(Palo Alto, Calif.) — Just weeks before her fifth birthday in 2003, Reina Villarreal went into cardiac arrest at her home in Petaluma, Calif. Within minutes, her father was performing CPR as the paramedics were called and on their way. Reina was soon airlifted to a hospital where she was placed in a drug-induced coma for four days as doctors determined what was wrong.
Reina was diagnosed with catecholaminergic polymorphic ventricular tachycardia, a rare and inherited arrhythmia syndrome that can cause sudden death if left untreated. Reina’s parents were unaware that Reina had a heart condition prior to this diagnosis. Patients with CPVT have structurally normal hearts, but have potentially fatal arrhythmias.
For treatment, Reina received an implantable cardioverter defibrillator, a surgically placed device that continuously monitors a patient’s heart rate. The ICD consists of wires that carry electrical signals from the heart to a small computer inside a metal box. The tiny box also contains a pulse generator and batteries that need to be replaced every seven to 10 years. If the ICD detects an abnormal heart rhythm, it sends low-energy electrical pulses to get the heart to beat normally again.
“These devices have been lifesavers for people,” said Anne Dubin, MD, medical director of pediatric electrophysiology at Lucile Packard Children’s Hospital Stanford and Stanford Medicine Children’s Health. “Prior to these devices, there were only medications that didn’t work very well.”
Dubin, professor of pediatrics at the Stanford School of Medicine, said there have been many improvements since the devices first became available 10-15 years ago, allowing cardiologists to put them in younger patients, including newborns.
Now age 16, Reina, a sophomore in high school, has been living with an ICD for 12 years. Two years ago, Reina’s ICD was near the end of its life cycle and needed to be replaced. Since receiving the new device from the hospital’s Electrophysiology and Arrhythmia Care Team, she hasn’t had any arrhythmia episodes.
Patients with ICDs still need to monitor their physical activities and stress levels, though, to prevent arrhythmia episodes. For instance, if Reina’s heart rate gets too high, the ICD will deliver an electrical shock to get her heart to beat normally again. These shocks can be quite painful.
“Reina has to watch her physical activity so she doesn’t increase her heart rate,” said Jorge Villarreal, Reina’s father. “She can’t do activities like going on roller coasters or playing organized sports in case the device area is impacted and the wires are damaged.”
In addition to the physical restraints, patients must also find ways to reduce stress and anxiety, which can trigger an arrhythmia episode. Reina practices yoga and does biofeedback therapy to help keep her stress level down.
Providing stress-reducing techniques is a big part of overall care for patients who have ICDs, said Lauren Schneider, PsyD, a Lucile Packard Children’s Hospital psychologist who regularly counsels patients who have these devices.
Schneider provides cognitive behavioral therapy to her patients and teaches them biofeedback techniques.
“We give them skills that address many aspects of the challenges they might face, whether it’s the stress, anxiety or the activity restrictions,” said Schneider, who is also a clinical assistant professor of psychiatry and behavioral sciences at the School of Medicine. “We provide them with a tool kit that they might use in certain situations.”
Connecting with other patients who have similar medical conditions has also proven helpful. That’s why the Pediatric Electrophysiology program has an annual daylong “Connecting Event” to bring patients and their families together in a supportive and informative environment.
This year’s event, which was held at Stanford University March 14, included activities for the patients, their siblings and parents. The families learned about biofeedback techniques, the importance of genetic testing and how to perform CPR, as well as participating in support groups. They even had a bocce ball faceoff with Dubin, Schneider and the care team. (The patients won.)
Jorge said he enjoyed watching his daughter interact with the medical staff on a different level, one that didn’t involve a procedure or doctor visit.
“It was great to see Reina play bocce ball with her doctors and interact with kids who have with the same type of restrictions,” he added. “She even was able to connect with a girl who has the same heart condition as hers. Where we live, we don’t know any other kids with heart disease so kids don’t really understand her limitations and what’s she’s going through. This event really helped her to connect and to not feel so alone. We are already looking forward to going back again next year.”
Discover more about our Children’s Heart Center or call (650) 721-2121.
