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90270 tn?1199334469

Muscular dystrophy and CF

Hello, thank you for your time in advance. This question is more out of curiousity than anything as I have never read a case report like this. I have had neurological issues since 2000, at that time my respiratory function also decreased,low MIP and MEP. Prior to my problems, I worked full time, am asthmamtic and had frequent exacerbations with pneumonia. I was put on O2 at that time(2000) due to hypoxemia, 24/7. Since 2000, I have had two proximal muscle biopsies, no real abnormality (no glycogen storage disease, mito or myotonic issues). My resp. situation declined rapidly, My PFT's showed restrictive/obstructive pattern and ABG's worsened. Needless to say, I was vented in 2004 due to severe resp. failure, and am chronically so at this point. Now comes the interesting part. I have developed pancreatic insufficiency, have diabetic issues, grow B. cepacia, P.A and steno, sometimes Staph. Sweat tests borderline high, Cl greater than Na and I have frequent pneumonias requiring IV's about every 3-4 months.I see a CF doctor,he treats me as a CF patient.I also have distal myopathy (EMG),just what kind is a mystery,I am to have another biopsy (distal) soon to help diagnose the neuro problem. Now..my question is, have you read any case studies that involve a person with CF that also has neuromuscular disease (myopathies specifically)?I do know that certain illnesses can elevate the sweat tests..I have been trying to tie myopathies with CF to no avail.Genetics done on me are inconclusive. Needless to say, I am a medical mystery. Any light shed on this would be appreciated. Thanks!
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251132 tn?1198078822
MEDICAL PROFESSIONAL
I could find no reports of a relationship between cystic fibrosis (CF) and neuromuscular disease, including myopathy.

People with CF are likely to have lung infections.  Common causes of these infections are B cepacia, Pseudomonas aeruginosa and Staphylococcus.  It is also common for people with CF to have diabetes.  It is thought that scarring causes the loss of beta cells in the pancreas.  This is one of the principal causes of diabetes in people with CF.
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90270 tn?1199334469
Note: I am 34 years old, female.
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