Evelyn's Story - Congenital Diaphragmatic Hernia (CDH)

For expectant parents, the 20-week ultrasound is one of the most exciting and nerve-racking moments of the pregnancy. We had an uneasy feeling as the usually cheery ultrasound technician was having difficulty getting a good view of Evelyn’s heart. As she excused herself to bring in another tech, she undoubtedly alerted the OB as to what was waiting for him. He sat us down and told us he believed Evelyn had a diaphragmatic hernia and he admonished us not to google anything about the diagnosis. He had no information for us regarding likely outcomes and just warned us that this was a rare and extremely serious condition, one with which he certainly couldn’t help.

When we met with an MFM specialist the following week, after many gut-wrenching days of anxiety, fear and tears, she sketched on a piece of paper the position of the organs and wrote down next to it “how do we help baby breathe?” We considered ourselves lucky, in that no one recommended termination of the pregnancy as the most logical solution. Countless expectant parents are told that the outcomes from CDH are so poor that the best thing to do for everyone is to terminate. The MFM specialist explained to us that it was important to find out whether or not there were any associated genetic anomalies, so we agreed to an amniocentesis. She then gave us a glimmer of hope. She told us she had done her residency under a surgeon, Dr. David Kays, at the University of Florida who specialized in CDH and had remarkable survival rates for babies across the spectrum of severity. She promised to do her best to track him down that afternoon and send along our contact information.

Would you believe later that evening we received a call from Joy, Dr. Kays’ RN and program coordinator? She was so understanding and compassionate and hopeful. One can simply imagine the number of times she’s reached out to families in turmoil. She thought it was so important to make sure she talked to us as soon as possible so we could start to believe in not only the possibility of a good outcome, but in the likelihood of a great outcome! Joy wasn’t the only call we received that night. Dr. Kays himself called to not only explain what CDH is or detail his treatment philosophy or boast of his success rate, but he called so that he could learn more about us. His first question was to ask for the baby’s name. He wanted to know who we were, what we did for a living and what worried us the most. The man’s genuine first priority was to start building a relationship of trust and understanding so that he could feel good about us and our baby and we could feel good about putting her life in his hands. I have never experienced a doctor, a surgeon no less, who emphasizes the personal relationship as much as the technical application of medicine. We talked with Dr. Kays easily for 45 minutes and at the end of the discussion, we knew deep down in our hearts that this was the man who would do anything to ensure a positive outcome for Evelyn. My wife and I relocated to St. Petersburg from Melbourne, FL and Evelyn was born via scheduled induction at 38 weeks gestation. She was limp, seemingly lifeless, and blue as they carried her to the isolette in the corner of the delivery room.

She was quickly stabilized before being whisked upstairs to the CVICU. Dr. Kays came to see us that night and told us he planned to do her repair surgery the next morning. Morning came and when we arrived at her room to see Evelyn before surgery, Dr. Kays had discouraging news. She had not had a good night and was too unstable to risk the repair. Instead she would be going on ECMO, which is a heart and lung bypass machine. Evelyn tolerated being put on ECMO and she had her repair surgery the next day. Approximately 40% of her liver was up in her chest, along with stomach, spleen and bowel. She was on ECMO for 10 days and had done well as Dr. Kays worked to wean her off the machine by lowering the flow rate of the pump, allowing Evelyn’s heart and lungs to do more of the work. Finally, when the flow rates could not be lowered further, the ECMO circuit was clamped off and Evelyn’s lungs were put to the test.

Evelyn did not do as well as we had all hoped. Her oxygen saturations dropped and Dr. Kays decided to put her back on ECMO. Evelyn had other plans, however, and suffered a pulmonary hemorrhage before the circuit could be restarted. Dr. Kays took total command of the situation and calmly and efficiently brought it under control. He directed the use of a different kind of ventilator and Evelyn started to slowly get better. Within two weeks, Evelyn was breathing completely on her own and taking breast milk feeds orally, through a bottle. As her strength and endurance built, she increased her feeding to the point that she no longer needed the feeding tube (which ran from her nose to her intestines). Her mommy and daddy are so proud of what a fighter she is and how determined she is to live! We were discharged after 51 days, without a feeding tube and without needing supplemental oxygen. There are a few defining traits about Dr. Kays: he’s a fighter and he believes in the potential of these babies. He fought day and night for our Evelyn. My wife and I wondered if he ever slept. Dr. Kays provides a continuity of care that cannot be equaled anywhere, period. There is no night shift or weekend doctor taking over for him. These babies are his undying passion. He does this to give them the best quality of life possible and to show the rest of the medical community how strong, amazing and wonderful these babies are. He never doubted what Evelyn was capable of. Each of these children is uniquely different and he manages them accordingly. His focus is always on the long-term physiological and neurological outcomes. There is no one-size-fits-all plan for CDH babies. His broad depth of experience is not something you can find anywhere else. Each baby he treats adds to his knowledge and we have seen him apply lessons from other children to Evelyn’s care. We are eternally grateful to Dr. Kays for saving our little girl’s life and giving her an opportunity to thrive!