In a Heartbeat

Expert care, innovative technology get 5-year-old back to health

Marques

Marques and his mother, Liliana Cabrera. The first clue that Marques had a heart condition— known as long QT syndrome — was when he collapsed at his preschool.

When the phone rang that November morning in 2012, Liliana Cabrera couldn’t be prepared for the news she would hear. Her son, Marques, then 4, had collapsed at preschool, and two teachers were performing CPR trying to save his life.

“It took a few seconds to process what they were saying,” recalls Liliana, whose son had no known health problems. “He was fine earlier that morning, running around and being mischievous as usual.”

Crying and nearly hysterical, she called her husband, Louis Abraham, who was on his way to work. He raced to the hospital, arriving just as a gurney whizzed by with a medic pumping his son’s chest. “I ran after them and watched as they worked to stabilize him,” says Louis. “I stayed calm. I’ve served in military combat and that training allowed me to stay in the moment and not panic.”

A Dangerous Disorder

Liliana and Louis soon learned the cause of their son’s distressing state – a heart condition known as prolonged (or “long”) QT syndrome. The disorder affects the heart’s electrical system and can cause rapid, erratic heartbeats that may lead to sudden fainting, seizure or death. They were stunned by the diagnosis.

“Most people aren’t aware there’s a problem,” says James Perry, M.D., director of the Electrophysiology Program at Rady Children’s Hospital-San Diego, who made the diagnosis and took over Marques’ care after he was transferred from Palomar Medical Center in Escondido later that day. “People are outwardly well and healthy until suddenly they are not,” he adds, explaining that long QT syndrome is most often diagnosed through an electrocardiogram (EKG) and/or genetic test, both of which are not part of routine child health screenings.

In fact, Marques had received a clean bill of health at his regular checkup, just a few months before his cardiac arrest on the preschool playground.

“He was fortunate for the fast-thinking help of his teachers and of the paramedics’ quick arrival with a defibrillator,” says Dr. Perry, also a professor of pediatrics at UC San Diego.

To protect against the possibility of another heart event, Marques needed an implantable pacemaker/defibrillator device, says Dr. Perry, adding that internal defibrillators constantly monitor heart rhythms and deliver electrical energy, if needed, to restore proper functioning. He notes that the heart rhythm team at Rady Children’s cares for nearly 300 children and young adults with pacemakers and defibrillators.

Marques’ surgery by Eric Devaney, M.D., went well, and Liliana and Louis appreciated the concern and sensitivity shown by the Hospital staff.

“They kept asking us if we were OK. And they really kept us informed,” says Liliana. “The fact that they walked us through the [defibrillator implantation] process beforehand
was really reassuring,” adds Louis.

Smart Heart Device

Marques was soon back at home playing with his 6-year-old brother. Through wireless technology, installed in the family’s kitchen, Marques’ condition can be checked on a schedule. He also takes medicine to treat his long QT syndrome.

“All Marques has to do is spend 10 minutes within six feet of the monitor for it to take a reading,” says Sue Shepard, R.N. B.S.N., C.C.D.S., a cardiac specialty nurse overseeing wireless monitoring for patients with implantable cardiac devices at Rady Children’s. The monitor collects information from Marques’ defibrillator, which tracks heart rhythms and other data, and checks for equipment problems.

“This is a good example of the importance of wireless medicine,” adds Shepard. “This equipment gives us the ability to troubleshoot (if any problems are detected with the device) as well as to monitor the condition of patients with a defibrillator.”

After about two months of recovering at home, Marques returned to his preschool. That same day – Feb. 14, 2013 – was proclaimed “Marques Cabrera Day” by the city of Escondido, which held an event celebrating the fast action of the teachers and fire department paramedics who saved Marques. Fittingly, the event also included CPR demonstrations.

Dr. Perry says Marques, who is now 5 and started kindergarten this fall, can lead a pretty normal life, although certain competitive sports will need to be avoided. On this day, Marques can be seen hurrying through the family’s home, with a playful grin, as he heads outside to play.

His parents still marvel at his great recovery.

“Some children with this disorder suffer severe side effects, such as brain damage, but Marques has no damage,” says Liliana, adding that the family is very thankful for the wonderful care their son received at Rady Children’s. “They were amazing.”

Predicting future heart problems

In the 2002 science fiction movie “Minority Report” starring Tom Cruise, specially gifted beings could “see” catastrophes before they happened, thereby allowing the incidents to be thwarted.

Researchers at Rady Children’s Hospital-San Diego are trying a similar feat using the power of science in hopes of predicting – and ultimately averting – dangerous heart rhythms.

“We are looking at heart rate predictors that would let you know 10 to 30 seconds ahead of time of a dangerous problem,” says James Perry, M.D., director of the Electrophysiology Program at Rady Children’s. “The concept is that eventually if those patterns can be detected quickly enough, then an intervention can be
introduced that may prevent the dangerous rhythm from occurring,” he explains.

The research is based on an analysis of cardiac patient data, and the initial results are encouraging.

“Our pilot study showed that these predictors worked reasonably well 75 percent of the time,” says Dr. Perry. “It was a surprisingly good first attempt.”

Further studies are planned, and the project is one of several studies at Rady Children’s focused on ways to better understand and control abnormal heart rates.
Originally published in U-T San Diego