Vaughn’s CDH Story
When we decided to have a 4th child, we assumed it would be like their siblings before — they would be born, and we could all barely skip a beat! On April 27th, at only 16 weeks, I went to an ultrasound in Pittsburgh and was told that Vaughn's stomach was up by his heart and that his heart was pushed all the way to the right. I couldn't comprehend what was happening, so I told them to call my husband at home with the results and that we wanted to receive the news together. I was at the scan alone because we had a first-trimester screening just four weeks before, and I was told that everything was going great and our baby was thriving. I have struggled to be able to fully pinpoint all of the emotions I felt leaving that appointment that day.
Later that evening, we got a call that it was a Congenital Diaphragmatic Hernia and that his prognosis was unfavorable. Like most parents, we had never even heard of CDH before being diagnosed ourselves. The images they obtained also told us he had an issue with his kidneys and a flat profile. Both of those were ruled out later, but after hearing your baby may not have a profile, it was hard to forget. We were given a few possible scenarios, and they told us should return for more testing. We had our prior children with this same OB, and we trusted him, but he was not optimistic. He stated that they don't see CDH often but that Vaughn's case was the most severe he had seen. I think it broke his heart to have to be the one to give us this information. We gave ourselves that evening to cry, and the next morning we decided that we would do all that we could to save our baby. We went on the offensive and immediately found that even though CDH is life-threatening, there is hope outside of Pittsburgh.
We got to work looking for second and third opinions. A few days later, we were at CHOP (Children's Hospital of Philadelphia) for follow-up testing. They saw that Vaughn looked to be an isolated LCDH with liver, spleen, stomach, and bowel up. They estimated a three to four-month hospital stay for Vaughn and a 70% chance of survival. 70% seems like good odds until it is truly life or death for your baby. They offered us an experimental procedure called FETO that had the potential to open up his left lung, but we would have to be in Philly by 23 weeks gestation. We left knowing that although CHOP was a great hospital, we felt Vaughn's best chance to survive and have the best quality of life was in Florida at Johns Hopkins All Children's Hospital. There were times when we questioned if we really could relocate with three kids and two cats. But I am thankful for the hope we found that if we could get to Florida, we felt like we had a fighting chance!
We got in touch with Tiny Hero and applied for the financial grant. We were able to obtain a home and made a move from Pennsylvania to Florida on June 17, 2022. We spoke to Joy and Dr. Kays' team over the phone and felt so confident in our decision to relocate before we even had our consult. While many families from all over the country temporarily relocate for JHACH's unique CDH unit, due to the ages of our other children, we felt the best way to keep the family together while considering their needs was to move permanently!
Our friends and family rallied to help us make this cross-country move. It would not have been possible without everyone's help and support. Our three older children, ages 6, 4, and 1 at the time, couldn't have been more patient and supportive. We were age-appropriately honest with them the whole time about their brother. We wanted to be able to show them photos of Vaughn and know that they have already been made aware of how this would look.
A few weeks later, we had our MRI and consult — it was confirmed that Vaughn was severe and on the severe side of severe, but Dr. Kays felt confident we would bring our baby boy home. Those were the only words we needed to hear that day. He gave Vaughn a 90-95% survival and a 75% chance of requiring ECMO.
Vaughn was born on September 15th, at 36 weeks and 6 days, via an emergency c-section. We showed up for a routine parental appt and found that my blood pressure and Vaughn's scan that day showed some issues. Bob and I had left our house that afternoon, telling our kids we would be home for lunch, not knowing Vaughn would be born that day! My mom had come to stay with us just a few days prior in preparation for her being with our older kids for delivery.
Once Vaughn was here, he was quickly intubated and taken to the CDH unit. He went on ECMO that night and stayed on it for eight days. He had his repair surgery on day two — he had 25% liver up and missing 85% of his diaphragm. For Vaughn, everything was on cruise control after that point while we waited for him to stabilize. Our next significant milestone was extubation on day 19 of life. I got to hold my baby for the first time when he was 20 days old! From there, he was brought down from CPAP to wall oxygen. We are so lucky that Vaughn took all feeds orally and was even breastfeeding while inpatient! He has dealt with well-controlled reflux and continues to be orally fed at home. We hope to start solids soon!
With three older kids and no family living in Florida, I went to most of my prenatal appts and testing alone. Thankfully we had help with delivery and the tough few weeks after! Bob and I would alternate half days at the hospital every day so that one of us was home with our older children and one of us with Vaughn. While inpatient, Vaughn got to experience his first category-four hurricane. We locked down for four days! We had only been in Florida for a few months and got initiated quickly.
On day 39, we brought our baby home, which was well under Dr. Kays’s prediction especially given Vaughn was on ECMO! We have never been able to come up with words enough to thank Dr. Kays, Dr. Smithers, Dr. Stone, and the entire team of APPs and nurses. Having a team that believes in your baby is the best possible thing you can do. We were able to bring Vaughn home on 0.1 liters of oxygen (the lowest oxygen setting) which he was able to ditch just a few months later.
Our story did not end at discharge, though. Vaughn was diagnosed with Bilateral Congenital Cataracts at three months old. BCC happens in 1-3 out of 10,000 births. I am sure that the BCC and CDH are not a common combo! He would go on to have both cataracts removed, and he now wears contacts! Contacts can give him the best vision until he is old enough to get permanent lens implants. After his first cataract-removal surgery, Vaughn also had a bowel adhesion that caused an obstruction. They also repaired two inguinal and an umbilical hernia during that surgery. He has been able to overcome these obstacles and continues to smile! He is very close to meeting all his milestones at only six months old with the help of OT and PT. With his vision back, Vaughn is unstoppable! We know there can always be more bumps in the road, but we have the best doctors on our side! We are so thankful to Tiny Hero and the JHACH team for believing in our family and Vaughn!
There is SO much hope… when it feels really dark, I hope stories like Vaughn's can be just what these new families need to pull through.
Love,
Vaughn’s Fam
