The doctors in Michigan weren’t optimistic in discussing an early diagnosis of congenital diaphragmatic hernia (CDH) with Ashlee and Jordan. They didn’t believe the baby would survive very long after birth.

Ashlee, an emergency center nurse, was not to be deterred. She sought hope. She, her family and her friends, all immediately began researching this mystifying disease that involves a hole in the diaphragm, which allows the shifting of organs into the chest cavity and often stifles the growth of lungs before the baby is even born. Ashlee found David Kays, M.D., and the team at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida. Instead of discussing unpleasant options, Joy Perkins, the nurse coordinator for the CDH program at the hospital, asked what the baby’s name would be.

Olivia became the first patient treated in the new Center for Congenital Diaphragmatic Hernia at Johns Hopkins All Children’s Hospital. The 15-bed unit, believed to be the first in the country to devote 24/7 care exclusively to CDH patients, was formally unveiled in a ceremony Oct. 24 in the hospital’s Research and Education Building.

Unconventional Approach

CDH affects 1 in 3,000 births, and few doctors have consistent success treating it.

Kays, medical director of the Johns Hopkins All Children’s CDH program, has been treating CDH for more than two decades. He takes an unconventional approach, performing surgery to shift the organs and repair the hole just a few days after birth, earlier than most. Follow-up care promotes lung and neurodevelopment. While many recommend these pregnancies be terminated, Kays believes nearly all of these babies can survive with early intervention and dedicated follow-up care. A network of elated parents across the country help tell of his team’s success. More than half of the program’s patients come from outside Florida.

Infants treated at Johns Hopkins All Children’s, even severe cases, survive at rates in excess of 90%, which greatly exceeds national benchmarks.

“We thought at first this is too good to be true,” Ashlee says. “Are we sure they are that good? But we found they are that good and the rest is history. We kind of just knew that was the place we wanted to go.”

“This isn’t just fixing the hole,” Kays says of his approach to CDH care. “This is a disease process. The operation is very important, but what we bring to the table at Johns Hopkins All Children’s is the whole concept of care. It’s long-term. These babies deal with secondary effects of having small lungs, of not being good eaters, they are smaller and they carry this physiology for years, but the good news is they get better and better as they grow.”

The Right Choice

Ashlee does not regret her decision to drop everything and temporarily move from Michigan to Florida to give birth and be seen by Kays.

“He was very upfront with us right from the start, and that’s exactly what we needed,” she recalls. “From day one, he had a plan and his confidence meant everything to us. We completely trusted him.”

Kays is direct, but he exudes a warmth that explains his office, which is filled with photos of happy, healthy toddlers whose parents sent updates, and cross-stitch patterns that say, “You’re the best,” likely stitched from the comfort of a patient-room couch. There are even blue baby footprints next to the words, “Greyson loves the CDH team.” You get an idea this CDH crowd is one big happy family.

Olivia, meanwhile, was deemed a “moderate” case by Kays, but he warned the family her low birth weight may require some time on extracorporeal membrane oxygenation (ECMO), a heart-lung bypass that helps the baby’s lungs develop. In Olivia’s case, she also had an intestinal perforation, which set her back a bit, but Kays was able to complete the repair while she was on ECMO, and even closed the intestine in the same operation. Olivia did beautifully and made it back to Michigan just in time for Christmas.

Ashlee thinks fondly of the team in Florida, not just Kays and Perkins, but also Stacey Stone, M.D., the nursing team and others.

“We don’t ever praise just Dr. Kays for this because we know it’s a whole team approach,” Ashlee says. “The nurses sat by my kid’s bedside for hours and hours. Even after Olivia was discharged, we are able to get questions answered.

“Dr. Stone sat by her side on ECMO for four hours. If it wasn’t for the whole team, I don’t think Olivia would be here.”

The CDH center is an expansion of the program that has been seeing more patients each year since Kays’ arrival. Surgeons such as Stone, Keith Thatch, M.D., and C. Jason Smithers, M.D., are joining as are specialists from neonatology, perinatology and other disciplines. Patients come from across the country for this expertise. The program is happy to take them all on.

“The misconception is that these kids go home damaged or do poorly,” Kays explains. “Pediatricians say they are surprised these kids come to them looking so good. Olivia is going to do great. That’s what we do here.”

Olivia celebrates her first birthday this month. She’s crawling and on the brink of walking. She’s a little small, but otherwise a typical baby, her mom says.

She also made a recent trip to Florida, a check-up and a visit with the team where it all began in the Center for CDH.

The Kishinchand Chellaram Foundation supports the Center for CDH and research within the program.