"CDH is not a death sentence. There is hope. If you’re facing a CDH diagnosis, find hope. " - Olivia's CDH Story
The day I found out we were pregnant, I thought my whole world turned upside down. I was excited and nervous but so ready to become a mother. I could barely wait to tell Jordan, but I held in my little secret until he got home from work. We were elated. This baby that we had planned and thought of and dreamed of was finally becoming a reality.... and until May 15, 2018, everything was going great.
14 weeks pregnant we were at our first ultrasound, seeing our precious little one for the first time. We were having a great time, until the ultrasound tech stepped out and got our OBGYN. He came in with the most somber look on his face. “There’s something going on with your baby. I can’t believe we even caught it this early. I’m shocked, to say the least.” This is the day our world really turned upside down. He went on to explain what a congenital diaphragmatic hernia was, and how we couldn’t deliver in our home town, and how serious it could be since we were so early in pregnancy. “You have to deliver somewhere that is ECMO capable. The closest is University of Michigan”. He then set us up with a consult at U of M for 2 weeks later.
After an ultrasound and amniocentesis, all the geneticist wanted to talk about was termination. She gave me a book about other parents who chose to terminate. “You have to think about the baby’s quality of life...maybe this will help you decide” she said. We went home that afternoon in a fog, overwhelmed with the information we received. The only joy from that day was that we were expecting a baby girl. We chose a name for her on our way home. Neither Jordan nor I could find the words to say. We yelled at each other when the other person asked what we should do, we were hurt, lost, and desperately wanted this baby. I cried, and he sat there in a stony silence looking completely defeated. We didn’t speak for most of the evening because we just didn’t know what to say or do.
As a nurse, I research, ALOT. In our night of silence...I found the first shred of hope that I could hang on to. I found Tiny Hero. My best friend had sent me their link about questions to ask your doctor, and as I dug into that I started to read every single story on their website. I kept reading of a doctor who had tremendous success. I didn’t say anything to Jordan, but I sent the contact form out to who I would later learn was Joy Perkins. Joy called me the next day.
Joy asked me what I knew about our baby. She asked what we were going to name her. I told her we had chosen Olivia. From that point forward, she only referred to our baby by name. Our baby had a name; she was real; and there was hope for her. She told me of Dr. Kays and his success, how they treat CDH babies a little differently to save their lungs AND their brains, and how CDH babies don’t have to die they can not only survive but can THRIVE. Talking to Joy on the phone that day was the first time I had felt peace.
About 10 minutes after I spoke to Joy, Jordan called me. Before I could even say hello, words were flying out of his mouth “I don’t want to terminate our baby. I want her. We want her. Please, we have to do something.” I cried. And then I was able to tell him that maybe there’s something we can do, maybe there is someone who can help save Olivia...but it’s going to mean a lot of changes and moving across the country. I could hear a sigh of relief on the other end of the phone. We would do anything to see this doctor. Anything for Olivia.
At the end of July at 24 weeks, we flew to St. Pete and spent 2 days going through testing... MRI, Fetal echo, and another ultrasound. Our MRI showed a left sided hernia with stomach, spleen, intestines, and colon in the chest, and Olivia’s heart was pushed to the right side of her chest. At that time, her liver was still down. We sat down with Dr. Kays, and he explained CDH in more detail, told us that he could give Olivia a 98% chance of survival and 15% chance of needing ECMO! This blew my mind. Because 10 weeks before that, our OB told us that she maybe had a 50% chance and even if she did survive her quality of life could be poor. That day we made the decision to go to Florida for Olivia’s birth. I carried out the rest of my pregnancy as routinely as possible. Olivia was having a little trouble growing, but thankfully she was only measuring behind by a couple weeks.
September 30th, 2018, we packed up the car and drove south. 18 hours from Kalamazoo, MI to St. Petersburg, FL.... at 34 weeks pregnant, what a drive that was. We settled in at the Ronald McDonald House and started what we thought would be a 4 week wait for our precious girl.
While I desperately wanted to keep her safe inside of me, my body and Olivia had other plans. She decided she needed to arrive on October 16th, at 36 weeks by way of c-section due to preeclampsia and intrauterine growth restriction (IUGR). She weighed in at a tiny 3 pounds 15 ounces. She cried twice before they could intubate her. The most wonderful sound I have ever heard.
After 3 days in the CVICU, we were moved to the brand-new Center for Congenital Diaphragmatic Hernia (CCDH) Unit at Johns Hopkins All Children’s Hospital (JHACH). That night, Olivia almost went on ECMO. Dr Kays and his team were able to keep her off and get her through to day 6 when she had her repair surgery at the bedside. Surgery went great, even though she was born with about 10% liver up. We thought after the initial rough 24-48 hours post-op we would be on the road to recovery, but by day 10 we were having other issues. Around 3 am at 10 days old and 4 days post repair, Olivia was placed on ECMO. The smallest baby at JHACH to go on ECMO. She was under weight and most other hospitals (including University of Michigan) might not even have tried to put her on. ECMO saves her life that night. An x-ray early that morning showed free air in Olivia’s abdomen. An emergency surgery on ECMO brought an intestinal perforation to light. Day 17 brought a third emergency surgery (again still on ECMO) where a hematoma (like a blood clot) was removed and a wound vac was placed, along with a chest tube. Then we finally saw significant improvement. At 21 days old, after 11 days on EMCO, Olivia was able to safely come off and thrive on her own. And thrive she did. Even with her tiny, less than 4-pound body, she blew away her medical team. We were discharged from JHACH on December 12th after 56 days with .1 liters of oxygen and no g-tube. We flew home 4 days before Christmas.
There are a thousand other little details and things that make up our CDH roller coaster, but the greatest thing that sticks out in my mind is that Dr. Kays always had a plan. When things looked bad, he always had an idea of what to do next, something else to try, something else to keep her alive. Him and his team never doubted our feisty little girl, and they fought and protected her just like she was their own child. The care and continuity we received from Dr. Kays, Dr. Stone, and all our ARNPs was impeccable. They saw her every day, and we consistently had the same nurses who noticed even the slightest change.
We started this journey scared out of our minds. We were terrified we wouldn’t bring home our baby. But we are firm believers that Olivia came home with us because of Dr. Kays and the way he treats CDH. Because of that, Olivia is thriving, and growing, and acting like a normal baby.
CDH is not a death sentence. There is hope. If you’re facing a CDH diagnosis, find hope. Find the people that will help your child. We could not have made a better decision than to relocate and fight for our baby.