The outlook for Beau was grim so his parents decided to put their baby’s life in the hands of a man they had never met.

Beau was in a Minnesota hospital on a form of heart-lung bypass support called extracorporeal membrane oxygenation (ECMO). Doctors there wanted to wean Beau off ECMO before performing surgery to repair his condition — congenital diaphragmatic hernia (CDH).

CDH is a life-threatening birth defect that occurs in about 1 in every 3,600 births. It happens when the diaphragm — the muscle that separates the abdomen from the chest — does not develop completely. This creates a hole where abdominal organs such as the liver can migrate into the upper part of the chest, which can inhibit the growth of the baby’s lungs.

With Beau struggling, his parents — Bri and Jay — began considering alternatives.

“The hospital made several attempts to wean him off ECMO to do the repair surgery, but it was not successful,” Bri says. “Jay and I were not very confident with his progress, so we started doing some more research. To be honest, our outlook for Beau was pretty grim at this point.”

 A New Hope 

As they reviewed web pages, blog posts and input from CDH support groups around the country, Bri and Jay discovered the Center for Congenital Diaphragmatic Hernia at Johns Hopkins All Children’s Hospital in St. Petersburg, Florida. The program is the only unit in the country dedicated solely to the care of CDH and has a success rate above 90%, well above national benchmarks. Medical director David Kays, M.D., has cared for more than 700 infants with CDH.

“After we made an inquiry at the hospital, Dr. Kays called us personally and assured us that he was confident that he could save Beau,” says Bri, tearing up.

Kays has vast experience managing ECMO cases. He frequently performs repair surgery, which involves putting the organs in their proper place and repairing the hole in the diaphragm, while babies are on ECMO.

“When a baby like Beau is on ECMO, the blood is thinned out and does not clot, so doing major surgery on a baby whose blood doesn’t clot is a scary thing,” Kays says. “Some hospitals are not comfortable doing that.”

In most cases that come to Johns Hopkins All Children’s, families from around the country learn about Kays and the Center for CDH before the baby is born and travel to St. Petersburg before birth. Kays often attends the birth and reliably performs repair surgery within the first week.

Beau’s case presented an unusual wrinkle. He had been born about 1,500 miles from St. Petersburg, was in a fragile condition and needed to be transported while on ECMO.

“When we were contacted about coming to get Beau and doing his repair surgery while still on ECMO, we said, ‘Yes,’” Kays says. “Doing this type of surgery while a baby is on ECMO is part of our normal management.”

Fragile Cargo  

Kays and his team flew to Minnesota on a medical transport plane to personally supervise Beau’s trip to Florida.

“We had never met Dr. Kays,” Jay says. “We talked to him on the phone for about 10 minutes. And now we are putting all our trust in him to fly up to Minnesota and save our baby. That first phone call was on a Monday and by the next Sunday, he’s telling us his team from Johns Hopkins All Children’s is coming to get Beau.

“I was thinking about how fortunate we were that someone was willing to put down everything they are doing, fly to Minnesota and pick up our baby. We asked the doctors and nurses at the hospital back in Minnesota if they had ever had a baby transferred out on ECMO. They all said no.”

“This was our last hope,” Bri says. “It was humbling to know we found someone like Dr. Kays just in time.”

Kays acknowledges it’s unusual to take a medical team by jet aircraft across multiple states to pick up a baby on heart-lung bypass.

“It’s not routinely done, but it’s not new to our team,” he says. “We’ve done this six or seven times already. We may be the only CDH program doing this, but we believe fundamentally that these babies can survive, so we were happy to make the trip.”

In babies with CDH, the risks are generally with the lungs and especially the brain, which if deprived of sufficient oxygen, may not develop properly.

“I was upfront with the parents when I first spoke to them,” Kays says. “I told them we can repair the CDH, but I can’t tell you for sure there hasn’t been any lung injury or brain damage before we took over his care. You can’t predict the outcome as well when you don’t care for the baby from birth.”

A Successful Trip  

Kays performed Beau’s surgery four days after they arrived in St. Petersburg. Ten days later, Beau was off ECMO.

“It took a little longer in Beau’s case because we had to coach his lungs for them to get stronger,” Kays says.

Kays says he expects Beau to have a good outcome. “It’s very gratifying for us as a team,” Kays says. “It’s a slow process, but he’s getting a little stronger every day.”

Bri and Jay are grateful.

“It’s amazing to see how far Beau has come,” Bri says. “We had to wait two months just to hold him for the first time. Now we are looking forward to the day when he is an active, rambunctious little kid.”

“We hope the strategies that Dr. Kays uses at Johns Hopkins All Children’s can be shared with other hospitals,” Jay says. “We understand we were super fortunate. Everything fell into place for us to come to St. Petersburg. I hope in the future, families won’t have to travel this far to get this level of care.”