During my two-month hospitalization with Guillain-Barre Syndrome in Saskatoon, I was exposed to severe and unnecessary risks. Like all rare diseases, Guillain-Barre manifests itself as medical illiteracy in the medical mind – in family physicians, ER physicians, even physiotherapists. My steps in counterpoint:
James A. Murtagh, the Vice-President of Operations at St. Paul's Hospital, Saskatoon, firmly accepted my 4-page set of notes for permanent attachment to my hospital chart. This material remains relevant as a teaching vehicle. The Notes appear below.
Tuum Est Webmaster Georgena Sil
Physicist and Technical Writer
Patient: Georgena S. Sil
Physician: Dr. Wesley Hugh McKee, Saskatoon
Location: St. Paul’s Hospital, Saskatoon
These notes record events for clarification, and medical data for reference. I expect these notes to achieve:
When medical needs are simple or ride along at the status quo, Dr. McKee provides compassionate and adequate medical care. There come times, however, when the patient develops a new medical problem, or a new disease, so that medical needs go beyond the status quo. My concern lies with Dr. McKee's conduct in these situations.
His practice desired to remain a millpond, Dr. McKee instantly hates anything that threatens to roil it. If Dr. McKee can convince himself within his own mind that the new medical complaint is somehow a problem the patient has to solve, rather than a problem the physician has to solve, then he is absolved of responsibility in relation to time spent and in relation to grappling with technical modern medicine.
Quite a payoff for the physician, but unfortunately this minimalist tactic does place the patient at considerable and repeated physical risk.
The association between Guillain-Barre syndrome and my symptoms of hypertension and tachycardia should be made clear.
Before contracting Guillain-Barre, my physical health was far from perfect. I have been diagnosed with two uncommon physical diseases: Interstitial Cystitis of the bladder, and Osteitis Pubis of the bone. Although faced with their chronic nature I continued to take care of my general health, including emphasis on physical fitness within my limitations, so that my blood pressure, heart rate and respiration rate consistently measured normal.
But when Guillain-Barre developed in mid-September 1989:
Standard textbooks and review articles describe the clinical picture of Guillain-Barre syndrome. Autonomic dysfunction is considered to be a core symptom of the disease, right alongside the progressive symmetric motor weakness. Standard sources state:
“Features strongly supportive of the diagnosis Guillain-Barre: Autonomic dysfunction. Tachycardia and other arrhythmias, postural hypotension, hypertension, and vasomotor symptoms, when present, support the diagnosis.
The hypertension [of Guillain-Barre] may respond to [blood pressure medication] as may associated headache.
[Source: B.G. Arnason, p. 2067 and 2069]
“Autonomic disturbances [in Guillain-Barre] are common, may be severe, and are sometimes life threatening; they include tachycardia, cardiac irregularities, hypotension or hypertension, facial flushing, abnormalities of sweating, pulmonary dysfunction, and impaired sphincter control.
[Intern Dr. Hubert's textbook, p. 612]
“Autonomic dysfunction [in Guillain-Barre], including BP fluctuations, inappropriate ADH secretion, cardiac arrhythmias, and pupillary changes occur in more severely involved patients. The respiratory paralysis and the autonomic defects may be life threatening.
[Textbook Neurological Disorders, p. 1446]
My concern arises from Dr. Hugh McKee's acceptance of my case without concomitantly undertaking to read the briefest clinical picture for the Guillain-Barre syndrome. While I fully understand that technical matters of this disease belong to the neurology specialist, there definitely are areas related to general health which fall under the family physician's umbrella. Autonomic symptoms belong in the latter category.
Facing down Dr. McKee's rigidity and anger was not easy, but upon finally agreeing to read the above quoted paragraphs, Dr. McKee placed me on a beta-blocker (Tenormin 25 mg) to bring down the excessive blood pressure. This was on October 6, ten days after I was admitted on September 27, and fifteen days after I first presented to St. Paul's Emergency on September 22, 1989.
The blood-pressure treatment brought major changes consistently from that date of October 6, 1989:
Before this problem was solved, I was forced to lie prone much of the time as this was my only means of controlling the breathlessness and tachycardia which instinctively felt so hard on the body. Solution to the problem in the form of Tenormin brought as a side benefit increased mobility, as I was now able to sit up longer, get around in a wheelchair, and periodically use my walker for short walks (before and after the nadir).
Any good nurse can describe much better than I the benefits to the patient in facilitating mobility. These benefits include full expansion of the lungs, prevention of muscle atrophy, and lubrication of the joints. These benefits accrued to me in addition to the primary benefit of relieving the physical strain and risk to the body of autonomic symptoms with respect to my ongoing Guillain-Barre.
Guillain-Barre syndrome produces symmetric motor weakness which develops quickly over a period of days/weeks, enters a plateau phase, and remits gradually over a period of weeks/months. During the development phase, once an area or muscle group is hit by demyelination, the resultant motor weakness is not seen to fluctuate but will exist until the body restores and repairs a large percentage of the myelin sheaths affected in that area.
This is a very slow process, thus the motor weakness is constant rather than intermittent. It is puzzling therefore to see references in the chart to the physically and logically impossible feat of “intermittent motor weakness.” Said references are found in the early section of my St. Paul's Hospital chart for Guillain-Barre syndrome.
It would be fair to surmise that the force of Dr. Hugh McKee's emotion and rejection of the seriousness of the presenting complaint, led to passing observations and information through a distortion filter. This not only by Dr. McKee but by those initially in contact with him, judging by perusal of the earlier chart section.
A statement of fact with respect to constancy of the motor symptoms is therefore summarized here: The motor weakness associated with Guillain-Barre sees only gradual change. There are also some adaptive effects to note: After an area is stretched once via passively-assisted motion, it may be easier to do it next time as the joint loosens up and the tendons stretch.
In Guillain-Barre syndrome, the feat of ‘intermittent weakness’ is physically and logically impossible.
The motor weakness heals very gradually – but there are adaptive effects to note.
Additionally, a motivated patient will quickly use one's intelligence to adapt and alter one's body alignment for balance or to let stronger areas compensate for the weakest muscle groups (for example, sliding backwards onto a stretcher replaces stepping up on a step-stool). This is an ongoing learning process, so the patient's ability to do tasks may improve even though the disease is constant.
I have also observed that my muscles become weaker and stiffer through repeated use, so that short bursts of physical activity may be interspersed with enforced periods of rest. This adaptive effect is reported to be characteristic of Guillain-Barre syndrome.
It is important also to take an accurate view of the motor weakness in relation to patient risk re falls and accidents. In a scenario of intermittent motor weakness, physicians might reasonably veto day-passes from the hospital if the patient were at risk for an unexpected attack of weakness which might lead to accident and injury.
On the other hand, in a scenario of constant motor weakness, the patient learns by experience the limitations faced, which discovery enables living within those limitations, so that the risk of falls and accidents is reduced after a reasonable period of time.
Tuum Est - It Is Up To You
The original typewritten notes were four pages long, prepared laboriously and with patient access to textbooks, during my two-month hospitalization with Guillain-Barre.
Page 1 was an introduction: its purpose was to place the conduct of GP Dr. McKee on record where he held hospital privileges (St. Paul's Hospital in Saskatoon).
The balance of the notes are scientific and focus on two areas: autonomic symptoms, and the monophasic profile of Guillain-Barre. This content remains relevant today as a teaching vehicle.
James A. Murtagh, the Vice-President of Operations at St. Paul's Hospital, Saskatoon, accepted the full four-page set of notes for permanent attachment to the medical chart of Georgena S. Sil.
The fulltext document, as it exists in the hospital chart, is available in PDF form at: Sil Notes for St. Paul's Hospital Chart
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