March 23, 2023

It's been a while since our last update, but a lot has happened since our last update in January.

Two weeks after the suspected FPIES scare, Teddy had dangerously low hemoglobin on his regular labwork, so we ended up having to head to UVA for a transfusion and to figure out the cause. There was SOME blood in his stool, but not enough that we would've been able to notice (doctor confirmed he wouldn't have expected us to), but not enough to explain the sharp drop over 2 weeks. They scoped Teddy and found an ulcer in his duodenum and several pre-ulcer spots in his stomach. They clipped the ulcer to stop the bleeding and took some biopsies of the spots in his stomach. It was assumed until further notice that the ulcers were due to the aspirin transplant patients take to keep blood flowing easily though their new organ, so aspirin was removed from his meds line-up and Omeprazole put in its place. Teddy was discharged on 1/26 but ended up being readmitted to our local children's hospital on the evening of 1/29 due to a black stool. After 2 days of observation and 1 day of a clear fluids diet and the second day having formula and some applesauce, it didn't occur again and his labs were all holding steady, so he was discharged again on 1/31. On February 1, we learned that Teddy's ulcers were actually caused by EBV and that the biopsies were consistent with PTLD (post-transplant lymphoproliferative disorder), a type of lymphoma that can develop in solid organ transplant recipients on immunosuppressants, usually who have tested positive for EBV. Teddy's brand of PTLD has been diagnosed as Diffuse Large B-Cell Lymphoma. We started an aggressive decrease in his tacro to be off completely by the following Monday.

Unfortunately, Saturday, Teddy was acting ill. No fever, but lethargic, some vomiting, and not wanting to eat. We asked if this should be expected while dropping the tacro, we were told it wasn't, so Teddy and dad high-tailed it to UVA and Teddy's sister and I joined the next day after we were told Teddy tested positive for norovirus. Thankfully, for what it's worth, no one else in the family seemed to catch it, but that doesn't help when the one person in the family who has the potential for the worst reaction to a virus is the only one to get it. Teddy ended up in the PICU for a week after having "more diarrhea coming out of a child his size than [the doctors] have ever seen." I was told this several times by several different doctors and nurses. Teddy needed aggressive hydration, was given 4 rounds of IVIG to try and boost his immune system (one time having a pretty intense reaction after the infusion was complete: he started having chills, then had a fever of 101.5, given Tylenol and after 45 minutes, had a fever of 103.5 before finally settling out after about an hour and a half - it was terrifying), and puffed up like the Stay-Puft Marshmallow Man due to all the fluids he was receiving. He had 3 IVs, one had already been blown, so he had a less than ideal one in his leg around his ankle and the other two in either arm. By the next Friday, he was given a PICC line and had a CT scan to check if the EBV was causing any issues or if everything was made worse by the norovirus. CT scan didn't show them anything they weren't really already expecting, but knew that the after effects of the norovirus wouldn't let them judge his EBV as clearly as they would like.

By Monday, Teddy was moved to the intermediate care ward and his labs started to show improvement but also that his immune system was attacking his red blood cells, due to it going in overdrive trying to attack both the norovirus and EBV. Because of this, and knowing they needed to get the EBV out of his system to keep the PTLD from getting worse, Teddy was started on an infusion called rituximab, used to treat some cancers and PTLD. His first dose was that Tuesday. The following day was Teddy's birthday. We were less than thrilled that Teddy's first birthday was spent in the hospital and was relatively lackluster. He got some presents from us and some cards from his grandparents and donating aunt, but we really wish we could've done more. Plan is to do something for him later this year for now. He was discharged that Friday and scheduled to come back once a week for 3 more weeks for a dose of rituximab. We also had an allergy appointment as well to figure out the FPIES thing and any other allergies.

Rituximab isn't a quick process, it's treated the same as chemotherapy. It starts slow and is slowly increased in speed as long as it's being tolerated. Thankfully, Teddy didn't react much immediately to cause concern there. He would have a lower heartbeat that would steadily improve over the next 2 days the first 2 times. Then he had some peeling skin. After the last one, I thought he had kidney issues or something because he was peeing and eating a ridiculous amount, but his labs all showed that he was okay. His last planned dose so far of rituximab was March 6, then he had a CT scan last Friday to see the progress. Some things seem to be shrinking, but his surgeons at UPMC hadn't seen his CT scan by the time we went in on Tuesday 3/21 to review how he's doing, so we're still kind of in the dark as to our next moves. Especially as we won't really know too much more until we get labs again in April to see how his EBV is doing. As off the day of his last rituximab infusion, as expected by his doctors, the EBV was showing as Not Detected. Hopefully that holds up during his labs in April so we can put this all behind us. This lymphoma seemed pretty aggressive considering. He first tested positive for it on 12/19/22 but was clear of it on 12/6/22. They think the FPIES episode was actually the first manifestation of the PTLD/EBV ulcer activity, and by 1/23, he already had the ulcer and confirmed PTLD. That's less than a month that damage was being done and we didn't even know because he wasn't acting different in any way.

Teddy went from just toeing the line at 21lbs on 1/31 to being just over 19lbs by the time he was discharged. Lost a little more weight after that, but is now back on his food game. He's almost 23lbs now. We're still not sure what is going to happen here in the future as we would need more plan info, but so far, this is what we've got going on for us. Taking it one day at a time.

January 13, 2023

Apologies! It's been a while since we've updated!

Teddy's been doing well overall since his transplant! His liver enzymes and bilirubin are at the best levels they've ever been and he's a super-happy little guy. He's cutting through 4 teeth at the moment, so he's not super-happy all the time right now, but he's being plenty distracted by his older sister. Also trying to figure out what to do for his birthday next month(!).

On New Year's Eve, though, Teddy started vomiting after his nap and fell asleep after getting out pretty much everything in his stomach. We kept trying to give him some Pedialyte or formula but he would immediately vomit it back up. He also fell asleep pretty much immediately after vomiting. After 2 hours, I brought up the possibility of FPIES from his oatmeal in the morning, connecting the dots between the one scoop of oatmeal I'd been mixing with his purees to add texture and more substance, and the reflux he'd started having a few weeks earlier that didn't get better with changing his formula. This was the first time he'd had a full mixture of oatmeal, though I had mixed it with peanut butter, so could also be a possibility, but it doesn't line up the same. His liver team said they would look into it but agreed he needed to go to the ER when he still wasn't getting any fluids down and got so dehydrated that he started acting delirious. We went to our local children's hospital (where his Kasai procedure was done) and after a few hours, his team at UVA wanted him to go out there so they could have an eye on him directly as they didn't know what the problem was, but being only 7 weeks post-transplant, wanted to make sure there wasn't a problem with his liver or from surgery. That meant on Sunday, Teddy and Dan were medically transported from our ER to UVA's ER before being admitted in the afternoon.

Teddy steadily improved and was discharged on Wednesday with a potential diagnosis of FPIES as his liver was happy, the connections were happy, and no viral studies or bacterial checks came back positive and some of the other things that were off with his labs along with his symptoms on Saturday are considered classic FPIES symptoms. We go back in late-February for an appointment to start checking that out as he also had some reactions to milk and wheat on regular allergy tests. For now, we're being very careful of what Teddy is eating to avoid this situation again as it was the most scared I think Dan and I have ever been ever, including Teddy having had relatively major surgery twice already in his life.

We've since switched him to a hypoallergenic formula just to avoid any discomfort until we learn more about any possible allergies. FPIES is something that can be grown out of, so hopefully that is a thing because he's already going to have enough going on being immunosuppressed, I would hate for him to also have several allergies to be concerned about on top of all that.