It’s been a little more than a month since I was admitted to NYP Columbia to wait for a new heart. It’s a little more than ten years since I got the ICD (implantable cardioverter defibrillator) that has kept me from expiring (a weird way to say dying), on multiple occasions. Having the ICD is paradoxical. On the one hand, I’d be dead without it. On the other hand, I’ve lived in fear of getting shocked by it every day since it was implanted.
My ICD has about two months of battery life remaining, so now it’s a race to see if I get a new heart before I need a new battery. If I’m lucky, the former will happen first, and I’ll be thrilled. The surgeons will just take the entire device out during the transplant surgery. If the latter happens first, the electrophysiologist will have to open me up, remove the device, replace the battery, and sew the device back into a small pocket under the skin on my chest. There it will stay for however long it takes to get me a heart. The heart failure team would rather not have me undergo this procedure so close to a potential transplant. My insurer even more so.
Whatever happens, it’s a good excuse to tell the story of how I came to have the ICD. I’m not sure whether to call it a “sliding doors” moment, fate, luck, or divine intervention. Whatever you call it, I owe my life to the ICD and the story of how I came to have one still floors me every time I think about it.
First, some background. People ask, what’s an ICD? Is that like, a pacemaker? Well, yes and no. My device does have a pacing function that delivers an imperceptible electric pulse to the heart to restore it to its natural rhythm. If the heart rhythm gets into a sustained crazy phase, called fibrillation or tachycardia, my device cardioverts, or, delivers a 900 volt shock to my heart. Not surprisingly, it hurts to have 900 volts delivered directly to your heart (think paddles, and CLEAR!!! a la ER or any hospital show). If you’ve ever been shocked while changing a light fixture, it’s like that, but a lot worse. I’ve been shocked dozens of times since I got my ICD. It has an alarm that goes off before I’m shocked, so I almost always know it’s coming, but I’m always surprised by how much it hurts.
An ICD is now considered essential treatment for people with severe cardiac rhythm disorders. To understand the difference and ICD makes, it is worth examining the outcomes of the rapid onset of tachycardia in two cases. First, consider the Loyola Marymount basketball player, Hank Gathers, who did not have an ICD and died on the court during a game. Second, consider Anthony van Loo, a Belgian footballer who, like Gathers, collapsed in the middle of a game. Van Loo, however, had an ICD and it saved his life. Query why he was playing soccer at that level with a deadly cardiac disease. This is a question worth examining in depth, but for now, it’s worth noting that my own life with ARVD has many parallels the cases of van Loo and Gathers.
ARVD was first studied by doctors at the University of Padua, Italy in the late ‘80’s. While researching a cluster of mysterious sudden cardiac deaths among a statistically signficant number of young people around Padua, they discovered that the disease is often found in patients who died during vigorous exercise. This discovery happened just a few years before I was playing basketball in Iowa and suffered the tachycardia episode that nearly killed me and set off this seemingly endless chapter of my life. By 1999, doctors at Johns Hopkins established an ARVD program and established the first patient registry. Our understanding of the disease, its genetic roots, and the treatment options, including ICDs, has only accelerated since then.
It was in late winter/early spring of 2008 when my Dad, returning from his law firm’s DC office, boarded a crowded Acela bound for Newark. He took the window seat in the only empty row. When the train stopped in Baltimore, a distinguished looking gentleman, perhaps in his fifties, sat next to him in the last open seat in the car.
The two made small talk, as business travelers do when they can’t find a seat in the quiet car. “So, what do you do?” is the question one always arrives at in this situation. “I’m a patent lawyer,” said dad, “what do you do?” The answer, remarkably, was that his fellow traveler was an electrophysiologist. Not just any electrophysiologist, but Dr. Hugh Calkins. It still shocks me that the man who sat in the last seat on the train, the one next to dad, was the preeminent electrophysiologist in the country and the head of the department at Johns Hopkins University hospital. Is it a coincidence? Luck? I often wonder if I deserve such good fortune. For years, I took it for granted.
By the time Dr. Calkins sat next to Dad, I’d been living with ARVD for more than fifteen years. Dad explained the story of my diagnosis to Dr. Calkins, who asked if I had a defibrillator. “No, he’s been on a variety of beta blockers since the incident in 1994,” dad said. He was, as dad puts it, “aghast” to learn I did not have an ICD. Dad explained that I’d been presented with an ICD as a treatment option upon diagnosis, by Dr. James Martins at the University of Iowa Hospital, but rejected it as too invasive. They talked for the rest of the trip; about ARVD, about technological advancements in ICD tech, and about changes in our understanding of the disease, its genetics, and how to treat it. When they reached Newark, Calkins gave Dad his card and insisted that I come to Baltimore for an examination.
I, being a somewhat reckless person, especially as it relates to matters of notable significance, tossed the card into a desk drawer and did not think about it again, except when my dad would ask, “have you called Calkins?” I hadn’t called, and did not call, until it was almost too late. He would ask, frequently at first, then more sporadically, until he finally gave up and figured I’d get to it when I got to it. Maybe it was a lifetime of his telling me what to do that made me not want to do this one thing that he really wanted me to do. I don’t know, but I can only imagine how difficult this was for him.
I am a terrible patient. I’d been told in Iowa to avoid strenuous exercise, but for years after the event, I still played basketball regularly, surfed regularly, and partied like I was twenty-five. By the time Dr. Calkins sat next to dad on the Acela, I was a 38 year-old lawyer, and had two kids. It’s hard for me to explain why I acted like that; why I ignored a life-threatening cardiac condition that had already nearly killed me at least once, but more likely half dozen times.
I’ve talked with my mother about this, and the “ignore it and it will go away” trait is one we share. Not coincidentally, we also share the genetic mutation of the plakophilin 2 (PKP2) gene, which has been identified as the primary cause of inherited ARVC. Lucky for Mom, she’s asymptomatic. I drew the short straw and inherited the gene, and the symptoms, and the dangerous trait of hoping bad things will go away, if I just ignore them.
At some point in the early spring of 2010, I went surfing with a friend in Long Beach, NY. I recall the day as being a gorgeous one, with cloudless blue skies and a nice, clean, little swell running across the sand bar at Pacific Ave. I was riding a new board, one I’d shaped myself. My buddy Adam and I were among the few people out in the lineup, as the water was still freezing cold and the waves weren’t really big enough for some people to bother with. We were having fun, though, trading waves and joking around. We’ve lost touch in the last few years, but Adam is a one of a kind type of guy. He’s pure Brooklyn, born and raised (Bay Ridge, I think). Under his tough exterior, he’s smart and funny, kind and generous. Did I mention funny? He’s a hilarious guy.
I remember saying something to him at one point, telling him I was feeling a little woozy. “Maybe you should get out and take a break” he told me. I remember paddling toward the beach, and slowly walking out of the water. My head suddenly heavy and my breath labored.
The next thing I remember, I was waking up on my back on the edge of the sand, with waves washing up around my feet. I was blinking up at the blue sky while Adam was slapping my face. “Jesus Christ, man, I thought you died. Nearly shit in my wetsuit.”
He’d called 911 and the Long Beach EMTs trudged across the sand with their portable cardioverter, but by the time they got there, I was up walking around, shaking it off. They wanted me to go to the hospital, but I refused, against medical advice, as they say. I signed a paper, they left, and I eventually drove home and did what I always did back then; ignored what just happened. I’d forgotten all about the event until, about a month later, a bill arrived from the Long Beach Ambulance Service.
“What the fuck is this?” Laura asked me. I hadn’t told my wife anything about what happened in Long Beach. “Did you have an episode when you were surfing? Were you in the water? Jesus, Andrew.” She was upset; yelling and on the verge of tears.” You could have fucking drowned. You’re lucky you didn’t die. You don’t live alone, you know. Do you have any idea how selfish that is?” I was caught. What could I say to her? I dug through that desk drawer, the one I’d carelessly tossed Dr. Calkins card into, found the card and called for an appointment.
In late May, I took the train down to Baltimore and went to Hopkins for a work-up with Calkins. Dr. Calkins looks different than I’d thought he would look. I can’t think of why I thought this, but I think I pictured him as a tall man with a commanding presence. In fact, he is short, wears funny wire-rim glasses, and salt and pepper hair.
“I remember your Dad very well,” he told me. “I’ve been waiting for you to call.” His team ran a battery of tests: stress tests, EKG, echocardiogram, cardiac MRI. I had been there most of the day by the time I finally sat down with Dr. Calkins in his office. He told me my ARVD was dangerously advanced (its a progressive disease) and insisted I get an ICD. I agreed and told him I could schedule something for the following month, after an appellate brief I was working on got filed. “Oh no. I mean tomorrow,” he said. I told him it was essential I get this brief filed.
“OK,” he said, “but if you leave, it will be against medical advice.” He told me the story of a female patient with ARVD he treated a few years earlier. He wanted her to have an ICD, she wanted to put off implanting for three weeks, as she was getting married, and did not want the visible scar on her chest in her wedding pictures. He relented, and she died from a ventricular tachycardia episode on her honeymoon. So, I took his advice, we got an extension on the brief, and I spent the night at Hopkins, where they implanted the device the following day. Driving home from Baltimore, I promised Laura I would turn over a new leaf and start taking care of myself. I did, eventually, but it took about another seven years.
Six months after that ride home from Baltimore, Laura’s father was in from Cleveland for a visit. It was winter and I had a terrible cold, probably the flu, with all the regular symptoms; cold sweats, excruciating headache, full body pain, you name it. I remember laying on the couch, watching “Great Balls of Fire,” the Jerry Lee Lewis biopic starring Dennis Quaid, when I heard a faint series of electronic beats. “Do you hear that beeping?” I asked Laura. Before she could answer, I’d been shocked. I remember suddenly being on the floor. “Uh, that was my defibrillator,” I said, in a panic.
We rushed to the local hospital and I was whisked into the ER. My device would fire that night more times than I care to remember. My dad winces when I ask him about it. I cannot imagine what it would be like to watch my own child writhe in pain while getting repeatedly shocked. I spent the next five days in the cardiac ICU. What happened? It was a combination of severe dehydration, and a drop in potassium levels due to the flu, that threw my heart into an arrhythmia it couldn’t break out of, even with dozens of shocks.
That was the first time it saved my life, but not the last. Not by a long shot. Over the next ten years, the device would fire, with increasing frequency. In the last three years, it increased to the point that the doctors finally decided I need a new heart. And so, here I sit and wait.
They say it is better to be lucky than good. Nothing about my disease has anything to do with luck. I inherited the gene and got the symptoms. That’s just the facts. But how should I describe what happened since my diagnosis? If Dr. Calkins hadn’t sat next to Dad on that Acela, would I have gotten the ICD that has saved my life? Or would I be dead? And now, for me, getting lucky means someone has to die so I can keep living. It’s probably better not to think about it, but that’s just not possible.