pma

[lucy]

I have had PMA for ten years and it has progressed from my right arm across my shoulders and now to my left arm. my legs seem fine but I do have fasiculations which scares me. I had fasiculations in my arms and chest for 15 years before atrophy started. Will it move to my legs?

As Jean said, no one knows what parts will be affected and when. My husband has had a very slow progression(thank the good Lord) but he pretty much had symptoms in his arms and legs from the beginning. His fasiculations were a lot more rapid in the beginning. From what you descibe, are you sure that you have PMA? Could it be benign fasiculations? If you do not have weakness, I would question your diagnosis....

 

[jean]

Fasciculations by themselves can be normal ,but only having weakness in the arms with no effect on the legs doesn't mean the diagnosis isn't right. PMA in a few people stays confined to the arms only and is known as flail arm syndrome.(bi-brachial amyotrophy). It can stay in the one region indefinitely ,or eventually involve other areas. It makes it very difficult for neurologists to accurately predict what will happen in individual cases.It is a watching and waiting game for everyone,which is very frustrating both for those of us with the disease and for the doctors and carers looking after us. How are you doing Lucy? Have you been able to get some help with all you are having to do? I hope so.
very best wishes
Jean

 

[lucy]

Jean,

Thanks for caring. My husband has hired someone that comes into my home twice a month to clean the house. That has allowed me a little more freedom to get out and do fun school activities with the kids. I have also had more (me) time which I usually use downstairs on the treadmill several times a week for a half hour. I feel so much better when I can excercise. I use to belong to the Y and would exercise all the time. Over the last 3 or 4 years I have kind of let myself go. I have probably put on 50 lbs. between the lack of exercise and "stress" eating. I am actually seeing my doctor once a month to try to get my weight back down. I know that I would feel much better if I could get this weight off. In the grand scheme of things though, that is not important. It is all about my husband and learning new routes to use when something he is doing no longer works and caring for his needs. Right now it is the use of his joystick on his wheelchair. He is having a terrible time with that but we are in the process of finding out what the next step is after he is unable to use it anymore. How are you doing? I hope well!

Lucy

 

[jean]

Hi Lucy
I am so glad that you are finding things a little easier with having some help in the house. It is important for both you and your husband that you are ok and so you need some quality time of your own. This disease takes its toll of both partners.
I am doing fine at the moment. I know I am fortunate in having slowly progressive disease and whilst disability is frustrating I am still able to do most things.I concentrate mostly on the present and try not to think of what will happen in the future.Possibly that is easier for the person with the disease than their carers,but I suspect it would be good for both to try and dne day at a time is an easier hill to climb i think!
Anyway look after yourself as well as your husband.
best wishes
Jean

 

[lucy]

Hi everyone

We just got back from our dr.s appt. at Wash U. Once again my husband got a breathing test. It was 77% the first time, 78 the second, and 79 the third. When we started going 2 years ago it was at 88% so that means it has dropped about 10% in 2 years. He is getting over a cold which could contribute to some of the drop. His arms and legs are both pretty much useless now and his neck showed some weakness this time. I asked him on the way home why we continue doing this to ourselves. We know with the diagnosis of PMA that it is degenerative but it still doesn't make it any easier to hear. I asked them when patients usually start to feel like they are having breathing difficulities and they said it differs but around the upper 50's. After it dips under 50 they said they start to be concerned and that is probably when we would get sent to a pulmonologist. Hopefully after a good nights sleep we can wake up with a more positive attitude. Right now we are sitting with our 2 beautiful kids not saying much but at the same time acting like nothing is wrong. I hope all is well with you all.

Lucy

 

[patricia1]

Dear Lucy My understanding is most patients % goes down every two month 12 % so your husband is doing well after two years He can get a bipap machine but ins company pay after your % is under 50% I would think if you want one pay out of pocket because it does make a difference in your breathing and can prolong your life so look into it. But your husband is still at a safe % Good LUCK Pat

 

[lucy]

12% every two months? That sounds like sooooo much. Would that be the people with the true als (has bulbar symptoms)? My husband luckily has only been affected from the neck down. He has primary muscle atrophy which is a variant of als. It supposedly progresses a lot slower. He has had a slow progression but at every 6 month check up he has definite signs of worsoning.

 

[patricia1]

That is people with ALS Pat He still is doing fine he is still at a slow pace no matter what he has.Hang in there Pat

 

[lucy]

hello to all

That is people with ALS Pat He still is doing fine he is still at a slow pace no matter what he has.Hang in there Pat

As I said before we went to our 6 month check up last month and our breathing test went to a 77% vs. 83% 6 months ago. I was hoping this decrease had something to do with his cold he had been fighting. The more I check into this, the more I get concerned. Lately he has been noticing that he could be having some problems with his breathing. I wonder if it could be from the appt. and them telling him his breathing capacity has went down 6%. I have been researching early signs of respiratory distress and he definitely has some.... He has vivid nightmares, voice seems low volumes at times, and headaches off and on. Could this be our imagination after receiving this breathing test? Surely he wouldn't be having breathing difficulties at 77%? Let me know if anyone can shed any light.

Thank you!

Lucy

 

[jean]

Hello Lucy
I am so sorry to hear about your husbands breathing problems.I think you have to remember that the 77% was one moment in time and that it depends on all sorts of factors ,such as how tired he is at any particular time. It probably goes up and down quite a bit. Sleeping might be causing it to dip and so there might be a build up of CO2especially at night. Is your husband wanting to consider bi-pap,because if he is getting CO2 retention ,using it at night could helpand make him feel better during the day.It might help with the nightmares and headaches.
best wishes
Jean

 

[manfred]

pma general comment

Hi this note is "general", but especially for Brenda, Sorry to hear that you have "accepted" the obvious outcome of your condition.One of the most important tools in the fight against this illness is,DO NOT ACCEPT THE EVENTUALITY OF IT ! stay positive,stay strong and fight back with everything you have in you!The Mind is a powerful ally!My daughter was miss diagnosed several times and eventually she was told that she had P.M.A......She also doesn't like to read up on it as it would make her think of her situation...so I do the research and when I come up with something that is useful,we discuss it.If you are positive then the people around you are positive..(I know its hard to be upbeat when you have m.n.d.'s)..and positive energy helps!This is not to give you more concern butI feel that I should bring it up.In my research(I've downloaded more than2000 pages on this illness) I came across a web site that had quoted a neuropsychiatrist who stated that the worst thing m.n.d. patients can do ..is to accept their time line (they are after all different for different people)...it seems that when the mind accepts this "time line " the body will program itself to shut down within that time frame!The more one researches this illness the more one can understand it ...understanding what you're dealing with makes it easier to fight back!here are unfortunately no cures yet,but there are things that can help to delay the progression of this illness.My daughter has had this M.N.D. for close to 5yrs now..and she has progressed slowly...she does however have foot drop in her right foot..(she wears a prothesis for this when she goes out,it really works well and can be worn without it being noticeable.)She was also put on rilutek,and V-E and has subsequently combined other anti-oxidants with these..she goes to pilates twice a week and does other minor exercises given her by her therapist.....Since she started this (jan..2007) she has shown noticeable improvement,(she still tends to slur her words when she is cold or tired and has had to adjust when doing chores that require her hands ,but even to this she has adjusted well.She is stubborn ,(runs in both sides of the familly)and she's a fighter...when she was first diagnosed with pma.I asked her to promise me that she would never accept nor give up ...so we all stay positive for her(and all of us too!) always tell her to stay positive ,stay strong and fight back...........so what I'm trying to tell you is pretty much the same........DON'T ACCEPT .....STAY POSITIVE,STAY STRONG and FIGHT BACK with everything you have in you.... Manfred

 

[manfred]

general

HiI've only been on this forum for a short time and givensome of the queries and sy mptoms of some patients over their stated time frame .............I'm curious,is anyone taking any medication,exercise/therapy..if so what?Manfred.

 

[ianjudd]

hello Jean,

I am not sure if you will get this because i am new to this but my father has been diagnosed with "Flail Arm Syndrome". I live in Essex England and my Dad lives in Cambridge England and i am trying to find out as much information about this Flail Arm Syndrome. After reading your message earlier it seems that you might know about this so if you can get in touch with me i would appreciate this.

I look forward to hearing from you.

All the best IAN

 

[jean]

Hi Ian
Sorry you have had to wait a while for this message, but I haven't been on this site for a while.

Flail arm syndrome is also known as bibrachial amyotrophy and is a variant of PMA. For some people it seems to remain in the arms and not spread elsewhere and even if it does, it seems to have a longer course than most, with good walking being maintained for a long time. As with all rare variants there isn't too much in the literature about it and reseachers vary in their opinions from it being a rare variant of ALS and fully progressive , to it being a relatively 'benign' disease ,( although I personally find it hard to believe that inability to use your arms can be called 'benign'!)

Has your dad had symptoms for long and is he free of any weakness everywhere else in his body apart from his arms? I hope he still has some use of his hands and arms and so able to do somethings for himself. If he uses a computer , he might be interested in the e-mail based support group for PMA/PLS which operates via the MND Assoc in the UK and can be accessed via .

Hope that is of some use.

Best wishes

Jean