Riley program drastically reduces death rate for babies with rare heart condition

INDIANAPOLIS (WISH) — Finding out your unborn baby has a heart condition can be a heart-wrenching experience for parents-to-be. 

Nearly a decade ago, Riley Hospital for Children launched a program to help parents cope with the stress and responsibility of hypoplastic left heart syndrome, but what they’re finding out now, is that it’s literally saving lives. 

February is American Heart Month and the babies at the center of this program are born with essentially half a heart. Without medical intervention, they would likely live just days at the longest, but when it is diagnosed in-vitro, the babies are treated and undergo a surgery within the first days of life. Then they must hang on until six to eight months old before undergoing yet another imperative surgery and it’s often up to the parents alone to make sure the babies make it to that point. 

“You hear everyone talk about how it changes everything and it really does,” Jon Jones said. 

He and his wife Ashley Jones were expecting their first child in 2017, when a routine ultrasound showed something unusual. 

“I know their world is going to be shattered the minute I start saying your baby has heart disease,” Dr. Anne Ferrell said. 

Baby Jaxson has hypoplastic left heart syndrome, meaning half of his heart didn’t develop properly. 

“It’s an experience that you wouldn’t wish on your worst enemy,” Jon said. 

Jaxson arrived and one week later went under the knife. That’s when the Jones family was quickly swept up by a medical team at Riley Hospital for Children. That team trains parents to have the knowledge to take their baby home and continue care for them until that second surgery months down the road, with constant monitoring by hospital staff through secure technology. 

“Before we had the home monitoring program these parents were just at the mercy of looking their child without any objective way to know if anything was changing and had to rely basically on their own intuition to some extent to be able to alert medical staff that they felt something was different or changing,” Ferrell said. 

Farrell and Dana Hartman lead the program. Hartman serves as a 24-hour go-to for parents. 

“I get anywhere from probably 30 to 50 texts a day, frequently phone calls,” Hartman said. 

She also receives data, pictures, and videos through an iPad each family is supplied. 

“I had a love-hate relationship with that iPad, but it would have been more stressful without it,” Ashley said. 

She’s a nurse, but still felt the weight of the world when taking her baby home between surgeries.
 
“No nurses. No doctors. Nobody’s here. It’s just us,” Ashley said. 

“It’s a scary time, because you know that any illness any setbacks will put him back in the hospital, so you’re really careful about who he interacts with, taking him out into public, keeping him healthy and as good as he can be,” Jon said. 

The Jones family said the home monitoring system gave them a mental and physical safety net when caring for Jaxson, which allowed them to bond with their baby and enjoy his early months

“It decreases their anxiety to a level that I think has totally changed the level of care for these babies and most importantly for their parents,” Ferrell said. 

Perhaps that lessened stress is enough to call the Riley home monitoring program a success. That’s all Farrell and Hartman had to point to before. But with the start of 2019, the pair finally had enough data from enough babies to see if their work is changing the outcome for those with hypo-plastic left heart syndrome. 

Nationally, 18 percent of babies with this condition die between their two surgeries. That’s about one in five. But at Riley, babies under the home monitoring program had a mortality rate of just five percent. That’s one in 20. 

“That’s huge. However many babies that now are here and so that’s very heartwarming and encouraging for us,” Hartman said. 

Babies like Jaxson, who just celebrated his first birthday and is living a happy healthy life. 

“He’s great. He’s smiling all the time. He’s laughing,” Jon said. 

“I mean it’s just that’s mind-blowing honestly. To have a program like that and to be able to monitor these kids in that way… It’s just technology is wonderful, but it’s changing lives too,” Ashley said.