I kept meaning to update after our last Cincinnati trip in August, but I kept waiting for more… things. But there aren’t more things. Just more questions. More unanswered puzzles.
I’m left rather unsettled and disappointed by our whole trip, to be honest.
Teddy had a sleep study. Yes, in Ohio. The local sleep centers, though initially all “oh, sure, we’ll do a sleep study on a 7 year old” changed their tune after the doctors called in the orders for the study, specifying what they wanted. And though I was pissed about it, ultimately I think it’s ok. I’d rather drive out there and get good, useful, accurate information than do it here and risk that something got missed. Also the sleep center at UI is smelly.
Anyway, so he had a sleep study. It was awful. So awful. That child has trauma issues that we need to address regarding people coming at his face with stuff. We kept having to retape the things because he couldn’t stop crying and his tears were making the tape lose its stick. And they don’t get the NASA channel and they don’t want you to use devices after x time, so we couldn’t stream the NASA channel, and without NASA, there’s really no sleeping. Sometimes there’s no sleeping WITH NASA, but it goes much better with NASA.
The results were not bad, not great. The ENT said that basically, there’s no emergency here, but it is concerning, we should do something about it, and we can’t drop the ball on this. He seems to have both obstructive apnea and central apnea. He was going to talk to the neurologist more and then we’ll chat at our next clinic about the central apnea component (essentially – his brain isn’t keeping him breathing at night).
The obstructive component – you know, he hadn’t had a sleep study before his tonsillectomy in 2013, but everyone knew he was having apnea episodes, and his tonsils were GIANT. (The tonsillectomy was because of really high EBV levels, which can cause PTLD, the cancer of nightmares, and PTLD often starts developing in tonsils, and you can search this blog for more info about that.) It was assumed that the tonsillectomy would help the apnea. It did. Some. But not completely.
The lingual tonsillectiomy in November last year was both an attempt to help stop all the croup episodes (or croup-like episodes) as well as help the snoring/apnea. I’d say the results were mixed. The doctor, being a doctor, is very certain that the reduction in the croup like episodes was entirely his doing. Because sometimes it’s hard for doctors to realize the actual size of their heads. But the fact is, he did also start the IVIG around the same time. It could be either or both.
Anyway. He still has obstructive sleep apnea and they want to see what’s going on in there now. We’re going to go back later this month for another set of scopes, see how things are now, and they’re having the surgeon remain handy in case they decide that they think they can do anything with the laryngomalacia. We’ll see how it goes.
Then they’re going to want us to do another sleep study in the spring, and another round of clinic appointments. My understanding is that we’re in for years of sleep studies, which is the opposite of fun. And probably will need a CPAP, especially depending on what the neurologist says. Which, again, is the opposite of fun.
(Teddy has trauma issues related to masks. We are going to need a professional to help us get him to be ok with sleeping with a mask. It’s really, really bad. Just sheer panic. Even when he’s really sick and needs the medicine at the ER and he can barely sit up, that child can fight the terrifying mask.)
We also saw genetics, which was a really interesting discussion, but yielded, again, tons of questions but zero additional answers. Just… maybe additional perspectives.
And then we saw immunology. Immunology was pretty sure that he does NOT have NK Cell Dysfunction because I guess it gets overdiagnosed, despite it being diagnosed really so rarely. So they wanted their own labs. Their own labs showed that his NK cells are few in number and the ones he has don’t work (golly, I’m so surprised), but they think it’s caused by some over-arching issue. But they also discovered high EBV levels, so they want to repeat labs when his EBV is zero.
But they need to repeat the labs at THEIR lab.
They were unable to tell me how they anticipate this working, because I want accurate answers and a better idea of what’s going on with his immune system, but I don’t want it enough to drive 20 hours round trip for labs. To drive 20 hours round trip for labs, on the off chance his EBV level is zero when we arrive.
They had zero ideas on that.
The rest of the appointment was interesting. They had some interesting things to say. Some interesting thoughts to share on immune suppression and innate immune function. They’re either insanely smart, or they’re insane, and that’s really the task now for me. Which one are they? They want us to see the nephrology team in Cincinnati, and we were able to add on an appointment when we’re there later this month. They want us to talk about his immune suppression.
Which leaves me with a quandary. If they recommend another course of action with regard to his immune suppression…. what do we do with that? Do I come back to UI and say, “hey, so…” Because I’m not super comfortable with asking UI to manage a medication regimen that they’re not comfortable with. Do we switch to letting Cincy be his primary nephrology team for a while? Golly, THAT wouldn’t be awkward at all. And I’m not sure I am super comfortable messing with his suppression. His (current/former – she’s on maternity now and then is moving to Ohio, but to Columbus) nephrologist had considered a few different changes in an attempt to finally get rid of the CMV, but ultimately, we kept coming back to the fact that this particular drug regimen has been *REALLY* successful. We’re six years in with NO rejection episodes, and function still amazingly good – hanging in there at stage 2. That’s really, really above average for babies with kidney transplants. Nobody wants to rock that boat.
And us transplant moms talk about how nephrologists are sometimes pretty famously against boat rocking, but in this case… it’s really hard to argue with success. Would other meds be even better? Maybe. But maybe they’d be so much worse.
I don’t know.
We’ll see what they say. And we’ll see what research studies they have to back up whatever they recommend. Hope they’re prepared, lol.