Julia | Living with ARVC
Santa Rosa, CA | Born January 1992
“This is something I’ve had to deal with every single day for more than two years. I have had to mitigate. Life has really had to change. I came out of this a completely different person.”
The Sonoma County Superior Court house in Santa Rosa, California, stands like a relic of architecture from over a half century ago. Its austere concrete and brick façades are fading and crumbling. A new one is being built to replace it, and a massive fence surrounds it, blocking the view into the active construction site.
Julia emerges from the courthouse into the glare of afternoon sun. She works as a court clerk, doing the fastidious tasks that keep the institution functioning, regardless of its rough exterior. However, it’s Friday, and Julia has other plans on her mind.
“Julia!” She hears her name and turns around. It’s Joel, her dear friend who works at the same courthouse. He comes over and gives her a hug.
“So, you’re still coming over tomorrow?” Julia asks.
“For sure,” he replies. “Should I bring any games?”
Joel and Julia first bonded over board games. “At his house there was literally a wall of over 200 boxes of games,” recalls Julia. One of Joel’s roommates collected them, and over time, friends would come over to try and work their way through his library.
And while the two still love playing board games together, their bond has been forever deepened after Julia’s cardiac episode—a near death experience that unexpectedly placed her life in Joel’s hands. It was an event which, despite its frightening nature, forged an unbreakable connection between the two. For Julia, it led her to a diagnosis of arrhythmogenic right ventricular cardiomyopathy or ARVC, a rare genetic heart condition, that had, without warning, almost taken her life.
In early February, 2020, Joel and Julia were both feeling the need to blow off some steam together. It had been a rough few years for the two residents of Santa Rosa. In 2017, the Tubbs Fire, at the time the largest wildfire in the state’s history, consumed much of their hometown. Julia had been living in her grandparents’ house with her brother when the flames approached.
“At first, I was like maybe the fire won’t come to us, but that was wishful thinking,” she recollects. Her grandmother was on vacation, and the local authorities gave people around five minutes to collect what belongings they could from their houses before evacuating. “There were embers flying everywhere and I needed to shield my eyes,” she remembers. Julia managed to grab some important documents and her grandfather’s medications until the honking of neighbors’ cars signaled that it was time to go.
She dropped her grandfather and brother off with extended family, and then decided to go it on her own. “I ended up being homeless for a bit,” says Julia. She slept in her car by the river, waiting for the worst to pass, ready to flee further if necessary. “You couldn’t buy gas anywhere,” she recalls, “but I had some in my tank—not enough to get really far—but enough to go some distance.”
The aftermath of these events had wide-reaching implications for Julia and her family. Not only did they have to rebuild a house, but with people becoming more geographically scattered, there was a need to re-establish their family dynamic. Things slowly started to improve. The housing market in California was tight, but Julia fortunately found a place she could afford—although that meant working overtime to pay for it. Little did she know then, but her high-stress levels were compounding a still-unknown issue with her heart.
But on this particular day, in February 2020, these were the issues that Julia was trying to avoid thinking about in order to enjoy her and Joel’s escapade to the city. They visited the Academy of Arts and Sciences before descending on a speakeasy style bar for some drinks in the evening.
Sitting around the table, laughing with friends, Julia began to feel faint. She lost track of the conversation and suddenly found herself fighting her body’s overwhelming desire to pass out. “My chest was feeling painful,” Julia remembers. “But not bad enough to go to the hospital.” Always tough, Julia pushed through, traversing the hills of San Francisco on foot for the rest of the night. “I thought I would feel better the next morning,” she says.
However, the next day, Julia still felt off and decided to head home early to Santa Rosa. Joel was happy to accompany her on the long trip via public transit. That morning they headed to the trolley stop just as a train was approaching. They made a mad dash—20 yards or so—and caught the train doors just in time.
“Everything was fine until we got through the doors,” recalls Julia. In an instant, her body began to shut down. She lost her balance and gripped onto Joel’s arm to stay upright. “Everything around me collapsed. I couldn’t hear. I couldn’t feel. Every sense was taken away from me in a half a millisecond.”
Joel looked at his friend, confused. Her eyes were open, but their gaze was unsettling—like a person holding on for dear life. Suddenly, he understood that something was very wrong. With the next stop quickly approaching, he made the split-second decision to get off the train. Julia was leaning on him heavily, but he managed to get through the doors and back out into the fresh air.
Joel sat Julia down at the edge of the train station and dialed 911. “Joel?!” Julia looked at him with utter confusion in her face. She started to fall over and Joel again caught her before she hit the concrete. He held her up against the trolley stop glass with one arm, the other grasping the phone, trying to describe his location to the emergency responder on the line.
A few minutes later he could hear the ambulance sirens approaching. It would take some help from bystanders to direct the responders Joel and Julia’s exact location. When they arrived Julia was put on a stretcher and loaded into the ambulance, Joel jumped in back as they sped off to the hospital.
In the ambulance, the paramedics worked to keep Julia alive. They shocked her with a defibrillator though were not able to restore her heart to a normal rhythm. When they arrived at the Emergency Department minutes later, Julia was immediately wheeled into a room.
“None of the medication they were using was working on me,” recalls Julia. Once she was transferred to the ER, her heart was in the midst of a constant storm of ventricular tachycardia (VT), a dangerous rapid arrhythmia that inhibits proper blood flow to the rest of the body. Her heart was beating at an alarming 236 bpm and would not slow down.
A doctor approached Julia and told her there was still one remaining option, and that there was no guarantee it would work. They could stop her heart completely, putting her into full cardiac arrest, in hopes that when it restarted, she would return to a normal rhythm.
“I remember holding his hand, and saying, ‘If you have to do it, you have to do it.’ Then, I was gone.” Doctors let Julia’s heart stop completely for 30 minutes, supporting her body systems with machines in the meantime. “Then, they shocked me—200 joules and my heart decided to come back on, and was stable.”
Joel sat in the waiting area, anxiously awaiting any news. “All through this time, I had no idea what was going to happen to my best friend,” he explains. “I was in this zone of postulating, theorizing, but just waiting, not really having any idea of what was going on.” He had talked to each of Julia’s parents: Dwight, who lived locally in the Bay Area, and Ann, who lived in Southern California, and they were both on their way. When doctors told him he could come and see Julia, he was shocked to find her fully lucid. Dwight, Julia’s father, arrived minutes later and was much relieved by her conscious state as well. With more family arriving, Joel returned home.
Julia was transferred to Kaiser Hospital in San Francisco where an electrophysiologist ran a slew of tests including an electrocardiogram (ECG) and a cardiac MRI to try and determine the cause of her VT storm. The MRI showed an abnormal amount of scar tissue on her right ventricle, while the ECG showed inverted T waves, a hint to faulty electrical activity.
The next day Julia was diagnosed with ARVC and handed a single page of information about the condition. She was also told that she would need to get an implantable
cardioverter defibrillator (ICD) placed to protect her from future arrhythmias. Dwight, who worked for Medtronic for over 20 years—a manufacturer of ICDs—implored the medical staff to install one of their devices in his daughter. “If anything was going to go wrong with the device,” he said, “I knew exactly who to talk to.” Within a week, with a Medtronic defibrillator in her chest, Julia left the hospital and returned to her life.
Julia survived her first major cardiac event from ARVC and is eternally grateful for the role Joel played in helping to save her life. The disease struck with little or no warning. Despite being a lifelong athlete, neither Julia nor her doctors had any prior indication that her heart wasn’t working properly. Her fortunate outcome was the result of the quick actions of Joel and the doctors who managed to restore her heart to a safe rhythm. “I was lucky,” she reflects. “I don’t know if I’m going to get lucky again.”
Two and a half years later, Joel arrives at Julia’s grandmother’s house to a dining room table full of family, and Brenton, Julia’s partner. After hugs are exchanged, Julia deals Joel in to a game of rummy—a family tradition as long as anyone can remember.
While sliding the cards across to her grandmother, from whom Julia got her first name, and no doubt her competitive spirit, Grandma Julia taunts jocularly as she makes her moves, relying as much on her luck as her undeniable swagger. Her husband, Fred, sits to her left and shakes his head as his wife steals his cards again.
Regardless of who wins or loses, simply being together is a gift that distance makes all the sweeter. Julia’s parents, who have been divorced since she was four years old are both present. Dwight recently relocated to Mexico City, and Ann still lives in Orange County. Despite separating while Julia and her brother were young, the two are still friends and have remained involved in their children’s lives. To fill out the table is Mark, Julia’s cousin, who has remained close with her since the two were little.
Everyone quiets down as Joel begins to retell the story of the fateful day just two-and-a-half years ago, when he helped save Julia’s life. “I did not know that,” quips Ann, jaw agape, at another aspect of her story. Many here have not heard the entire tale told from his perspective, rich with new details.
Slowly the conversation morphs into talk of Julia’s earlier life: different bits of nostalgia from her family—her boisterous attitude, and ability to always get into trouble. She was a brilliant young athlete with a future in sport. “They called her ‘The Beast,’” says Ann, who’s daughter followed in her footsteps as a formidable soccer goalie. Julia would get out of breath sometimes after long runs, but consistently pushed through physical challenges with impressive ease. “There was a point when we thought she was asthmatic because I’m asthmatic,” Ann adds. Julia played soccer in high school and college, but her soccer career was cut short prior to her diagnosis, due a series of concussions she sustained on the field.
In fact, looking back after diagnosis, the only early warning signs of ARVC were the fainting episodes that Julia experienced. “I would have vasovagal episodes when I was young,” says Julia. However, these occasional spells of lightheadedness or dizziness were attributed to a sudden drop in blood pressure. “It was never to the point where they seemed concerned about it,” she says. “No one ever thought that was something that could have alluded to this.” Julia now thinks it likely that these fainting episodes were in fact related to her ARVC.
Even after her diagnosis, Julia held onto a sliver of possibility that she could play soccer again. Her doctor,
admittedly knew little about the condition, and how any activity that would elevate Julia’s heart rate would put her in immense danger of another arrhythmia. “I was told to go back to living normally,” recalls Julia. “I went back to running for two or three months.”
However, at a follow-up appointment, Julia learned that running was actually endangering her and leading to more scar-tissue buildup in her ailing right ventricle. “I progressed my disease because of all that running,” she says. Since then, she has learned much and changed her entire lifestyle to lessen the damage of ARVC. “I miss pushing my body,” she relays. “It’s something I find very relaxing and exciting.” However, the realization that she could no longer do the physical activities she loved meant that Julia needed to find other means of emotional release.
Now, much of this comes from more creative activities. Julia shows off several of her paintings of various sizes and styles. Her inspiration lies heavily in the natural world, including representations of one of her favorite creatures: the octopus. Others are less objective scenes tinged with natural beauty. Starlight skies meld into swirls of purple, and sparkling textures reflect an oceanic iridescence. Her prolific use of color and texture is pleasing to the eye and on further notice , many of her paintings are fully on display at her grandmother’s house.
“When we moved in, we had nothing on the shelves,” relays Grandma Julia. Following the fire, in 2017, she had her house rebuilt, but the vast majority of their belongings, including such memorabilia, had vanished. Julia had been filling her grandmother’s walls for years with her artwork, but then had to start from scratch. “I lost all of my drawings from school,” Julia laments. She has a degree in geology and paleontology at Sonoma State college, and there she specialized in creating scientifically-accurate illustrations of the rocks, fossils, and extinct species she studied. “I love making art for my grandmother,” says Julia. Since the fire and her need to recalibrate her life to a rare cardiac diagnosis, she has begun painting more frequently.
Another important factor for Julia’s health was improving her relationship with alcohol. “I’ve been sober for over a year-and-a-half now,” she says. What once functioned as another decompressing activity, started to hinder her mental health. So, when she learned that alcohol also didn’t pair well with ARVC, she took it upon herself to stop drinking entirely, yet another transformation that has taken place.
“This is something I’ve had to deal with every single day for more than two years,” Julia says. “I have had to mitigate. Life has really had to change. I came out of this a completely different person.” She has also begun to see the long-game involved in ARVC, and the eventual challenges that the disease will pit her against. “It just kills me for those people that are 45 and 50 with this disease who have heart failure. I just don’t want to go through that.”
Learning to manage her ARVC responsibly has also unleashed a new desire to be involved in advocacy. “If I can be part of the solution I absolutely will. I’m in a good position. I’m still fairly healthy.” Julia keeps abreast of developments in treating ARVC through gene therapy, and is utterly thrilled to know that ARVC patients with her genotype, via the PKP2 gene mutation, are among those being researched right now.
Talking about her condition is something that brings new meaning to her family gatherings as well. Due to the timing of Julia’s diagnosis with the start of the COVID-19 pandemic, neither of her parents have been tested yet for the PKP2 mutation, leaving more mysteries to uncover, especially as both Dwight and Ann remain physically active and neither have shown any signs of the disease. Hearing Julia talk about her experience has brought sincere gratitude to the gathering today, reifying their desires to have genetic tests done.
After wrapping up the card game inside, everyone heads out into the sun to try their best on Grandma Julia’s cornhole set. Ann and Dwight face off as the others cheer them on. Julia remains immersed in looking through her portfolio of paintings, but takes special note of how the game develops. “I’m not a sports person anymore, but I’m definitely still competitive.” When the first game wraps up, she puts her artwork down and heads over to the pile of bean bags to face off against her longtime nemesis, cousin Mark, who happens to be a professional bowler. Unintimidated by his accolades, Julia stares him down before she sinks her shot. Julia, fearless competitor, and proud nerd, is always playing to win, regardless of her opponent.
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.