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Neuromuscular disease and lung issues

I was diagnosed with Jo-1-positive polymyositis several months ago. I am a 48YO female. My HRCT did not show overt ILD. It showed minimal volume loss at the base of both lungs, mucus plugs, and calcified granulomas scattered throughout both lungs. I am under the care of a pulmonologist, who performed another PFT yesterday. Here are my results, in part:

Fvc % Predicted: 116    
      Post Fev1/Fvc: 82    
      Fev1 % Predicted: 124    
      Fef 25-75% Pre Bronchodilator: 102    
      Fef 25-75% Post Bronchodilator: 125    
      Tlc: 145    
      Dlco: 115  

My MVV was 66%.

I had an esophageal manometry test two days ago and am awaiting the official results. The RN who performed the test said I have weak pressures, causing swallowing difficulties. I had to swallow two or three times (rather than once) to get her bolus of water down each time. The pulmonologist said yesterday that must be the cause of my intermittent SOB -- the food being stuck in my esophagus puts pressure on my trachea. I had a sleep study recently that showed very mild OSA but was remarkable for hypoxemia (in the 80s) throughout the night, even when I was awake. The pulmonologist and neurologist believe my weak chest wall muscles cause this problem when I am lying down.

My official diagnosis from the pulmonologist is reactive airway disease. I am concerned I am losing function because of my neuromuscular disease. I take CellCept and prednisone for the polymyositis. Does anything about my PFT look significant? Why is my MVV so low? Can I do anything to improve my muscle tone? I have a young child and am concerned about long-term health issues.

Thank you very much for your help.
1 Responses
Avatar universal
MEDICAL PROFESSIONAL
Hi, understand your predicament. Respiratory muscle weakness due to neuromuscular disease is best managed with BIPAP. Bilevel positive airway pressure, is similar to CPAP therapy. However with BiPAP therapy there are two different pressure settings for the patient. It can be described as pressure controlled ventilation in a system allowing unrestricted spontaneous breathing, as the pressures vary for inspiration and expiration. So, it could help improve your O2 sats. Also, this can be supplemented with oxygen therapy. Please discuss this with your doctor am sure he will provide further assistance. Regards.
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