Resident Rock Star: Facing Rare Diagnosis, Jack Scaramuzzino Fights Through Adversity to Thrive

“Can you live without a whole heart?”

That’s the first question that raced through Chelsea Scaramuzzino’s mind when she first learned something was wrong with her baby. She was 20 weeks pregnant, lying on the ultrasound table, holding her husband’s hand.  Moments before, the couple was excitedly waiting to hear if their first child was a boy or a girl.  

But the ultrasound technician had much more serious news to deliver: She could only see two chambers in what should have been a four-chamber heart.

“My stomach just sunk when I heard that,” said Chelsea. “I assumed the baby wouldn’t make it. I thought: ‘Of all things, why does it have to be his heart?’”  

Doctors told Chelsea and her husband, John, that their baby boy had hypoplastic right heart syndrome, one of the rarest cardiac defects seen in newborns. His heart only had one pumping chamber, and once he was born, he would need three open heart surgeries to survive.   

A rollercoaster recovery 

Five months later, the Scaramuzzinos and their new baby boy, Jack, were calling Pediatric Cardiac Intensive Care Unit (PCICU) at Golisano Children’s Hospital home. The PCICU, manned by a team of specialized providers, plays an invaluable role in caring for children with weakened hearts, often helping them recover from surgery, or survive until they’re ready for surgery — or in Jack’s case, both.

When Jack was just 5 days old, he underwent his first operation, performed by George Alfieris, M.D. and Francisco Gensini, M.D., designed to reconstruct his aorta and allow blood to get to his lungs.

He rebounded quickly. Nurses and doctors in the PCICU began calling him their resident rock star.

But it wasn’t long before he took a turn for the worse. 

“It was flu season, and Jack caught parainfluenza. Usually, babies’ hearts can withstand the added stress that comes with an illness, but Jack’s heart is far from normal,” said Karen Powers, M.D. “Even ordinary baby activities, like crying or straining to poop, can cause problems for his heart.”  

The paraflu was the first of many bumps in Jack’s road to recovery. A few days later, his condition deteriorated further.  He stopped breathing effectively, and his skin became cold to the touch, taking on a blueish hue.

“He was suffering from a pulmonary hypertensive crisis, which can lead to heart failure or cardiac arrest,” said Jill Cholette, M.D., who was called emergently to Jack’s bedside. “It was certainly traumatic and scary for Jack’s parents, but we were able to intubate him right away, improve his heart function, and control his breathing.”  

Once the PCICU team stabilized him, doctors took Jack to the catheterization lab to get a better look at his heart. There, he was diagnosed with pulmonary hypertension, meaning he had high blood pressure between his heart and his lungs — a dangerous complication for any newborn’s heart, much less Jack’s.  

It took him five weeks to recover. When Jack was finally able to go home, he was two months old, and he still needed around-the-clock oxygen and his feeding tube.

“I was so afraid to take him away from the doctors and nurses in the PCICU who knew him and knew his heart,” said Chelsea. “But we were leaving the hospital, and for a while, we weren’t sure if that was ever going to happen.”

‘We had our boy back’

At home, Jack faced another slew of complications. He was having trouble feeding, and testing indicated that his body wasn’t getting enough oxygen. After he suffered another hypertensive crisis when he was three months old, doctors determined that Alfieris would need to perform Jack’s second surgery two months earlier than scheduled.

His recovery was almost as difficult the second time around.

“There were many steps backward, even when there were some steps forward,” said Chelsea, recounting how Jack struggled to come off his ventilator after the surgery. “But finally one day, Jack started to do one of his favorite things: suck on his fingers. I knew then that we had our boy back.”

Jack will still need a third operation sometime in the future, but today, he’s doing well. He skipped crawling, Chelsea jokes, and went straight to walking. He only has to come back to the hospital for visits every six months, and when he does, the nurses and the doctors in the PCICU who cared for him barely recognize him.  

“We care for the sickest of the sick, and we see babies like Jack when they’re most vulnerable,” said Erin Ward, R.N., a nurse on the cardiac team who cared for Jack during both of his hospitalizations. “To be able to see him big and healthy, it’s just so refreshing. This is why we do what we do.”