PMA progression?

[Lena]

Hey, I'm not seeing much here on PMA. I have voice and spine and respiratory involvement, misdiagnosed as Bulbar Onset ALS, then PMA in November 2020. Progress seems relentless; though I remain coordinated I am barely able to stand and can hardly talk. It started with twitching and ridiculous cramps in 2018 but those are rare now.

I don't think I'll last much longer.

What about you?

 

[Nikki J]

Welcome. Sorry you had to find us.

I think PMA is sometimes called by neuros LMN dominant ALS for insurance / ssdi reasons I am not sure how many here carry the PMA label. Some of us are LMN dominant. I am but my progression has been slow and started in one leg. I don’t have bulbar signs yet and minimal respiratory involvement so far.

I look forward to getting to know you

 

[Lena]

ya, quite a club! it's good to meet you, Nikki, I've read some of your posts, very helpful. glad your progression is slow. weird how many different manifestations there are. I may have had it long before obvious symptoms. I was a dancer but also disabled with rheumatoid arthritis, so I have a long history of adaptation.

Am I understanding that you have considerable ALS in your family?

Elena

 

[Nikki J]

Hi again. Yes I am FALS. The sixth out of 7 affected in my lifetime.

 

[Lena]

I'm sorry for the abundance of horror. does it help you to be active here? it seems like there are so many people wanting to be relieved that they don't have ALS. a little hard to take.

I feel so so so much weaker in the evening. is it like that for you?

Lena

 

[Mon]

Hi Lena

My dad is LMN dominant. He has a regional subtype of PMA called flail arm. Its started in left arm, 1 yr later went to right arm. He is over 2 years from onset now. His shoulders are completely atrophied. Id say it got much worse past 6months versus the first 1.5yrs. Hoping he is plateauing a bit now. My dad gets weaker as day progresses for sure. Physiotherapist suggested doing most appointments or things that require more energy in morning when you are fresh.

 

[Lena]

Thanks, Mon. yeah, I treasure my mornings. My arms and shoulders are pretty weak but the worst is my neck and spine. I started with bulbar involvement--voice, breathing, and they said I had bulbar onset als. but I'm at 3 yrs post onset. Before this I had rheumatoid arthritis and osteoporosis so the total disability is pretty bad, but I was a dancer and adapter so I manage a lot. my legs are ok but balance sucks when my spine feels like a garden hose holding up a bowling ball. I probably need a good brace but anything medical is aversive to me now.

How are you and your Dad coping with it all?

 

[Mon]

Im so sorry to hear Lena but thats good you can manage and adapt. You kinda have to figure it out right? So you started with bulbar onset? And the other areas you listed have since become involved? I actually havent heard of bulbar onset PMA.
We are managing, one day at a time. He often feels tired. His arms feel heavy when he is walking. Its frustrating for him, those are his words today. He wants to do more and just cant. He cant lift his arms too high at all. In past 6months, cannot drive and needs help showering. He needs help opening a bottle, pouring a drink, needs to use straws- cannot lift a glass. He can still write, type and feed himself( he lowers his head towards to food because he cant bring utensil to his mouth. Im doing ok, but it hurts to watch him lose abilities. He has a slow progression, so thxful for that.
I very sorry you are also dealing with this illness. Sending good vibes your way.

 

[Mon]

My mother in law had RA and i know first hand how disabling that is, but to have PMA/ LMN ALS too, is just unfathomable. So sorry.

 

[Lena]

your validation and support mean a lot to me. deep thanks!