Congenital Diaphragmatic Hernia: Jackson's Story
It is closing in on midnight.
United flight No. 800 out of Dulles International Airport is at cruising altitude, destined for Tampa.
In seat 9C, Jacqueline, a new mother, cannot stop crying. She’s not sure whether the hot tears on her face are out of fear or relief, or the mingling of both.
Somewhere in the night sky ahead of them, Jacqueline’s fragile and critically ill newborn lies in the belly of a cramped Cessna airplane—a Citation II—bound for St. Petersburg.
What this mother wouldn’t give to be with her baby boy right now.
This had never been a part of Jacqueline’s birth plan. But neither was the devastating diagnosis she had received 23 weeks into her pregnancy–that her baby would be born with congenital diaphragmatic hernia (CDH) … a malformation of the diaphragm that results in abdominal organs being pushed up into the chest, choking off lung growth. Her baby has what specialists call a "right-sided CDH" with "liver up," meaning it is among the more rare and severe of CDH cases.
Doctors at the University of Maryland Medical Center in her hometown of Baltimore gently explained her odds and her range of options.
“They didn’t so much say I should terminate, but they said a lot of parents do terminate,” says Jacqueline.
Jacqueline wasn’t going to terminate. She quickly launched into research on how she might pursue the best possible care for her baby. Her quest led her to Johns Hopkins All Children’s Hospital in St. Petersburg, where the CDH program, directed by David Kays, M.D., is achieving remarkable outcomes.
Nationally, for babies with CDH who need ECMO (extracorporeal membrane oxygenation—an advanced form of heart and lung life support), the survival rate is 50 percent. But CDH patients at Johns Hopkins All Children’s Hospital who require ECMO survive at rates exceeding 90 percent.
After one visit with the team at Johns Hopkins All Children’s Hospital, Jacqueline was certain that she wanted to have her baby in St. Petersburg. The plan was for her to relocate from Baltimore three weeks before her delivery date. She’d have access to all of the best care this nationally acclaimed program has to offer.
As it turned out, Jacqueline’s baby had other plans. At 1 a.m. on Feb. 27, six weeks early, her water broke. Son Jackson was brought into the world in Baltimore. He was as critical as expected, and within 24 hours was placed on ECMO.
But here was a mom who didn’t give up easily. Jacqueline emailed one of the CDH team members from Johns Hopkins All Children’s. "So here’s what’s happening. Is there any possible way we can get him down there?" She knew the odds were against her.
“I just didn’t know how you transport such a sick baby,” Jacqueline says. “I didn’t know how you do that. But if it was safe, and if it was possible, I wanted to do it.”
At 2 a.m., nurse coordinator Joy Perkins gets Jacqueline's email. She answers it with cautious encouragement but makes no promises. Perkins informs Kays, and the wheels start turning. Can they do this? Should they go pick up a critically ill infant and bring him back to Johns Hopkins All Children’s? It was not something this team had ever done before.
“We had an opportunity to help a baby,” Kays says. “We knew the child had a better potential outcome in our hands if we could safely get him here. That was the question. I felt the odds were good.”
Kays reached out to Lisa Moore, senior director of nursing in the cardiovascular intensive care unit, via text:
Kays: Want to do an ECMO transport? There’s a CDH on ECMO that the mom wants to transfer here.
Moore: Lol. You’re killing me!
Kays: I’m only half-joking.
Moore: Which part is the joke?
Kays: Not sure. I was hoping you could tell me.
Moore: How can I help?
The next few days are a scramble. Kays is gratified to receive support from hospital leadership, but there are moments when the details involved seem almost insurmountable.
In record time, they must secure a plane outfitted for medical transport and a highly skilled flight crew. Saturday turns into a planning day for a Sunday flight. There is an extraordinary amount of equipment necessary to transport a baby on ECMO. Could they fit all the people and equipment they needed on a small Cessna and make it work?
At 10:40 a.m. on March 4, two pilots and a crew of four take off for Baltimore. On board are Kays, Michelle Chadbourne, ARNP, respiratory therapist Dean Shepherd and perfusionist James O’Brien, who would be in charge of the ECMO equipment.
The plane is stuffed with seven IV pumps, two sets of medical gases, the ECMO pump and the massive tubing that goes with it, a heating unit for the ECMO circuit and three monitors.
“I couldn’t tell you the weight of all the equipment, but it is the most I’ve carted on a transport in thirty years,” Shepherd says.
The team flies into Baltimore on the heels of a nor’easter. With the last of the high winds still in play, the pilot maneuvers the plane down onto a private airstrip at BWI Airport.
It takes two ambulances to move their equipment from the tarmac to the University of Maryland Medical Center. Their challenges are just beginning.
Cut and Paste
The clinical staff at the University of Maryland Medical Center are supportive of Jacqueline’s wishes and ready to assist—but the highly complex job of preparing an infant on continuous heart-lung bypass for air transport would present some challenges.
Kays learns that baby Jackson is on a different blood thinner than he is accustomed to prescribing. That would have to be factored in.
A small spot on Jackson’s brain had shown up in an earlier head ultrasound that is now a source of concern. Could they keep him stable during the flight so as not to exacerbate any potential brain bleed, if there is one at all?
Then there’s the critical task of switching from the University of Maryland Medical Center’s ECMO to the Johns Hopkins All Children’s team’s ECMO equipment. It is a complex cut-and-paste job. They make it happen.
Then, another challenge—keeping the baby warm enough as they move him to the plane. A piece of equipment called the "heater/cooler" keeps the baby’s blood at an optimum temperature when it’s connected to a power source. But unplugging and moving the infant even a short way across a tarmac in 34-degree weather proves daunting.
“The baby’s entire blood volume is circulating outside of his body into the ECMO circuit, which is not insulated,” Chadbourne says. “That was a big challenge.”
The final test is maneuvering a sick and fragile infant attached to a massive amount of equipment into a small plane with an even smaller door.
“It was hard,” Kays says. “Getting the baby on. It was 10 or 15 minutes of just trying to … It was a lot of ‘Stop … go. Now left … right. And you’re moving an inch at a time trying to get it all on.”
Finally secured onboard, baby Jackson would experience his first flight—destination: Johns Hopkins All Children’s Hospital.
If an experienced physician, secure in his own skill level, would ever admit to worrying, perhaps it would be during this flight home. Was the baby’s blood too thin? Was his brain okay? Would the stress of moving him be worth the benefit Johns Hopkins All Children’s could provide? David Kays hoped and believed it would be.
“This is part of what we’re here for,” Kays says. “To push the envelope and do some things other people are afraid to do. I had to trust in our ability to take good care of him. I had great confidence that we would pull this off and do it well.”
The Cessna touches down close to midnight at St. Petersburg-Clearwater International Airport. Baby Jackson is transported to Johns Hopkins All Children's cardiovascular intensive care unit (CVICU). A skilled clinical team is ready and waiting for him.
An hour later, mom Jacqueline lands at Tampa International Airport and gets a fast ride to the hospital.
“Let’s just say my Uber driver did not go the speed limit,” confesses Jacqueline.
She rushes up to the CVICU, anxious to reunite with her baby. She’s not completely sure of what she’ll find. When she reaches his room, she sees that her baby boy is all set up and stable and ready to be made well.
Mom can exhale. And she cannot stop smiling.
One day later–a head ultrasound shows Jackson is fine. No new signs of a brain bleed.
A few days later—Jackson is weaned off of ECMO. Kays performs a successful surgery to repair his diaphragm.
At three weeks—Jackson is extubated (tubes removed) and Jacqueline gets to hold her boy for the first time. She holds him for hours, making up for lost time.
At four weeks—Jackson is learning to eat and learning that he likes it.
Just recently—the best sound ever. CDH babies usually don’t cry at birth because they are unable to take in a full breath. But Jackson has found his voice.
“It’s kind of awesome to hear,” says Jacqueline. “It shows how strong he is.”
The Center for Congenital Diaphragmatic Hernia at Johns Hopkins All Children's Hospital
At the Johns Hopkins All Children’s Center for Congenital Diaphragmatic Hernia (CDH) in St. Petersburg, Florida, families find hope during a time that may otherwise feel hopeless.
Led by CDH expert David Kays, M.D., our team combines compassionate care, innovative techniques, and cutting-edge technology and equipment to treat babies with CDH.