Myasthenia Gravis A Manual for the Health Care Provider James F. Howard, Jr., M.D., Editor Table of Contents Chapter 1 - Historical Notes Chapter 2 - Physician Issues James F. Howard, Jr. Chapter 3 - Nursing Issues Tina M. Vassar, Madeleine Batenjany, Wilma J. Koopman, Marilyn M. Ricci Chapter 4 - Anesthesia Issues Brian Barrick, and Robert Kyle Chapter 5 - Emergency Care Issues H. Alexander Krob Chapter 6 - Psychosocial Issues: From Diagnosis to Lifetime Management Kimberly M. Johnson Chapter 7 - Physical Therapy Issues Michael Thomas Chapter 8 - Occupational Therapy Issues Timothy Holmes Chapter 9 - Speech Pathology and Swallowing Issues Susan G. Butler Chapter 10 - Dental Care Issues Loren L. Patton Chapter 11 - Guidelines for the Pharmacist James F. Howard Chapter 12 - Myasthenia Gravis Foundation of America Chapter 13 - Appendices 2 Myasthenia Gravis Foundation of America Readers are advised to check the most current information 355 Lexington Ave, 15th Floor available. It is the responsibility of the health care provider, New York, NY 10017 relying on their own knowledge and experience to make diag- noses, determine appropriate treatment, doses and schedules Myasthenia Gravis: A Manual for the Health Care Provider and overall best treatment plan for the pateint. ISBN: 0981888305 To the fullest extent of the law, neither the Publisher, Editor Copyright © 2009 by or Authors assume any liablility for any injury and or damages Myasthenia Gravis Foundation of America to persons or property arising out or related to any use of the All rights reserved. No part of this publication may be repro- material contained in this book. duced or transmitted in any form or by any means, electronic The Publisher or mechanical, including photocopying, recording, or any in- formation storage and retrieval system, without permission in Library of Congress Cataloging-in-Publication Data writing from the publisher other than photocopies of single chapters for personal use as allowed by national copyright Library of Congress Control Number: 2008932503 laws. Myasthenia Gravis: A Manual for the Health Care Provider, Permissions may be sought directly from the Myasthenia edited by James F. Howard, Jr. Gravis Foundation of America, 355 Lexington Ave, 15th Floor New York, NY 10017, USA: phone: (212) 297-2156, 1st ed. email: [email protected]. Cover photo, page 28 by: Mario Beaurgard / Stock Connection Includes biographical references and index / Jupiter Images ISBN: 0981888305 Notice 1. Neurology 2. Nervous system - Diseases 3. Myasthenia Gra- Knowledge and best practice in the field of Myasthenia Gravis vis I. Howard, J. F. (James Francis) Printed in USA is constantly changing as new research and experience broad- ens the knowledge base. Changes in practice, treatment and therapy may be necessary or appropriate. Preface 3 Chronic Condition Program – Specialty Groups Acknowledgments Blue Cross Blue Shield of Michigan This book is the product of the efforts of many people. First Susan G. Butler, Ph.D., CCC-SLP and foremost we thank the MG community who has taught us Associate Professor about Myasthenia Gravis. Their stories and insights into the Department of Otolaryngology problems faced by myasthenics helped shape our understand- Wake Forest University Baptist Medical Center ing of this disorder. Winston-Salem, NC MGFA wishes to express its gratitude and thanks to the Wis- Timothy Holmes, OTR/L, COMS consin Chapter of the Myasthenia Gravis Foundation of Amer- Department of Physical and Occupational Therapy ica, Janice Edelman-Lee at Chodoy Design, and Ron McFar- UNC Healthcare lane at MedPro Rx, and all of those whose generosity and sup- The University of North Carolina Hospitals port have allowed this project come to fruition. Your commit- Chapel Hill, NC ment was the driving force to develop the best possible re- source for community health care providers. James F. Howard, Jr., M.D. Distinguished Professor of Neuromuscular Disease List of Contributors Professor of Neurology & Medicine Chief, Neuromuscular Disorders Section Brian P. Barrick, D.D.S., M.D. Department of Neurology Assistant Professor The University of North Carolina at Chapel Hill Department of Anesthesiology School of Medicine The University of North Carolina at Chapel Hill Chapel Hill, NC School of Medicine Kimberly M. Johnson, M.S.W., L.C.S.W., C.C.M. Chapel Hill, NC Department of Clinical Care Management Madeleine Batenjany, R.N., M.S.N., ANP-C UNC Healthcare Case Manager The University of North Carolina Hospitals Premium Blue Health Connection Chapel Hill, NC Preface 4 Wilma J. Koopman, R.N., B.Sc.N., M.Sc.N., T.C.N.P. Marilyn M. Ricci, R.N., M.S., CNS, CNRN C.N.N.(C) Adjunct Faculty, Neuroscience Clinical Nurse Specialist Advanced Practice Nurse Learning Institute London Health Sciences Centre St. Joseph’s Hospital & Medical Center Assistant Professor and Lecturer School of Nursing Faculty of Phoenix, Arizona Health Sciences Michael Thomas, L.P.T. University of Western Ontario Department of Physical and Occupational Therapy London, CANADA WakeMed Health & Hospitals H. Alexander Krob, M.D. Raleigh, NC Instructor and Fellow in Neuromuscular Disease Tina M. Vassar, R.N. Department of Neurology Chapel Hill, NC The University of North Carolina at Chapel Hill School of Medicine Chapel Hill, NC Preface Robert W. Kyle, D.O. The mission statement of the Myasthenia Gravis Foundation Assistant Professor of America (MGFA) is to facilitate the timely diagnosis and op- Department of Anesthesiology timal care of individuals affected by myasthenia gravis (MG) The University of North Carolina at Chapel Hill and closely related disorders and to improve their lives School of Medicine through programs of patient services, public information, Chapel Hill, NC medical research, professional education, advocacy and pa- tient care. Since, at present, there is no cure, there must be a Lauren L. Patton, D.D.S. consistent, interdisciplinary effort by all allied health profes- Professor sionals to appropriately evaluate and treat patients with MG. Director, GPR Residency Program Department of Dental Ecology This handbook is written as an aide to all healthcare person- School of Dentistry nel who are involved in the care and management of patients The University of North Carolina at Chapel Hill with myasthenia gravis. Sections are included to assist the phy- Chapel Hill, NC sician community in their evaluation, diagnosis and manage- Preface 5 ment of MG. Every effort has been made to outline the varying and will be able to supply the physician with more detailed in- opinion of the experts and it is recognized that more than one formation about current treatment efficacy. approach may be appropriate. The over riding principle that The pharmacist plays an important role in the safety and edu- patients with MG are not in a vacuum and must be evaluated cation of the myasthenic patient. Today’s healthcare system and treated based on their entire health care picture is neces- often depends upon the specialist who has little knowledge of sary. The perspective of an anesthesiologist and dentist are other disciplines. The ever growing list of drug-drug interac- also included because their interaction with the myasthenic tions that may be harmful to the patient places the pharmacist patient is unique and special considerations are necessary. in a central position to be the watchdog and keep the patient Nursing plays a critical role in the day-to-day management of out of harms way. the hospitalized and community patient. A team approach to This coordinated, interdisciplinary approach will promote in- the patient will inevitably result in the optimal care of the my- creased quality of life for those affected by myasthenia gravis. asthenic patient. The following guidelines are a culmination of over 35 years of The patient with a chronic illness faces numerous obstacles in clinical practice in university-based teaching hospitals. It is their every day life. The clinical social worker is invaluable to the authors’ hope that these guidelines will challenge all allied the patient (and the healthcare team) in navigating the bu- health clinicians who work with patients diagnosed with myas- reaucracy and hurdles as it pertains to insurance, financial as- thenia gravis to critically evaluate their current practice with sistance and psychosocial well being. respect to these patients. We submit that incorporation of The allied health evaluations should be objective and quantita- these recommendations into the evaluation and treatment rep- tive for each individual patient and should assist the physician ertoire of allied health professionals will enhance the quality in diagnosis, classification and effectiveness of medical treat- of care provided to people who have myasthenia gravis. ment. These evaluations should enable the allied health profes- sionals to determine a functional baseline and an appropriate exercise prescription. The patient will then be able to utilize a program of moderate-intensity exercise to improve his/her level of fitness, which will diminish the effect of exacerbations. Through the use of self-monitored exercise, each patient will have an improved sense of his/her own functional limitations Preface 6 1 Historical Notes James F. Howard, Jr. The unraveling of the molecular mechanisms of Myasthenia Gravis (MG) over the last three centuries has resulted in what we know today about the pathogenesis of MG and the rationale for its present and future treatments. The first descriptions of MG cases occurred more than 300 years ago, yet it was not until a series of discoveries in the early and mid 1970s that an understanding of and the general consensus that MG symptoms are due to an autoimmune response to the acetylcholine receptor (AChR) complex on the post-junctional membrane of the neuromuscular junction The first two cases of a disease that was likely to be MG were and died in 1664. Marsteller concluded that Opechankanough described several centuries ago; one on the American conti- developed MG late in life when he was between 70 and 90 nent and one in Europe.