Jan 17, 2020 Latest post:
Apr 13, 2020
Hello! We're putting together this site in the weeks leading up to Wilder's second airway surgery on February 20th, 2020. He has a long and complex medical history, starting the day of his birth. We're hoping this will be a place where we can keep everyone updated on what he's going through and how he's doing as that changes day to day and he has some pretty big events coming up.
To (not so briefly) summarize what he's been through so far in his life:
He was born at 29 weeks after my water broke at 28 weeks. Although my doctors did their best to keep me pregnant for longer with lots of meds pumped into me and bedrest at the hospital, I developed an infection that required an urgent c-section. He spent 9 weeks in the NICU, and for the most part did great there. He did take a while to come off respiratory support, it took several attempts every time we tried to wean his support down. At the time we thought he just needed a little extra time to grow and get stronger, but looking back this was our first indicator that something was wrong with his respiratory system. Once he was finally off respiratory support he took to breastfeeding so well, it seemed that we'd be home soon with our healthy baby. However, he continued to have apnea events that required him to stay on caffeine. We were told after several attempts to take him off it that we would be able to bring him home on the medicine along with a pulse ox monitor to check to make sure he wasn't continuing to have apnea events. Discharge was just around the corner finally. In the couple days leading up to our planned discharge date he started struggling more with eating, he would choke and his oxygen levels would dip some. SLP came to consult and thought he just needed more time to work on his suck/swallow coordination. It still seemed that we would be able to go home, we would just need to watch him carefully and pace his feedings. Then as we were getting ready to go home, literally packing up his stuff, one of the doctors came in and told us she didn't think she could discharge him. Nurses had noticed him wheezing some, there were concerns about his feeding, and they felt he needed more time in the NICU just to be safe. After tears, a lot of tears, we convinced her that we could manage him at home and would be following up closely with our pediatrician. We finally got to bring our baby home!
Once we were home, unfortunately things didn't get better. He was having frequent desats, especially during sleep. His wheezing increased and he started displaying other signs of respiratory distress such as retractions and some color change around his mouth. We brought him in to the pediatrician many times in our first month home, and were even sent to the ED once from there to have him looked at. Every time doctors would be slightly concerned, but when they would check his oxygen it would be ok at the time and we'd get sent home to keep monitoring him closely. After a month home, we brought him to our first pulmonology appointment. Both his pulmonologist and neonatologist who we saw that day were concerned enough about him to have him admitted directly from the appointment. We spent the rest of that week in the hospital having many tests done and having him on monitors 24/7. It was acknowledged from his sleep study and observations that he had some respiratory concerns and we were scheduled for a bronchoscopy. That revealed "very very severe" tracheomalacia and mild laryngomalacia. Tracheomalacia means a floppy airway in the lower region of the airway, and laryngomalacia means floppiness in the upper part of the airway. We were told that he would outgrow both of these by the time he turned 1 most likely. We were sent home with instructions to have him sleep on his belly to help to keep his airway from collapsing and to keep him on the monitor when asleep or eating.
We continued to feel extremely worried about him. He appeared to be struggling to breathe more often than not. In addition, feeding had gotten harder for him and he was choking and having blue spells while eating much more often. At this point we had added several more specialists to his care team. We were seeing neomed, pulmonology, ENT, SLP, cardiology (for a minor heart defect that was found during his NICU time), an eye doctor and his pediatrician. At yet another clinic visit these concerns were brought up and he was scheduled for another swallow study after having 2 previously that he "passed." A couple days later we went to the swallow study and after letting him feed for a little longer and playing back the recording they were able to see that he was in fact aspirating. Calls were made by SLP to his doctors and it was decided that he would be admitted that evening for a feeding tube to be placed. We were inpatient for a few days while an ng tube was placed, we learned how to use it at home, and plans were made to set a surgery date for his g-tube. About 3 weeks later we were back at the hospital for his surgery, which as nerve wracking as it is handing your child over to go back to the OR, it was a relief to get rid of the ng tube and be able to use the g-tube.
We were discharged home with the plan to just keep doing everything we were doing, using the g-tube as needed and monitoring his breathing while we wait for him to grow out of everything. I did not feel comfortable with this "watch and wait" plan as we weren't seeing any improvement, if anything he was only getting worse. We had also found out during one of his admissions that he had developed high blood pressure for unknown reasons, so we were now seeing nephrology as well and having his blood pressure checked weekly during our home nurse visits. I started researching his conditions, mainly his airway, and found that Boston Children's Hospital was the best place in the country to go for treatment. We were lucky we weren't that far away and I contacted them to set up our first appointments. Through my research I found several parent support groups for kids with airway disorders and feeding tubes which still serve as lifelines many days when I need advice, a place to vent, or to celebrate milestones achieved.
While we waited for the date of our Boston appointments to arrive, one of the scariest moments occurred. One night after the big kids were in bed and I was holding Wilder, he stopped breathing in my arms. I was able to perform CPR on him and he started breathing again as Ryan called 911. We spent a week in the hospital after that where tests revealed how bad both his obstructive and central apnea were. We were sent home with a vent and he had to be on bi-pap whenever he was sleeping to ensure he was safe. Two weeks after discharge from that admission we were on our way to Boston. We had lots of clinic visits and then a triple scope where the team took a look at his whole airway as well as his esophagus. His airway surgeon, Dr Jennings, came out after to talk to us and told us that while his airway wasn't the worst he's ever seen, it was really bad. He had 100% collapse in a few different regions and he was definitely recommending surgery to fix it. Although the surgery was major, it was a relief to hear that there was something we could do to help him as we feared we were heading for a trach if there weren't any other options for him. We spent a few days inpatient in Boston after that, getting his vent settings correct on his bi-pap and then came home to start the process of scheduling his surgery.
We spent the next few months at home, trying to keep him healthy before surgery. We did transition to 100% tube feeds as he was choking more and more and developed aspiration pneumonia. His lungs needed to be healthy before surgery, so although it was hard to stop breastfeeding it was definitely the right call. We were also approved for home nursing care due to the amount of equipment and care that he needed. This was, and still very much is, a life saver for us. It allows us to still live life, take care of the big kids, all while keeping Wilder safe. A week after his first birthday we headed to Boston for his surgery. The surgery itself went well. It was complicated, as his surgeon said, and there was an area of collapse that he couldn't access, but we hoped it would be enough for him to be able to breathe well on his own. Recovery at the hospital took a while, we spent 5 days in the ICU before being moved to the floor for another 5 days. We were discharged home finally for the rest of his recovery.
Once we were home and things settled down I felt that his breathing still wasn't great. He was still having desats at night and lots of noisy breathing. He got sick a few times in the winter and spring, requiring oxygen at home for and a PICU stay at UVM once. After that we knew that his airway was not where it needed to be still and we scheduled follow up appointments in Boston. These appointments were more often than not frustrating as doctors conceded that he still had lots of collapse in his airway, but weren't sure that surgery was the right approach. During this time we had also discovered that he has a compromised immune system. He does not produce enough white blood cells, specifically neutrophils, on his own which leaves him very susceptible to infection. We added hematology to his list of specialists, moving from UVM hem/onc to BCH hem due to the specificity of his condition. We also started seeing neurology and genetics as his developmental delays were becoming more and more apparent, despite working with him in therapy. He was now delayed well beyond adjusting for prematurity, again without knowing why. We were going to clinic visits very often, but not getting many answers or direction. We knew his health, and overall quality of life, needed to improve.