(CNN) — When 6-year-old Jack Foley crossed the finish line last month at the Long Beach Kids Triathlon, it was more than an athletic achievement. His completion marked a remarkable recovery from a rare heart condition.
The journey began before Jack was born.
At a routine 20-week sonogram, doctors told parents-to-be Rob Foley and Lauren Kiefer-Foley of Long Island, New York, that the fetus’ heart wasn’t properly developing.
“The doctor told us, ‘we can’t see the left side of the baby’s heart,'” Kiefer-Foley recalled. “He told us, ‘you need to get to a cardiologist first thing in the morning.'”
Further tests would confirm a diagnosis of hypoplastic left heart syndrome, a rare birth defect affecting the heart’s ability to pump blood through the body. The Centers for Disease Control and Prevention estimates that fewer than 1,000 babies are born with the condition in the United States each year.
Doctors informed the couple about the risks and challenges their child could face.
“We were advised by many doctors to terminate our pregnancy because the condition is so severe. They told us that most babies don’t even survive birth, let alone the first surgery,” Kiefer-Foley said.
Nonetheless, the couple decided to give their baby a chance.
After Jack was born at full-term via a scheduled C-section, doctors took him straight to a neonatal intensive care unit to administer the level of care he would need before his first open-heart surgery days later.
“When the doctor said ‘it’s a boy!’ I didn’t get to see him for hours after that,” Kiefer-Foley said.
They would also have to wait until after Jack’s first surgery to hold him for the first time.
“It just sucked, for lack of a better word, to not be able to see him for so long, and I didn’t get a hold of him until he was 9 days old,” Kiefer-Foley said.
With a successful delivery behind them, it was now up to Dr. Emile Bacha, chief of congenital and pediatric cardiac surgery at Morgan Stanley Children’s Hospital of New York-Presbyterian/Columbia University Medical Center, to guide Jack through a series of surgeries to repair the newborn’s heart.
‘Super Jack’s’ remarkable recovery
Central to the circulatory system, the heart is made up of four chambers and valves that allow blood to flow through the body. Abnormalities in the heart’s structure — such as a valve not functioning properly — weaken its ability to work properly.
In Jack’s case, half of the chambers — the left atrium and ventricle — were underdeveloped and unable to pump blood.
“So you’re born with not only half a heart but the weaker of the two sides. And that’s a very, very severe diagnosis,” Bacha said.
When this happens, the right side of the heart is left to do twice as much work, pumping blood to the lungs as well as throughout the body. The heart of a newborn can compensate for only a few days or hours post-delivery.
Left untreated, the condition is invariably fatal. However, it is treatable with multiple surgeries to increase blood flow to the body and bypass the poorly functioning left side of the heart.
Four days after birth, doctors inserted a shunt into Jack’s heart to keep it open for blood flow. A second surgery at 4½ months would reroute his blood flow. The third-open heart surgery would complete circulation throughout his body.
“These procedures have a significant risk associated with them. The first one, in particular, has about a 10 to 15% chance of dying from surgery, and then the second surgery has another 5% risk of dying, and the third has the same thing. So you have a cumulative risk of not making it that’s pretty significant,” Bacha said.
The goal, he says, is simply to survive. But as one of the NICU nurses told Jack’s parents, his recovery was far better than expected.
“She said to us one day, ‘you know, kids with this normally don’t do this well.’ She goes, ‘you might have a little Superman on your hands.’ And then I just looked at him and I went, ‘that’s right!’ I was like, ‘you’re my Super Jack,’ ” Kiefer-Foley said.
A major accomplishment
Six years and three open-heart surgeries later, Jack isn’t letting the fact that he has half a functioning heart stop him from leading a normal life. The active boy enjoys roller and ice hockey and just competed in his first triathlon.
Adorned with his Superman-inspired logo, Jack completed a lap in a pool, did a short bike ride around a park and ran the bases at a baseball diamond to complete his first triathlon in Long Beach, New York, on June 17. He completed the race in his signature ‘Super Jack’ t-shirt with the words ‘half the heart, twice the fight’ on the back. The competition would be a major accomplishment for any child, but this race bore extra significance for Jack’s family.
Kiefer-Foley’s brother, Michael Kiefer, competed in his first triathlon just two days before he was killed on September 11, 2001.
“We lost him on 9/11. He was a city fireman, and it was a very first triathlon he ever did, and he came in second in his age group. He was only 25,” she recalled.
To Jack’s mom, there was no question about where he gets his athleticism.
“Oh, of course, I cried,” Kiefer-Foley said. “And when he crossed that finish line, he ran into my arms, and I just melted.”
Despite his condition, Jack continues to meet and exceed developmental milestones. “Regarding outcomes, Jack is the kind of kid, when you see him, you don’t notice there’s anything wrong,” Bacha said.
Jack’s doctors encourage him to pursue normal activities and tell his parents not to be alarmed if he gets winded before his peers or is in need of extra rest. “I always consult with his cardiologist, and I always ask, ‘is this OK for him to do?’ And he says ‘absolutely.’ They want him to be active,” Kiefer-Foley said.
“There’s no acute risk to him,” Bacha said. “And we we recommend that the children try to have a normal life as normal as they can.”
But even with successful surgeries and speedy recovery, infants are not cured; they may have lifelong complications.
As Jack gets older, there is a chance that he will require further operations such as a heart transplant, and that, of course, makes his mom nervous.
“I am going to take a cue from my husband and remain positive and hope that medical science will come up with something,” Kiefer-Foley said.