The Willems Family

In December of 2021 my wife Bryanna and I found out we were pregnant with what we thought was our third child. At one of the early ultrasounds we discovered, much to our surprise, that we would be having identical twins! We have a four year old girl and three year old boy at home already so this was quite the shock at doubling the number of children. At subsequent ultrasounds we later found out that they would be boys and around week 16-18 my wife had a routine ultrasound appointment and that's when we discovered that one of the boys has Tetralogy of Fallot. We were devastated by the news and what the treatment would entail. We couldn't believe that our little boy would eventually need open heart surgery. We were so focused on the eventual open heart surgery and trying to process that on top of having twins and our toddlers at home that we were completely unprepared for the road ahead. My wife was scheduled for induction on July 28th, 2022 and after we were settled in and they started the pitocin and we were ready to go! Within a few hours of starting the induction our nurse came in and said that they would be stopping the induction because triplets were just born and took the remaining NICU beds. Long story short, we ended up staying in the labor and delivery room overnight and late the next morning the started her induction again. July 29th at 2:16pm our son Daniel was born and 2:21pm Isaac was born just shy of 38 weeks. Isaac was breech but Bryanna was able to deliver both without a c-section! She's amazing! Isaac was doing great after birth and they said he was a "pink tet" and we would probably be home within a week. Shortly after birth he was taken up to the CVCC (Cardiac Critical Care Unit) for monitoring due to no NICU beds being available yet. After a few attempts to get an umbilical IV they were unable so he needed peripheral IV's which caused problems later down the road. After a few days in the CVCC we were moved down to the Special Care Nursery(SCN) to help Isaac learn how to eat. While in the SCN he was eating ok and doing well but he progressively started to have more episodes where his O2 saturations would drop and would recover without turning blue but eventually he began to drop his saturations more frequently and started to turn blue. Initially they said they didn't consider them "tet spells" but eventually they diagnosed it as such when it became frequent and his extremities and core started to turn blue. After maybe a week in the SCN we were then moved back up to the CVCC for monitoring and we began to have more discussions with cardiologists, neonatoligists, pulmonologists, surgeons, etc. Immediately before he was moved to the CVCC he had a chest x-ray in the SCN which by the time we were moved up to the CVCC was read and they informed us that he has a right sided pneumothorax and very hazy looking lungs so they put him on a higher flow cannula to treat that and by his next chest x-ray the next day it was significantly better but his lungs still looked really hazy so they wanted to do a swallow study to see if Isaac was aspirating. The swallow study was not good. They immediately saw he was aspirating and even after attempting to thicken the formula he was still aspirating so bottle feeding was stopped and he eventually needed to have an NJ tube placed to feed him and avoid the reflux and aspiration. The NJ placement was difficult and took multiple attempts to get it positioned properly. In the meantime he was put on TPN and lipids for IV nutrition but due to being premature and his fragile vasculature he infiltrated every peripheral IV he had. Because he was also getting IV dextrose and hourly heel sticks to check his glucose when a peripheral line infiltrated they would have remove it, give five injections around the IV site to neutralize the dextrose and then get another peripheral line. This was a recurring issue until he was eventually able to not have the IV dextrose and was difficult to watch him be poked and prodded so much. Isaac was still having the "tet spells" and more frequently now and even after resolution of the pneumothorax and giving him pulmicort for inflammation of his lungs he continued to trend downwards. Finally August 10th at 12 days old the cardiologist spoke with my wife and I and said after reviewing his most recent echo his pulmonary artery stenosis looked more significant and that they would like to give him PGE (prostaglandin) to hopefully open up his PDA to get some more blood flow to his lungs and body but this also would mean that he would require an open heart surgery in about a week to place a shunt. He warned us that the medication could cause apnea so they would give IV caffeine with it to counter that side effect. After they gave the PGE Isaac eventually became apneic and was rushed out of our room down to the more critical area of the CVCC and was intubated. Even after he was intubated and on the ventilator the cardiologist said his O2 saturations were only in the 50 percentile. He also said because of that they had an ECMO team standing by to possibly put him on ECMO because of his poor saturations. Nursing staff also requested that the in house spiritual counselor should be paged down and hearing this we thought the worst. The cardiologist came back a little while later and said that his saturations were improving and after discussion with the surgeon the wanted to do the open heart to place the shunt by the weekend but, again, shortly after this he came back and said his saturations are very poor again and they would like him to be stable for about an hour and then do the open heart. We were extremely terrified and scared for our child's life by this point but things got even worse. The cardiologist rushed in the conference room we were waiting in and said that my wife and I should come back and see him because he was doing very poorly and they were emergently going to take him in to open heart surgery. It was very difficult to see Isaac intubated and with so many IV lines, ventilator lines and people working on him. His saturations were at 37% on the monitor when we were in there and you could just feel the sense of urgency with the way all these health care professionals were working calmly but with a purpose. We were able to see him for maybe a minute or two before he was taken back for his emergent open heart to place his shunt. We were told it would be about a 3-4 hour surgery in all and someone would periodically update us on the progress. The surgeon spoke with us prior to the surgery as well and said that he would be starting the surgery and that another surgeon was currently driving up from Rochester to assist. It was a very scary 3-4 hours because everything we were told with Isaac going into the emergent surgery basically equated to "bigger babies do better" and "he's on the small side for ECMO and the surgery" and with it being done so emergently we didn't know what to expect. Thankfully the cardiologist came in one final time to update us that everything went well and the procedure is done but they would leave his chest open for a few days until the swelling goes down before they close him back up. He was eventually brought back to the CVCC where we could see him and, again, it was tough to see him in that condition but he was finally stable for the large part. after a few days he went back in to surgery to have his chest closed and within another few days he was extubated and doing well and we were thrilled to still have Isaac after all he had gone through to this point. He was watched very closely for awhile in the more critical side of the CVCC and was getting post surgical care, medications, BiPap etc. until he was eventually moved down to the other side of the unit. He did well for a few days but again started to run into some complications. The first day I was going to go back to work I was just about to walk out the door at 5:45am when I received a call from the CVCC and the nurse said that Isaac's blood gasses were getting worse and that he is going to be intubated again. That was devastating news since he was progressing so well. When we got back to the CVCC the nurse told us that they saw a pocket of fluid in the right side of his chest but they weren't able to drain it to see what it was because it wasn't enough fluid and with the open heart surgery they were concerned about a chylothorax. Soon after on a chest x-ray they noticed a significant increase of fluid which collapsed his lung down severely so they needed to place a chest tube to drain the fluid. Once the chest tube was placed and the fluid was being drained they knew immediately that it was chyle which now meant that Isaac would need to be on TPN and a special lipid formula for IV nutrition because they also saw what they though was pneumatosis/NEC on an abdominal/chest x-ray. Luckily on the x-ray 12 hours later whatever issue they saw in his intestines seemed to have completely resolved and they did not diagnose it as pneumatosis/NEC. The following days were battles with trying to keep his ABG's and lab values in a normal range while he was on BiPap and his chest tube drained. They also wanted to remove his central line in his groin to reduce chances of infection and place a PICC line but they had a lot of difficulty placing it. They measured a few times with ultrasound and his vasculature just wasn't big enough and when they were finally able to attempt they had difficulty threading it through is SVA and kept going up his neck so they decided to pull the PICC back and leave it until they could advance it with Interventional Radiology. His chest tube eventually stopped draining and they thought that they may be able to remove it soon but instead found on an x-ray that there was another significant fluid build up in his chest and found that his chest tube was obstructed so it had to be removed and replaced. After that his chest was draining again and put out a significant amount of fluid and they were finally able to remove the chest tube after over a week. We were hopeful at this point we cleared the hurdles and would be working on feeding and getting home but, again, something else popped up. While they were watching his chest x-rays to follow the chylothorax they noticed one night he had an elevated temp over 38.5 so immediately started antibiotics and started to look into the cause of that. On the x-ray saw a pneumatosis/NEC that seemed to be pretty significant and we were notified of this one morning by phone and that they had stopped all feedings and were back to IV nutrition and were starting some antibiotics. They also drew labs to culture which grew E. Coli within the first 24 hours. Fortunately they were able to catch it quick enough where the antibiotics worked and it began to slowly improve. While this was going on the medical team was also sporadically getting elevated temps on Isaac yet and increased inflammatory markers and weren't sure why so they did echoes of his brain, heart, abdomen and all extremities. They discovered what they called a "globular mass" in or near his right atrium where his PICC line was as well as a clot in his groin where they removed his central line once they were able to finally get a PICC line placed. By this time he was already on multiple antibiotics and heparin because of the pneumatosis/NEC and wasn't able to have tylenol so he was already receiving the treatment for those. The mass in the heart had the doctors stumped and they weren't sure if it was a clot, endocarditis or a vegatative infection from the PICC line. Isaac would eventually put together some good days where progress was made but then ultimately would again have elevated temps and have fevers. All the cultures they drew didn't grow any bacteria and his viral panel came back clean so they wanted to look at some things with another echo. The echo showed that he had something on his liver but, again, they were not sure if it is a hematoma, an infected abscess or something altogether different. After a repeat echo the mass on the heart hadn't changed size and neither had the spot on the liver. Isaac continues to have elevated temps and an occasional fever with his CRP labs continuing to rise and fall repeatedly. There was multiple discussions about the possibility of removing the PICC line and placing a new one to try and positively remove the question of an infected PICC line and, as of this writing, sounds like they want to remove and replace it but if they're unable he will have to get another central line with a Broviak in general surgery which would mean being intubated again. Right now we're just frustrated that it seems like every week there's something new that Isaac is dealing with and despite being on multiple antibiotics he's still having temps, fevers and elevated inflammatory markers on top of the mass by his heart and the mass on his liver that the doctors are having difficulty diagnosing. At the time of this writing Isaac was born 47 days ago and has been in the hospital this entire time, mostly in the CVCC and it's been difficult for my wife and I to see all he has gone through thus far and everything he's still currently fighting and all this to know that he still has his full repair to come yet and no end in sight for getting him home. The doctors and surgeons seem pretty confident that we'll be there probably over a month yet with everything currently ongoing with his elevated temps and we still haven't started feeds through his NJ tube because of the pneumatosis/NEC and they don't want to start enteral feeds until they get the current issues and his CRP labs figured out. While all this has been going on we've stayed mostly at hotels, a Ronald McDonald House room once and even rented an apartment for a week so our toddlers and Isaac's twin brother could stay with us and we could be close to the hospital. The past few weeks though we've stayed at home and made the hour drive so either my wife or I could be home with the other children because all the new places, people and not knowing when mommy or daddy were going to leave and come back and who would be watching them just became to much of a burden on them. It's been without a doubt those most difficult time in our lives and for our family as a whole this past month and a half and we've just been praising God for Isaac still being here and still fighting. We're also so grateful to our families that have helped take on so much of the burden with us and for our Church family as well. Despite everything we still feel blessed and are hopeful Isaac will finally get healthy and get home before his full repair in the future.