Reagan's CDH Story - RCDH

We were very excited for our 20-week ultrasound. I knew that I was having a girl this time; my symptoms were drastically different from my two previous pregnancies with my boys. At the ultrasound, we were initially told that Reagan’s heart was not in the correct place. We were then told to come back for an ultrasound in 4 weeks. At that appointment, things were much worse, and they were not sure what was causing it. So we were sent to MFM, and they completed another ultrasound. We were told that she could have a CCAM or CDH at this appointment, and we were sent for a MRI. She told us CDH was the worst-case scenario, and that we would talk more about this if in fact this was what it was. A week later, we were told the news that in fact it was CDH. We had never heard of this before and our hearts dropped as we learned more and more about this devastating birth defect. The radiologist at our local hospital told us that they thought she had no right lung tissue what so ever. Her LHR was less than 1, which we knew was not a good sign. Fast forward a couple of weeks and we meet with a group locally that would be treating us if we stayed in Indiana. They gave us a very grim outlook and even asked us if we were for certain that we wanted to do everything possible. They were very pessimistic about her outcome. I was completely devastated. Luckily, we had already found Tiny Hero (through my mother’s online research), and we had already worked with the wonderful, Joy, to get scheduled to see Dr. Kays for a consult. My heart was breaking because the excitement of having a girl was quickly overshadowed by her serious condition.

When meeting with Dr. Kays, we immediately felt that he was the right choice to care for our daughter. The more he talked the more I felt confident with our decision to relocate. Now don’t get me wrong, her results from her MRI did not look good, and he gave her a high likelihood of the chance of going on ECMO, and that she had substantial amount of liver up, 60% to be exact. She also had part of her bowel up. He was matter of fact but was compassionate at the same time. He was interested in our baby and our story. Being in the medical field, I had never met a surgeon like this. He answered all of our questions, and we actually left that visit with an optimism and hope that we had not had in a long time.

My family: my husband, myself, and my two older boys relocated to St. Petersburg from Noblesville, Indiana. It at first did not feel feasible to be able to relocate from Indiana to Florida, but it worked out and everything fell into place. We were lucky enough to stay at the Ronald McDonald House and to have family and friends that rotated in coming down to helping out.

EF922463-7AF1-4F1B-966A-AE63A7FA070D.jpg

Reagan was born via C-section at 37 weeks 6 days.  We heard a small little cry, and then she was whisked away to be taken to Dr. Kays and his team to start stabilizing her. Once stabilized, they walked her by us so I could see her before they took her upstairs to the CVICU. She somehow managed to keep herself off of ECMO, for 5 days, although those days were touch and go, and Dr. Kays slept at her bedside on multiple nights during this time. Dr. Kays had decided that we could go ahead with surgery, and she had surgery on that 5th day. That was when everything changed. During her repair surgery, they found that her liver actually had developed around the tiny strip of diaphragm that Reagan did have.  She ended up with a cleft in her liver that reached all the way down to the vessels. When repositioning her liver to put it in the correct anatomical position, her body reacted poorly, and she was declining fast. She was placed on ECMO in the middle of her surgery. What was supposed to take 3-4 hours turned into about a 10-hour surgery. Dr. Kays had said in his years of experience, he had never had to put a baby on ECMO in the middle of surgery, and he had never encountered anything like her diaphragm and her other anatomical abnormalities that she had.  Reagan was missing about 95% of her diaphragm, had a thin strip of normal diaphragm in the middle, and a sac which helped to keep her other organs down. She had a large hole on the right side on top of the other abnormalities with her diaphragm. Reagan was then on ECMO for 13 days and intubated for 31 days.  She then started to make progress and luckily made a smooth transition from being intubated, to CPAP, to wall oxygen. She was not able to come off of oxygen and went home on the lowest setting possible. But she is eating from a bottle and continues to grow and thrive.

IMG_2720.JPG

Dr. Kays’ continuity of care is unmatched. He follows these babies through their entire stay and post discharge.  For an abnormality like CDH, you definitely need the continuity of care that he provides. He has so much knowledge and experience with this that cannot be surpassed. That was another thing that made us feel confident, was that he would be following Reagan throughout. Our local hospital stated that they just could not provide that. That the doctor on call would be making decisions, and those decisions may be different from some of the other doctors in the large group that they had. They were not able to provide that same continuity that Dr. Kays did.

Reagan is a thriving, smiling, happy baby, full of energy and who loves to watch and laugh at her big brothers. We still struggle with gaining weight, and we are still on oxygen, but we are plugging away and make little improvements daily. I cried multiple tears throughout our stay in Florida but had tears of joy when we were able to take our baby home. We owe that to Dr. Kays, and we will forever be grateful for the care that Dr. Kays has given Reagan; it is unmatched.

 

 

1.      She had liver up.

2.     ECMO needed.

3.     Right.

4.     LHR <1.0.