St. Pete hospital believes it has highest survival rate for rare birth defect

National survival rate 50%, doctor's is 95%
Posted at 6:15 PM, Jun 01, 2018
and last updated 2018-06-01 20:13:39-04

ST. PETERSBURG, Fla. — A Bay Area doctor is revolutionizing treatment for very sick babies. A St. Petersburg hospital believes its best practices give them the highest survival rate in the world for a serious birth defect.

Whenever Arabella cries, parents Mindy and Christopher Koehler only feel joy. That’s because of a devastating diagnosis they got during an ultrasound.

“My child has a problem," mom Mindy Koehler described when she got the news. "I felt like so confused and lost.”

Their doctor told them it was congenital diaphragmatic hernia or CDH. A birth defect that impacts one of every 3,000 pregnancies. For babies with CDH, the diaphragm does not develop properly. Abdominal organs move up through the resulting hole in the chest, squeezing off healthy lung growth. CDH basically leaves babies with dangerously small lungs. Only about 50% of them live past their first six months. The severity impacts survivability with worse cases as low as 10% and the less severe cases as high as 70%, on a national average.

2-month-old Arabella had the most severe form of it. Doctor after doctor advised the Koehlers to consider aborting because she would only have a 10% chance of living. These parents were left hopeless and heartbroken until a CDH support group pointed them to Johns Hopkins All Children’s Hospital and Dr. David Kays.

“And he said ‘I will tell you that she’s got enough lung to survive’ he said. ‘she may not win the marathon but she will participate.’ He gave us an 85% chance.’" said Christopher Koehler of his conversation with Dr. Kays.

“We believe we have the highest survival rate in the world for CDH," said Dr. Kays.

At more than 95% it's sparking a surge of patients from around the world. It convinced the Koehlers to travel from coast to coast. They flew in from California for Kays' help. Part of the doctor's secret to success is taking the risk of surgery. Nationwide doctors are reluctant to do surgery for fear of making the sick babies worse.

“Doing a major operation like a CDH repair on a baby whose blood doesn’t clot is a scary thing and leads to a high risk of bleeding," said Kays.

This is the part where Kays differs from other doctors. He believes repair surgery is critical to helping these infants use their lungs. Kays wants other hospitals to be as successful that's why he's working on publishing his data in a medical journal. Hoping this will give more of these newborns back their ability to cry and save their lives.

“I was waiting just the whole time to hold her," cried Mindy Koehler.

She only got to touch Arabella once the tubes were removed. More than a month and a half after she was born.

“It used to be that the families didn’t believe us but it’s happened enough times that they believe it," said Kays.