia Chu, MS, RD
Nutritionist
Forbes Norris MDA/ALS Research Center
California Pacific Medical Center
San Francisco, California
ix