Hirayama's Disease

[bbuttelmann]

Thanks for the replies. Yes, warm hands helps. A warm climate helps a LOT. Does exercise help? No one knows. It can't hurt.

It is REALLY great to be able to connect with others that have similar experiences. I'm 71 and clearly the old guy in this group. I am going to post a pretty long set of experiences which I hope will be a sober encouragement to the rest of you.

I think we still need to identify some "centers of excellence", as they say in the medical field, where there is experience in diagnosing and treating Hirayama's disease. I WANT TO PLAY MY GUITAR AGAIN!

One point: Hirayama's and SMA (spinal muscular atrophy) are most likely different: SMA is known to be genetic and there is a genetic test. Google it. The Muscular Dystrophy sites have information. I don't think anyone knows exactly what Hirayama's is.

[bbuttelmann]

Living with Hirayama’s Disease.

I am posting this to encourage others. I hope it will help: For what it’s worth, here is my experience living with Hirayama’s disease. This disease is so rare that without the internet you probably feel like you are the only person in the world with your peculiar set of problems. If it will help you feel connected by reading this, read on. Be of good courage - You CAN lead an almost normal life.

As of October, 2008, I am 71 years old and have lived with what is most likely Hirayama’s disease since I was a teen, if not before. Other than the slow wasting away of my hands (right much more than left), I am in reasonably good health, have no known life-threatening illnesses and still live an average normal life. Up until the relatively recent occurrence of these blogs, I thought my condition was entirely unique and paid little attention to it, aside from the obvious ever-present problem of my right hand slowly becoming almost useless and my left recently beginning to lose some strength..

I think the first evidence that my hands were different happened around age 12: I had begun piano lessons about six months earlier, and my teacher was frustrated with the fact that I could not get the third finger on my right hand to play with any strength or control. She believed, and I accepted as true, that I was simply not trying hard enough or practicing well. The rest of my fingers were fine, and there was no noticeable muscle deterioration anywhere in my upper extremeties. After a few frustrating months of her yelling at me, I gave up the piano. Later, at about age 14, I noticed that in the winter I would lose control of my right pinky. Other than these two anomalies, I was your normal frustrated neurotic teenager.

During my childhood I was a standard active boy, played all the usual kid’s sports, and especially loved baseball, but could never handle a basketball well enough. I was a good runner and made a decent football running back and receiver – caught my share of touchdown passes – but couldn’t throw a pass. I am right-handed, in baseball was a good hitter and fielder, and never had any noticeable problem pitching, throwing, or batting. Perhaps that’s because a baseball is smaller and easier to grip than either a football or basketball. I played baseball and softball for over 50 years, maintained a good batting average, usually batted leadoff (which means I was recognized as a batter who frequently got a hit, but not often a home run), and I played a good left or center field, and pitched slow-pitch softball. I had a good reputation for catching outfield flies and being able to throw out a runner at the plate. I was able to do all this well into my fifties. My wife and I had five kids (yes, be of good courage - Hirayama’s disease has NO effect on your sex life ). I am mentioning all this to encourage everyone that Hirayama’s disease does not have to make you treat yourself as an invalid. You CAN lead an almost normal life. I have been able to teach my sons and daughter to play ball, handle a jib sheet and even sail a sloop, and was able to coach little league baseball and soccer.

By the time I was in my middle teens, I noticed that the fingers of my right hand simply didn’t have the dexterity that my left fingers did. That became a problem since I am right-handed, and slowly I developed the ability to use my left hand for fine motor activity – handling change, keys, etc. But I continued writing with my right hand all my life, and still do, although since about age 60, it has required using my left hand to support my right. I look weird having to use two hands and write very slowly, but that hasn’t stopped me. Typing has become a REAL problem in the last few years, since now I can only use one or two fingers on my right hand, while the others just sort of get in the way. It makes typing slow and laborious, but not impossible. Recently, a very good occupational therapist made a splint for my right hand that helps with typing, but it makes me look a little like Freddie Kruger, so I only put it on when the family’s out.

One of the beneficial side effects of living with a somewhat dysfunctional right hand is that I have become almost ambidextrous. Makes one a pretty darn good handball player, and I have won my fair share of handball tournaments. I played handball in my thirties and forties. My wrists and arms were strong – only my right fingers were weak. To make a strong hand in the game, I would tape all my right fingers together into a web.

For fourteen years in my thirties and forties I was a university professor (computer science, Ohio State, in case you’re wondering). The only effect Hirayama’s disease had on me that I can recall is that I was probably the only professor who wrote slowly enough on the board for my students to keep up. During that time I enjoyed playing on a softball intramural league every Summer, and in several handball and racquetball tournaments. When I turned forty I took up classical guitar and played well enough for my own satisfaction. Around age fifty I could no longer strum with my right fingers, and the guitar still sits unused in my basement. I haven’t given up the hope of playing again. During my forties and fifties I also played the recorder, a rather simple woodwind from the Middle Ages, and finally stopped when my right fingers could no longer hit the notes. In my early thirties I took up sailing, and continued until around my middle fifties, when I could no longer handle the sheets (ropes to you landlubbers). I’ve had my entire family out sailing many times with no worries about safety, but would not do so today.

Beginning in my thirties I sought medical help, but it was not until my late fifties that the Mayo Clinic finally diagnosed my condition as Hirayama’s disease (their best guess). That was about 1997, and at the time they could give me no hope of treatment or cure. Today, my right hand has deteriorated significantly and my left hand is weak but useable. Both hands show significant muscle atrophy. Both arms are thin but not skinny. My right forearm shows some muscle atrophy. I have almost normal strength gripping with the fingers of either hand. Most of the weakness is in not being able to extend my right fingers, and to a lesser extent, my left.

So, what may have caused this illness? Is it genetic, infectious, or the result of some trauma? Is it really a “disease”, or just a condition that has resulted from some unusual event? I don’t think the experts know yet, but I do know that when I was about twelve I was in a serious car accident. At the time the only consequence that was discovered was a hairline fracture in my left ankle. Back then (late 1940’s), we didn’t know about whiplash or how to detect spinal injuries. Did a car hitting me from behind at 45 mph cause this? Probably. Can I be sure that these symptoms only appeared after the accident? Frankly, I don’t remember that accurately. I was only a kid. Who knows?

Don’t give up. With some adjustments you can live a long and happy life. May you live and prosper.

[josephblayil]

Thanks for that. I'm 25 now and I feel great after reading through your post. Let's al be positive and optimistic and we can live a normal life. Thanks again for that great post.