Ryder’s Story - Congenital Diaphragmatic Hernia (CDH)

That day you first learn the words “Congenital Diaphragmatic Hernia” is one you will never forget. May 10, 2019 will forever be a hard day for me. Only two days before my first Mother’s Day, our world was shattered. We had been having a great pregnancy, my symptoms were fairly mild, and we had a blast surprising our families with our announcement. Ryder would be his grandparents’ first grandchild and his great-grandfather’s first as well. Everyone showered us with love, support, and excitement! We were floating on cloud nine! I have Crohn’s disease, so my doctors gave us the choice of whether or not we would like to go to the high-risk doctor for a thorough exam to be sure, but we had no reason to believe it was necessary. We decided it was worth checking everything fully to ensure my long medical history was not affecting our pregnancy. Little did we know they would find something completely unrelated to my health. They told us Ryder had a two-vessel umbilical cord (normally, there are three vessels) and CDH. They gave us an estimated 80% survival rate, but all I could hear at the time was that there was a chance he would not survive. The rest of the day, I was hormonal and completely shut down emotionally, but my husband began to do some research. Through various Facebook support groups, we found hope! We learned about the various organizations supporting families through this journey, and we learned about Dr. David Kays at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida. We had a consult at our local hospital, Children’s Healthcare of Atlanta, and my husband’s hometown hospital, Children’s Healthcare of Philadelphia, but when Dr. Kays gave our baby a 98% chance of survival, there was no looking back. And we are beyond grateful! We had to relocate early because I began having contractions at 31 weeks due to double the amount of normal amniotic fluid. We are lucky we did because my water broke only one week after we arrived - at 33.5 weeks, I was admitted to the hospital’s antepartum unit.

Ryder Jackson Coonley was born on August 29th, at 34 weeks, weighing 5 pounds 13 ounces via cesarean due to being in a breech position. He only had 15% of his left diaphragm, which allowed his stomach, intestines, and 15% of his liver to move into his chest. This pushed his heart and lungs to the right and prevented his lungs from developing fully. He had about 15% lung volume at birth and was immediately intubated (breathing tube through his mouth). He was put on ECMO, the highest form of life support that bypasses the heart and lungs, about six hours after birth and had his repair surgery the next morning to move his organs back to the correct place and patch the hernia in the diaphragm. He had a great first week and was about to come off ECMO, but he had a pulmonary hemorrhage (a bleed in his lungs – completely filling his lungs with blood) only about 12 hours before they were going to take him off (September 5th). His progress went completely backward. It was absolutely heartbreaking! For two weeks, we saw little progress and had to take everything extremely slow to allow his tiny body to recover. Still being a hormonal mess and a first-time mom, I struggled to say the least. Luckily, I had already made some amazing CDH mom friends in Florida who helped me through those first few weeks.

We tried to take him off ECMO again on September 18, but he had a second pulmonary hemorrhage within 30 minutes of trialing him off ECMO. This was the first time his surgeon had ever had a baby throw a second pulmonary hemorrhage. I will always be haunted by the words “oscillator STAT” being yelled from my son’s hospital room. They immediately got him on an oscillator vent (a special gentle ventilator that can deliver more frequent smaller breaths to help him recover) and back on ECMO. It feels like just yesterday we were standing in that hallway waiting for our son thinking we had lost him. But, in true Ryder fashion, he was fine and tougher than ever. On September 20, just two days later, we were finally able to successfully trial him off ECMO while he was still on the oscillator. He had a rough first 12 hours. We had to add Nitric Oxide and a vasodilator to relax the inner muscles of his lungs to widen the vessels, which helped him successfully stay off ECMO. We removed the cannulas the next day (September 21), and he adjusted to life without ECMO. We also switched him to a regular vent off the oscillator. It was the best feeling to watch the ECMO machine being wheeled out of his room and know we were getting so much closer to holding our baby.

Again, he had to throw us another curveball in total Ryder fashion. Just one day after removing the ECMO cannulas, Ryder accidentally knocked the breathing tube out of his trachea. It took two attempts to get the breathing tube back in, and he was down for longer than he should have been. He had to be bagged for what felt like an eternity. All while my husband and I stood there watching, scared to death, in the corner of the room, just trying to stay out of the way. The sight of seeing my child with little to no oxygen going in his tiny body will haunt me forever. But he pulled through as he always does. What does not kill you makes you stronger, right? Wow, we must be able to lift a semi-truck by now…

The two lung hemorrhages and the accidental extubation were the scariest moments of our lives, but Ryder is a fighter – he pushed through it all! He was finally extubated on day 35 and learned to breathe on his own with light oxygen support in his nose. We finally got to snuggle with our baby after waiting over a month! Let’s just say I hardly ever put him back down for the rest of his hospital stay. The wait made those first snuggles sweeter than words could ever explain!

Eating is the hardest part for most CDH babies during the first few years of life, and Ryder was no different, unfortunately. On November 4th, Ryder had another surgery for severe acid reflux, a feeding tube placed in his belly, and two hernias in his groin repaired. We were finally discharged after 85 days in the hospital and a total of 103 days living in Florida. Since getting home on November 28, 2019, Thanksgiving Day, we have been working very hard playing “catch up” and helping him achieve his milestones. We had significant feeding setbacks in the first few months at home, but we slowly worked through it all. Ryder was on oxygen support at home for a few months and ate most of his food through his feeding tube for the first year after discharge. After months of semi-weekly feeding therapy, we were finally able to wean him off of the feeding tube! He went from completely tube-dependent to 100% oral in only three weeks. It was a difficult year trying to get him to eat, but now he is a hangry toddler, just like any other kid! He completely blows us away time and time again with how much he eats now. Weight gain is still slow, but for a CDHer, he is doing amazing! We still go to speech, physical, and occupational therapy as he is still working to overcome some developmental delays from being intubated for so long on top of being premature. However, he is catching up quickly, and we don’t expect him to need therapies for much longer. Ryder is a little firecracker – full of energy and personality – and still does everything on his own timeline. When people meet him for the first time, they have no idea he ever had anything wrong with him. It is absolutely amazing how far he has come!

To all newly diagnosed parents, I know it’s scary. I know it seems impossible at first. But nothing is impossible for these resilient babies! Since our diagnosis, we have seen babies with less than a 15% chance of survival not just survive but THRIVE! And once you’re in the CDH community, you are never alone. We all work together to help the next family going through this roller coaster. And luckily, there are several amazing organizations out there to help you with resources, grants, and support along the way.

With tons of love and support,

Ryder, Leah & Aaron Coonley

2023 update: Ryder is now 3.5 years old, blowing everyone away with how far he has come. He had his feeding tube removed a few days before his second birthday and had the stoma closed surgically a few months later. While he still doesn’t love to eat, he eats more than enough to maintain his growth curve. He is definitely smaller than his peers, but I’m only 4’10”, so he was never going to be a big kid. He attends a special needs preschool three days per week and a private preschool 1-2 days a week. The special needs preschool is helping him with potty training and he gets speech while he’s there. He is still in OT for weakness in his left arm and shoulder as well as PT for some weakness in his right leg. But for the most part, he has caught up developmentally. We are just fine-tuning some things and trying to get him ahead before Kindergarten. Ryder has been known to pull other kids around in a wagon or push them around in the little cars they have at school. His little lungs don’t prevent him from living life to the fullest! He also now has a baby brother and he LOVES his baby!