I don’t even know where to start. Let’s call this post “We’re missing something.”

We’re missing something.

At least one something.

Here’s a general update:

  • Croup. Teddy continues to get croup monthly. His regular Otolaryngologist decided that he’s above her pay grade at this point, so had us see aerodigestive at UI. (and we want to see aerodigestive at Cincinatti – and I think we’re still headed that direction – but I don’t regret going ahead and seeing them at UI.) That doctor did an in-office scope on Teddy and discovered a few things. a) his left nostril dead ends. That seems like a big thing to miss for 6 years, eh? That’s going to be a new thing to tackle but not just at the moment.   b) his airway is just awful looking. Very angry tissue.  c) he didn’t see any stenosis.

    So what the heck? Now he has no stenosis? Did he outgrow it? Maybe. But there goes the “he has subglottic stenosis so that’s causing the croup” theory.  (which means the “he probably needs surgery to correct this” treatment plan is also out the window for the time being, which makes me happy.)

    He thinks his airway looks like severe reflux. Even though reflux was ruled out by a pH probe. Even though he’s been on reflux meds before with no noticeable difference. Even though, even though, even though. And this isn’t the first time we’ve heard it, and it was even a theory I had. Even after the pH probe (because they aren’t 100%). So I’m on board with this as a working theory. We’re trying a PPI again and we’ll see. He said to do it for 2 months and if he hasn’t had croup in that time, we can figure it’s working, but I’m assuming that we’re also going to do another scope. In the meantime, after 10 days on the PPI, he got croup and ended up back in the hospital, so….

  • Transplant is concerned that he’s getting sick that often – and then I told them that this doesn’t even include when he’s sick and doesn’t get croup. I don’t even keep track of that. We’ve been working with a new therapist for about 8 months now and she said this week that she has not seen him NOT sick – and she comes weekly or every other week. He’s sick ALL THE TIME. People want to know why we never go places and do things, it’s because Teddy’s ALWAYS SICK. They’re concerned, so they had us see immunology. Immunology basically wanted to run a bunch of labs, but based on the labs they’d run the last time they saw him (pre transplant when he kept getting line infections), they had an idea of what they’d see and had worked out a treatment plan that we’d proceed with when the labs came back looking as expected. The labs have come back. They’re not as expected. I don’t have the first idea what they mean, because immunology labs are HARD, man. But some things are WAAAAY of kilter.
  • Genetics. Meanwhile, I requested to see genetics again. Last time we saw them, he had far fewer diagnoses and they were really just looking at liver things, or liver/kidney things, mostly to rule out big things that could affect transplant. They never did any general overview stuff. He has SO MANY diagnoses right now, it’s hard not to wonder if something doesn’t tie a lot of things together. I have a few theories from Dr Google, and I think they’re good theories. We’ll see what they say. Microarray came back normal.
  • Weight/eating. He recently started a new med that has, as a side effect, appetite suppression. He has stopped eating by mouth almost entirely, and he’s lost a not insignificant amount of weight. We’re trying to stop that weight trend by increasing tube feeds, but I think we have to increase them more than I realize, or else something else is going on.
  • CMV He continues to have elevated CMV levels. He’s been on a treatment dose of Valcyte for a month now and the level’s finally gone down. He’ll be on treatment dose for another month, then down to a prophylactic dose for a while. Again. Me = not a Valcyte fan.
  • There’s just something else. Something. He’s struggling. And I don’t know what it is, what we’re missing, how we’re missing it. I’m hoping Genetics, neuropsych, immunology, and Aerodigestive can suss this out. If it doesn’t happen on its own, and if nobody has any major breakthroughs on their own, I’m going to ask if everyone – neuropsych, transplant, genetics, aerodigestive/ent, immunology, and either letters from or conference calls with OT and possibly his BHIS worker – can get in a room together and share ideas. We have so many pieces. So many. How do they fit together?

Feeding Tube Related Issues

So, Teddy has been complaining of pain from his button since fall. When we were in Iowa City for another appointment, I asked if someone from GI could pop over and measure his stoma to see if he needed to size up his button, figuring that would be an easy answer. And as it turned out, his button was quite small and he needed to size up quite a bit.

But the pain didn’t stop. So fast forward to January and I asked again if someone from GI could see us for an actual appointment this time, while we were in town for another appointment. So the same doctor saw us.

And he said that Teddy was faking pain for attention.

And we’d had such a bad day, and this was a the end of that very bad day, and I just didn’t have any “what is your actual problem, you big giant jerk” left in me that day, so I said essentially, “well, that doesn’t seem right, but fine, whatever” and left. (Honestly, the nurse brought me a free parking coupon because that is how beat down I looked. And felt.)

Anyway, so I made an appointment with a GI locally. I didn’t even care which one.  (Not Dimeo, who we’ve seen before, because evidently he won’t see you if you’ve also seen another GI doctor, and I don’t put up with that crap. Sorry, but you don’t get to boss me about how many professional opinions I get to seek.)  Anyway. I see Peds GIs at this point as completely fungible and essentially worthless, so I decided I didn’t care who we saw. We ended up with some gentleman originally from India who works in the Mercy Subspeciality Office.

Miracle of miracles, he listened to what I had to say about the pain, he asked Teddy about the pain. We discussed the possibility of referred pain from whatever’s causing it to hurt when he pees. We discussed the possibility of constipation (and ruled it out). Discussed what he felt was more likely – evidently you can develop abdominal wall pain from a Gtube that doesn’t really have any specific cause. And I can see that, I guess. It seems like the standard GI cop-out answer, but we’ll accept it as a possibility. He discussed imaging – ultrasound to look at abdominal wall for anything out of the ordinary, and another set of scopes. We’re not going to do scopes. It’s not that bad to warrant all that. But we’re going to do ultrasound. And if he needs another CT or another set of scopes for anything ELSE, then we’ll think about adding on looking at this gtube from inside if he’s still having pain. The doctor is also going to check with their surgery clinic to see if they have any thoughts, since surgery is who mostly handles Gtubes at their clinic.

It was nice to be listened to and taken seriously.

Viral Issues in Transplant Recipients

Just an informational post because the husband was having trouble coherently explaining this. 🙂

Solid organ transplant recipients obviously take immune suppressive drugs and that makes them more vulnerable to essentially everything that the immune system would usually fight. Viruses. Bacterial infections. Cancers.

A handful of viruses in particular tend to cause more trouble than others – mostly, the type that hang out in your body. A typical person is exposed to these viruses, often doesn’t develop any symptoms – or just mild cold symptoms, and then the virus lies in their body, dormant, for all time. Sometimes, even in healthy people, the viruses can be reactivated. Transplant recipients, because of the suppressed immune system, are more prone to viral reactivation. Also, if they weren’t exposed pre-transplant (obviously more common in kids than adults), their first encounter with one of these viruses can be troublesome.

This article gives a nice run down, if you’re interested in more detailed information using longer words: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3152274/

We monitor Teddy for three of these viruses on a regular basis (technically, we monitor viral load). Epstein Barr Virus (EBV), Cytomegalovirus (CMV) and BK Virus (which doesn’t actually stand for something – it’s just the initials of the first guy who ever had it).

CMV. I don’t know what to say definitively about Teddy and CMV right now because we’re not entirely sure what’s going on. It looks like his CMV levels go UP every time he has another illness. That’s not ideal, but as long as it comes back down every time, it’s better than not coming down. One of the things that was going on when he was admitted in the spring was that his CMV level was pretty high. Since then, we’ve noticed slight bumps associated with other illnesses. The risks with CMV infection are numerous. Primarily, it can cause rejection. It can also permanently damage the kidney. CMV viremia (for simplicity’s sake, “viremia” would be “high” viral load – what he had in the spring) carries with it a fairly scary mortality rate. CMV is treated by lowering immune suppression and taking a fairly nasty antiviral medication called valgancyclovir, which really does a number on your bone marrow (making you very anemic very quick, at least if you’re Teddy).

EBV. I want to say it was the first year post transplant, T got EBV for the first time. There were a lot of other things going on, and he ended up getting a tonsillectomy and a CT scan – primarily because the biggest risk factor with EBV in an immune suppressed patient is development of PTLD. PTLD – the acronym that strikes fear in the hearts of transplant moms everywhere. PTLD is a pretty aggressive cancer that transplant patients get with an awful mortality rate. It’s pretty rare… but transplant moms are so far past “rare” already – I mean, peds renal transplants are ALSO rare. So at our house, we tend to refer to EBV as “the scary cancer virus.”

BK virus. I know the LEAST about BK because we’ve never had to deal with it. BK virus primarily affects the kidney – causing permanent damage and/or graft loss.

Teddy is never going to graduate ever

<note, that’s my sarcasm talking>

37% of children with mild hearing loss failed a grade by 3rd grade.

35% of teens with ADHD drop out of school.
30% of teens with ADHD have failed a grade.

60% of kidney transplant kids have had to repeat at least one grade by the time they are teens (this study wasn’t exclusive to kids who had been diagnosed as infants or in early childhood).

Another diagnosis I’m not quite ready to talk about yet has a 61% likelihood of failing a grade, with 83% failing to meet academic standards to move on, but being moved on socially.

Add those all up. (which, yes, I know you would never ever do because that’s not how it works.) This kid is DOOMED.

<insert emoticon of choice here to indicate that I’m being sarcastic and kind of humorous, and not serious.>

OK, but being serious here for a minute. Look at what he’s working against. Look at how much the deck is stacked against him.

Going along with all those statistics are others I didn’t report. What % in each case develops depression or a conduct disorder from being so discouraged, from being ostracized, from always being the bad kid, from feeling stupid. Those numbers are also huge – higher than the percent of kids who have failed or been held back – because so many of these kids eek by academically but struggle – academically and socially and with self-esteem and self-worth and by the time they hit junior high, they already perceive of themselves as being bad and friendless.

Now why on EARTH would I ever want to put him in a public school? With those kind of statistics? I truly don’t understand the bais doctors have towards public school.

Guess what? At home, he doesn’t feel like a bad kid. At home, he experiences success. At home, he is challenged but not frustrated. If he doesn’t get something, we have the freedom to try again, to try a new way, to put it off for a while and try again later. At home, I can talk to him one on one and he doesn’t have to struggle to hear and see and focus with a classroom full of other wiggly kids. He stands the GREATEST chance of success at home.

And at home, he’ll never fail. Ever. His education might look vastly different than it did for his siblings, but that’s OK.

Hearing Test, Finally Concrete Answers

Well, Teddy had his, I don’t know, 300th hearing test this week. He’s never passed one. Ever. He passed the sedated ABR but he’s never passed an awake hearing test in the booth. And the question has always been whether he’s failing the tests because he can’t hear, whether he’s failing the tests because he can’t understand or follow the instructions, or whether he’s failing the tests because he isn’t paying attention. And if it’s that he can’t hear, is it because he has fluid in his ears or because he has an unrelated hearing loss? We’ve spent a LOT of time at Speech Therapy working on following verbal instructions. And he’s older. It isn’t allergy season and he doesn’t have a cold. We had high hopes this time.

And it worked. He followed the instructions. He remained engaged.

He failed.

But it was a good, solid fail. He definitely has a hearing loss. He didn’t have any visible fluid.

Mild. Both ears. Something in his middle ear. When she stimulates the cochlea directly, he hears perfect.

She said she wouldn’t give hearing aids to a homeschool kid with a mild loss at his age and likely level of cooperation.

But the more I read… the more I’m pro-hearing aid. A huge percent of  learning happens accidentally, by overhearing things, vs being specifically taught them. It’s hard to know if this is attributable to his hearing loss, but man, do I notice this with him. The things that kids have usually managed to pick up just from being alive… he hasn’t picked up.

Kids with an untreated mild hearing loss are so much more likely to be held back a grade than their peers… that says to me that treating would be beneficial.

Especially in a kid who already has SO MUCH working against him. I can’t do anything about the kidney failure/transplant. I can’t do anything about the drug side effects. I can do something about this.

So we have an appointment with Otolaryngology and Audiology in January in Iowa City. We’ll see what they say and go from there.

“When I was a baby…”

So. Last night, Teddy was sleepy and asking me in a sleepy voice to tell him about when he was a baby. What else did I do when I was a baby? (It started when he asked me when we got his IV pole.) So this led to lots of discussion about his infancy. Most of which is depressing. Every third thing I said was that he really liked to snuggle, but he didn’t want to hear that over and over, so we talked about hospital, and dialysis, and Nurse Jen, and Dr Jetton, and driving to Iowa City, and taking baths, and getting his tube, and getting other tubes and getting them out. We looked at pictures on my phone. 80% of which are in the hospital. And we talked about a lot of non hospital stuff, like that he loved Genna and would just stare at her all day. But man, so much of his life happened in the hospital. Heck, my favorite Funny Teddy moments are all medical-related. And it’s fine. It’s his life. I need to get past always feeling sad about his early years because I don’t want him to feel sad about them.
So today we’ve received another packet from the government about Teddy (it’s related to Medicaid). “How has this disability affected your life?” Also, I have less than an inch of space to answer that question.
How HASN’T being born with renal failure affected his life? I mean… there isn’t a single aspect of his life TODAY that isn’t directly impacted by that. Developmental delays. Sensory processing disorder. Eating. Balance and body awareness issues. sleep. speech issues. adhd. cognitive issues. anxiety. motor delays. Well, OK. The heart defect and the subglottic stenosis aren’t likely related. Do you suppose “in every way” would suffice? I don’t even understand this question – isn’t the nature of disability such that it affects your life fairly completely? Especially if you’re born with it? It’s like saying “how has being Caucasian affected your life?”

Croup, apnea, and hearing

Soooo… in my ongoing effort to keep this blog a place where I can go to remember T’s personal history, while also keeping friends/family current, here is an update.

T’s been getting croup every month since May. May, June, July, and August were all mild enough to only require a few days of prednisone but not ER. They mostly started in the MORNING, which means I can get on top of it with pred before night. (And yes, croup is actually worse at night, your body’s natural levels of something I’ve forgotten the name of lower at night, and that lets croup get worse. Will be discussing with God in the future.)

About Oct 10, I allowed myself to observe (in my head, not out loud, I’m not THAT stupid) that he hadn’t had croup in September. Oct 20, he had the worst croup episode to date. *note to self: never notice anything ever again*

This is from my Facebook: “So. This morning. T woke up at 5ish with a slightly stridory cough. I thought ‘I will need to remember pred before bed tonight.’ About a minute later, ‘no, pred right now.’ Went potty, came back, he was REALLY struggling to breathe and decided on er. Halfway to hospital, I decided I wished we had called 911. We got to er 15/20min after he woke up with the slight cough. O2 of 70 with a doc standing by to intubate.”

That is how fast he can crash with croup.  (struggling to breathe: retractions at collarbone and ribs, drooling, unable to sit or stand, coughing with every inhale.  Then halfway to hospital, he stopped coughing. He couldn’t.)

And when I say “doc standing by,” I mean, the doctor brought a chair and sat at the foot of the bed. Out of the way of the nurses and RTs, but RIGHT THERE. For an hour or so.

And then as I run down to the bathroom after he’s stable to a) pee and b) have a moment, I pass the doc quietly saying to another doctor that that was scary. Ha. Then he came back in to tell me “that was a pretty significant episode of croup.” I think he could see the crazy in my eyes that told him I was already well aware of that.

The thing about airways, especially in kids, or so I am told, is that they’re fine until they’re not. Even once we got his airway open enough to get his sats up, he was still NPO for several hours just in case, because having an open airway at that moment still wasn’t any guarantee that he wouldn’t suddenly collapse.

So, back to otolaryngology we go. Fortunately, we already had an appointment with them because his apnea’s been markedly worse lately, and the monthly croup plus worsening apnea seemed to be related/concerning, plus the whole Teddy-cant-pass-a-hearing-test thing. Does he need tubes again? Does he need hearing aids? I’m tired of being put off by local docs, I want this solved. (detectable fluid in ears doesn’t seem to be evident every time he doesn’t pass a hearing test, so…) So we’re seeing audiology and oto in January, which was the soonest they could get us in.