Cylas Thriving Thanks to an Innovative Care Approach for Congenital Diaphragmatic Hernia (CDH)

Life with Cylas started “a little rocky.”

His mom, Sarah, was 17 weeks pregnant with Cylas when an ultrasound showed a severe congenital diaphragmatic hernia (CDH), a birth defect involving an opening in the diaphragm, which is the muscle that helps with breathing.

A hernia in the diaphragm causes the contents of the belly—including the stomach, spleen, liver and intestines—to push up into an infant’s chest. This leaves little room for the lungs to develop and grow. Instead, it causes them to remain small and underdeveloped.

In Cylas’ case, his hernia developed on the left side of his diaphragm, the most common side for CDH.

Sarah’s OB/GYN referred her to a doctor who specializes in high-risk pregnancies, but the doctor “didn’t have many good things to say,” Sarah said. Because Sarah’s cousin had given birth to a baby with CDH a few years earlier and had been cared for at Cincinnati Children’s, Sarah asked for her advice.

“She told me I needed to go to Cincinnati Children’s,” Sarah said.

A New Focus in the Treatment of CDH Babies

Around the same time, in the summer of 2023, the Congenital Diaphragmatic Hernia Program was in the midst of making dramatic changes in its approach to care.

Paul Kingma, MD, PhD, CDH specialist and director of Cincinnati Children’s Bronchopulmonary Dysplasia (BPD) Center, led the program’s overhaul.

“We’ve always worked to protect the lungs of a baby with CDH,” he said. “But over the past year, we’ve taken it to a much greater level—to the point where everything we do centers around protecting the lungs. Our philosophy is that if you protect the lungs and minimize the amount of injury that occurs to them, the lungs will have the greatest opportunity for growth, and lead to the greatest long-term potential for babies.”

In addition to doing everything possible to avoid injuring the lungs, the CDH team began taking steps to help them grow.

One of these efforts included increasing the use of fetoscopic endoluminal tracheal occlusion (FETO). This procedure is performed prenatally to block the trachea, which causes fluid pressure to build and the lungs to grow. Because it can help the lungs more fully develop, FETO can increase a baby’s chance of survival.

Although FETO isn’t new—it was first performed at Cincinnati Children’s about a decade ago—the incidence with which it’s done has greatly increased. Today, Dr. Kingma says the CDH team performs between eight to 10 FETO procedures each year.

“If you go somewhere that isn’t used to caring for CDH, they may look at a baby with a severe case and say the likelihood of survival is basically zero,” he said. “Whereas we know that even babies with the most severe cases can survive and do relatively well. We also know that you can change a case of severe CDH into a mild or moderate case of CDH using FETO.”

FETO Helps with Cylas’ Lung Growth

Nervous but hopeful, Sarah was admitted to Cincinnati Children’s Fetal Care Center, which treats women with high-risk pregnancies and babies with complex fetal conditions.

The care was “immediately completely amazing,” Sarah said.

She had an ultrasound and an MRI, then sat down with her team to discuss her choices and what to expect.

“It was all pretty scary, but Cincinnati Children’s never made me feel like my only option was to terminate my pregnancy,” Sarah said.

After learning about FETO’s benefits, Sarah opted to have the procedure. The result: Cylas’ lungs more than doubled in size while he was still in utero, giving him a much better chance of a positive outcome after birth.

Born a few weeks shy of his due date, Cylas was quickly transferred to the newborn intensive care unit (NICU), which has a team of neonatologists who specialize in caring for babies born with CDH.

“The first time I was able to see him, it was very emotional,” Sarah recalled. “The care team in the labor and delivery room was amazing. They’d let me go visit Cylas whenever I wanted.”

Minimal Respiratory Support Needed

One CDH specialist, NICU nurse practitioner Christina Hastings, MSN, APRN, remembers “instantly” connecting with Sarah and noticing something right away the day Cylas was born.

“Cylas was unique. In contrast to other patients at the time who did not have FETO, Cylas required very, very minimal respiratory support,” Hastings said. “That was something new we were seeing after implementing the changes in our approach to care.”

At two days old, Cylas had surgery to repair his diaphragm, move his organs back into place and remove the portion of his liver that had herniated outside his chest wall.

A short time later, he had another surgery to insert a tube into his trachea (called a tracheostomy, or trach) to help with breathing, and to insert another tube into his stomach (called a gastrostomy tube, or G-tube) to provide nutrition. 

“Once his trach was placed, he was a different baby,” Hastings said.

An Accelerated Road Home

Soon, Cylas was healthy enough to leave the NICU and, after seven months in the hospital, go home with his parents. “He’s doing absolutely incredible today,” Hastings said. “He’s on track developmentally and hitting all his milestones. He’s absolutely thriving.”

Caring for Cylas, who was the fourth patient to go through the revised CDH program, was a group effort, Dr. Kingma said. “There were several neonatologists, pediatric surgeons, and cardiologists with a strong interest in CDH and an even stronger interest in taking care of Cylas who were all working together on his care,” he said. “That’s the whole purpose of the CDH team—having a group of experts who are all on the same page.”

Cylas also taught his care team a few things, including how to treat a baby who doesn’t require much respiratory support.

“FETO and non-FETO patients are very different, and it’s evident from the very start,” Hastings said. “They’re able to oxygenate and ventilate better. They’re able to breathe easier. Because of that, their pathway is often expedited.”

Dr. Kingma reports that, over the course of the past year, the team has noted a 40% decrease in babies’ time on a ventilator and a 12% decrease in the number of days they stay in the hospital.

'Ready to Go Move Mountains'

Over the past few months, Cylas has “taken off,” Sarah said, noting he recently turned 1 and desperately wants to walk and do everything his 5-year-old brother and 7-year-old sister are doing. Even though he still needs his trach and feeding tubes, with the help of therapies—including physical, occupational, speech and feeding—he’s talking and making strides.

“He’s just the happiest little boy,” Sarah said. “He’s ready to go move mountains.”

She added: “There were days when I didn’t know if we’d make it to the next day, if I’d ever get to take my baby home, when things were not promising, but Cincinnati Children’s never made me feel like giving up hope. I think they’re the reason I got to keep him and build memories with him.”

(Published October 2024)