respiratory muscle weakness

[grace77]

I am 42 yrs old and have been dx'ed with a myopathy( probable mitochondrial) with associated proximal and proximal muscle weakness. My pft's show a restricted pattern with MIP 25 % of predicted and MEP of 21 % of predicted. I experience air hunger and dyspnea on exertion. The myopathy is thought to be a progressive disease. I am worried about further decline in my resp. muscle weakness. My dlco is also reduced. I follow closely with my pulmo. doctor. I also have documented poor exercise tolerance on my stress echo. I have mild cardiac involvement with EF 45%, mild lt atrial enlargement. My sats are always good.
In your experience, what are the sxs of further decline in respiratory function that I may experience? It is difficult at times to lay supine due to dyspnea.
Thanks for your help.

[Sunny602]

I just want to tell you that I know what you are going through. I too have respiratory weakness (failure) due to a myopathy (distal with diaprhagmatic involvment- said to be rare) I think that everyone progresses at different paces...I happened to progress quickly. I too had a very difficult time sleeping with my bed flat, I slept with 10 pillows. I ultimately was on oxygen as well as a machine called BiPAP to give me extra support. The results that you give in your PFT's resmembled mine, I got chills reading them. I was just like that. In fact, my low MIP,MEP is what made my docs think neuromuscular instead of asthma, which is what they origininally thought. How long have you been this way? Years or has this happened very quickly? You also said a mito disease, I bet they have done a muscle biopsy along with other icky tests on you...I get EMG's done at least once a year to track my distal muscles.
Like I said, everyone progresses at a different pace, my case is very rare and most neuro diseases do no progress as quickly as mine did, I hope that I do not scare you as to what I am going to say next. Within two years of being symptomatic, shortness of breath, especially with stairs, hard time walking, lying flat was impossible, my oxygenation suffered., and then living on BiPAP..I finally progressed to the point to where I had to make a decision...either live on the BiPAP 24/7 or become ventilated. I chose the later and am okay with the decision. I am able to breathe finally, although it is with the help of a mechanical unit. I do have a trach..I get out in the world with a wheelchair and do most anything a "normal" person would do (yes, I do get stares)..Again, I don't mean to frighten you, I am just telling you what I went through personally. If you want to email me, let me know, I will be happy to do so.Just so you know as well, not everyone with respiratory weakness winds up on a vent, just some support might be needed at times. I know people who have diaphramatic issues, they get short of breath at times but for the most part they pace themselves and avoid doing things that make their breathing worse- they also don't usually wind up on a vent.  I am thinking of you...sunny

[knightshotter]

A related discussion, respiratory weakness secondary to pulmonary was started.

[Sunny602]

It took a long time for a diagnosis...alot of it was see and wait because they really could not find a concrete answer. They monitored me closely, every 3 months with PFT's, abg's and yes, went through muscle biopsies as well as EMG's. The proximal biopsies weren't conclusive but the distal was. The EMG's have definitely changed over the years. Amazingly, my initial EMG's and neuro tests were almost normal, just my PFT's and respiratory status was affected. I literally had to go on oxygen, I had been working full time a short period before being put on O2....what a shocker. You also may email me...***@**** like we have things in common. Grace, I will email you as well. It is always good to chat with someone else who is going through what you are. Sunny

[National Jewish]

With progressive disease, you are likely to experience further limitation of exercise tolerance, with associated increased shortness of breath for a given degree of exercise.  You may also experience difficulty with mucus clearance and be more prone to lung infection.

The air hunger might be relieved by the use of assisted ventilation.  You might want to discuss the options for non-invasive assisted ventilation with your pulmonologist.  During that discussion you might also ask about respiratory maneuvers, such as 'huff coughing', and simple respiratory devices that can assist with mucus clearance, such as the Acapella or Flutter Valve.  Another, much more expensive but effective device used primarily by persons with cystic fibrosis is the Vest.

Finally, try to remain as physically active as you can and work with your doctor to develop a regular exercise program.

[grace77]

Feel free to email me and I can give you the specifics. I also have a low DLCO.Looking  forward to hearing from you and Sunny.

[maggiemag]

Well, well....very interesting!  I, too, have recently been diagnosed with some sort of myopathy.  I already had cardiomyopathy since 2002, and now it seems I have a generalized type, which is affecting my respiratory muscles.  I too get SOB on exertion which was out of proportion to my heart failure.  After a couple of years of PFT's showing a restrictive pattern and low DLCO, and having been on oxygen at night for about 3.5 years, I finally had MIP's and MEP's done which show, I think, about 50% of normal for my age.  They think I also have generalized skeletal myopathy too but will need further testing.  How did they diagnose you guys, EMG's and muscle biopsy and what else?  I also choke now and then which I hope isn't related!  My question is how fast will it progress?  Grace, you say your sat is always fine?  Have you had an overnight oximetry?  I also need oxygen when I fly which is a major pain in the neck.  I have been told that I will need CPAP at a minimum eventually and maybe bipap.  I just know that if I go out to a party, for instance, and laugh and talk a lot, that occasionally I feel like I can't breathe, that I need a vent.  It is scary, and I need to learn to pace myself better.  May I email you too sometime?

[grace77]

I also wanted to add my ERV has decreased significantly and is quite low now which my doctor says reflects the muscle weakness also.

[grace77]

I would love to email you. My email is ***@****. Climbing stairs is horrible for me as far as dyspnea and tachycardia. Email at your convience. Thanks!