After years of waiting, we finally have Trikafta in hand. After all that time, and given how expensive the drug is, I had expected the last step to be more of a hurdle, but instead it was totally seamless. Our doctor wrote the prescription, I signed some paperwork to enroll in Vertex's patient support program, they contacted me and found out what specialty pharmacy we wanted to use, and bam. A couple of days later the medicine showed up.
Lemon has been a champ about taking it so far. The main challenge is that it has to be taken with some kind of fat-containing food. At night, it's no problem, because he is about to do a tube feed so even if he doesn't eat much dinner, we know the formula will take care of it. In the morning, though, he does have to eat something, and for those of you who have been following along for any amount of time, you know that Lemon and "eating something" are not always concepts that go well together. But, we have found that he is usually willing to at least drink a glass of whole milk, so smooth sailing thus far.
I have to say that at least up until now (we are still only on our second week) I haven't noticed any marked effects, either good or bad. I feel like the big results for us will come when Lemon goes back to in-person school in the fall. It is my greatest hope that the Trikafta helps him weather all the colds and other viruses that will undoubtedly be circulating in great abundance once everyone is back together again, so that he won't be constantly sick. So, we shall see.
Meanwhile, the summer has gotten off to a good start. The kids have had their first couple of weeks of camp, which have been pretty successful. I am definitely feeling good about the decision to just send them for half-days, that seems to be all the structure their little minds can handle just yet, and they like having their afternoons at home to bop around. With any luck, they will eventually warm up to the new (very nice, perfectly competent) sitter that we hired for the summer.
Lemon is participating in a big study of the effect of Trikafta on kids. We had the first study visit the week before he started the drug, and I was pleased to find out that he was our hospital's very first research subject for the study. They hand-picked him because he has done so many other studies that they thought he was their best shot at having _their_ first attempt at this one very big, complicated study visit go well. And, he did not disappoint, he was amazingly cooperative, and since almost all the tests were familiar to him, he didn't balk at anything and we were "only" at the hospital for 4 hours, as opposed to the 7 projected in the study protocol. They took samples of pretty much every possible bodily fluid, did multiple different types of breathing tests, and trained us on a home spirometry device so that we could do even more breathing tests at home. We have to go back in another couple of weeks to do the next visit at one month after starting Trikafta, Unfortunately we won't get to see any of the data until the study is published, but Lemon is still very proud to be one of only 150 kids from around the country who are participating in this.
To top off a generally excellent month, we were also treated to a visit from some cousins who live in Maryland and haven't seen in person since the summer Lime turned 2. It was wonderful to have them with us for a few days, the kids had an uproariously good time, and cried when their cousins left to continue their road trip. We can't wait to see them again, and to be reunited with everyone else that we haven't seen in far too long.
