Rhiley’s Story - Congenital Diaphragmatic Hernia

My husband and I were elated when I found out we were pregnant in January of 2022. I was so excited to be a mom finally and couldn’t wait for my little baby to make their entrance into the world. I went to my eight-week prenatal appointment and saw my little gummy bear (legitimately looked like a gummy bear in the ultrasound) for the first time. A few weeks later, we found out via NIPT testing that we were having a girl! The NIPT test also revealed that she didn’t have any genetic disorders that it tested for. The weeks went on, and my little belly continued to grow, and so did my love for my sweet Rhiley.

On May 23rd, I went to my twenty-week anatomy scan nervous but excited to see how much my baby had grown and to be sure she had all ten fingers and toes. Watching the ultrasound on the screen, I was amused and brought to laughter as the ultrasound tech chased Rhiley around as she wiggled and turned with every swipe of the probe. After the ultrasound, I watched as the tech chose normal from the drop-down box for Rhiley’s arms, legs, head, etc., but once she got to the abdominal section, she stopped. I didn’t think much of it then and continued to admire the printed ultrasound pictures of my baby. I then went to the exam room and waited for my doctor to come in and tell me everything was fine and how healthy my sweet babe was.

Instead, he told me he thinks she may have something called Congenital Diaphragmatic Hernia, or CDH for short. Not knowing the full spectrum of severity or what the heck CDH was, I broke out in tears. I called my husband and had the doctor explain to us both what this meant moving forward. He was not 100% sure and referred us to an MFM doctor for further testing. Two days later, I had an appointment with the MFM doctor, and they did an ultrasound and confirmed that she did, in fact, have CDH. I also opted to do an amniocentesis to test for genetic and chromosomal disorders not tested with the NIPT testing, which returned negative. I was told that the hospitals in Shreveport, Louisiana, do not have level 4 NICUs or ECMO equipment that Rhiley may need so I would have to go to Houston to give birth there. I was referred to the Fetal Center at UT Physicians. I found out that they had one of the best clinics for babies with CDH.

I started to travel back and forth to Houston once a week for appointments. We were told that Rhiley’s defect size on a scale of A through D, A being mild and D being severe, was between a B and a C. They believe she only had a 30% chance of needing to go on ECMO once she was born and had a 75% chance of survival. Still, Dr. Harting stated that she would have about 80% survival odds at CMHH. After follow-up ultrasounds and a two-hour MRI, they estimated 21% of her liver, stomach, and intestines were up.

We knew it would be a tough journey, but we had faith and knew Rhiley was strong and would kick CDH’s butt.

At twenty-eight weeks, while driving to Houston for an appointment, I began to have contractions. Once I arrived at my appointment, they did tests to monitor my contractions and an ultrasound to measure my amniotic fluid. It was determined that I was in preterm labor, so I needed an amnioreduction and medication to stop the contractions. I was admitted to the hospital and had the procedure done a couple of hours later. I was in the hospital for four days so they could monitor Rhiley because an amnioreduction could also cause preterm labor. I made it through the four days but was an absolute nervous wreck. The doctors told me the chances of my fluid levels rising to the same amount it was pre-amnioreduction was high, so I was told I needed to relocate to Houston around 34 weeks in case I went into labor early. We relocated at 33 weeks to be safe.

At 38 weeks, Rhiley was born on September 26th at one of the best hospitals in the world with a team of doctors who are dedicated to helping babies born with CDH. She was placed on ECMO at six hours old, and it was a shock because she only had a 30% chance of needing it. She was having a hard time blowing the C02 off her lungs; her C02 gasses were extremely high, making her blood very acidic. On day two of life, she had her CDH repair surgery, and they discovered that her defect size was a D-sized defect, but her lung volume led them to believe her defect size was smaller. The eight days she was on ECMO felt like the longest days of my life.

She went from being intubated, extubated to CPAP, and then weaned to high flow in 2 months. She had severe acid reflux, which caused her progression to slow, so on December 1st, she had another surgery to place a g-tube as well as a procedure called Nissen Fundoplication (the top of the stomach is wrapped around the bottom of the esophagus to help reduce severe acid reflux or GERD).

She is still working on respiratory support and weaning how much support she needs. We are not sure when we will be home, but we are thankful to have such a wonderful team of doctors, surgeons, and nurses here at Children’s Memorial Hermann Hospital, helping our sweet baby girl. We are lucky to have the support of the Ronald McDonald House here in Houston as well.

For all of the CDH parents out there currently in the thick of it like us, know that these babies are stronger than you can imagine. They fight like hell and are so resilient. Also, remember to give yourself grace and know that a whole community is available to help get you through what is most likely one of the hardest things you will ever experience.