This past June was my 7th anniversary of having my ventilator, my BiPAP, which I rely on for breathing support. A ventilator is sometimes used by patients with generalized myasthenia gravis (MG) who have weak respiratory muscles. It’s different from a CPAP, a much more common breathing support device.
BiPAPs provide both inspiratory and expiratory pressure support. Because breathing difficulty is related to diaphragm weakness in MG, not the lungs, patients with MG need support for both inhaling and exhaling. Getting my BiPAP 7 years ago made all the difference for me, and I don’t know where I would be without it. I use my BiPAP 10-15 hours daily, all night and throughout the day. It is even more difficult for a weak diaphragm to function when lying down, so sleeping with it on is crucial.
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Before I had my BiPAP, I had constant insomnia because I couldn’t breathe well enough to sleep, which, in turn, only made me weaker and made my condition decline faster. My form of MG is atypical. I had difficulty breathing very early on as my disease developed, and it is still a symptom I struggle with all the time. I had no idea why I struggled so much to breathe initially, but doing multiple spirometry and pulmonary function tests years ago was how my pulmonologist discovered that my diaphragm is extremely weak.
My lungs are in great shape, yet my respiratory muscles are frail. That is part of the cognitive dissonance of neuromuscular disease.
Once I got my BiPAP, I couldn’t believe how much it helped; however, I initially found it challenging to sleep with it. That is common. It can take time to adjust and find a tollerable mask. I always recommend to others with MG to be patient and not give up on finding ways to tolerate it and be able to sleep with it on. It took a couple of tries with different masks to find one comfortable enough for me. I have a respiratory therapist who works for the company that owns my machine. Over the years, the company and my respiratory therapists have been great sources of support and guidance with using my machine successfully. I rely on them for advice and adjustments to my machine.
I have since completely adjusted to wearing it at night and throughout the day. My BiPAP is so much a part of my and my husband’s life that it’s like a family member. My husband does the care of cleaning and managing it. Whenever I have to travel for appointments or if we get a rare excursion for fun, it always comes with us. I even use it in the car. It’s been with me every day these last 7 years. The only times I did not have it were my last couple of hospitalizations the last few years. I was not allowed to have it with me because of the pandemic and had to rely on the hospital’s BiPAP. Using it every day is just a normal part of life for me.
My ventilator has helped me through countless respiratory flares and MG crises. It helps me stay out of the hospital and have the energy and strength to participate in life as much as I can. It’s an essential medical device that can help patients with more serious, generalized forms of MG. Respiratory weakness is the most serious symptom of the disease, and it’s essential patients with MG have support in helping to manage this symptom. A ventilator can really make a difference for some patients.
