Jason | Living with ARVC
West Newbury, Massachusetts | Born July 1984
“I used to hike three or four peaks in a day and not really see what’s going on. Now, I spend more time smelling the flowers on a leisurely jaunt—looking more at the little things and the vistas, but not going for the miles. I’m more of a quality over quantity person now.”
On a pleasant spring evening, Jason, along with his wife, Meg, and his seven-year-old son, Bryce, take a stroll in the woods near their home. The spring ephemerals are pushing out from the earth with gusto as warm spring air settles into New England. Together they pass the spot where, last year, they observed an owl’s nest, with a brood of baby owls. “We watched them grow up,” says Jason. “There was something really cool about it—to see them so regularly. It helped to center me and gave me something to focus on at the time.”
“A couple years ago, I’d be running up this hill,” says Jason. Instead, today he stops halfway up the trail to take a breather. “I used to hike three or four peaks in a day and not really see what’s going on,” he says. “Now, I spend more time smelling the flowers on a leisurely jaunt. Looking more at the little things and the vistas, but not going for the miles. I’m more of a quality over quantity person now.” Together, everyone listens for birds and takes note of the signs of woodland creatures emerging from their winter slumber.
This change, among many others, came about when Jason was diagnosed with arrhythmogenic right ventricular cardiomyopathy in 2020. ARVC is a genetic disease where a malformation of cardiac muscle tissue puts him at an elevated risk for potentially deadly arrhythmias. In response, Jason has been forced to completely change his mindset in how to approach adventures outdoors.
There are many bright sides to lifestyle transition. No longer the type to bag multiple peaks in a day, Jason is poised for more family-friendly visits to the woods close to his home on the North Shore of Massachusetts. Not only are the hikes less strenuous, he is within driving distance to multiple hospitals, including Mass General, where he sees specialists for his ARVC.
Meg knows well that getting outside is important for his, and their family’s, mental health. “Every little bit helps,” says Meg. Throughout Jason’s recent troubles with his heart, she has remained steadily by his side, providing continuous support. “They say it takes a tribe to raise a child, but it also takes a tribe to support someone with this disease.”
Jason and Meg’s love grew against the backdrop of nature, something they wanted to share with Bryce. Together they are planning camping trips for the summer ahead—going to the American West. By balancing his passions with his physical health, Jason strives to remain healthy and be the husband and father he wants to be.
However, Jason will be the first to tell you how fortunate he is. “I tell my story because a lot of people don’t know about ARVC,” he says. “And they learn how lucky I really am. Many other people learn about this disease from an autopsy. I made it through everything pretty well.” Like most people who discover they have ARVC, Jason was once a dedicated athlete, which put him at an acute risk to which he was completely unaware.
The next day, Jason arrives to an empty house in the mid afternoon. Typically, the first to get home, this was often the moment when he would change into his running clothes and go out for a jog. However, running, or any athletic activity that would raise his heart rate, is something strictly out of bounds. It would put him at risk for ventricular tachycardia (VT), an irregular electrical impulse that causes his heart to beat dangerously fast.
Running was where Jason’s journey with ARVC started. In 2010, Jason moved from North Carolina to Massachusetts and had a lot of free time on his hands. He and Meg were in the process of relocating to be closer to her family, for support as they started their own.
Meg’s father had convinced Jason to apply for work at the naval shipyard. Jason and Meg both worked as teachers in North Carolina and he held little inclination toward the Navy, but much to his own surprise, after a year-long screening process, Jason landed the job and began work as a shipwright. With a job in hand, Jason moved to Massachusetts before Meg and outside of work and living with his in-laws, he didn’t have a lot going on.
“I did a couch to 5k program. Then, I got the running bug.” He progressed from 5ks, to 10ks, to half-marathons and beyond. He ran his first marathon in Newport, Rhode Island in 2017. “It started dumping buckets of rain on me,” he recalls. Although the experience was mildly miserable, it would not deter him from doing it, again and again.
On a rainy day in April of 2020, while out for a routine 5K run, Jason felt his first symptoms of his completely hidden ARVC. However, about halfway through his route, he felt off. “My heart would just not settle down,” he remembers. “I didn’t know at the time, but I was definitely in VT.” He decided to walk the final mile-and-a-half home and took the rest of the day off. After he took a shower, his heart rate slowly returned to normal.
Starting in high school, Jason began working with the local volunteer fire department in Northeastern Ohio, where he was raised, and he continued working with local firefighters ever since. Due to his years of training and experience as an EMT, he was aware of different types of arrhythmias. “Some I knew were life-threatening, some aren’t,” recalls Jason. He reached out to a friend, a local paramedic, for advice and support if needed.
He waited two weeks to try running again. On another drizzly day, he tried another 5k. Just as the last time, his heart began racing and would not slow down. He called his friend, the paramedic. She hooked Jason up to a heart-rate monitor. “She told me, ‘You’re in VT, you need to go to hospital right now!’”
Meg drove him to the closest hospital in Portsmouth, NH. No one there could believe that he had a serious arrhythmia. “I present very well in VT,” he explains. “Then, they put the pulse ox on my finger and saw that my heart rate was at 190 bpm.”
Doctors surmised that Jason had had a heart attack. A cardiologist did a heart catheterization, but it came back inconclusive. “He told me, ‘I don’t know what’s wrong with you, but I would trade hearts with you right now because yours is extremely healthy.’”
Without more information, Jason was let go from the hospital after a few days. He made an appointment with an electrophysiologist, and was told not to run in the meantime. In June, Jason visited the electrophysiologist. His EKG results were slightly abnormal (inverted T-waves) and an MRI showed tissue damage to the right ventricle. The final test would be a cardiac stress test, where Jason ran on a treadmill for 15 minutes. “It felt so good to run, that I asked the nurses if I could keep going a little longer,” remembers Jason. They agreed, but in only a few more minutes, Jason’s heart began racing—he went into VT. The nurses acted quickly, anesthetized Jason, and shocked his heart back into rhythm.
Jason ended up with three major diagnostic markers for ARVC, and the electrophysiologist made the diagnosis despite Jason not having any known family history of the disease. He explained how important it would be for Jason to avoid raising his heart rate and the necessity of getting an ICD. The only problem was that the clinic that diagnosed him was not set up to perform the procedure. “They sent me home with an external defibrillator,” says Jason. “It was big and uncomfortable.” Ten days later, Jason traveled to Boston, to Mass General Hospital, where he received his ICD and got a procedure known as an ablation, where surgeons burned scarred muscle tissue in an attempt to thwart future VT.
Subsequent genetic tests revealed that Jason had a genetic mutation to his PKP2 gene, a relatively common genotype that leads to ARVC. Tests also revealed that he had inherited ARVC from his father, who had never shown any symptoms. The big question that lay ahead next was whether or not their son should get tested for the genetic marker.
Bryce arrives home from school and instantly the house is filled with youthful energy. In the downtime before lacrosse practice, father and son go out to the yard to warm up by playing catch. Jason tosses the ball to Bryce, who returns accurately. An occasional errant throw does not send Jason diving and running after the ball, but instead he simply walks to pick it up.
As much as ARVC has changed Jason’s life, he is not yet ready for it to potentially change Bryce’s. “Our doctor recommended that we wait until 10 to test him,” says Jason. “I’m still very nervous about testing Bryce. That’s something I think about all the time.” However, for now, the strategy has been to let their son participate in whatever activities he likes, including team sports. “I want him to be a kid and run around, to play soccer, basketball, and lacrosse,” continues Jason. “And if he tests positive at 10, we will have to reel back those activities. I would rather have him experience that for some of his life than never at all.”
Jason meanwhile, was forced to alter not only his recreational activities but his professional ones as well. Over the years, he worked his way up from shipwright to an engineering technician for the submarine weight and stability branch at the naval shipyard. Essentially, when a submarine needs to be drydocked for repairs, his job is to make sure the vessel is precisely landed on the docking blocks in the dry dock. From a tower above the port, he directs exact movements until the submarine is secure and balanced on its cradle.
His job used to require voyages on the vessels as they descended several hundred feet below the surface. However, now with the risk of cardiac complications, Jason remains on shore, where medical services are easier to come by. Likewise, while Jason still helps out at the volunteer fire department in his town, you won’t see him running into a burning building anymore. Instead, he helps assess situations and mentors fellow firefighters.
Despite all this, avoiding strenuous activity is no easy feat, and he has made costly mistakes. Only a month after getting his ICD placed in August 2020, Jason was cutting logs with a chainsaw for a fire pit in his backyard, with Bryce by his side. He started to feel funny and then BAM! “I felt something punch me in the chest. Wow. That was a surprise. I was not expecting that. It was very painful.”
That winter Jason had a more serious series of shocks from ICD after snow shoveling the driveway. In an instant, he went from pushing snow around to getting a ride to the hospital. He received three shocks before being admitted for 10 days and receiving a second ablation just before Christmas. “That was miserable,” he remembers. “Both my hospitalizations were during COVID, so the whole time I was there, no one could visit me.”
These were hard lessons, but Jason remains optimistic that even as his ARVC progresses, he will only get better at managing it. He has not had a shock since December 2020, and hopes to keep it that way by finding the right balance between his personal safety and sources of joy. For now, he is leaning into his role as a husband and father, while seeking adventure that doesn’t come with needless risk.
Jason now uses his free time to work on the teardrop camper in his driveway. “I’ve always been a tinkerer,” he says. Back inside, there is a map of the US on their kitchen wall, check marks next to the national parks that they have visited as a family, with more to come.
Jason looks into his future and knows that, like many with ARVC, he will have to remain proactive to monitor his heart for signs of heart failure as he ages. “I am trying to do everything I can to prevent that from happening while still living my life. I don’t want to live in a bubble—that’s not a good idea for anyone.” Remaining proactive is important, but he does not want the disease to define his life. “I’m just very thankful for the time I have,” says Jason. “That I’m still able to do what I do for work, spend time with my family, and live my life.”
This story was written by Living in the Light, a patient advocacy initiative producing unique and engaging content that educates the biotech industry and medical communities about the realities of rare and chronic diseases and the profound effect they have on families and daily life.