This article, first published in The Medical Post, describes my personal journey with Guillain-Barre Syndrome, an acute demyelinating neuropathy. This is a true story: a candid account of a family doctor who panicked in the face of a rare disease, and the patient who forged a partnership with him.
32 THE MEDICAL POST
Georgena Sil at age seven, sprightly in a ballerina dress. She was active and healthy from birth to her mid-30s.
At 17: Sil’s strongly developed diaphragm from playing the bagpipes may have saved her from a respirator during Guillain-Barré.
At age 23, Sil was planning her career in physics.
At time of writing: Although Sil is recovering, she walks with a cane and cannot work outside her home in her chosen profession.
In September 1989 I developed Guillain-Barre Syndrome. Its onset constitutes a medical emergency. In absence of supportive treatment, mortality reaches 30% to 40%; with treatment, the rate falls to 5%.
Yet the rare nature of this disease marks up the latter price-tag. In the midst of a modern city boasting three full-size hospitals, it was my insistent participation in my own medical case that played the larger part in keeping me alive.
That September began bustling as any other. Working at breakneck speed, I moved house on the first of the month.
Mid-Sept.: Tingly numbness creeps from my toes across the soles of my feet. My toes look dusky in color.
Sept. 19: I awake to feel surface numbness and intense tingling pervasive in legs, buttocks, perineal area and, disconcertingly, my tongue. Pain bludgeons my upper back. Surely these oddities can't last!
Sept. 20: No change. With aerobics and light weights, I try revving my circulation.
Sept. 21: The previous day's exercise clearly set me back, not forward, for no matter how much I concentrate, or relax, I can walk only in slow motion. The sensory symptoms extend to hands and fingers. A cough develops. My period arrives much too early (a first).
Sept. 22: I visit Emergency of the hospital near my home. The ER physician advises “a muscle spasm strong enough to pull the spine out of alignment is causing the pain.” I should use heat, and see my GP first thing Monday.
Sept. 23-24: I survive the weekend by using heat like gangbusters. Heat, coincidentally, soothes Guillain-Barre's pain.
Sept. 25: It's Monday. Hanging on to walls and countertops to keep from falling, and with the blessed kindness of a taxi driver, I arrive in my GP's office, calm with hope.
He is irritable. This youngish man, in his late 30s like me, provided compassionate and adequate medical care when the problem was familiar, well defined. But my present symptoms fit no known clinical picture in his repertoire. To exempt himself from a potentially challenging diagnostic quest, he convinces himself that my condition represents a problem the patient has to solve, rather than a problem the doctor has to solve.
He first measures my vital signs. My blood pressure is very high at 156/90, my pulse 90 beats/minute. This was, in retrospect, the onset of life-threatening autonomic symptoms.
He then takes the history of my sensory symptoms over the past few days. His diagnosis: “You are simply hyperventilating. That's the only thing that causes hand-in-glove numbness.” He adds, “Look how deeply you're breathing.”
I explain that I played the bagpipes for a decade when younger, and my diaphragm is strong. I breathe deeply but slowly.
I go on to defend my overall medical history. In 1984, I was diagnosed with Interstitial Cystitis, a serious but rare autoimmune disease of the bladder causing supra-pubic pain and frequency of voiding. Diagnosis came late, two-and-a-half years after disease onset. Following cystoscopies and treatment trials over several months, remission was induced with silver-nitrate installations.
But the rigorous treatment required to attack the advanced disease in my bladder left me with a chronic, and painful, bone complication named Osteitis Pubis. Also rare, this created its own struggle for diagnosis through 1985.
Both diseases are mainly inflammatory, the symptoms physically manifested; neither have stress-related or behavior components. There was no reason to doubt the validity of my present distress.
My GP reluctantly agrees to investigate further. He taps my Achilles tendons; there is no response. He has me splay the fingers of my right hand, telling me to maintain the spread against his mild pressure to close them together. My hand folds immediately. Reflexes in my knees, elbows and elsewhere are bypassed. Yet his chart will state “Full reflex testing, with all results normal.” His systematic denial has begun.
Desperately, I return via taxi to the hospital Emergency near my home. There the chart will show very high blood pressure, and pulse 118 beats/minute. All my peripheral reflexes are recorded here as absent, kaput. My knee, for example, is tapped with a rubber mallet 20 times, and nothing happens. The ER physician phones my GP's office, but receives this vehement response: “Make sure you tell her it's nothing anatomical!”
The meticulous measurements now get brushed aside, as does my right to a second opinion. But I know my symptoms are real and deserve answers. After five cruelly exhausting hours of waits and argument, I am grudgingly awarded an appointment with a neurology specialist, scheduled four days hence.
Sept. 26: My weight is four pounds down. I am weaker.
Sept. 27: I fall climbing stairs in my home. It takes 10 minutes to claw my way to my feet. What if I'm too weak tomorrow to crawl out of bed for help … ?
I return to hospital Emergency. The ER physician, a different one this time, senses something serious. But the hospital requires a primary-care physician to direct any given patient's case, making my GP's approval mandatory for admission.
The hospital needs, and uses, tenacity. I arrive in Emergency at noon. It is dusk before arduous argument persuades my GP, and I am wheeled by stretcher through lengthening shadows to a bed on the medicine ward.
Sept. 28: The neurology appointment is moved up. A spinal tap shows abnormal elevated protein in my spinal fluid.
Sept. 29: Tests at the EMG Clinic reveal that nerve conduction in my muscle fibers is badly slowed. In some muscles, the amplitude of the signal attenuates to zero.
My neurologist is sure I have Guillain-Barre syndrome, a rare autoimmune disease in which the body destroys the myelin sheath, and sometimes the core, of type A nerves. The disease moves upward with deadly speed, plateaus, then spontaneously regresses over months or years. I've now entered a grave waiting game.
Cards and chrysanthemums, gestures of love, brighten my bedside table as old friends, my extended family, rally round. My pastor drops by with increasing warmth. One day, I cry in his arms. The medical system is getting to be too much.
My neurologist had, at my request, copied a textbook summary of Guillain-Barre. Devouring the page, I was stunned when I came to “autonomic symptoms.” Yes, my blood pressure was high, now 155/110, enough to bring on severe headaches. My resting pulse surged at 95 beats/minute, climbing to 120 with the minimal exertion of sitting up.
My respiration quickened; driving my muscles made me puffy. My face was flushed and sweating was abnormal; one night, I literally drenched the bed.
The textbook concluded: “Autonomic dysfunction occurs frequently in Guillain-Barre, can be severe … and is closely related to sudden death.”
I approached my neurologist, who advised that generalized symptoms are the GP's bailiwick. But my GP rebukes, “Your elevated pulse and blood pressure just mean you're frustrated with the hospital.”
In the comfort of my pastor's arms, I decide I need more information. A hospital pass is dickered for, reluctantly given, and I struggle by wheelchair and taxi up to the Health Sciences Library of our university campus. I am no stranger to this library, having in past years researched my two other uncommon diseases here. This work would not so often fall to the patient if Medicare compensated doctors for library study.
POURING OVER the recent Medicus Index, I now list promising papers on Guillain-Barre. My letters are large, looping, sloppy, my fingers barely grip the pen. I can hardly move the wheelchair on the library's carpet.
I am cold, so cold, and I ache horribly. Why, oh why, did I come here? I shouldn't have to do this! Shivering, I can't tug the hardcover journals off their shelves. A librarian rushes to aid, accomplishes the copying, while I feed her nickels. Then, near collapse, I shunt back to my hospital bed.
The research papers are explicit. All authors concur: “The two principle causes of mortality in Guillain-Barre, split 50/50, are respiratory failure and autonomic dysfunction.” My GP listens now.
Following my diagnosis, he considered it crucial to monitor my respiratory capacity, watching for chest muscle failure. Now, the other deadly 50% is recognized. A beta blocker is prescribed, and within 24 hours my wildly elevated vital signs stabilize.
Disillusioned with the medical system, concerned about what else may be unknown or overlooked, I seek permission to review my own hospital chart. My GP is not yet ready to be this liberal, but my request gets the full blessing of the head nurse who contacts Administration's vice-president of operations. His verdict is quick: A full copy of my own chart is given to me to keep and work with.
The chart shocks me. Incorrect entries date right from my first attendance in Emergency. But, in absence of judicial legislation in this area, the hospital is powerless to compel physicians to correct chart errors. The patient's only option is to formulate one's own notes for attachment to the chart. It takes a week of study and painfully awkward writing, but my notes, when complete and typed, and firmly accepted.
That was the turning point with my GP. He had a partner now to share the thinking over this rare disease, and his panic subsided. I was able to offer, as Guillain-Barre continued its relentless course, ideas aiding my own comfort and treatment. Methods of physiotherapy and pain control, for example, specific to this disease.
I spiral downward. Four weeks from onset I reach the nadir. Paresthesia extends from toes to crown. Swallowing is stiff. My bowels block. Gravity defeats everything. I can no longer grip a pen or hold up a newspaper, nor am I self-mobile in a wheelchair.
Guillain-Barre attacks striated or voluntary muscle, leaving alone smooth muscles such as the heart and diaphragm. My (infamous) diaphragm, highly developed from the bagpipes, may be what saves me from a respirator, for by now my chest muscles are shudderingly weak.
I reach a plateau, and days drag bleakly by. Practical nurse I'll call Valery, who happens to neighbor my new home, surfaces for daily visits. But her conversations, first light, show increasing tinges of aggression. One day, bolder, she demands my car keys, ostensibly to reposition my Honda to allow her more parking room. Weak, I yield without skepticism, but discover my car keys are at home. This enrages her.
The head nurse, face sober, approaches later with a quiet question: “Did you know Valery expects to drive your car while you're laid up?” and a protective lecture: “Don't make any property decisions until you're stronger.”
Valery is replaced by practical nurse Kent (not his real name). Cheerful as a chipmunk, humming Pavarotti, Kent also loves bawdy, off-color jokes. An enema, for example, is “get out the garden hose.” Kent uses his gusto for life to strengthen us.
And suddenly! My fingers one day curl into a weak semi-fist. Dawn of my gradual reversal.
October and November pass. The Berlin Wall comes down, and I regain my own freedom, able to negotiate the hospital wing gingerly in a wheelchair. Our Saskatchewan Roughriders, after a decade of monumental incompetence, clinch an uproarious Grey Cup, and I clutch my own laurel, a walker with which I painstakingly increase my yardage, first to the ward door, then down the hall.
At November's end I am discharged with supportive help to complete my recovery at home. Home Care for housework, volunteers for grocery shopping, and Meals on Wheels, enable this transition.
Our health is of significant value; losing it precipitates a grieving process. I long ago made that difficult journey when chronic illness first struck in 1984. Guillain-Barre syndrome, though taken in stride, had new flavor: I came face to face with my own mortality – and won.
As I write this in 1992, I walk with a cane, but cannot yet work outside the home in my chosen profession of physics. Currently I pursue a secondary career – writing – from home, holding patient advocacy as a major goal.
Georgena Sil lives in Saskatoon
The Impatient Patient: Coming to Grips with Guillain-Barre Syndrome
was first published by Georgena Sil in The Medical Post 29(5):32
The Saskatoon Star Phoenix reprinted this article as What to Do When Doctor Doesn’t Know Best (by permission). In the same edition of the Star Phoenix, an accompanying column by their in-depth editor Kathryn Warden spoke on behalf of patient involvement:
“Georgena Sil's horrific tale (today's Lifestyle Extra cover story) is a reminder modern medicine is not perfect.
It's also a revealing look at what can go wrong in the doctor-patient relationship, especially when a paternalistic physician encounters a new breed of activist patient who's convinced her symptoms are real and isn't going to be told otherwise.
Sil's case may be unusual, but no patient should have to go to such lengths to be taken seriously by a doctor. Near collapse, she had to dig out the information herself that led to diagnosis.
Only after she insisted on seeing her own medical chart and found errors in it, did her doctor recognize she was a partner in her own care.
Patients should feel heard, crucial because patients may be unwilling to listen to their doctors unless they are listened to themselves.
A Patient Charter is not a statute and has no force in law. Rather, the Charter acts as a catalyst to resolve individual patient concerns. The best-designed Charters also spur system improvement.
Just under half of Canadians who have a regular GP are considered engaged in their care. These engaged patients tend to be female (58%) and age 50 or older. The future: Structural changes to health delivery (team-based care, and electronic health records) will help relieve time pressures that impede a working alliance between physicians and patients.
Patients must still take the initiative – and do the work – when errors need correction in their medical chart. On this point, little has changed since the Medical Post article was written.
Tuum Est - It Is Up To You
This article was originally published in The Medical Post, issue 29(5):32.
The Saskatoon Star Phoenix reprinted the article under the title What to Do When Doctor Doesn’t Know Best (by permission).
In the same edition of the Star Phoenix, in-depth editor Kathryn Warden wrote a column to introduce the piece and comment on patient involvement in medical decisions.
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