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A story about a heart rhythm disorder

Cameron It was 7:11 a.m. on March 26, and Mark Knowles had just returned from his job as a Dispatcher/EMT with the North Greece Fire Department. As he came toward the house, his younger son, Zachary, opened the door for him, inadvertently setting off the burglar alarm. Little did Mark know, but at that precise moment the heart of his other son, 7-year-old Cameron, had stopped.

Mark turned off the alarm and began chatting with his wife, Becky, while she got ready for work.

Soon, Becky grew surprised that the burglar alarm hadn’t woken Cameron. He was sensitive to loud noises and, once awake, would normally be up and bounding with energy. Since it was a school day, Mark decided to go in and check on him.

Cameron was still in bed. Mark touched him gently to wake him up, but Cameron didn’t respond. Mark looked closely at his son and realized he wasn’t breathing. Mark yelled to Becky, who rushed into the room.

“He was gray and had no pulse,” Becky recalls. She gave Cameron two quick rescue breaths, as Mark raced from the room to call 911.

Becky pulled Cameron onto the floor and started performing chest compressions. She was screaming to Mark, “Why is this happening?” Still, her training as an EMT/nursing student enabled her to stick with the critical task at hand: Keeping Cameron’s heart pumping blood until help could arrive.

Meanwhile, Mark had decided against calling 911. In this instance, he realized there was an even better option: He called the Greece Ridge Fire Department, where he had once worked. He told them, “My son is unconscious. Get the squad to my house.”

The fire department was at their house in two minutes, and they administered a shock to Cameron. A few minutes later, Monroe Ambulance arrived. The EMTs gave Cameron two more shocks as well as a shot of epinephrine. The crew loaded him in the ambulance and took him to Unity Hospital. Cameron now had a pulse and was just barely breathing on his own. A call was put in to the Pediatric Intensive Care Unit at Golisano Children’s Hospital at the University of Rochester Medical Center.

Mark was overwhelmed by what Becky had done. “He kept telling me, ‘Thank you! You saved him!’” Becky recalls. “But I couldn’t accept it. I just kept wondering what kind of life Cameron was going to have. I didn’t have real hope.” As a cardiology technician and EMT, Becky was well aware that brain damage was common in cases where a person was without a pulse for more than a few minutes.

Though she had no way of knowing it, that same thought was on the mind of the PICU team that had picked up Cameron and was now transferring him to the medical center. That team, consisting of Dr. Andrea Hinkle, Dr. William Parilla and Dr. Elise van der Jagt, was already considering using a radical new therapy in hopes of preventing brain damage in Cameron. Once at the hospital, he would be put into a medically induced coma and his body would be cooled to 92° F. This would decrease swelling and bring the body’s functions to a near-halt, helping prevent the cascade of events that can lead to irreversible brain damage.

Cameron’s body was kept cool for 24 hours. Then, over the next 14 hours, his body was gradually warmed back to its normal temperature. Still, there was no way to know if the cooling protocol had worked. When Cameron awoke he was restless, yet because of the breathing tube he was unable to speak. Nurse Staci Goldstein came up with a plan to assess Cameron’s mental state.

“I asked him, ‘Cameron, do you like the Yankees?’” Staci recalls. Cameron shook his head “No”. Then Staci said, “Cameron, do you like the Red Sox?” Cameron shook his head “Yes!”

Becky says, “That was one of the best days in our life. We knew we had our boy back!”

There was still more to learn, though, about what had caused Cameron to go into cardiac arrest. An EKG examination of Cameron revealed that he has long QT syndrome. In this rare condition, part of the heart’s electrical pattern—the QT interval—is slightly elongated. When a person with long QT becomes excited or stressed, this slight irregularity can cause the heart’s ventricles to fibrillate—that is, quiver—instead of pump. This ventricular fibrillation can lead to death within minutes.

Many young people with long QT have succumbed during the excitement of athletic events. But in Cameron’s case, it took even less: Just the sound of the burglar alarm going off at 7:11 a.m. (It was by knowing this time, and the exact time of Mark’s call to the fire department, that the family was able to determine that Cameron’s heart was stopped for at least 12 minutes.)

Though Cameron was recovering well, the chance of another cardiac arrest had not diminished. Fortunately, he was in good hands. Long QT syndrome had first been studied in depth a quarter century ago right at the University of Rochester Medical Center by Dr. Arthur Moss. Several years ago, in a landmark study from the University of Rochester, Dr. Moss, along with Dr. Wojciech Zareba and Dr. James Daubert, had shown that implantable electronic defibrillators were highly effective at preventing sudden cardiac death in people with long QT syndrome.

On April 4, in an operation performed by Dr. George Alfieris and Dr. James Daubert, Cameron had an electronic defibrillator implanted in his chest. If Cameron’s heart should ever stop for more than a second or so, his defibrillator will immediately shock it back to its regular rhythm. On April 11—his brother Zachary’s birthday—Cameron was back home.

Becky was certain that Cameron had survived for a reason. Four weeks to the day after Cameron had nearly died, that reason would become very clear: A report from the American Heart Association suggested that there was link between medications used to treat Attention Deficit Hyperactivity Disorder (ADHD) and sudden death from conditions like long QT. Cameron has ADHD, and had been on three medications to help control it. New guidelines now recommend giving children EKG exams prior to and during use of certain medications for ADHD.

“We don’t ever want any other family to go through what we did,” says Becky. “So if his story can help others, then that must be why he was given another chance at life.” As a start, Cameron will be part of a press conference on National CPR Day, to help people understand the value of learning CPR.

Now, Cameron’s life is nearly back to normal. He enjoys riding his bike, using his new golf clubs and playing video games—though he has to stay away from cordless devices like the Wii because of possible interference with his defibrillator. And he’s more than happy to show just about anyone the scar on his chest from when his defibrillator was implanted.

“He calls it his ‘chick magnet’,” says Becky. “Dr. Alfieris told him to call it that.”