Wednesday, June 7, 2017
--> Today I have an appointment at the cystic fibrosis clinic in the hospital where I had my lung transplant. I haven’t been to a CF clinic in at least ten years. And previous to transplant I only had one CF doctor my entire life, from my diagnosis to his death. Dr. Thomas Doland at Yale. He died shortly after my transplant. And I never saw a need to find anyone else. I belonged to my transplant team at that point. The interesting thing about having a lung transplant is that, for me at least, you sort of leave the CF camp and move to the transplant camp. Of course, I still have CF, and of course it still affects so many things, but my lungs no longer have CF, so it kind of eliminates the need to be followed continuously by a CF doctor I guess. Instead of seeing a CF doctor, I see my transplant doctor and a million other specialists—sinus ENT, ear ENT, cardiologist, several endocrinologist, kidney specialist, gastroenterologist, infectious disease and many more I can’t even think about. The roster is long—each doctor taking care of their specific organ.
But now my ear has really brought CF to the forefront again. There are several reasons I can’t get rid of this infection, but there is only one reason I got the infection to begin with, and that is cystic fibrosis. The pesky dumb aggressive little bacteria that is in my ear is pretty much a bacteria you can only grow if you have CF, a healthy person could lather in it; it wouldn’t do any harm. It’s the same bacteria that ate away at my CF lungs time and time again. And it’s the same bacteria that like to wreak havoc in my sinuses, and then found its way into my ear. But the point being CF has again found a way to rule my world, even though transplant is supposed to somewhat alleviate that from being the case, and trust me it did, I’ve been on IV medications, a rotation of three different antibiotics and one antifungal since December 19th. I’m over it. And like all my ENTs like to say: we can’t transplant your _____ fill in the blank with ENT things. We have all done everything we can but we need a forever solution.
Since my transplant there have been a lot of advancement in medications that target the actual problem with CF—we can’t transport fluids and sodium properly in and out of our cells. This is something your body needs to do to be a normal functioning healthy person. There are two medications available now that try and correct the actual defect in the protein that causes the problem. My lungs don’t have CF so in essence I wouldn’t really benefit from a cure for CF, I now need a cure for transplant rejection—this is now my terminal disease. I hope this makes sense. So back to the ear problem that has led me here! These two medications Kalydeco and Orkambi COULD help my ear! So a few weeks ago my genius sinus surgeon told me he has seen big improvements in the sinuses of CF patients on these medications. I told him I didn’t even know what my mutations were.
So here I am a mixed jumble of emotions. I know I have a genetic disease. But it’s a different thing to actually find out specifically what mutations I have in my gene. I am entering back into a world that I haven’t been to in so long, even though I suppose it’s been there in the background the entire time. There are a lot of mutations and a big chance I don't have the right one, of course I’m scared of disappointment here. But this is the way medicine is headed and it’s thrilling to be honest, even if it doesn’t exactly mean a cure for my own lungs and me. The drug pipeline is exciting (https://www.cff.org/trials/pipeline)
There is more to it though and I’ve been trying to understand why I am SO emotional about this experience. And it’s because I got these CF mutations from my parents—neither of them alive anymore, neither of them with me. My mom should be with me for this. She should be sitting with me in the exam room like she always was before she died. I’ve never even seen a CF doctor without her and this is bringing it all back, everything we went through together my entire life. And it’s a visceral punch to my stomach. At the end of the day this loss is always circling me. The progression of life, these sorts of milestones, will always make the big losses slowly burn again, the ache roaring back. But I have to keep going and I have to keep facing these things and luckily I don't do it alone.