I know I sound like a broken record, but when I was a kid, it was way back in the late 60s. The only pulmonary function test they had was at the MDA clinic, and I absolutely hated it. They’re very archaic way of testing a person’s breathing abilities was that little contraption with the balls in it that you are supposed to make float to the top. I would blow & blow and never get the balls to move even a smidge! It was demoralizing as well as irritating (an understatement). When I turned 18, I told my parents I was no longer going to the clinic because I didn’t feel like it did anything for me, and I didn’t have a pulmonologist anymore until a year ago.
Although I’ve had many pneumonias, I just haven’t bothered getting a pulmonologist because I figured they wouldn’t know any more about SMA than any of my other doctors, so I wasn’t in the mood to teach someone else about my issues. I figured I would just stick with the GP. After getting in a group for people with MD on Facebook last spring, I learned that a PFT was essential for someone with muscle weakness from a neuromuscular disease, especially while I was well, to get a baseline. I researched for a pulmonologist that would listen to me and who might know a little bit about SMA. I chose a young woman who had been practicing about 5 years, thinking her age and proximity to med school might give her newer information about therapies and devices. I chose right.
When I went for my 1st appt, I planned on letting her do all the talking so I could see what kind of knowledge she had. That was difficult because I’m really used to taking the lead in all of my medical care. In her exam and wrap-up, she told me that I needed to get a PFT, and that I might need a BiPAP if my carbon dioxide levels were too high. I told her another pulmonologist had tried to get me on a CPAP instead (an idiot obviously), and she said CPAP’s aren’t the proper treatment for someone with SMA. Joy! I had someone who knew what the heck they were doing!
I did get a PFT, and my levels were not good. My carbon dioxide was around 38, and my lung function was very, very low (9 sticks out in my head, but I seem to have blocked it from memory.) She put me on a BiPAP for nighttime sleeping, gave me a suction machine, and a cough assist (those last 2 were my request), but I wasn’t able to handle the BiPAP. I think I just don’t have it in my head that I need it yet because I don’t have any morning headaches, sleepiness, or feelings of exhaustion. My psyche would not allow me to get the whole breathing rhythm thing down, so I gave the machine back and told the respiratory therapist that maybe I would try again in the future. She didn’t look very happy, just shook her head, and packed up the machine.
I know my Dr. is going to want an explanation for why I’m not doing it, but as with so many other things, she’ll just be one more in a list from whom I’ve ignored instructions. I’m not saying I’ll never do it or that it’s a stupid idea. I’m saying I’ll keep the possibility in my back pocket for future consideration when my breathing gets really bad. Unfortunately, I do realize that this method of dealing with the problem is probably going to cause me to bypass the BiPAP and go straight to the vent when the time comes.