September 21, 2024
Unfortunately, about a month after our last update, Teddy's liver numbers elevated to a point he had to be hospitalized again. After a liver biopsy the following afternoon, it was confirmed that Teddy had mild acute rejection and was started on a bolus of steroids and back to all his original post-transplant medications, plus Valcyte added to keep his EBV at bay to avoid his PTLD making a return. Teddy was quite unhappy being restricted to a hospital room and often asked to go on walks, which we did sometimes. We'd go to the playroom, we'd take trips down to the cafeteria as we were able to take his wagon, which is probably his favorite item in the world. But on the down side, his IVs failed quickly - he was admitted on a Tuesday and by the time he was discharged on Saturday, he went through 3 IVs, which were fine for pushing fluids, but didn't do well for blood draws as they would start collapsing the vein, so he needed a new stick each time a blood draw was required, so at least twice a day. Blood draws usually meant either his dad or I holding him down while a nurse and the phlebotomist held his arm to get what they needed. Plus, 'roid rage doesn't only exist with body builders or baseball players from the late-90s/early-2000s. It also meant that he would wake up by about 2am or earlier every night and could take a while to fall asleep again. We were very happy to see his liver respond positively to treatment and being able to be discharged. Teddy continued doing mostly well for the last two months. He continued waking up in the middle of the night until several weeks after he stopped the steroids. His tacro randomly spiked really high at one point, so we were able to even that out pretty quickly. But then 2 weeks ago, his liver numbers spiked back up a bit and made improvements last week without change to anything. We go back at the end of the month to see how they're doing then. Through all of this, his doctors keep asking if he's acting off in any way, but he isn't. When I arrived at his transplant hospital, during intake, the on-call pediatric GI asked if Teddy was acting off in any way. All I could say was, "Not in the slightest. He's jumping off the couch daily, he runs around the house giggling, he eats well, he sleeps fine - not too much or anything, his stools are mostly normal - just the occasional weird one here and there, but nothing truly concerning. We wouldn't have had any idea his numbers were anywhere close to as bad as they were when he was admitted. His doctors were just like, "Cool. Cool cool cool. That's fun." It was a point where all I could do was laugh like, 'It is what it is.' It sucks that Teddy got rejection again and his liver doesn't seem to want to stay happy for too long with his 2-year Liverversary only 2 months away. I'm hoping it settles out soon and accepts that Teddy is it's home now.
Outside of just his liver, the results from Teddy's sleep study in spring were finally available in mid-summer. It didn't show any signs of obstructive sleep apnea, which I was excited about as maybe that meant he could avoid a tonsil/adenoid removal (though, the adenoids I'd probably be okay removing as they do actually seem to have been a bigger cause of his breathing issues last winter). It's because of this I was taken aback when I received a message that the number of central apnea events per hour he had that night gave them cause to want to proceed with a brain MRI to see if they can find out the reason for that, so that is scheduled for not long after his liverversary, which is about a year after his breathing issues first started. It is interesting how long this process has taken, and it was even sped up as once I asked his transplant team for a referral to ENT, we finally got scheduled in December - for an appointment at the end of May. It's only because of Teddy's team requesting ENT prioritize him due to his history of PTLD that he had an earlier appointment to fast-track this process for us. I want to know what's causing his central sleep apnea, but I also obviously don't want anything concerning showing up on his MRI. Hoping for a relatively easy end to the year as the last two winters have been a little rough, it would be nice to have a smoother one.
May 8, 2024
Been over a year since our last update! Teddy had a generally calm summer, numbers bumped a few times, but EBV was kept in check. Unfortunately, in November, his EBV was detectable again, but low and not in a worrying range. Around the same time, though, his breathing got weird and he started snoring a lot overnight. We got an appointment with the ENT team in January, as adenoid/tonsil hypertrophy can be a symptom of PTLD in post-transplant patients. The night before, his breathing really concerned me, but got under control when he fell asleep. His appointment was around his nap time, so he did end up falling asleep then and they heard him and said his tonsils looked fine but sounded like his adenoids were enlarged. He started on Flonase 2 days later and after a week, he went back to normal. He had a sleep study 2 weeks ago to check if he might need to have his tonsils and/or adenoids removed. We follow up on that next month.
Unfortunately, his liver numbers have not been stable. They got pretty high in late-February/early-March. We went to the ER to follow up on a set from the week before in the beginning of March and thankfully, his numbers dropped dramatically. Not sure what caused them to rise in the first place though and they've been all over the place since. He had a fever 1 week after his sleep study that resolved after 24 hours and had labs today to check where he was at since his last ones, which were better than the labs before, but still not in normal ranges. This week, they're out of whack again, but so are other levels that indicate he's been fighting an infection, but other than that day with the fever, he hasn't acted like it in the slightest. He wasn't acting off when his LFTs spiked so high in March either, so it's hard with a toddler that can't tell you if anything is wrong and they're not acting any differently than they normally do, including jumping and running around the house with their sibling.
He does also seem to be showing some signs of eczema, but we don't know for sure, though it's not unheard of with transplant patients so another thing on the list to try and figure out. Hoping his LFTs calm down soon so his poor little veins can catch a break as he hasn't gone longer than a month since he was born without needing blood taken for lab work.
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.
November 29, 2022
Haven't updated here recently, but have on Teddy's Instagram (@ourteddysunshine), but Teddy had his transplant on November 10! Everything went as expected and he's been healing up very well surgically! He still has some hypertension we're trying to settle out, which the team thinks is most likely due to some of his necessary meds. Otherwise, his liver enzymes are trending in the right direction, which we hope continues! We're also trying to settle out his anti-rejection meds to reach his goal range, but hopefully that won't take too long.
He's started crawling on all 4s instead of army crawling everywhere only a week after surgery and once we got home, he was immediately interested in solids, which we didn't get him to even look at before surgery. Now we just need him to chunk on up like how we thought he would before we learned he had a liver disease that kept him from absorbing fats correctly.
We were able to send the specialized formula cans and ready to serve bottles we had leftover to other families who needed them, so that felt nice to be able to give back, and we hope Teddy's story is helping other families with a child with BA to see more stories and understand every baby is different with this disease, but any amount of knowledge is helpful on your own journey.
Thank you so much for supporting our little guy! It means the world to us :D
November 11, 2022
Teddy had his transplant November 10th! The surgeons were happy with how everything went and Teddy's aunt's surgery (his donor) is also doing well! We have a long road of recovery and getting medication doses figured out and any other bumps that may show up along the way, but we're so grateful that he had this opportunity to help him get healthier! Thank you again to everyone wishing him well, keeping him in your thoughts/prayers/vibes, and who have donated and/or shared this campaign!
November 9, 2022
Teddy has been admitted for his transplant that is scheduled to take place tomorrow! We can't thank everyone who's donated enough, it means so much to all of us. Any good vibes, prayers, whatever you believe in would be much appreciated!
November 6, 2022
Teddy has a Bonfire shirt campaign going now for Giving Tuesday! If you would like to support Teddy in the wild, one of these shirts will help!
https://www.bonfire.com/teddys-new-liver-fundraiser/
October 22, 2022
Teddy's getting his transplant!
We learned officially this week that Teddy's Aunt Paige has been approved as a donor match! The transplant is scheduled for only a few weeks away. We're scared, terrified, and everything in-between. There are a lot of unknowns on this journey, but we thank you all so much for helping in any way you can, by donating or sharing the link, it's meant so much to all of us.
October 11, 2022
Today (Oct 11), Teddy had a CT scan of his abdomen requested by his transplant surgeon, so we're hoping this is good news and means maybe he'll get his transplant soon. The nurses loved him and were giggling at the fact that he would not stay sedated. They told me (Mum) that it would be about 5 minutes, but about 20 minutes or so later, they were finally picking me up from the waiting room saying every time they thought he was finally under, he would wake up and start blabbing at them, so it took them a while to get what they needed. When we went back to the recovery room, he was wide awake and very hungry and all the nurses walked by laughing like, "You wouldn't even know this kid was just knocked out. He's acting like nothing happened at all! Amazing." He was a little loopy but happy for sure.
He also received a Vitamin K injection yesterday and will be going every 4 weeks until his transplant to keep his levels in order. He is definitely not a fan of these, but thankfully he gets over it pretty quickly and is back to being happy and playful :D
