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Progressive Supranuclear Palsy:
Willy Gommel's Personal Perspective
Edition of July 9, 2004
On December 1, 1997, I got sick. Really sick. What was it? I don't know, and neither did the doctors at the time.
The symptoms were as dramatic for their absence as for their severity. The main symptom was fever: close to 105° F. Other symptoms included mild respiratory distress, though that could as easily have been my usual life-long condition intensifying with the fever. The other main symptom was terrible muscular pain and general weakness, to the point where it was nearly impossible for me to get out of bed at all, for any reason, for two or three days. And, although I hesitate to list it as a "main" symptom, certainly a major one was my intense blood pressure attack: it was at least 220/140 for the days I was so weak, despite its being fairly well controlled otherwise by my medications at the time.
In the weeks that followed, it became apparent that some of the damage was permanent: I soon became aware that it had gotten significantly harder for me to type at work than it had been due to persistent weakness in my arms, making it much more difficult to hold them up to the keyboard.
It also emerges as a question mark whether I should have said "was" or "is" back there. By this I mean two things. The first thing that emerged was that this is some sort of ongoing neurological condition that continues to develop to this day. That gives me the first word in the diagnosis: "Progressive." The second is a bit more subtle: the realization that this disease has in fact been with me all of my life. As I track the present much more severe symptoms of the disease, I recognize all sorts of little ways that I was unusual, or substandard, or both, starting from earliest recollectable childhood: such things as the fact that no amount of practice on the piano would ever render me able to play a fast-paced piece; likewise, skill on the typewriter (on modern computer keyboards, speed does not depend so much on regular rhythm as it did on older mechanical typewriters); explosive high energy in my earlier years at a rate that other boys could not begin to match, offsetting my equally dramatic slowdown in more recent times, etc.
In fall of 2000, I consulted a neurologist. My symptoms were not yet far enough advanced to permit of full diagnosis. Neurological diagnosis is a tricky business at best. To obviate $2,000 worth of tests plus a substantial trip to the place having the required equipment, a tentative diagnosis of Parkinson's Disease was tried (by testing its remedial medication on me); the only result was no results, which essentially blasted the theory. By 2001, my employer had noticed my slowing down. By early 2002, it was becoming apparent that I was no longer able to put in a full work day, and that my work during the hours I could put in was becoming commercially nonviable. Back to the neurologist I went. Now he found the diagnosis much easier to make. The "Supranuclear" refers to the site of nerve damage relative to a region in one of the older brains; the "Palsy" refers to the concomitant muscular weakness. The statistics indicate that about one in 10,000 are so afflicted. Since that time, I have been living on disability benefits.
So, fairly typically, my balance is impaired; my speech and fine motor coordination in my hands is impaired; and my muscles have all gotten weak to the point where most motion hurts. One surprise for me (though probably not for MDs) has been the extreme limitation of motion resulting from all of this in my shoulders in particular, which earlier in 2003 deprived me of my ability to steer an automobile, thus of my ability to drive at all. I've had to get a small step-stool to reach even eye-height shelves in my kitchen and elsewhere. I sit in my study trying to type such essays as this. Lately I've found and downloaded a program that makes my computer keyboard sound like an old platen typewriter, and that has helped out quite a lot. Still, by the older standards to which I had become accustomed, it works very slowly indeed for me -- and I cannot get much done because my days are so short after a bit of extra sleeping that seems needed and also the slow motion that services my end-of-day bodily functions and also the fact that I must still eat.
Is the lifestyle frustrating? Of course! But many friendships and people's wonderful attitudes have made my life far, far brighter than it would be if the frustration loomed large enough to capture my entire life experience.
My final retirement is now two years old. In that time I have had to learn to cope with circumstances that do prevent a normal life style. I have also learned that the coping requires frequent recalibrations due to the changing pace of my body's capacities. I have also learned -- with some grace, I hope -- that no man is an island, for reasons which change with circumstances. This realization has led me into, and circumstances have required, my permitting of involvements of others which in earlier years I would not even have thought of inviting.
Here are a couple of links for anyone interested in learning more about this particular affliction:
NINDS, an operation of the NIH agency of the U. S. Department of Health and Human Services, offers a first-rate public information service about all sorts of neurological disorders:
This is the general listing, on which you will find
this listing for PSP itself.
Here is a listing for the Society for Progressive Supranuclear Palsy.