Link to High Res on Youtube
At 13 weeks pregnant his mother's water broke. The doctors at that time did not believe he would survive without amniotic fluid long enough to be born alive. She carried him another 13 weeks and he was born at 26 weeks and 6 days. He was not expected to make it out of the delivery room because amniotic fluid is needed for the lung development to take place. When he was born They were able to get a breathing tube placed and get his lungs open. We were able to see him for the first time for a few seconds on his was to the NICU a from the delivery room. Soon after were counseled by the doctors at 90 minutes old to take him off of life support because even in the best of circumstances they only expected him to survive a few hours and they were giving him a lot of support. The decision was made to give William a chance and not to withdraw support.
He had pulmonary hypoplasia (underdeveloped small lungs,) pulmonary hypertension and a few days later developed PIE pulmonary interstitial emphysema which is where air gets into the lung tissue where it does not belong due to rupture of the air sacs in the lungs from high ventilator pressure. There is a catch 22 though because he needed the pressure to ventilate his lungs even though it was also causing damage. They switched his ventilator to an oscillator which is gentler on the lungs and can help with PIE. up to 95% of babies with pulmonary hypoplasia do not survive their first month.
At one week old William took a hard turn for the worst. The doctors were asking the family to come up so we would have a chance to see him and say goodbye. Someone was with him all weekend and our pastor and a friend came up to see him and prayed for him with us. Our church had a night or worship and a few members came together beforehand and prayed for William. All that weekend we prayed and the next week things turned around and he started getting better. Throughout the next month they slowly weaned his medications and he started gaining weight, just a few ounces but he was getting stronger and had signs that the PIE going away.
After 34 days on the ventilator he was extubated and was on a SiPAP breathing machine and he did well. After a few days we were able to hold him for the first time. During the next two months William started growing and overall did pretty well. He spent some time on just a nasal cannula with oxygen eventually having to go back to the SiPAP most of the time. He did have ROP, an eye disease of prematurity made worse when kids are on supplemental oxygen. At first they thought he might require eye surgery, but as time went on he looked better with each eye exam.
After reaching his "due date" around 3 months old, they started to talk about him needing several surgeries dues to his need for long term ventilation: 1. Tracheostomy tube 2. Gtube for feeding into his stomach and 3. Nissen surgery where the stomach is sewn in place around the esophagus to prevent digestive fluids from coming up the esophagus and potentially entering his lungs. William was transferred to the Johns Hopkins Hospital at 3 1/2 months old. After Transfer, he started requiring more O2 and was intubated again on the conventional vent. after 4 days he was transferred to the PICU and diagnosed with a bacterial infection in his lungs and started on antibiotics. He was also diagnosed with a adenovirus on top of everything and not doing well. He was put back on nitric and they ended up going back on the Oscillator for 1 1/2 months. Most viruses don't have any medicine for treatment, they can only do supportive care. For Adenovirus there was an antiviral medicine but there were risky side effects. They eventually did a course of Cidofovir and he finally got over the virus. At 4 1/2 months old he a tear in his lung (pneumothorax) causing his lung to collapse. They put in a chest tube to remove the air and put him back on a conventional ventilator while it healed. The entire recovery from the virus took about 2 months - at this point he is 5 months old. This video highlights his first 5 months: https://www.youtube.com/watch?v=dTPxU03GToQ
The next 2-3 months had a lot of ups and downs in William's care. He needed the tracheostomy surgery, but he needed to be on his back at least 5 days to recover. He would not tolerate even 48 hours on his back. The doctors felt he needed the surgery but was too sick to survive it. He did have surgery to place a central line, but that didn't require the same recovery time on his back. In the weeks following the central line surgery, William started to tolerate more time on his back, going several hours at a time to several days and then 2 weeks. Finally they agreed to do the tracheostomy surgery. 3 times infections prevented the surgery from taking place. Finally 1 day before turning 8 months old he got the trach surgery. The recovery went well, but a week later his central line was pulled out and he had to urgent surgery to have a new line placed. This one was a more permanent cuffed line with antibiotic protection.
The month following the tracheostomy surgery William did really well overall getting the oxygen percentage they were giving him down and being weaned off some of the sedation and paralyzing medicines. He did have 2 pretty bad infections but bounced back afterwards. We were able to hold him again for the first time in 5 long months.
The week after Christmas, just 9 months old, things started going downhill. His oxygen requirements which had been in the 50-70% range were in the 90-100% range eventually staying at 100%. This has been going on for the past 8 weeks and only briefly have we been down under 100% oxygen. There have been several bad infections and blood oxygen desaturations down to the single digits. There were several times we were not sure he was going to be able to get his oxygen levels back up. We have spent many hours and nights by his bedside reading to him and praying for him. He is now just past 11 months old and currently being treated for another infection. He is still on a lot of sedation as well as medicines for Pulmonary Hypertension. They are trying to come down on the paralytic medicine and adjust the others accordingly.
Toward the end of March 2015 nearing William's first birthday they had successfully weaned him off the Cisatracurium paralytic! He was missing it at first, but slowly getting used to the new freedom of movement. He was still on 100% FIO2 for the most part but tolerating some weans. We still seemed to be going back and forth with fevers and loose stools. He turned 1 year old 3/26/2015 and even shared some smiles:
Link to High Res on Youtube
Easter weekend he had another pneumothorax and had a chest tube put in. It was a rough day and William had spent a good bit of time in the 30's and 40's for sats before they got the chest tube in. After the chest tube they were able to get the FIO2 down over the next couple of days. Later that week they even got to 60% for the first time since December! Still dealing with fevers.
Since the pneumothorax and the chest tube coming out William has been on the upswing overall. No infections the past month or more, He's been smiling a lot and laughing. FIO2 has been averaging 50% BUT They have been raising the sat goals! First 80, then 85. When sleeping he's usually satting in the mid 90's! Quite an improvement since January. We have come a long way. 14 Months in 5 days!
On Thursday July 16th William had surgery to get a Gtube, Nissen and hernia repaired. No more ND tube! He was scheduled to go a month prior but had an aspiration pnemonia that caused an infection that took a month to fight though.
Link to High Res on Youtube
After the gtube nissen and hernia repair William overall was doing well. The focus was on weaning the IV sedation meds. In August we switched to the LTV ventilator, changes his feeds and continues to work on weaning Dex, Midaz and Morphine. Dex was turned off in Sept, Morphine and Midaz went off in October and on a sunny day in November William went outside for the first time ever. Things were looking pretty good.
In December of 2015 things started to go in the wrong direction.William's lowest average FIO2 settings were in October 35-40%. In December 75-100% was where we were. There was no identifiable cause the Hopkins PICU could pinpoint. While sleeping his sats were worse than when awake. Going to CHOP in Philadelphia was suggested. The rationale was that William might need a lung transplant and CHOP was the closest hospital for that. The other reason to go was Hokins felt like they had done everything they could and that a fresh set of eyes could be good for William's treatment.
We transferred to CHOP from Hopkins on 1/31/2016. We started off in the PICU and worked with the lung transplant team. There were tons of doctors that stopped by for evaluations and testing. After 11 days in the PICU I was shocked to find out they were transferring us to the PCU, their Progressive Care Unit for trached and vented kids. At first I was worried since we had been in the ICU for 19 months and it was all I knew.
Well the PCU was perfect. Everyone there was really nice and supportive. The best part was the training and the things that we as caregivers were able to learn how to do to become somewhat self-sufficient. Since going to the PCU, William has been doing great.FIO2 has been averaging 30% After a few weeks we met with the transplant team and the pulmonary hypertension team the decision was not to do a transplant because he is too well. There was nothing that disqualified him otherwise so that if he were to get sick in the future and need a transplant he would still be eligible.
From a pulmonary hypertension standpoint they felt like his current medication was keeping it well-controlled and they thought that maybe he could actually grow out of the need for some of those medications.
The other thing that came up in the transplant testing was Craniosynostosis which is basically when the cranial sutures close to early and prevent the skull and brain from growing properly. This can lead to neurological problems and developmental issues. They are taking him to the OR and doing a procedure to make an incision in his scalp and drill a hole in his goal and insert a pressure probe to measure the pressures and he will remain in the PICU for 24-48 hours while they monitor the pressures. This should give us the data to determine how to move forward.
The monitoring showed elevated pressures while awake and active but normal low while sleeping. Most of their data on high or low pressures comes from kids that are sedated or under anesthesia so they are not as worried about the higher waking pressures. They do feel like he needs the surgery to increase his skull volume. They just think it can wait to let his lungs heal. So we will wait at least 3-6 months and continue to monitor which is a good thing overall.
After 2 months at CHOP it looks like we will be heading back to MD and hopefully we can work towards finally getting him home. We will certainly miss everyone in the PCU, the Transplant team and the Cardiac team and the other docs and staff. Everyone has been great to work with.
I need to update this a little more, but the short version is that we came back to MD and went to Mount Washington Pediatric Hospital for a few months and then went home for the first time after 27 months inpatient. We have had our ups and downs but other than one long stay in 2016, we have been home and doing well.