Hirayama's Disease

[charlize29]

Hi there,

Here is a case of Hirayama's disease (also known as Juvenile Muscular Atrophy of the Unilateral Upper Extremity )of a 16 year old boy who had undergone surgery. Cheer up and do not lose hope, things will be fine. Prior to your surgery, do some visualization & relaxation exercise. Goodluck to you and take care!!

Here's the link:http://www.ncbi.nlm.nih.gov/entrez/q...&dopt=Abstract

Regards,
Charlize29

[wastingaway]

hey guys,

i just wanted to reply because i have recieved a few emails over the last couple of months about this.

i did undergo my surgery and have been going to occupational therapy for the last 6 months or so. the doctor told me he thinks he solved the problem and i will give him some credit. before, my hands got tired very fast with any sort of activity. i couldn't even finish a song on guitar hero becaues of it. i can now play for quite some time with out them getting tired (maybe 30-45 minutes)...clumsy, but not tired which is odd because even my friends' hands get tired after a few songs. along with that improvement, i have also gained some muscle back on the outer pinky finger. im not sure of the name but my left hand has recovered some of it. don't get me wrong, it is by no means back to normal and my hands are still rather bony, but it is improvement none the less

unfortunately those are the only improvements i have noticed. at occupational therapy they do measurements every month or so and it shows my hands are still loosing strength, slowly but surely. i have lost almost all the muscle between my index finger and thumb in the left hand as well which makes it very hard for me to bend my index finger fully. my therapist is trying to e-stem it back. (i think this is how she got my muscle back on the outer pinky) i'm not sure how the e-stem really works but if it DOES work i will be one happy camper! also, i have developed a twitch in my left forearm that comes and goes, i am hoping it is a sign of muscle growth however though i believe my grip strength last time was 4 pounds in the left hand and 25 in the right.

besides that not much is new. im stilll hopin things get better but in the meanwhile ive been tryin to live life to the fullest and get the most out of it. it is rather depressing when you have no control over your body, but i think the best thing to do is try to continue living. ive probably done more fun, crazy things in the past 6 months or so than ive ever done. you just have to try to stay positive

i wish everyong the best of luck and i hope you guys had a good new years! also if anyone has anything theyd like to share feel free to post. there really isnt too much information on this on the internet and a lot more people are effected by it than i realized.

[HandShake]

Hello WastingAway,

Hang in there buddy. I've had Hirayama's for about 17 years now. It started when I was 15 and I went to all the top neurologists in New York. They gave me every test imaginable and then gave me & my Mom blank stares and the obscene bill. No one knew it was Hirayama's. I only know that's what it is now because there is a fair bit of data on the web recently.

I have it in my right hand. I'm glad to hear your condition has improved. Even if it's only a little bit, that is incredible. I've been stable for at least 12 years now. It may have gotten worse recently after moving and a lot of heavy lifting, but I'm not sure yet because it periodically gets weaker than normal and then VERY SLOWLY returns to... Normal weakness. LOL. I can't write for very long and I have to hold a pen in a very strange way. My hand is pretty weak. I can hold a light bag of groceries for about 3 blocks before I have to switch hands and give my right a rest. Eating is kind of difficult. When cutting food (like steak), I have to hold a knife like I'm gonna stab somebody "Phycho" style and it makes me look like I have no manners. Or like I'm a 5 year old.
I can however mouse and work on the computer for as long as anyone else. =) Thank God for the modern age! PSP or any other kind of 6+ button controller gaming is out for me which reaaaallly sucks. But thankfully Nintendo Wii is out there. =p

I can bend my fingers and make a fist, but my index & pinky dont bend all the way in. Some things I've tried which have a positive result include accupuncture and shiatsu deep tissue massage. Sadly, the results only last a day perhaps and then it's back to "normal". I always wonder if constant massage will help, but I can never afford it.

My hand gets a hell of a lot weaker in the cold to the point of almost being unusable. But then again, my left hand gets weaker in the cold too, so I guess it's just an exagerrated malaise in my right.

I lift weights and work out so that at least the rest of me is strong. I've found that my atrophy actually extends to my right tricep as well, but not enough to be noticable. The tricep is still strong enough for me to work it out more than the other to acheive some kind of symetry.
I've found that though the disease only affects my right hand and forearm, there are many smaller things wrong with me that are all on the right side. For example, my right shoulder used to dislocate A LOT and I had to have surgery to keep it in the socket (it popped out painfully during sneezes and once while I was getting a blowjob - Not Cool!). I also injured my right ankle and it's been funky ever since. So while these things aren't technically connected to the Hirayama disease, I wonder sometimes if my left brain just hates itself! Or at least the side of the body it's responsible for.

All in all it hasn't really gotten in the way of my daily life, but you can get used to a lot of things I suppose. The ONE thing I would give up 10 years of my life for is to be able to shake someone's hand with a good firm grip. As it stands, I have to shake with both hands (so at least I can apply pressure with my left) and many people notice right away that something is wrong wit my right and I lose a lot of first impression points. Especially since nothing seems wrong until that moment. The look in people's eyes when they realize always cuts me to the core. That is the one thing I've never gotten used to...

So I periodically check on Google to see if some brilliant surgeon has figured out some breakthrough procedure so that I can give a good firm handshake again.

I often wonder why God gave me this affliction. I was once friends with a really great girl who suffered in her youth from polio which made her left leg weak. I forced her to walk with me everyday for years until her leg became strong and her limp turned into the sexiest hip-swaying strut. When we started, she could only muster 4 blocks. Now she does 3-4 miles no problem. When I was friends with her, it seemed to give my disability a purpose. She couldn't pull the "you don't understand" card on me and thus I was able to help her strengthen. Now we are no longer friends and I'm back to having no reason for this thing...

Anyway, that's been my experience for what it's worth.
If you have any questions (as weird or mundane as you want), feel free to email me. I think it is ultra-cool that you got surgery and have physical therapists helping you regain your strength - and that your doctors gave you some sort of solution. Rock on Brother. Rock on.

[canada]

Hey guys

I was diagnosed with monomelic amyotrophy aka. Hirayama's disease in 2003 and have now been going for 5 years or so. Unfortunately it affects both my hands, left much worse than the right, so that I also cannot play video games anymore hahah. Typing is manageable, but I find small everyday tasks a pain: ie. doing up buttons, shoe laces - notice that?

The thing that bothers me the most, besides the handshake like you pointed out, is that I can't play hockey or golf with my friends anymore... what I would give to for some sports again.

I've just recently been in touch with some doctor's at the Mayo Clinic in the US and hopefully will fly down there soon to get re-evaluated. I've read a few reports about mis-diagnosis and wondered if I may be lucky enough as well.

As to the post above about the surgery - how are you doing now? What did they do?

[Confused/Afraid]

Hello Everyone,
My name is vicky and i am a transgender female,my life has been very difficult as it is. I struggled to get were I was for it all to come crashing down on me.
I was diagnosed with Hirayamas Disease and have all the symptoms.It started with my right arm and now my left but this started when i was 30 not in my teens nor my 20`s like most. I`m 36 now. Is that normal, also they say it happens most to Asians or Japaneese or one`s from India I am latin, is that normal. I was a cosmetologist and a Dental assistant for about 20 years or so, my right is totally non functionable and my left is now getting weaker.I had applied for Disability cuz I can`t do much any more and I have been denied twice. I am now going in front of a judge for an appeal. What I want to know is if there are any of you who have applied for disability, or is there any one who is recieving disability. Should I just stop trying. There is no one out there who will hire some one who can not use there hands and arms. I need to know that there is hope for me and if there is any one who would help me thank you all for your support.

[musman100]

Hi to all I'm musman.. i found this great forum in sites and i knew this is a right place for me.

[billshakes]

Hello I'm a 36yr old English guy based in Barcelona and it looks as though I have Hirayama's disease which is causing wasting in my left hand. The official diagnosis is still a few months away but I've had MRI and Electromyography (EMG) tests and symptoms suggest this disease.

Just wondering if anyone has some advice on physical therapy that I can do myself to improve general mobility of the hand? What part of my hand should I exercise and how?

As far as I understand it from my Spanish health care, with a typical MRI result ie there is nothing out of the ordinary, there is no treatment or solution so if anyone knows otherwise then please let me know. I would also like to know what sports are considered ok to do as under doctors advice I have given up anything that strains the neck e.g. tennis, yoga and swimming (front) but am still running.

Also wondering to what degree the wasting could continue, it's been gradually getting worse over a 2 year period and hasn't reached a plateau yet but I'm obviously hoping it will and stop at the hand... Anyone else with experience of the condition I welcome your input be it positive or not, how far has it progressed with you and where.

Good luck all

[Shuntsman]

Hi I would like to talk to you about Hirayama disease my email is [email protected] thanks.