Community CPR, EMS and expert heart doctors work together to save boy with rare condition

“I have a box in my tummy,” says 5-year-old Eli Conyers, lifting his shirt as proof. You can’t actually see the “box” through his skin, but it’s there – a defibrillator implanted by doctors at MUSC Children’s Health to prevent a morning like Sept. 28 from ever happening again. 

That morning, Eli ran out of the door to catch the school bus as it was pulling up alongside his home in rural Colleton County. He tripped and fell, but popped back up and kept running. His mom, watching from the door, couldn’t see what was going on at the bus, but noticed it seemed to be idling for longer than usual. Then her nephew Josiah came running back.

“He said, ‘Auntie, Eli is playing dead by the bus,’” recalled Elicia Ford. She raced outside and found Eli lying on the ground, his eyes half open. Ford called 911, then scooped Eli up and carried him back to the house.

“I’m like, ‘Eli, wake up! Wake up!’ And he’s not responding at all. And then his breathing starts to get different,” she said.

Her older brother, Eric Ford, had served eight years in the U.S. Navy as an electronics technician. CPR was part of his annual training, but he had never used it outside of those training sessions. That morning, he had to perform CPR on his nephew as the family waited and waited for help to arrive – 30 agonizing minutes, they said, before paramedics were able to make it to the scene.

The entire time, Eric Ford kept saying to himself, “Just keep going. Just keep going.”

“I had to do it, so I just did it,” he recalled.

Once EMS arrived, the crew spent more time working on Eli so he could be airlifted to MUSC Shawn Jenkins Children’s Hospital while his mom drove to Charleston.

In Charleston, Eli’s heart stopped again when the helicopter touched down, and then again when he was moved into the hospital, and then again when he was moved to the Pediatric Intensive Care Unit, Elicia Ford said.

Doctors ran multiple tests, including a COVID-19 test, to rule out possibilities. The issue that became apparent over the course of a few days, though, was a condition that is relatively rare, yet something that doctors think about whenever a child shows up with heart problems seemingly out of the blue: Catecholaminergic Polymorphic Ventricular Tachycardia, or CPVT. The good news for Eli: He was at a children’s hospital ranked in the top 10 in the nation for its pediatric cardiology program, according to U.S. News and World Report.

CPVT is caused by a genetic mutation that affects how calcium moves through the cells in the heart, said pediatric cardiologist Lanier Jackson, M.D.

People are born with the condition, but it’s usually diagnosed in the childhood or teen years when the adrenaline from exercise, stress or emotion causes arrhythmias, or fast and irregular heartbeats. Until then, patients probably don’t realize anything is wrong.

“Before that there was nothing. Nothing at all,” said Elicia Ford. Eli’s a very active boy, “constantly playing, always running around,” and she had never seen any sign of a problem before.

 

Eli’s fall when he was running to catch the bus could have been because of the CPVT, or the extra adrenaline that kicked in when he fell could have triggered the CPVT, Jackson said.

Either way, once his heartbeat was out of sync, he was in danger of his heart stopping altogether. Adrenaline, in the form of epinephrine, is commonly given to people suffering cardiac arrest to get the heart going again. But in the case of CPVT, that adrenaline instead triggers more irregularity.

“You can often have repeated arrhythmias, and those arrhythmias cause your heart to stop beating,” Jackson explained.

The multidisciplinary team at the children’s hospital had to work together to use alternative medications to get Eli’s heartbeat under control, Jackson said. A child with a cardiac condition would normally go to the Pediatric Cardiac Intensive Care Unit, but since Eli had first been sent to the PICU, the two teams worked together to stabilize and care for him in place.

Meanwhile, once Elicia Ford heard the possibility of CPVT mentioned by doctors, she immediately began researching the condition and potential treatments. Having information helps her stay calm, she said. By the time doctors came in to talk about surgery, she was ready to have a conversation about cardiac sympathetic denervation.

The procedure, also called a sympathectomy, decreases the amount of adrenaline getting to the heart, Jackson said. In addition, doctors wanted to implant a defibrillator in Eli’s chest. The defibrillator acts as a back-up, in case the sympathectomy and his medication fail to prevent an episode.

But, Jackson noted, although Eli had all the classic features of CPVT, the condition can be definitively diagnosed only through genetic testing, which takes several weeks. The medical team thought that Eli needed surgery sooner than that. Using a patient-centered approach, there were open conversations between the cardiology team, the intensive care team and the family, Jackson said, to determine the course of action.

A week after the initial incident, pediatric cardiothoracic surgeon Minoo Kavarana, M.D., operated on Eli.

Being in the hospital, Eli said, was “not good.”

But more than a month after that morning – one that Eli doesn’t remember but his family will never forget – Eli runs and plays with his little sister and cousins. 

"Everything the emergency department did, what the ICU did, what we did, helped him.
But really, the lifesaving measures were in the beginning, with the community.”

Dr. Lanier Jackson

If Eli goes 10 years without an episode, that will indicate the medication is working and they can probably remove the defibrillator, Elicia Ford said.

In the meantime, he’s returned to kindergarten and is very much his old self, his mother said.

Jackson said that, given how well Eli came through the episode, they hope for a high quality of life for him for a long time.

And Jackson particularly credited Eric Ford, who performed CPR while waiting for EMS, for making that life possible.

“That was really what saved the blood flow to his brain and saved his life,” Jackson said. “And EMS did the same thing, providing an appropriate shock. Everything the emergency department did, what the ICU did, what we did, helped him. But really, the lifesaving measures were in the beginning, with the community.”