Tomorrow is a big day, the first day of the new school year, with Lime off to 4k and Lemon starting first grade. At least based on our clinic visit Wednesday, I feel like we're in the best position we could hope for to be taking on this new challenge. Over the past 3 months, Lemon has grown an inch and packed on 5lb. Amazing what 4 cartons a night of Boost Kids 1.5 can do. He definitely has the on-board reserves that we wanted him to have going into the school year, and now all we can do is keep our fingers crossed that it is enough to give him the foundation he needs for winter.
Lemon also blew his best-ever PFTs. I don't ascribe much clinical meaning to the numbers yet. At this stage I think the values are all about how well he cooperates. But, this time, in addition to the regular PFT machine, he was offered the option to try another type of test where you sit in an enclosed booth. He really wanted to try it, so he gave the regular PFT his absolute all, and the results showed.
One interesting thing that happened was that, as I mentioned on a previous episode, Lemon had a liver ultrasound over the summer. The notes that we got back electronically said that the ultrasound and elastography read as normal. So, I had expected that the GI doctor would say to try to taper the urosdiol, since that was the plan we'd agreed on if the bloodwork and the imaging looked good. But, instead, what the nurse practitioner said at the appointment was that the ultrasound showed mild fibrosis and that we should continue the ursodiol. I think I was too surprised in the moment to ask any questions, but I did schedule a follow-up with the GI doctor (for January, his next available, sigh) to try to figure out what's going on. Somehow playing "telephone" with a combination of the electronic health records and the nurse practitioner in the middle isn't working.
My other big goal for the appointment was to begin the process of getting Lemon enrolled in the clinical trial for the new drugs that treat the underlying cause of CF in people with his mutations, since there is a study site in Minneapolis. Our clinic was very supportive and reached out right away to their contacts in Minneapolis. Unfortunately, we got some bad news on that front--the Minneapolis site is already fully enrolled. We did get our names put on a waiting list, but the study coordinator there said our chances of getting a spot are essentially zero. The other sites are too far away from us to be practical. So, sadly, it looks at the moment that we won't be able to participate in the trial after all.
Still, it is great news that the site is already fully enrolled and that the trial is moving along at a good pace, since ultimately the goal is just to get the medication approved by the FDA so we can begin using it. Maybe sometime in the next couple of years? We can hope!
