Back in August, I introduced you to my fellow Pompe warrior, Brad Crittenden. Recently, Brad shared some very exciting and encouraging news with me and has agreed to let me publish an update about the progress he has made since starting enzyme replacement therapy (ERT).
Because Pompe can attack the diaphragm and other muscles needed for breathing, patients undergo pretty regular pulmonary function tests so the doctors can monitor just how low our pulmonary function is getting and to let us know when we’ll need to go on a ventilator or other assisted breathing device. Or at least that is how the story went in the past, before ERT was available to us. Now, Pompe patients are tearing up the old progression path of Muscular Dystrophy and paving a new path. Some of us are getting better and stronger! Brad is one of those people who is showing improvement after only a few treatments!
Here, in his own words, is Brad’s update:
A funny thing happened the other day. I had a pulmonary function test that was actually better than the previous one! I honestly can’t remember the last time that happened. In the world of Pompe, we get used to the disease chipping away at us. This time, that didn’t happen. What changed, you might ask? Three months of enzyme replacement therapy!
I had been eagerly waiting for this test. Each time I went in for my infusion I was feeling a little bit better. The last couple times I was able to recline in the chair and still breathe, which I couldn’t do before. Even my nurse has noticed that my oxygen is better than when I first started ERT.
I arrived early for the test. Okay, I was a lot early but I was feeling pretty good about this! First was my volume. I knew as soon as I saw the first number that it was going to be a good day. I was up 7% and I’m thinking, “Holy Smokes, that’s even better than I was hoping!” It’s not as good as I was last fall, but sure better than 3 months ago. Then we did my inhaling and exhaling pressures. I was blown away (pun intended!). One was the same as 18 months ago and the other was even better.
We had one more test, my volume lying down. This is the one that I really don’t like because I just can’t breathe enough. Last time it was only 27%. Since I use a bi-pap when I sleep, I had no idea what to expect. So, I’m flat on my back and I do the first blow. The tech asks me if I’d like to take a break between the three reps and my inside voice said, “Heck no, you’re not getting me down here again!” My outside voice said, “I’m okay, I can do it”. So I do it two more times and sit up with anticipation. I got 39%! Now if I were in college, 39% would not look so good. To me though, it was like a home run! That means that my lung volume when lying down is almost half again what it was three months ago. That seems like pretty good news.
So now I’m confused. I thought the idea of these tests was for us to be depressed after doing them. I mean, I’ve been practicing that each time and now this happens. I’m going to have to change my whole outlook, but, I think I’m willing to do that. Love that the graph looks kinda like a hockey stick!
Thank you so much for sharing this inspiring news with us! I hope Brad’s progress gives you that extra bit of hope you’re looking for to keep fighting! The stuff (ERT) works! It really does!