It’s been a while again since I’ve given a full update on Wilder. A lot has happened this fall. He continues to be on oxygen full time, which has definitely helped with his shortness of breath and being uncomfortable, although overall we continue to see declines in his stamina and energy. Oxygen isn’t the easiest thing to manage with a toddler, but we’ve figured it out and only trip over his tubing a few times a day now. I still want to get him more mobile, so we’re getting a portable concentrator to let him move around our house easier and not have to be hooked up to the large concentrator as much. I wish we had an answer as to why he needs the oxygen now, but that remains unclear.

After our vacation in August we took him back to Boston for scopes. We were hoping for answers as to why he was struggling from both a respiratory and GI standpoint. His scopes looked mostly good. Which should have been good news, but didn’t give us a direction to go to help him feel better. It did show he had reflux, but that doesn’t account for all the GI issues he’s been having since his surgery at the beginning of the year. His airway, while not perfect, looked pretty good. No major collapse was seen. His tonsils and adenoids had gotten bigger and were obstructing his airway, so we decided to move forward with having those removed. We didn’t want anything else working against him as he was already working so hard to breathe. The trip to Boston was a frustrating one, for a lot of reasons. I was in a cast as I had broken my tibia, we got no answers as to what was causing his bigger issues, and we had to watch him struggle after anesthesia, both respiratory wise and with the medical PTSD he’s developed. 

We scheduled his tonsils and adenoids for early October, but it had to be pushed out a couple weeks as they didn’t have an ICU bed available for him for that date. I took him down last week for the surgery. We first had to have a cardio appointment to rule out some bigger cardio issues some doctors were worried about. He had a full workup, ekg and echo. Everything looked largely normal. Good news, but again, what is going on with his shortness of breath, desats, unexplained weight gain, lethargy, etc? We were cleared for surgery and went in on the 16th. His ORL surgeon, who I’ve had struggles with in the past, but is now truly one of his biggest advocates, came out after and said while the surgery itself went well he was hypoxic the entire time in the OR and no one could figure out why. She said unequivocally that we’re missing something here. There’s some larger syndrome that we haven’t identified that is causing many of his issues. She has seen him since he was 8 months old and sees the declines that we’re talking about. She said with his airway as open as it is right now he “shouldn’t” have had that trouble in the OR. He was intubated and they still couldn’t get his sats out of the 80s. They also had trouble extubating him and neither she nor the senior anesthesia team could explain what was happening. She recommended getting him back in front of neuro and for sure getting an MRI, seeing metabolic specialists, and rheumatology. She also called his feeding team at BCH to make sure they followed up with us as we’re still having so much trouble with his feeds as well as him taking any significant volumes by mouth. It was a hard conversation to have, but it also felt validating to have a doctor see what I’m seeing and say that I’m right, there’s something more/bigger going on.

We stayed 1 night at BCH after surgery. Even though he wasn’t in great shape, given his anxiety and PTSD around hospitals when they gave us the option to discharge we took it. He was puking a lot after surgery, which we thought at first was because of anesthesia, but after 24 hours we weren’t sure. They gave us zofran to take home in case he kept puking, and we hoped being at home would be the best place for him. He did well on the car ride home, but after only an hour at home he started puking again. I had to take off his nasal cannula as he got vomit in it and he very quickly turned blue. We suctioned, got him back on oxygen, hooked him up to the pulse ox and his sats continued to be low along with a high heart rate. He started having retractions, and also was developing a fever. After a quick call to his ped, I brought him in to the ER. They ran some tests there, did some breathing treatments, increased his oxygen, and decided to admit him.

Once we were admitted to UVMMC, he started to do better. He stayed stable on 1 liter of oxygen and IV fluids helped with his dehydration. Everyone was operating under the assumption that this was just a tough surgical recovery, possibly left over nausea from the anesthesia. That didn’t sit great with me, I felt like this was indicative of a flare up of all the symptoms we’ve been seeing for months. But, when he hadn’t puked in over 12 hours, was back to his baseline oxygen level and his fever was gone they weren’t sure what else to do for us. So, we were discharged again Sunday evening. He did ok at home, still pretty pitiful and needing pain meds as well as zofran around the clock to stay comfortable. On Tuesday he started to decline again. He was sounding junky, working harder to breathe, and then started puking again. Again, we brought him into the UVMMC ER. Although he wasn’t as bad as he was over the weekend, he wasn’t doing great. They weren’t sure what to do for him though. Thankfully, we had a good attending who saw there was something bigger going on and listened to our concerns. He had Wilder admitted to the peds floor again. I had to push the doctors hard to look at him through the lens of a complex kid with bigger, undiagnosed issues, rather than just a kid having a hard time after getting his tonsils and adenoids out. Everyone had a different opinion on what was happening, where his GI issues were coming from, why we were seeing declines, etc. Thoughts seemed to range from he’s basically fine to there is some bigger reason, possibly something progressive or degenerative, happening. We did more testing and labs, despite most doctors wanting to continue this on an outpatient basis for him. Although I hate being in the hospital with him, I couldn’t keep bringing him home without at least starting the process of figuring out what was happening. As more results came back it seemed like more doctors got on board with recognizing that he has something larger going on. We met with the palliative care team as a big goal is improving his quality of life and his comfort level. They seemed to listen, and definitely recognized that there needed to be better communication and coordination between specialists. Neuro also got involved again, although getting them to do an MRI, despite every other specialist thinking he definitely needs one, is proving to be impossible. Once he was doing better from a comfort level they decided to discharge again and continue everything outpatient.

I’m still frustrated with UVMMC and feeling like they don’t care and don’t want to listen. There are a few doctors we’ve seen who are great, but getting care for him across all specialists is incredibly hard. We have wanted to keep much of his care here in VT as traveling is so hard, but it’s not easy. We have a follow up with neuro in VT and GI/feeding in Boston. We’ll also be having hard conversations with his pediatrician and if we don’t feel like his team here is invested we won’t really have a choice except to move his care. Going to another hospital isn’t out of the question either. Cincinnati is on the table, possibly others if we can’t get answers. Wilder was the happiest, most tolerant little boy, and we’re losing that kid a little more every day. When we knew his airway was such a mess we at least had an explanation. It was something we could work to fix. Now we have no idea what is causing his issues. Things like muscular dystrophy, mitochondrial disorders and other genetic conditions have been talked about frequently. There are also some other things, such as Chiari malformation or adrenal issues that have been presented as many of his symptoms point that way. We’ve sent out for lots of genetic testing, so we’ll see what that reveals. Even though many of the possible diagnoses are scary, not treatable and may mean he will have a shorter life expectancy, we need to know what’s going on with him. The more information we have the better care we can provide for him. I just want the absolute best quality of life for him, whatever that looks like.