Ian's Story - Congenital Diaphragmatic Hernia (CDH)

Ian Bunch was born with RCDH on January 29, 1998. When I was 31 weeks pregnant, I went in for my normal checkup and the doctor measured the height of my uterus. He said because I was measuring 35 weeks, he wanted to do an ultrasound. The ultrasound revealed what was initially believed to be a problem with the heart. Our baby's heart was shoved way to the left of his chest. It took a couple of visits with level 2 ultrasounds to narrow the problem down. The doctor couldn't get a good picture of the baby and it was either a type of mass in the chest, or a diaphragmatic hernia. (Initially they thought there might be something wrong with the structure of the heart, but it was because the heart was being smashed up against the wall of the chest.) The doctor recommended that I deliver at a hospital that had ECMO and explained to us why. He gave us a couple of options, the closest of which was Gainesville, FL (we live in Jacksonville). We met with the OB specialist who did another ultrasound. He definitely could diagnose it as a diaphragmatic hernia. He said that it was right-sided and the intestines were probably up there and most of the liver. We then met with Dr. David Kays, one of the pediatric surgeons at Shands Hospital who also specializes and had done many repairs of CDH. He went through all of the scenarios that could take place. We all decided on a cesarean section, set for 38 weeks. I would be given two steroid shots starting two days prior to my cesarean. I started leaking amniotic fluid at 37 ½ weeks and went to my OB to check me. She called Dr. Kays and decided to get me to Gainesville as quickly as possible-by helicopter. After slowing my labor with medications for two days to allow steroid shots to help my baby’s lungs, they proceeded to do the cesarean. When they lifted my baby out, I heard someone say, "Judy, look quickly!" I saw my son for a split second before they handed him to Dr. Kays' team. I'll never forget that big "waaaa" face with no sound coming out, except for a quick little noise that I can't explain, sort of like what it sounds like when someone gets the breath knocked out of them.

I kept hearing someone call out each minute that passed, and knew things weren't going well. All this time, I was praying as fast as I could and straining to see my son. When four minutes had passed, I still hadn't heard anything. Finally, Dr. Kays came up to me and said that Ian was very serious but they finally got him stable enough and were taking him to NICU. After about an hour, someone brought me a picture of my son. He was on a ventilator and almost his entire body was covered with wires and tubes. Dr. Kays explained to me how serious Ian was. Ian's Apgar score had been 0 and 4. He said he went a long time without oxygen in the beginning because he was very hard to intubate. When I looked at Ian, the vent was working his body so hard that his chest was dramatically rising and falling. The nurse was there to answer any questions about all the wires or machines that we needed to ask. I couldn't stop crying every time I looked at him. He was so beautiful. He looked so much like our daughter when she was born. He was a good size (7lbs. 14oz.) which the doctor said was in our favor. Also that it seemed he had a strong heart. I would visit again and again. I couldn't stay away from him. It was like I was trying to engrave his face in my mind because I thought I would lose him.

At about 3 o'clock in the morning, I went to see him again - and there I saw the surgeon - Dr. Kays, still by Ian's side. That is when I realized that we were in the best place we could be. The next day, only 24 hours after he was born, Dr. Kays came to tell us that Ian was not doing well and that he had a small window of time to get his surgery done. He really didn't want Ian to go on ECMO without having done his repair yet, so he was going to do the surgery even though Ian was so marginal. He did Ian's surgery right in the NICU because he was too unstable to move. Ian made it through the surgery. Dr. Kays said that 85% of his liver and almost all of his intestines were in his chest. He said that Ian basically had no diaphragm and he showed us the Gore-tex that he used as a patch. He said it was the biggest patch he had ever done. The X-rays revealed that Ian's right lung was extremely small, about 30% of what it should have been, and his left lung was about 70% of what it should be. Dr. Kays said Ian's lung and liver were bruised somewhat because the vent was causing them to hit each other. The following day, Ian's blood gases were so poor that he had to be put on ECMO. Even though the ECMO program was the initial reason we went to Shands, I was hoping all along that it would not be needed. I was told that Ian's lungs had been worked too hard and ECMO allowed them to turn down the vent and give Ian's lungs a rest and that hopefully, his lungs would grow and expand with the room in his chest recently created by the repair surgery. I can't explain ECMO. It was the worst sight I've ever seen. I thought seeing him with all the tubes, vent, and other stuff was bad. However, it was nothing compared to this. A huge machine was parked right next to Ian's bed, with big fat tubes filled with blood going from the machine surgically attached to his jugular vein. The only redeeming quality was that for the first time, my son looked more peaceful because the vent had been turned down and it was not pumping his lungs like it had been. Life on ECMO was extremely hard. Ian would have one good day, two bad. Then have two good days, and one bad. At one point, we were very encouraged because the blood gas showed more oxygen than what they were giving Ian, which meant his lungs were beginning to work. He had to go through two circuit changes (that's when they change out the whole machine with another one filled with all new blood). They would have to do that because the blood in the machine started to clot after about five days, which was extremely dangerous. After both circuit changes, Ian would have a very bad day and a half, probably because it was a shock to his system. At about day 10, Dr. Kays tried a second trial attempt to go off ECMO - that's when they adjust the machine so they can see how Ian would do without it. Again, Ian's blood gases were too poor and he had to put him back on. Eventually, on day 16, it was time for another circuit change. Dr. Kays prepared the machine, turned down the flow for a few days, hoping that this time Ian was going to do it. He made it off and the next time I went in, the machine was gone. The next nine days, Ian was slowly weaned off the vent. When he was 22 days old, I walked into NICU and Sandy, Ian's nurse, had him dressed in a little shirt and had washed his hair for the first time. They put an oxyhood on him at first, then switched him to a nasal cannula. This was the first time I saw my baby's beautiful mouth. His cry was raspy and weak, but it was music to my ears. At this point, he started making progress in leaps and bounds. It was a continual positive progression from there. They did an upper GI to see how bad his reflux was going to be. The test showed minimal reflux. We started slowly introducing feeds by mouth. I had been pumping breast milk and when they first placed the NG-tube, that was the first real food (not IV) he received. Increasing the feeds by mouth was a painstaking process. Eventually I was even able to breastfeed. Since breastfeeding is not an exact science, every day I anticipated when he would be weighed, hoping for a nice gain. At the same time, the nurses taught me how to give Ian his meds by mouth since I would be doing that once I took him home.

After Dr. Kays was satisfied with Ian's weight gain and progress (50 days in NICU), we were able to take Ian home. He came home on portable oxygen by nasal cannula. I had a huge tank set up in his room and he had a 20-foot tube so I could walk around the house a little with him. He was constantly on a heart monitor for the first few months, then just at night. He had to take many meds by mouth every day. He remained on oxygen until his first birthday. In his first year and a half he was hospitalized for several respiratory infections. As he grew older, he became sick less often. Ian grew with no physical restrictions. He played soccer and basketball when he was younger, would ride his bike and skateboard, swim and go surfing. He would get more winded than some children when he would run long distances, but there was never anything he wanted to do that he couldn’t. He was age-appropriate for every grade in school. He did have challenges because of a memory problem that he had that is probably related to his lack of oxygen at birth, but it wasn’t too much to overcome. He is now nineteen years old as of this writing. Ian recently graduated from high school and just started college where he wants to major in something that incorporates his love for photography. Ian would not be here without his surgeon, Dr. David Kays, who is a pioneer in changing the way babies with congenital diaphragmatic hernia are treated. We are so fortunate we found our way to Dr. Kays. We are so lucky in many ways, and knowing how different it could have been, makes us count our blessings every single day for our miracle baby. - Judy Bunch