A heart for Hunter

Hunter weighed just under 5.7 pounds.

To date, Hunter was the smallest— and most complex—baby to go on the Berlin Heart at UF Health.

photo of Hunter in strawberry patch

Hope can look like a lot of things: A penalty kick during a nail-biting tie in a soccer game. Rain clouds appearing during a drought. 

For Nicole and Jonathan Fenix, hope looked like their son Hunter. Born just under 6 pounds, at 37 weeks, on March 14, 2018, Hunter was long-awaited, much-anticipated, and already very, very loved.

Before Hunter was born, his parents knew three things:

He had hypoplastic left heart syndrome.

Treatment required a Norwood procedure.

To date, there had been no successful outcome of a Norwood procedure in the Philippines, where they lived.

Hypoplastic left heart syndrome, or HLHS, is a congenital defect that affects how blood flows through the heart. During pregnancy, the infant’s heart’s left side forms incorrectly. Hunter also had another complication — a coronary fistula, where an artery and a blood vessel abnormally connect.

“You have two choices,” Hunter’s physician told Nicole after a somber ultrasound. “You can either take your son home and have him expire, or you can take him somewhere he can access the treatment he needs.”

The Norwood procedure, which focuses on building a newer, larger aorta, is one of the more complex open-heart surgeries, compounded by the size and age of the patient. It is one of three operations used to treat HLHS, each of which takes place at different stages throughout the child’s development. The time between operations can be fraught with complications and low survival rates.

The complexity of the procedure — and the critical postoperative period where mortality rates can increase — brought the Fenix family to UF Health Shands Children Hospital, which boasts some of the best postoperative and interstage outcomes worldwide. The post-surgical care process in the Cardiac Intensive Care Unit, or CICU, is a key component of their consistently high survival rates, Nicole Fenix said.

Jennifer Co-Vu, M.D., associate professor in the UF College of Medicine and director of the Fetal Cardiac Program in the UF Health Congenital Heart Center, first consulted with the family when they were still in the Philippines. Co-Vu leads an iPad-based platform that garners a 100% interstage survival rate by allowing doctors to monitor patients’ vitals after discharge.

“We teach them to weigh their kids every day — to get their pulse, oxygen saturation, and plot the data,” Co-Vu said.

This data provides health care teams with updates that indicate whether or not the baby is continuing to do well.

“This [program] was a large part of what led us to choose UF Health Shands amongst at least five other institutions in the U.S., Asia (Taiwan) and Europe (London),” Jonathan Fenix said.

But when Co-Vu conducted a second fetal ultrasound (fetal echocardiogram), she noticed Hunter’s HLHS was a rare subtype that would require total heart transplantation for him to thrive.

While Hunter waited for a transplant, he was put on the Berlin Heart, a machine that offers mechanical support for end-stage heart failure and acts as a “bridge” to transplant. At the time, he weighed just under 5.7 pounds–the smallest baby to go on the Berlin Heart at UF Health yet.

During the 26 weeks Hunter, Nicole, and Jonathan waited for a heart, they kept hoping.

They held that hope as the weeks turned into months. Then, they held that hope when Hunter was diagnosed with a blood infection, respiratory failure, and all of his organs shut down save for the brain, which was being oxygenated by the Berlin Heart — Hunter’s mechanical “safety net”.

Hunter’s physicians told her they would do everything they could, but it was up to Hunter to pull through, and for the Fenix family to keep hoping.

“Our hope is in a God who is never taken by surprise,” Nicole said. “The more impossible the situation got, the more we knew that Jesus would work to glorify His name. So however things turned out, we always looked back to our faith and this kept us hoping.”

On Sept. 12, 2018, Hunter got his new heart.

Due to the swelling from the infection he’d spent weeks battling, his tiny rib cage had expanded and was able to accommodate a transplanted heart that otherwise would not have fit.

Doctors called it luck. Nicole and Jonathan called it divine providence.

“God will cause all things to work together for the good of those who love Him and for those who are called, according to His purpose.” Jonathan said. “This is our hope.”

Before coming to UF Health, the Fenix family had done their research. They pored over statistics and outcomes from at least five renowned children’s hospitals around the United States. They spoke to countless physicians and read hundreds of articles about Hunter’s condition before making their decision.

Hunter’s pediatric surgeon, Mark Bleiweis, MD, has extensive experience in the pre- and postoperative management of newborns, including premature and low-birthweight babies. His expertise includes repairing complex, single-ventricle and valve defects, as well as treating patients who have underlying syndromes. 

There were other institutions that came with their own international reputations for leading-edge medicine and experienced physicians. But it was what came after the surgery that made Nicole and Jonathan know they made the right choice.

Hunter’s clinicians didn’t just provide his parents with updates, they welcomed them as members of his health care team. At every point, they were encouraged to ask questions and participate in his care.

“He is not just a patient, he is our son,” Jonathan Fenix said. “It was a true partnership between the medical providers and us, his parents.”

When they left the hospital, they felt confident that they could care for their baby. Nicole assisted the nurses daily, and their UF Health team was and still is only a call away.

“Many of the parents or guardians in transplant groups have questions I’m able to answer because I can easily just ask my doctors,” Nicole said.

Co-Vu’s approach to care revolves around treating the person, not just a patient.

“Our goal surpasses having babies be alive,” she said. “We want all of our patients to grow up to be adults who can aspire to be more than just alive, to have those quality-of-life outcomes as well as optimal cardiovascular ones.”

Hunter and his providers.

When she sees what parents do for their children, it makes it easy.

Nowadays, 2-year-old Hunter can tout a clean bill of health. Nurses and physicians from his team, like Frederick J. Fricker, M.D., and Biagio “Bill” A. Pietra, M.D., Hunter’s pediatric cardiologists, have become almost like family.

“His development is progressing incredibly well despite everything he’s been through, and he’s just one of the sweetest babies,” said Fricker, an eminent scholar and professor in the UF College of Medicine who pioneered the pediatric heart transplant in the United States in 1982 and monitored Hunter upon his discharge from the hospital. “It’s been a pleasure to be a part of his care.”

Due to Hunter’s immunosuppressants, Jonathan and Nicole travel with cleaning wipes, globs of hand sanitizers, gloves and face masks – accessories very much in vogue due to the COVID-19 pandemic.

“We’ve always had to be careful about infection due to Hunter’s condition,” Jonathan said. “So in a way, having precautions is our normal life, but now it is heightened.”