When Aiden Vayo found out that he had a rare genetic cardiomyopathy and an inherited arrhythmia syndrome, it actually came as a bit of a relief. Even though the diagnosis meant that his heart could suddenly give out, Aiden was at least glad to know what was behind the palpitations and other symptoms he’d felt for years. He was also relieved to learn that there was treatment available.
Aiden is an exceptionally bright, quick-witted teenager who deals with his condition with a dark but jolly sense of humor. “I once blacked out on my birthday and woke up in a bush,” he laughs.
Aiden is upbeat and cheerful even when discussing the tragic circumstance that led to his genetic diagnosis. In 2017, his father’s heart suddenly stopped while he was wakeboarding, and he died instantly. “After he died, it became way more important to look into it again.”
Making a personal connection with the Stanford Medicine Children’s Health care team
Aiden and his mother, Tanya, met with Scott Ceresnak, MD, Aiden’s cardiologist at Lucile Packard Children’s Hospital Stanford. They talked about Aiden’s continued palpitations, his father’s death, and the fact that Aiden’s grandfather and uncle on his dad’s side also had heart problems. Dr. Ceresnak ordered a workup, including exercise stress testing and echocardiography. Results showed that Aiden had diminished heart function and a different kind of arrhythmia than he’d had in the past.
Given his family’s concerning health history and the test results, Dr. Ceresnak put Aiden on medication and worked with the Packard Children’s Hospital genetic team to investigate further. Their testing showed a mutation that significantly decreases the heart’s ability to pump blood and causes irregular heartbeats.
“Aiden is a wonderful kid—an old soul who’s way beyond his years in his ability to process a life-changing diagnosis,” says Dr. Ceresnak. Aiden remembers that the doctors broke down the news for him in a helpful way. “They could explain it to me in a way that I could conceptualize it,” he says. Tanya remembers being put at ease by the fact that her son was being seen by experts at one of the country’s most experienced heart centers in caring for children and young adults with arrhythmias. “I felt so comfortable with everything they did and said,” she says. “They drew charts, shared detailed information, and had such positive attitudes.”
It’s not an exaggeration to say that Aiden had a pretty good time during his hospital visits, even having pun-making competitions with nurses. “My de facto means of coping is intellectualizing, and then using a morbid sense of humor,” says Aiden. “I appreciated that the Stanford [Children’s Health] team leaned into that. Once they knew I could make light of a serious topic, they could take on a lighthearted bedside manner, which was reassuring for me.”
“We have a fun, great medical team here, and we change our dynamic to address the needs of an individual, whether that’s joking around or being very serious,” says Dr. Ceresnak. “Aiden has an incredible sense of humor and also can be serious and thoughtful, and we try to adapt the way we educate and support a family in ways that help best.”
Weighing a big decision
Dr. Ceresnak brought up the option of adding an implantable cardioverter defibrillator (ICD) to Aiden’s medication regimen. An ICD is a device placed in the body that’s connected to the heart. If the heartbeat is too fast, the ICD sends a burst of signals or a shock to correct it and slow down the heartbeat to a healthy level. Aiden’s grandfather got an ICD, and it has saved his life.
These devices can be a great safety net, but it’s always a balance of risk and reward. “We’re not cavalier about putting in these devices, but when the risk of having a life-threatening event outweighs the risk of having the device, that’s when we have deep discussions with the family on carefully balancing those risks,” says Dr. Ceresnak. Aiden and Tanya agreed that having the ICD was the best choice, for both its physical and mental benefits. “I had a lot of anxiety around death before I got the ICD, so it’s had the effect of alleviating a lot of tension for me,” says Aiden.
The Packard Children’s Hospital team is amongst the busiest programs in the country implanting these devices in children. “I took a lot of comfort in the fact that this was relatively run-of-the-mill for my doctors,” says Aiden. In addition to the Betty Irene Moore Children’s Heart Center team’s experience with these devices, the Packard Children’s Hospital physicians use strategies that decrease the risk of the devices, such as programming them to minimize the chance of inappropriate shocks. The team brings together cardiologists, cardiomyopathy experts, genetic counselors, and a whole group of people, such as psychologists and social workers, who are thinking not only about the device, but also about what it means for the person who needs it.
“Having the device is traumatic for some families, and we take a holistic approach to what it means for the family,” says Dr. Ceresnak.
The benefits of an ICD
For Aiden, getting comfortable with the ICD, which looks like a small bulge under the skin on his chest, has been far easier than he expected. “Besides the fact that it could save my life, it has a lot of silver linings,” he says, namely the added lifestyle benefit of it.
The ICD automatically transmits data to his medical team at Stanford Medicine Children’s Health so that they can see if there has been any unusual heart activity. Each night, this data is automatically uploaded, and since Aiden has access to his online records, he can track how his heartbeat changes after safely experimenting with his levels of hydration and physical activity. Now, Aiden only needs to see Dr. Ceresnak and the rest of the Stanford Medicine Children’s Health team in person once a year for testing. He feels great, is an avid runner, and likes to exercise with his mom. “For me, there are a lot of wonderful things about where our field is going, and about being able to take care of amazing patients with unique heart conditions,” says Dr. Ceresnak. “But the best part is people like Aiden, who is so resilient and whose zest for life is so heartening. Kids like him are what makes our jobs so rewarding.”
