A Three-Year-Old Beats the Odds with Multi-Disciplinary Specialty Care at MUSC Children’s Health

As Jessica and Joe McDevitt prepared for the birth of their second son, they had no way of knowing they would bring home a champion fighter and trail-blazer in the arena of pediatric cardiology. “Like everyone, we assumed this would go pretty much like our first,” says Jessica. But that was before a pre-natal check-up found that their baby, Hampton, had multiple heart defects. “That news upended everything.” Their doctor referred them to the Advanced Fetal Care Center at MUSC Children’s Health. “It was nerve wracking to get a whole new care team in a much bigger hospital. But we were so touched by the warmth of everyone there. They were really there for us and our son,” says Jessica.

At their initial visit, the entire fetal care team met with them to explain the diagnosis. “We were devastated,” says Jessica. “It was very severe. Only half of his heart had developed because of tricuspid atresia, hypoplastic right heart, an undersized aorta, and transposition of the great arteries.” Frances Woodard, a pediatric nurse practitioner and the MUSC Fetal Care Coordinator for pediatric cardiology was at that visit. “It never gets any easier to deliver this news, it’s heartbreaking for them and for us. I admire these parents so much. I can only hope to have the grace and strength that they do.”

The McDevitt family had a long and difficult journey before them. “This is a very challenging constellation of cardiac conditions,” says pediatric cardiologist, Sinai Zyblewski, MD. “Hampton’s diagnosis requires a series of staged palliative surgeries–one as a newborn, another at, typically, four to six months, and a third at three to four years. The surgeons can’t ‘fix’ the heart, but they can reroute circulation so the child can live with a single ventricle heart.” 

MUSC Children’s Health is the only medical center in South Carolina that performs heart surgery on infants and children. Woodard coordinates the program, “I work with the referring physicians and the families to educate them about what to expect before and after the birth, and then coordinate their surgeries at each stage of care.” The MUSC program includes a comprehensive interstage monitoring program to follow patients closely through months or even years of surgeries. “The goal of interstage monitoring is to reduce mortality and growth failure. Since we initiated the program, our mortality has decreased from 15% to 5.7% and growth failure in our patients is 7-8% while the national average is 15%,” says Woodard. “We have a telehealth system for regular at-home check-ups, online daily progress monitoring through MyChart, coordinated in-home palliative care through Hands of Hope, and a dedicated dietician just for our pediatric heart defect patients.”

Hampton had his first surgery at only four days old. A few months later, as the team prepared for his next surgery, a CT scan found more bad news. Hampton had developed pulmonary vein stenosis (PVS). “It means the blood vessels that return blood from his lungs back to his heart are very narrow and obstructed. In fact, Hampton had completely lost flow in some of his pulmonary veins,” explains Zyblewski. “That’s on top of his already complicated diagnosis. It’s rare and especially difficult to have both single-ventricle anatomy and PVS.”

This new diagnosis meant Hampton could not progress through the usual pathway of staged surgeries. “The pressures in your lungs have to be very healthy for the staged single ventricle surgical palliation to work,” explains Zyblewski. When Hampton was four months old, he underwent surgery to repair his narrowed pulmonary veins. Unfortunately, the vessels closed again shortly afterward. With few options left, Jessica and Joe chose to pursue aggressive PVS treatment at Boston Children’s Hospital. Ultimately, however, his disease was too advanced, and Hampton’s care was transferred again to Children’s Hospital of Philadelphia (CHOP) where he was evaluated for a heart-lung transplant. “We don’t have a pediatric heart-lung transplant program here at MUSC, so we transferred them to Boston Children’s Hospital and he’s now wait-listed for a transplant at CHOP,” says Woodard.

While they wait, Hampton has stayed remarkably healthy and continues to be monitored by the MUSC team. “He’s almost four years old now. We wouldn’t normally expect a child with his diagnosis to live past about two,” says Woodard. “Hampton is really following his own path at this point–from a medical standpoint we’re in uncharted territory. We’ve never had a single-ventricle patient with PVS and only first stage surgery to survive this long.” It reminds Jessica and Joe of some advice Woodard gave them when he was first diagnosed. “She told us, ‘Don’t go on Google. You’ll drive yourself crazy.’ And she was so right,” says Jessica. “You absolutely cannot dwell on the statistics because every kid is different. Your child is not the average statistic. According to the statistics, Hampton should not be alive today.”

Their success navigating this difficult journey is also a credit to the MUSC Pediatric Palliative Care Program. Conrad Williams, MD, the program’s Medical Director, met Jessica and Joe at their first visit. “We get introduced to the family early in their process, so we can develop trust and a relationship which is really important because there’s so much uncertainty when a child has a serious diagnosis,” says Williams. Palliative care is a medical specialty focused on reducing suffering in serious illnesses. “Palliative care and medical care are not mutually exclusive,” says Williams. “We provide crucial support for the child and the family for as long as their disease journey happens to be–a few months, a few years, or into adulthood. Our focus is to make sure the family understands what’s going on with their child, assist with medical decision-making, and ensure that quality of life is always emphasized.”

Having the additional support of palliative care has been invaluable for Jessica and Joe. “When you’re going through this, you have a lot of questions, so it’s great to have another medical professional to talk things over with and to be a ‘go-between’ for the parents and the cardiologists and surgeons,” says Jessica. Zyblewski agrees, “The palliative care team is priceless. They’re the crux of our family support system and facilitate communication between the many providers and families. Our palliative care team helps us provide holistic care not just for the child but the family as well.”

South Carolina has a unique, statewide, home-based pediatric palliative and hospice care service, Hands of Hope, that provides in-home care for children with serious diagnoses. Because Williams works with Hands of Hope in addition to his full-time position at MUSC, Hampton had a smooth transition from in-patient to out-patient care. “As soon as we got discharged, Conrad was coming to our home. It’s so comforting to have that continuity of care,” says Jessica. “Having the palliative care team in your corner, it’s like they shoulder a little of that burden with you. You can really lean on them.”

For now, they’re waiting for the call that a transplant is available, and Hampton is enjoying time with his family and brothers. “For having a very serious disease, life is really good right now,” says Jessica. “His favorite things are trucks–he has an excavator that he plays with in the yard. He loves to swing and to walk down and watch the boats at the boat ramp. He loves watching airplanes.” Although no one can predict the future, the entire team at MUSC Children’s Health plans to keep providing the best care possible to ensure Hampton stays healthy and can keep enjoying life to its fullest.