I wanted to start by thanking everyone for your patience, I know it has been some time since I last posted an update.
For a quick recap, when I last posted on 22 March, Joey was being treated for inflammation due to an unknown hyper immune condition, and the state of his liver was unknown. We were waiting to see if the treatments for the immune condition were working, and for the results of a bone marrow biopsy. We were also waiting on liver biopsy results to get a better sense of the recoverability of his liver.
The bone marrow biopsy results came back first, and showed that HLH was not present (great news overall!). In order to determine if the treatment for his immune system was working, we had to wait for lab results to come back from a lab in Ohio. This is one of, if not the only, lab in the country that processes these kinds of labs. And because of this, it can sometimes take up to a week or more to get the results back. Those results finally came back, showing that the treatments were working. While waiting for the immune test results, we got the liver biopsy results back indicating that 40-50% of his liver was necrosed (dead cells, essentially). On top of that, his labs were indicating his liver was getting worse. So, on 26 March, we all decided it was time to relist him for another liver transplant (luckily he did not have HLH, or this wouldn’t have been possible). Like last time, he was listed as 1A, highest priority for receiving an organ. Unlike last time, the doctors and surgeons wanted Joey to receive a whole organ- this is preferred for second transplants, to give the second organ the best chance possible for survival. This was unexpected, and hit me hard. Harder than last time. It meant that his liver needed to come from a deceased child, and it also meant that his options would be much more limited than last time. Our doctors said he would still likely receive one in 3-5 days. Luckily, his liver numbers overall weren’t as bad as the first time he went into liver failure, so we were able to wait a bit longer than last time too.
Because Joey was relisted, it was decided not to close his abdomen until he was re-transplanted. On 27 March, Joey went back to the OR to have his abdomen cleaned and another biopsy of his liver, to keep gathering as much data as possible on its functionality. Oddly enough, the surgeons said his liver looked even better than the week before, but that was likely because the immune condition treatments were working and his inflammation was subsiding. However, the results of the second liver biopsy indicated that his liver was 80-90% necrosed, and not recoverable. Of course it wasn’t the news we were hoping for, but we were glad we had decided to get him listed before getting these results- every day counts.
While we waited for a new organ, Joey was treated for an infection from his breathing tube, and was given plasmapheresis almost daily. Your liver is responsible for processing ammonia in your body; when your liver isn’t functioning properly, your ammonia levels rise. High ammonia levels, especially if left untreated, can cause permanent brain damage. Plasmapheresis is one of the more effective treatments to remove ammonia from the blood. Until Joey has fully been weaned off sedation (more on that later), and likely gets a bit older, it will be challenging to tell if he has suffered any long-term cognitive damage as a result of his high ammonia levels. In addition to the ammonia, plasmaphersis helped address the fact that his liver was also not properly creating coagulation factors. When your coagulation factors aren’t in the right threshold, there is a high risk for bleeding. In Joey’s case, the doctors were concerned about brain hemorrhaging or some sort of internal bleeding. Either of which could have potentially disqualified him from receiving a transplant due to the instability of his overall health.
The first time we were waiting for an organ, he received it in three days. After three days passed this time, with no offers whatsoever, I started getting a bit worried. After five days, I was getting really worried. The Transplant attending physician on service reassured us that he had time, and that we could, and should, wait for a perfect match. While still elevated, his ammonia levels and coagulation factors were relatively stable with the plasmapheresis treatment. This all was reassuring, but still difficult for us, because at any point something could have gone sideways and disqualified him from surgery. Every day that went by without an organ offer was terrifying, we truly had no idea what to expect.
Aside from keeping his ammonia and coagulation factors stable, the doctors wanted to optimize Joey’s health as much as possible for surgery. One way to do this was to ensure his fluid levels were low. This was to make sure he didn’t have a lot of edema (swelling) before going to the OR, and would help with extubation. Another way to optimize his health for surgery was to actually extubate him. It sounds a bit counterintuitive, but ensuring he could breathe on his own before heading into a major surgery requires a level of strength that would be beneficial for him during and after. It also meant we could start weaning him off of the sedatives he had been on for weeks. So after relisting, the doctors started weaning his ventilation settings to prepare him for extubation. And on March 31st, Easter Sunday, Joey was successfully extubated. He was still sedated, but this was the first time in three weeks that we were able to really hold our baby boy, and to see him with his eyes somewhat open. My mom heart was so happy.
Although it was clear that Joey was uncomfortable and experiencing some withdrawal as the team continued to wean his sedation, we were able to enjoy four tube-free days with our baby… Because on the morning of April 3rd, we were informed of a potential organ match.
As excited as I was at the thought of Joey receiving this organ, I wept when I found out it was from a child who was a little younger than two, who passed unexpectedly. I thought about all of my friends who have children around that age. I thought about if it were us, which wasn’t an unlikely reality for us a handful of times over the last few months. Regardless of whether this organ was a good match, I wanted to teleport to this family, the parents especially, and hold them so closely. I still do, every single day. I wish I could surround them at all times with the love and gratitude we feel towards their family, and give them strength and comfort for the coming days/months/years as they grieve the loss of their child.
The surgeons flew out in the late afternoon on the 3rd to assess the organ. There was concern it might be a little big, but after inspection, they felt it would be a good fit. It was still on the larger side, but roughly the size of his first donor liver (just different dimensions). And because Joey had grown a bit in the two and a half months he had been hospitalized, there was a bit more room this time. And otherwise, everything else about the organ was a great match.
Given it had been over a week that we had waited, we all felt it was likely going to be the best offer we would get in the time Joey had left, so we decided to move forward with the transplant.
All day on the 3rd, we prepared Joey for the OR. He received platelets, plasma, blood, and for about 6 hours leading up to the surgery, he was put on continuous dialysis. This was an even more aggressive treatment to clear out ammonia, and to make sure he was as pristine as possible for the surgery. His kidneys had also taken quite a hit because of his liver failing, so the dialysis also helped relieve some stress on that organ for a bit too. While receiving dialysis, we weren’t able to hold him, which was really hard for me. I just wanted to wrap him up and make sure he felt so loved and safe before he went to the OR. Luckily, I was able to hold him for about 20 minutes before he went to the OR to do that.
We walked Joey to the OR around 12:30am on Thursday, 4 April. The whole day leading up to the surgery, walking him down to the OR, kissing him goodbye… it all felt like a much scarier version of his first surgery. Chris and I knew more about liver failure, the seriousness of it, and had a better understanding of the risks of the surgery… We were infinitely more nervous than last time. I was absolutely terrified as we parted ways with Joey at the OR.
Joey’s surgery was very similar to his first. It lasted about 6 hours, and it was complicated, but went well. Like last time, the surgeons decided to do the biliary tract reconstruction as a second surgery, and scheduled that for 8 April. Joey was re-intubated for the transplant, and it was decided he would stay that way until after his biliary surgery. The biliary surgery went well, so that afternoon, we all planned for extubation on 9 April.
After Joey’s transplant, and while he was still intubated, Chris and I decided to both go home at night. This was such a nice treat for us; we had only been able to get away from the hospital together a handful of times since mid-January. When we came into the hospital on the morning of the 9th, we were welcomed by an extubated Joey! This was a little surprising, because it sounded like he had been up a lot of the night, fighting sedation. And it was clear that he was extremely uncomfortable- he was breathing fast and hard, and vomited a few times shortly after we arrived. He was experiencing withdrawal. I was able to hold him and get him to settle down a little bit, but it was a really long and hard day for him. And us. A plan was discussed the day before regarding how to manage his medications, but it wasn’t really working. During his surgery the day before, the nurses were kind enough to bring in a big bed for Joey, that I could share with him to try and soothe him as he was going through withdrawal. So when the night came, I laid by his side and tried to keep him calm and comfortable. This worked well, until the respiratory tech came in around 12am to do chest PT (which helps break up secretions in the lungs, which Joey was experiencing). The chest PT put Joey over the edge, and he wasn’t able to calm down again after that. His oxygen levels started dropping, and after a few hours, they had dipped into the upper 70s (we want them in the mid-high 90s). He had tired himself out, and was too uncomfortable to breathe efficiently. So, around 3am on 10 April, he was re-intubated.
Chris and I were crushed. We were frustrated that everything seemed to have been done so haphazardly with regards to his medications. We questioned why they decided to extubate him given he had been so awake and uncomfortable beforehand. But we know that Joey had become a “sedation nightmare”, so we could appreciate that it was a difficult case. For some context, he was on opiates, benzodiazepines, barbiturates, and sedatives… and his doses were about 3 times what normal adults would start out with, because he had been on them so long and his tolerance had become so high. And even with all of that medication, he was still fighting through it and awake quite a bit.
Joey was scheduled to head back to the OR on 12 April to get closed up, which required him to be intubated. So it was decided to keep him intubated and rework the sedation plan. On the 12th, we learned his surgery was pushed to the 15th due to scheduling conflicts. So he was to stay intubated until after the surgery on the 15th. Then on the 15th, his surgery was pushed again to the 17th due to emergency surgeries for another little boy in the PICU.
We were glad he could be fully closed on the 17th, with all of his own skin (at one point the surgeons were talking about potentially using mesh to bridge the top layer of skin, or even a skin graft, and they would have used skin from his back to make that work). In the grand scheme of it all, it wouldn’t have been the end of the world if they needed to go a different direction, but we were really relieved that he could heal a bit more naturally.
But the surgery delays confounded our frustration. Because his extubation was being delayed, he needed higher doses of his medications. This then made extubation harder. It is a vicious cycle, but basically he needed to have enough medication to keep him sedated and calm so he wouldn’t accidentally extubate himself. But to extubate, you need to be awake enough to be able to breathe well on your own. Most of the meds he received were IV, but most PICU attending physicians want kids off most of the IV “drips” before they will extubate. So he needed to transition from the drips to oral, and get all the oral dosing right too. Add in the attending physicians changing over every 3-5 days… It was getting tricky.
But everyone wanted to give Joey a shot with extubation, and felt that he could potentially succeed. So on the morning of the 19th, he had a trail run of breathing on his own, the success of which would determine whether they would go forward with extubation. Unfortunately, he failed miserably, only making it for about 4 minutes before his oxygen levels started dropping. Any time he was too awake, he was too worked up, and was essentially hyperventilating. Similar to what happened the week before.
It was really disheartening, and I honestly felt like he was never going to get extubated. By the end of the day, there were discussions that if he didn’t seem to be doing better, he may need a tracheotomy. A tracheotomy would allow for him to move around more and start OT/PT, but the biggest upside is that we could start weaning the medications, even while he was on respiratory support. He would still require being attached to a vent for a bit, but it would be harder to accidentally extubate himself while not sedated due to the placement and reinforcement with sutures. However, it would also mean that he would have a trach in for a handful of months, as it is a slow process to get him to breathe with “room” air again.
We felt that Joey was so close to being able to extubate without the need for the trach, but just needed to get his medications dialed in ever so slightly. It was also at this time that we all agreed he was likely experiencing delirium (which can be really hard to diagnose in babies), and decided to start treating that with medication. We were hesitant about doing this at first (he was already on so many medications, we really didn’t want him to start on another), but after a dose or two, it became clear that he needed it. Over the weekend of 20 April, Joey was finally calm, even when awake, and did well transitioning off the drips over the next few days. And on 24 April, he was (finally) successfully extubated!!
Chris and I were thrilled! I cried (happy tears) as we, and all of the PICU staff, celebrated this big achievement for Joey. He was originally intubated in mid-march for a small procedure, and was planned to be extubated a day or so later. However, due to unforeseen circumstances, he ended up being intubated for 6.5 weeks (aside from the four days he was extubated). Joey has been through hell and back during that time; and we are so proud of our babe for hanging in there and being able to pull this off. He is the toughest person I have ever met, truly.
Everyone that has come by since Joey’s extubation has been so excited for him. Some of these folks have never really seen him awake, or even seen his chubby cheeks without a bunch of tape on them (which are even chubbier now because of the steroids). We are so lucky to have such wonderful people caring for him, and love how much he means to everyone! We are so grateful for the new family we have made here.
When he was extubated, he was put on a ram nasal cannula, so he is still getting quite a bit of respiratory support from the ventilator. But so far he has done well. He has also done well with the weaning of his final remaining sedation drip. Once he is fully off that, they’ll start weaning his oral medications. We have been told to expect at least a month before he’s fully off all of these medications.
In the last week or so, the team has also started reactivating Joey’s gut. He has been on IV nutrition for so long, but it is time to get him back to a normal diet. So far, that has meant putting milk into his intestines or stomach with an ND/NG tube. Some days he tolerates this better than others, and it seems to depend quite a bit on how severe his withdrawal symptoms are. But now that things are moving around in there, and he is less sedated, he is stooling again! It has been about a month and a half since this has happened, so it was a very exciting day in the PICU after his first bowel movement (I’ve never seen so many people get excited about poop!). It also means his liver, biliary tract, and intestines are working well, which is such great news! But now that he is pooping again, and his catheter is out (and peeing like crazy), it is back to changing diapers every couple of hours. I can’t say that we necessarily missed this part, but it feels good to get back to a sense of normalcy!
His high urine output is a good sign that his kidneys are recovering well, but we won’t know the extent of the damage for another couple of months, at least. What Joey really needs now is time to rest, recover, and get back to normal. Until then, it will be difficult to discern, with any real accuracy, how he is doing with regard to his kidneys. We are hopeful that we will see some more improvement there, and won’t have to worry about another organ!
Due to his extended intubation, Joey has become very weak from a physical standpoint. We have started working with OT/PT to rebuild his strength, and get him back towards meeting age-appropriate milestones. We also hope to start working with the speech-language pathologists soon, once Joey’s respiratory status is a bit more stable, so he can begin rebuilding his oral strength and feed by mouth again.
Regarding his liver and immune system- so far, things have been looking pretty good. Within the last few days, though, we have started to see some elevation in his numbers. The immune testing isn’t something normally tracked this closely after a patient is transplanted, so it is difficult to tell if this is normal behavior post-transplant, or unique to Joey. His liver numbers rising could be for a couple of reasons (for example, the introduction of feeding or some of the medications he is on), but because both these and his immune numbers are both rising, it could be an indication that he might be going into organ rejection. This sounds scary, but doesn’t seem to be a big deal here… They just bump up some steroids for a little bit. With Joey though, who knows, he never seems to follow the norm. So for now, we are waiting to see how the numbers trend, and praying for the best. As usual, we are taking this day-by-day.
Joey has a long road of recovery ahead of him, physically and mentally. We are grateful to all of the teams we work with here at the hospital, and their dedication to getting him healthy again. We have no doubt that he will get back there- he has proven over and over again how resilient and strong he is. He truly amazes us every day, and I am so immensely proud of him. Everything this poor baby has been through breaks my heart, but he is such a fighter. He wants to live, and he wants to be healthy. There is no doubt about that. I have no idea how we were lucky enough to be blessed with such an incredible child, but I am just so grateful he is ours.
As always, thank you to everyone for your love, encouragement, support, and prayers. And a special thanks to those who have already contributed to COTA for Joey’s campaign, and have shared his story! We are so appreciative to have you as part of Team Joey!!
All our love,
The Moschellas