"He was as severe as predicted." - Adam's Story
On a Friday in January 2014, we marched our excited family into our 20 week ultrasound to see our 6th baby in motion, and confirm everything looked normal. The ultrasound tech took longer than normal to scan, and we then heard the dreaded words, "Just a minute, I need to get the doctor. I’ll be right back.” Our doctor confirmed that they were seeing our baby had a congenital diaphragmatic hernia, and reassured us there was nothing we did to cause the defect. From that moment on, our world seemed to swirl around us, and our devastation was undeniable. About 10 days after diagnosis, we found out that along with his stomach, intestines, and spleen, 40% of Adam’s liver was also (up) in his chest, and that his chances for survival had now decreased to 20-30% (liver up, LHR less than 0.75). We had insurance that allowed us to relocate almost anywhere. We consulted with top-rated facilities including UCSF, Children's Hospital of Philadelphia, and even considered fetal surgery. At one point we were looking on the internet and we came across a parent-run website talking about a doctor named Dr. David Kays. He was in Florida, specialized in treating CDH babies, and he was getting higher than average survival rates. For a moment, it seemed that there was a sliver of optimism!
After talking to Dr. Kays and Joy Perkins (program coordinator) on the phone, flying down to meet them, and much prayer as a family, we knew there was no other place we would ever go for better care. From my experience as a registered nurse in the NICU, I knew that continuity of care (one doctor in charge), as well as better survival rates were key factors in deciding where we would go. We love Dr. Kays’ minimalistic approach when considering medications and interventions, and his attitude that these babies need to be able to act as normal as possible in the NICU. We moved our entire family (5 other kids) to Florida, arriving when I was 34 weeks along.
Adam was born about full term, on June 2, 2014, with a team of numerous people, as well as Dr. Kays, in the room to administer immediate life-sustaining care. He was as severe as predicted and the decision was made to have his CDH repair done on the day he was born, when he was about 6 hours old. This was an aggressive move, but knowing he would almost inevitably need ECMO, it was the right choice. It was a long and complicated surgery; he only had about 5% of his own diaphragm on his left side, and had excessive bleeding during the repair.
They were able to keep Adam off ECMO for 72 hours after surgery, which was a huge win for our team. We know this was due not only to Adam's will to fight and our family's will to fight for him, but also because of such an amazing doctor, nurses and a whole team of people caring for him. Adam spent 23 days on VA ECMO, and then was able to be weaned off the ventilator to regular oxygen, at 5 weeks old. He had a Nissen and G-tube surgery when he was about 8 weeks old, and was then finally able to eat. Mom pumped breast milk all that time, and Adam was able to successfully breastfeed and bottle feed at 9 weeks old!
Throughout our entire stay in the NICU, no one but Dr. Kays was ever in charge of Adam’s care. He would even personally change ventilator settings, make small adjustments in Adam’s positioning in bed to allow more lung expansion, and would check throughout the night to see what Adam’s most recent labs and vital signs were. Even when he wasn’t physically present, he was keeping track of Adam. Dr. Kays always talked to Adam as though Adam was listening, and saw him as a sweet baby boy.
We took Adam back to our home state of Utah after 90 days in the NICU where he continues to thrive in a loving home environment full of the sounds of family life. Partially due to our high elevation, he spent his first 18 months on oxygen around the clock, and has now weaned to just needing it when he sleeps. We assume that will go away soon too.
We fed him through his G-tube at night to get him extra calories for growth during his first year, and now he no longer needs his tube. He eats by mouth, loves food, and is medication free! He has never shown any signs of an oral aversion. He is beginning to walk, tears apart drawers throughout the house, and has even donned a new pair of glasses to help with some vision correction. Adam brings our family immense joy and happiness- beyond anything we could describe.
Dr. Kays has survival rates that are much higher than the national average, fewer babies with feeding issues, and babies who have a shorter length of stay in the hospital. Besides the fact-he is just amazing to watch in action, and has incredible compassion for the CDH babies as well as their whole family. Nurses often comment that he is a “CDH baby whisperer.” How could we ask for a better doctor than him?
We have an incredible amount of gratitude to God for Dr. Kays, Joy Perkins, and the amazing people in the hospital who cared for not only Adam, but our entire family during our stay. We feel quite sure that Adam would not have survived, had we not been in Dr. Kays’ expert care.