Respiratory Disorders Expert Forum
respiratory muscle weakness
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respiratory muscle weakness

I have been dx'ed with mitochondrial myopathy.(a neuromuscular disease) It has affected my respiratory muuscles with weakness documented by my pft's. My question is once you lose respiratory muscle strength- is there a way to strengthen these muscles? My pulmo doc is very concerned and is following me with pft's. I also have asthma for which I take advair and albuterol prn. But I cannot tell much difference. Do you have any suggestions that will help strengthen or prevent further weakness? Thank you!!
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251132_tn?1198082422
There have been no specific breakthroughs as far as I know.  For patients with specific gene defects there are some places that are working on gene therapy approaches.
In general the approach to therapy is:
Avoid triggers, such as fasting, exposure to cold, alcohol, tobacco, sedatives or anesthesia, and infections.
Stress can also worsen the disease.
Pacing and the avoidance of overexertion are important.
In patients with CPT deficiencies, a diet high in carbohydrates may improve the disease by shifting metabolism toward glycolysis.
A modest exercise program (60-80% of heart rate reserve) may benefit patients with mitochondrial disease in two ways. First, it prevents deconditioning, which worsens preexisting exercise intolerance and fatigue. Second, exercise may have a direct effect on the population of mtDNA within the muscle. Regular aerobic exercise improves muscle metabolism.
Treatment with riboflavin or coenzyme Q has been tried.

While reviewing the literature I found a good article you may want to read.  You may also want to contact the author, whose address is included.
Authors
D'Souza GG. Weissig V.
Institution
Department of Pharmaceutical Sciences, Bouve College of Health Sciences, Northeastern University, Boston, MA 02115, USA. dsouza.***@****
Title
Approaches to mitochondrial gene therapy. [Review] [94 refs]
Source
Current Gene Therapy. 4(3):317-28, 2004 Sep.
2 Comments
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Grace, Grace and more Grace. You have the name and numbers right.  What ever the out come, you sound like your OK.  So sorry for your illness.  My question is right under yours - I think we were both overlooked.
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