respiratory weakness secondary to pulmonary

[knightshotter]

Like others who have posted here, I have a progressive neuromuscular disease in my case progressive relapsing multiple sclerosis. I began having diapraghmatic weakness documented with flouoscopy and sniff test. i am on oxygen 24/2, very short of breath, at rest and at exertion, have 02 sats even with oxygen that often drop into 80s. PFTs show low DLCO and mip and MEP test 20% of normal.

Pulmo and neuro don't know what to do or have much to offer. Have never treated a patient like this. Another pulmo suggested a vest for breathing but didn't know where to get. Asked me to find. A different pulmo suggestd ventilation last year and i got scared and changed docs.

I have rheumatological involvement with inflammatory arthritis. To date, just worked up for lupus, sclerodoma, etc. and have some attributes of many but not all.

With my docs clueless, I feel the ball is in my court to find more knowledgeable doctors. Current docs seem to take the path that I will die eventually from choking or hypoxia etc. Where should I look for a vest to help with respiratory (I saw someone with a post polio vest) and where do i turn to decide when need to vent.

Current pulmo put me on bipap but it was extremely painful to wear and the muscles fatigued in diaphragm way too fast and doc acted like i was non compliant but had no solution i could comply with.

I was diagnosed with MS in 2004 at age 42 and I'm now 47.


This discussion is related to respiratory muscle weakness.

[Jenn12546]

Have the doctors considered BiPAP or some other non invasive ventilation methods (the curass)? I too have diaphragmatic weakness, have had this for 7 years now. The docs do not know why this happened to me, they have many theories but the pieces don't all fit to one specific problem.
Anyhow, over the years, I did get weaker...found that my sats and PFT's got progressively got worse. I too had O2, but ultimately BiPAP was added to the mix to help rest my muscles (that worked well). I am currently vented with a tracheostomy, have been for 4 years now. It has been a tough journey, but I feel that without the vent, I would not be here.  It was one of the hardest decisions I had to make in my life.
You sound like me in some respects as far as traveling to see docs. I too live in a very rural area and have to travel hours to get to university doctors. Thankfully, I have a wonderful team who looks into every option before going forward with a plan.
It sounds like you need to fish around to find a pulmo who is familiar with muscle weakness and its treatment. A neuro who partners with him or her would be excellent as they would be conferring with each other regarding your treatment. I guess forming a good team that actually talks to each other about your treatment is what I am saying so that everyone is on the same page.
If you have any questions please email me.
Jenn

[knightshotter]

Well I did plan to post a question on the doctor's forum but I guess I was surprised that there would have been a fee associated with posting there.

I have a "really good pulmo" specialist who has a little experience with MS. I previously saw another good pulmo but had no experience with MS. I am in a very rural town and even though i see a university respiratory team, there were no docs with specialty experience with respiratory weakness secondary to MS. As it is, I travel either 1 hour or 2-3 hours to see docs at the university hospitas but none have the expertise that you suggest.

The vest that you are talking about is indeed primarily for someone with cystic fibrosis or who needs help dislodging mucous. This is not the problem for me. I am hoping that some sort of vest/brace would help support the muscles so i could sit up straight and take deeper breaths. My breath is very shallow. The vest that you are suggesting made no difference. My neuro doc suggested i try and find a resource for such a vest but i don't know where to look.

[Victoireh]

I'm sorry to hear that your MS has made your quality of life to miserable. The only chest vest I have heard about are the ones used in physical chest therapy aimed at helping to dislodge the excess mucous from the lungs. I assume then that this vest would assist the diaphragm muscles to get the air in and out of your lungs. Have you tried posting your question on the doctor's forum side, to see if they have any suggestions?

http://www.medhelp.org/forums/Respiratory-Disorders/show/128

Also try to find a really good specialist who is very familiar with MS. I have asthma and had to switch pulmonary specialists, as the first one was not really on top of people who present atypically. I now see a specialist who works with about 12 other doctors of the same specialty, and they are well versed in all things to do with the lungs. But don't give up on trying to get the best treatment for you.