Drew's CDH Story - LCDH
The first time I had ever heard the words congenital diaphragmatic hernia were on August 24, 2016. It was our nine-year wedding anniversary, and we had planned to make a date out of it. This day was long awaited, and we were so excited. After our daughter, we struggled with secondary infertility. We had passed the 13-week mark and made it through some concerning issues around 15 weeks, so it should have been smooth sailing from there. We had a couple of ultrasounds at this point, one of them was just to reassure me that our baby was perfectly healthy and probably a boy. So, there we were, over the moon watching every beautiful movement our little baby made on the screen. He said “Its’ a boy!’ as he continued to scan. I kept wondering what the black hole that he kept labeling “ST” was. It was near his heart, and I didn’t remember the heart being so narrow on baby scans. I told myself that everything was fine and that I needed to quit worrying. He finished and said he was going to have the doctor review them and come talk to us. He came in and proceeded to tell us that Drew had congenital diaphragmatic hernia and that his stomach was in his chest. The “ST” I was worried about, was his stomach, pressing on his heart. He mentioned “pulmonary hypertension” and “pulmonary hypoplasia” and that it was a severe birth defect. He said he would likely be ok while he was in utero, but he would struggle after birth. I started bawling. I don’t think I heard much else of what he had to say. I asked if my baby was going to die. Andrew and I don’t remember what his exact answer was but it was definitely not “no”. He gave us a minute and had us taken to a consultation room where he came in and said he needed to ask if we wanted to terminate. We were shocked. When we said no, he had his nurse make us an appointment with a pediatric surgeon at Johns Hopkins All Children’s Hospital. Little did we know that this “surgeon” was THE Dr. Kays who families traveled from all across the country to see. Joy Perkins called me about an hour after our appointment. I hadn’t researched much ( the MFM told us not too ), and I didn’t even know what to ask. I didn’t even know whether or not he had right or left CDH. All I knew was his stomach was in his chest. What I knew after that phone call though, was that Joy knew a TON about CDH and had extensive history with CDH care and Dr. Kays. She was brilliant, and I couldn’t wait to meet her and Dr. Kays. I told my family I was going to hug him for moving from Gainesville to St. Petersburg with his practice. She told me about Tiny Hero, and it was a game changer for us to be able to read survivor stories. I felt like Drew was going to struggle, but that he was going to survive based on what I had read.
One week later, we had our perinatology appointment and met with Dr. Kays. We were told at that time he only had a “sliver” of liver in his chest which we later found out was 20% based on our MRI. Dr. Kays said he felt Drew had a 95% chance of survival and possibility of needing ECMO. He and Joy spent hours with us. Who does that right?! Dr. Kays talked to us about CDH, ecmo, his treatment, and why he could offer us better survival statistics that the national average which is about 40-50% for severe cases. Joy took us on a tour of the CVICU where Drew would be and introduced us to a few CDH families. Everything about finding Joy and Dr. Kays felt meant to be, and we were so grateful. Dr. Kays was the miracle that my family and friends had prayed for. Everyone at John’s Hopkins All Children’s Hospital was so wonderful to us. We kept thinking how blessed we were for everything to go so smoothly.
We had our amniocentesis to confirm that Drew didn’t have any other significant genetic abnormalities. Pregnancy with a CDH baby is a little nerve wracking. I had polyhydramnios and had contractions pretty much all day, every day. I was always worrying that we wouldn’t make it to the hospital in time. So, 1 week before our induction day we packed up and moved to RMH. We had family and friends helping with our house and animals as we were planning to stay at RMH until the day we brought Drew home.
At 39.5 weeks, I was induced. He was born at 1:24 pm and was a whopping 9.35lbs which I hear is big for a CDH baby. He was blue when he came out, but I had a complete peace and calm that I can’t describe knowing that the entire room was filled with people, specifically Dr. Kays, who were going to take the best care of my baby boy. I could feel the experience and confidence of the people around me. I knew Drew was going to be ok.
I was able to see Drew within a few hours of birth but thank God for iPhones because my husband was sending me pictures and videos of my beautiful, incredibly strong baby boy.
At about 12 hours old, Dr. Kays felt that he had gone through the list of interventions that he was going to try before putting Drew on ECMO. They had prepared us so well for ECMO that when they said it was time, it was a relief because we knew it was what he needed. We also knew that Dr. Kays felt that doing GOOD ECMO was extremely important, and that he was going to make sure he did everything he could to keep my baby safe on ECMO, protect his brain, and give him a good outcome. My husband and I were scared at times but Dr. Kays, Joy, and the extremely knowledgeable nursing staff had a way of calming our nerves. To have that comfort during such a scary time was so wonderful. Drew’s repair was performed while he was on ECMO. During the repair, he found that he did indeed have 20% liver up and his organs had actually started to shift across to the right side through his mediastinum. It was such a relief to know he had been repaired so he could continue making progress. He was slowly weaned off ECMO and came off ECMO at 8 days old. Our next goals were extubation, oral feeding, and discharge. We had heard it was a roller coaster and that it was.
Here’s a brief timeline of events:
January 5- delivery day
January 6- ECMO
January 7- Repair on ECMO
January 13- ECMO cannulas clamped
January 15- ECMO cannulas removed
January 24- Extubated to CPAP ( scuba mask )
January 26- We held him for the first time!
January 29- Graduated to RAM cannula
February 1- Switched to wall oxygen
February 2-16- Working with oral feeding
February 17- Nissen fundoplication, G-tube for severe reflux
March 2- We got to take our sweet boy home!
What you can’t see in this timeline are the meltdowns, the support of family, our daughter caring deeply for her baby brother, blood transfusions, moments that felt like “setbacks”, the “firsts” that were so different than our previous experience with childbirth, our son who never missed a chance to charm his nurse for the day, nurses encouraging me through the difficult feeding days, the “curveballs” that every CDH baby will throw, the exhaustion, the endless bottle labeling, the beautiful progress days, and so much more. Dr. Kays, the CDH team, nurses, and staff at John’s Hopkins All Children’s are like nothing I have ever seen and my family will always be grateful.
Drew is one year old now, and he is truly amazing. He is so smart and catching up quickly on his milestones. He crawled just 3 days before his first birthday and now seems determined to try walking soon. We still worry about him regularly, but I can honestly say it gets better over time. Drew has captured our hearts with his smile and we are his biggest fans.