A First Step - Understanding Guillain-Barre Syndrome

A

First

Step

Understanding

Guillain-Barre

Syndrome

Brian S. Langton

A First Step: Understanding Guillain-Barré Syndrome

Author: Brian S. Langton

Copyright © 2002 Brian S. Langton. All rights reserved.

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Cover and interior design and production by Pneuma Books.

Set in Minister Light 11/14.

Disclaimer:

The Author is not a medical professional. All opinions expressed are his alone, unless specifically attributed to others. No liability will be accepted for persons acting in any manner whatsoever based on information contained in this publication.

National Library of Canada Cataloguing in Publication

Langton, Brian S., 1928-

A first step : understanding Guillain-Barré syndrome / Brian S. Langton.

ISBN 978-1-5536-9411-3 (softcover)

ISBN 978-1-4122-4710-8 (ebook)

1. Langton, Brian S., 1928-—Health. 2. Guillain-Barré syndrome—Patients—Biography. I. Title.

RC416.L35 2002      362.1’9687      C2002-902256-8

10   9   8   7   6   5   4   3

Contents

Acknowledgements

Foreword

Author’s Preface

Part I What Really Happened?

1 A Rare Disease Hits

2 What Is This Disorder?

3 What Causes Guillain-Barré Syndrome?

4 Destination: Intensive Care

5 New Quarters

6 As Bad As It Gets

7 Patients and Their Televisions

8 The Art & Importance of Communicating

9 Communication Journal Started

10 Humour Has Its Place

11 Learning to Talk Again

12 Encouragement from an Unexpected Source

13 Some Reflections on Life in Intensive Care

14 Approaching Yuletide

15 Better Days

16 Breathing on My Own

17 Light at the End of the Tunnel

18 Another Big Step Forward

19 GBS Patients Are Neither Quadriplegics Nor Brain Damaged

20 A First Visit Home

21 Some Reflections on Life in the Rehabilitation Ward

22 Was Guillain-Barré the Best Name for This Syndrome?

23 Changes Needed

24 The First Steps

25 Homecoming

26 Starting to Live Again

Part II In Dreams...

Intro Dream Sequences

27 GBS Patient on a Secret Mission

28 Who Wants to Buy a Double-Decker Bus?

29 Submariner in Trouble

30 Guardian Angel in a Stetson

31 Sheriff’s Office

32 Ottawa Summers

33 Quicksand Floor

34 Precious Stone

35 Underground Canal

36 Heavy Snowfall Warning

37 Downtown Adventures

38 Part Time Patient

39 Daylight Robbery

40 Neck Brace

41 The Debt Collector

42 Flying Bombs

43 Across the Great Lake

44 A Game of Chicken

45 Jet Fighters On Call

46 Wild Dogs Attack

47 Catastrophe

Part III Epilogue

Epilogue Leaving the Dream World Behind

Part IV Appendices

Appendix 1 Endnotes

Appendix 2 Support Resources

Appendix 3 Internet Information Resources

Appendix 4 Reading List

Appendix 5 Guillain & Barré: The Namesakes

Appendix 6 About the Author

This book is dedicated to my wife, Sylvia, and our

daughters Ruth, Kim, and Sally.

List of Photographs

Photographs from Part I

Outside with Physiotherapists and Caregivers

Situated in the Car

Outside With a Caregiver

Two Occupational Therapists

A Physiotherapist

Outside with a Caregiver

Photographs from Part II

F-16 on Runway

Cockpit Check

Taking Off

View from Cockpit

Acknowledgements

"Friends are angels who lift us when our wings

have forgotten how to fly." (Anonymous)

Grateful thanks to my family, especially my wife, Sylvia, who stood steadfastly by my side and throughout many emotionally charged days and months maintained the journal that has provided the basis for this book. We were an incredible team, working our way through a most unexpected life crisis. A big thank-you, also, to our friends and relatives who rallied to support Sylvia and me at a critical time.

I also want to acknowledge and thank the following:

The doctors, nurses, and staff of the Intensive Care Unit at Foothills Hospital, Calgary, Alberta, Canada, for my early and accurate diagnosis and twelve days of thorough care and attention before being transferred to Rockyview;

The doctors, nurses, and staff of the Intensive Care Unit at Rockyview General Hospital, Calgary, who for seven months provided phenomenal care and were part of my extended family. I thank them all, as well as the doctors, nurses, and staff of the rehabilitation team of Unit 58 at the Foothills Medical Centre, Calgary, Alberta, who put up with me for almost four months as an in-patient;

Nancy, Elizabeth, Brenda, Julianne, Marlon, and so many others at Foothills for their encouragement and assistance;

Rosemary, my community physiotherapist, to whom I could always turn for advice.

Yi-nei and Kelly, who specialized in hand therapy at Rockyview General Hospital;

Emma and Katherine at the University of Calgary Rehabilitation and Fitness Program;

My dentist friend, Dr. Don Wallace, B.A., D.M.D., who not only took time out to visit, but also made ‘house calls’ to the hospital to provide needed professional care;

My business associates and colleagues for their giant-sized get well card containing over one hundred signatures and personal messages and for their concerns and assistance in so many very practical ways;

The players and staff of the then, and I hope soon to be again, English Premiere Division club, Nottingham Forest, for their good wishes and words of support;

My long-term caregivers, Cindy and Asunta for staying the course with their encouragement and caring support; and to Tara for her arm, which supported my first steps free of a walker;

Giselle LaBrie, for her care, encouragement and invaluable assistance in the planning and writing of this book at a time when ‘my wings had forgotten how to fly’;

Dr. Frank Warshawski MD, FRCPC, Director of Intensive Care, Rockyview General Hospital, Division of Respiratory Medicine and Department of Critical Care, Calgary Health Region, University of Calgary for his valuable support, advice, and encouragement and for his important contribution in writing the Doctor’s Foreword;

Dr. Joel S. Steinberg, MD, PhD, Vice President and Director of the Guillain-Barré Syndrome Foundation International for his encouraging remarks and permission to reproduce excerpts from GBS—An Overview for the Lay Person, written by Dr. Steinberg and published by the Guillain-Barré Syndrome Foundation International;

Dr. Kopel Burk, MD, for his good advice and kind permission to reproduce excerpts from his paper Communications and Altered Perceptions, originally published in the Journal of the Medical Society of New Jersey, New Jersey Medicine;

Luc Van Bavel, who kindly gave permission for the use of the F-16 and aerial photographs used in G.B.S. Patient on a Secret Mission;

Anne Shard, RGN, for her encouragement and kind advice;

Margot, my chest physiotherapist in Intensive Care at Rockyview Hospital, for her helpful advice and kind assistance;

Dr. J. B. Winer, MSc MD FRCP, Consultant Neurologist, University Hospital, Birmingham, England, for his kind permission to reproduce excerpts from his article, The Early Years, which appeared in the Tenth Anniversary Issue of Reaching Out, April 1995, published by the G.B.S. Support Group of the U.K;

The Nottingham Evening Post, for their kind permission to reproduce excerpts from a feature originally published in November, 1998;

Andrea Robertson, RN, BN, for her valuable advice and encouragement;

Kim McLeod and Debbie Berge, Rockyview Hospital, Calgary;

Tara Butler, for her assistance with proofreading; My editor, Jae Malone; and

Brian Taylor and the talented team at Pneuma Books.

I also wish to acknowledge all those friends, neighbours, or just concerned people, some of whom we hardly knew, who undertook acts of kindness in support of me and my family.

Foreword

This work by Brian is a true gift to humanity. Those who suffer this disease process have a unique experience, often very difficult and sometimes terrifying for themselves and their loved ones. To read this inside account is to have a chance to understand so much more, so much more quickly for patients, loved ones and care-givers alike. Yes, I certainly do not leave caregivers out amongst those who stand to benefit greatly from this clear and frank account. Guillain-Barré syndrome is not common and many medical personnel rarely if ever witness a case let alone act as a primary caregiver. Without experience or good reference sources we can all lose ground or suffer unneeded anxiety before expertise is obtained.

I first need to explain where I come from. I am an Intensive Care physician or now more commonly called a specialist in Critical Care. If you will, we are the general practitioners of the critically ill. We oversee all care coordinating the contributions of all the other disciplines of Medicine. We are responsible for anyone ill enough to require life support until they are stable enough to again be transferred to a bed on a hospital ward. Critical care specialists or ‘Intensivists’ come from a parent discipline such as Internal Medicine or one of its subspecialties including Neurology, Surgery, Anesthesia, Pediatrics or Emergency Medicine. We receive at least 2 extra years of specialized training and experience before assuming responsibility for critically ill patients. I trained in Internal Medicine including Neurology but subspecialized in Respiratory or Chest medicine before embarking on my career of patient care.

I explain the above to give you, the reader, a reference point from which to take my views.

To witness the struggle of Guillain-Barré, to truly involve oneself with it, is difficult for many physicians and other healthcare workers on several levels. On one hand you carry out daily visits to a patient who quickly slips into a state of apparent coma, unable to effectively communicate. Secondly, the daily work of care is carried out by many people—nurses, respiratory therapists, physiotherapists, social workers, spiritual advisors, ward aides and dieticians with communication and coordination being difficult to arrange. Finally change is seemingly glacial from day to day and new problems come up which can be extremely formidable.

To not overcome these hurdles is to miss the excitement of the disease, that of truly helping the unfortunate victim. To suddenly go from a healthy active lifestyle that you are working very hard to maintain with large expectations of great success, to helplessness in a matter of hours is frightening in the extreme. To many of the patients, going to prison would be easier and preferable, however these people have committed no crime.

Thus the focus early in the course is one of moral support whilst doing everything needed to rule out the other diagnoses of the differential, other conditions that can cause similar symptoms. Neurologists will spearhead this investigation with their strongest tools being the patient’s history, examination and a test of the electricity of the muscles and nerves (‘EMG’ or electromyography and tests of the electrical conduction of nerves). Caregivers taking the time to speak at length to the patients and families trying to explain what has and will go on can be priceless at this stage.

The focus then clearly shifts quickly to a mode of care that many medical personnel in acute care areas do not cope well with—that of chronic care! Communication with the patient is difficult. Communication with the family and significant others is also often challenging. Family members often quickly become very stressed facing the conflicting goals of being at the hospital and carrying out the mandatory duties of their daily lives. Firm answers for most of their questions are hard to come by and most of the answers you do have are as difficult to hear as they are to deliver—’We do not know what the exact cause is, cannot predict the course nor whether the treatments we offer, with their side effects, will help.’

For reference and reinforcement the reader needs a capsule summary of the medical facts of this entity. Guillain-Barré is an ‘idiopathic’ (meaning we do not know why), ‘acute’ (speaking to the abrupt onset of the disease not its course), ‘inflammatory’ (meaning it does its damage because of redness and swelling engendered by the body’s own defense mechanisms including specialized cells like white cells and chemicals that the body makes and releases), ‘de-myelinating’ (this is a tougher one—we in effect lose the biologic insulation from around our nerves in the body that carry signals out to tissues and back to the brain) ‘polyneuropathy’ (that is both motor and sensory nerves of different types are negatively affected). The brain itself is usually not affected, leaving the person, at first, entirely awake and aware but only variably able to sense their surroundings or react to them.

Guillain-Barré is characterized by progressive muscle weakness over hours to several days. Remission or improvement is really almost entirely ‘spontaneous’; that is, the body heals itself—if given the opportunity. Seventy-five percent of patients do not get severe enough to need the Intensive Care Unit but multidisciplinary therapy is vital to ensure the best possible outcome.

Significantly, approximately two thirds of patients describe a recent respiratory (breathing) tract or abdominal infection.

Very importantly it must be stated that although Guillain-Barré has been reported to follow vaccination, specifically flu vaccination, this is not proven and has been overstated in the recent past. The most solid studies done to date show a possible (and I stress possible) increase in incidence of one per two million vaccinations. The number of cases of flu that are prevented, therefore preventing disability and death from that illness, far outweigh the risk and problems of Guillain-Barré. Every year flu vaccination programs are getting larger, however there is no apparent increase in the incidence of Guillain-Barré. It is therefore becoming ever more probable that even the extremely weak association between the two is incidental, not cause and effect.

The infection most commonly seen to precede Guillain-Barré is diarrhea with a bacteria of a family called ‘Campylobacter’. Campy-lobacter bacteria are the leading cause of sudden diarrhea worldwide so it is difficult to avoid. It is a bacteria borne in food and carried in the bowels of a wide range of animals used for food all over the world.

If Guillain-Barré comes on in association with this bacteria it is also more likely that the patient may come down with the two most severe forms of the disease, that of ‘axonal degeneration’ and the ‘Miller-Fisher’ variant.

In the axonal form, that suffered by Brian, the insulation is lost off the nerves plus many of the nerve fibers themselves degenerate. This means in order to recover the nerve cell bodies in the spinal chord have to regenerate their nerve fibers before they can regenerate the insulation that covers them. Nerve fibers may not grow again or if they do, only very slowly, taking months to reform. In the time it takes this to happen changes such as muscle loss or contractors of limbs can be so severe that full function cannot be regained.

Miller and Fisher simply described cases of the disease that also affect directly some of the nerves of the brain. Some authorities believe the Miller-Fisher variant to possibly be a related but separate disease.

The deterioration of Guillain-Barré can vary in onset from hours to about two weeks; recovery is often within months but can stretch much longer. Besides weakness, difficulty with the nerves that control automatic body functions can lead to heart rhythm problems, difficulties with low or high blood pressure, unusual sweating, bowel function and bladder function and unusual sensitivity to some medications all of which must be managed carefully. These problems lead to a great deal of the problems (morbidity) and deaths (mortality) of this disease. A great deal of day to day difficulty can come from severe discomfort as nerves carrying sensory information (pain, light touch, pinprick, temperature and joint position) recover. Their messages can be somewhat scrambled leading to great discomfort. Brian at times suffered greatly with these ‘dysesthesias’.

Critical illness and being on life support often affects the brain and one’s perception of reality whether one suffers Guillain-Barré or the myriad of other problems that can lead to Intensive care unit admission. This brain dysfunction (or ‘encephalopathy’) of the critically ill is well known and characterized but not well understood. On a fundamental level it may be a defense mechanism of the psyche and represents the combined effect of anxiety, pain, sleep deprivation and the effects of drugs. Patients enter a state of altered perception of reality most often accompanied by amnesia. Brian’s accounts of his reality during his illness are indeed remarkable.

Therapy with intravenous protein antibodies (gamma globulin) or washing antibody proteins out of the blood (plasmapheresis) is experimental and of low general effect and may be carried out depending on the nature and resources of individual hospitals.

About 15 percent of patients recover completely. Another 65 percent suffer minor deficits, which do not interfere significantly with quality of life. Five to ten percent are disabled and in spite of the best of care about six percent of patients die often in association with concurrent chronic illnesses or advanced age.

You can see that a focus of my recommendations for care follows the proven and recommended practice of taking whatever time is needed to communicate with patient and family. The downstream benefits are well worth the investment in time for all parties. The fact that the reader has picked up this book attests to the need and quest for information and I applaud your mission.

Another aspect of care, which has been very successful, is to, as early as possible once a firm diagnosis has been made, establish a well-coordinated and documented plan of weekly care. All services and family are involved in the process. Then, even when staff turns over, the greatest measure of consistency and thoroughness of care can be achieved. An erasable board is erected in the patient’s room for all to see with a weekly schedule of times of various activities and goals.

As with all of patients, especially those of longer term, we entreat families to bring in photographs of the patient when they were well. This serves to help staff remember the patient’s identity and humanity even when they are at their worst. We also mandate speaking in the patient’s room always as though they were awake and cogent as, especially with this disease, you never know for sure when they are listening. Any discussions that, in your judgment, the patient should not hear are best held out of the immediate vicinity of the patient, in meeting rooms if necessary. This goes especially for medical staff that can sometimes wax lyrical without thinking of the impact of their discussion on partially informed ears that can take the words out of context. It can be confusing and at worst cause unnecessary anxiety on the part of the patient.

This information sharing and planning of care we hold always as a primary concern and we hold weekly or biweekly multidisciplinary meetings of staff and family to continue the process. If it appears the patient can cope and even participate these are held right in the patient’s room. Honest and forthright answers can be heard by all at once, greatly increasing the clarity and consistency of communication. Even in the face of staff turnover and different people explaining the same thing differently, true communication and understanding can be accomplished.

The fostering of teamwork cannot be underrated. Resources are increasingly limited in health care as budgets fail to expand and cost increases. Innovation is the only relief if it can be mustered and a great team is a much more fertile ground for innovation than a disgruntled group of isolated individuals.

Research is carried out in many Intensive Care Units but should only be carried out with full consent and understanding of the patient (if possible) and family with the knowledge that failure to choose participation in research protocols in no way affects quality or quantity of care. The process must be open and informed and there must be no coercion. A standard to strive for in any worthy institution. Because of the nature of this disease with its cause still unknown, research has been vital in giving us the ability to help as much as we do and will of course be needed to truly some day treat or prevent it. For the benefit of patients yet to come I encourage all to consider participation with an open mind.

Brian had the axonal form of the disease. His survival, recovery, and the production of this work despite disability represents a mammoth effort of will.

The diaries kept by his wife are straight from the heart, a true representation of how the process was perceived from the inside. We all have these thoughts but many times they are lost before being documented. We are so thankful to know our feelings are shared which always to some extent relieves the burden.

Finally, as a physician twenty-five years into the practice of medicine I must acknowledge that one recovers well from this disease not so much from the hard work of knowledgeable caring physicians, but because of the day to day work of the whole team with non-physician members and the patient doing the vast lion’s share of the work. The Critical Care environment is a great place to work simply because unsuited staff do not last. If you cannot do your job well and get along with others you quickly realize your talents are best suited elsewhere. We are left with a distillate of people who know and love their work and it is a privilege to be a part of it all.

I know this work can help relieve a burden for the reader, make life a bit easier and productive, and as always should be the aim of medical care, at least give some measure of comfort.

Frank J. Warshawski, MD, FRCPC Director of Intensive Care Rockyview General Hospital,

Division of Respiratory Medicine and Department Critical Care Calgary Health Region, University of Calgary August 2001

"I was never supposed to walk again, was I?"

"No you weren’t, but then you don’t listen, do you?"

—Conversation between the Author

and Physiotherapist, Brenda

Author’s Preface

After a few moments, the reader will quickly realize this is not a medical textbook. Having said that, I should also say that, being the frightening story of a rarely talked about disease told from the point of view of one who was suddenly stricken with almost total paralysis, it would not be out of place in the library of any medical professional.

While there is much technical information on the subject of Guillain-Barré Syndrome (G.B.S. for short) available in medical texts and journals, little has been written about the syndrome that explains, from a patient’s perspective, what a sufferer, his family, and friends have to endure. My experience as such a patient shows me that not all nurses and therapists are familiar with G.B.S. and the unique needs of a patient afflicted with the syndrome. It is my hope that this publication makes a contribution towards filling that void.

Guillain-Barré patients, like me, who are unfortunate enough to experience the almost total paralysis that comes with one of the most severe forms—acute, chronic axonal G.B.S.—have unique needs. In addition to the inability to control body functions, they are unable to communicate. In my case, I could not speak, move my lips, my eyes or my arms. Writing messages, therefore, was out of question. I could not even wiggle my ears!

Thankfully the paralysis stopped short of affecting my brain. Fortunately, this is typical of the Guillain-Barré Syndrome.

I am convinced that having an active mind while virtually all body functions had shut down, caused me to experience a whole range of dreams, nightmares, and hallucinations, as though my brain had a compelling desire to stay occupied. These forays into ‘alternate states’ were so realistic that, in many cases, it was difficult, if not impossible, to distinguish them from reality. I have spoken to other patients with G.B.S., and they claim to have had similar experiences.

Some of the dream sequences—and I will use that phrase throughout this book, although many were actually nightmares—were hilarious, somewhat hilarious, or just plain scary.

This book is comprised of two distinct