Lightning Flowers

The first time my younger sister passed out, she was eighteen, just beginning her freshman year of college at the University of Colorado in Boulder. One hungover morning in her dorm room in the fall of 2007, her phone rang, and she muted the call. She was sliding the phone back underneath her pillow when she blacked out and tumbled off the bed.

When she woke, everything was blurry, dreamlike. Her fan loud and big as a train. Christine's roommate found her crumpled on the dorm-room floor and raced for the resident assistant, who called 911. At the hospital, doctors checked her blood sugar, took a CT scan to look for epilepsy. Nothing was conclusive, so as the months ticked quietly on—football games and classes, mountain hikes and parties—her strange fall out of bed receded from view.

Then in December, just days before finals, it happened again. This time her roommate's phone went off, and she fell back into her pillow. When Christine came to, she called our parents in the Chicago suburbs, who began setting appointments for her over winter break. She would, after all, be home in just a few days.

Instead my sister, by then nineteen, spent those few days going into cardiac arrest over and over—first in the campus health center, then in the hospital, where a nurse noticed an abnormality in her heartbeat that pointed toward a dangerous genetic condition. The first time my parents received a call from a cardiologist named Dr. Sameer Oza at Boulder Community Hospital, he told them he could not release their daughter until she had a cardioverter defibrillator implanted. Christine had a rare arrhythmia, he told them, one that seemed to be activating cardiac arrest when she was startled. She had been lucky so far—her heart had restarted itself each time—but she might not be so lucky in the long term.

My parents bought plane tickets. Meanwhile my sister—surprised by a nurse in her doorway at the hospital—discovered the ceiling tiles spinning and woke suddenly with paddles poised above her chest.

"I have been dead," she told me years later.

Picture the sign posted on the door of her hospital room: DO NOT STARTLE THE PATIENT. SPEAK SOFTLY. And my mother creeping into the room whispering, "Christine, Christine."

I don't remember getting the call that she was in the hospital, but I do remember crying for her. That winter I lived in a one-room cabin on the property of a summer camp south of Jackson, Wyoming, five miles past the Hoback Junction, in a small valley rimmed by dark pine forests, where cliffs hung off the mountains like broken teeth. On the Broken Arrow Ranch, we had woodstoves, no cell service, and a bridge across the pale blue tongue of the Hoback River, which was slowly hardening into swaths of ice. The week my sister almost died in the Colorado foothills, we were deep in mud season up in Wyoming, tree roots jutting up at angles out of the first paltry snowbanks, trails slippery with melt. We'd already had a freeze over Thanksgiving that left my toilet tank solid.

It was the beginning of a life I'd dreamed of since childhood. I'd grown up in a suburb northwest of Chicago—not one of the leafy old towns along the lake, with their grand brick estates and quaint downtowns, but a subdivision farther out, forty-five minutes down the train line, where the trees were just gaining height, on land that had until the early 1960s been a tomato farm. All down my block, a few standard split-level and colonial houses repeated, each with different-colored shutters and brick facades, their grass trimmed, flowers blooming in rows every spring.

These were the years of expansion; these were the decades of sprawl. What had once been tallgrass prairie edged by forest, with bluestem so dense the horizon wavered, now disappeared beneath strip malls and subdivisions, until my neighborhood was sentineled by big-box stores and their vast parking lots. The concrete stretched most of the way to the Wisconsin border. And though I loved the way tornado winds blew off the plains into Chicagoland each May—ominous green-gray thunderheads crackling over the softball fields—these twists of wildness were rare. Most often the uniformity settled over me like a stranglehold. The flat gray of the sky during Chicago's seemingly endless winter; the stifling humidity of summers that left the house sticky. All around me I saw a place being plucked and prodded into form, the wild-growing plants weeded out. The deer that had once ghosted the scrap of wetland at the end of our street disappeared when more McMansions went in. Even as a child, despair filled me whenever I saw a SOLD sign on a wooded lot by the side of the road. I knew what came next, and I felt the loss in my very body.

When my parents first brought the three of us girls out West, I was in preschool, and though I spent as much time throwing up from altitude sickness as I did strapped into my tiny plastic ski boots, something of the big white massifs, the unruly pale rivers, imprinted in me. At first we went to Colorado every other year; by late elementary school, the trips were yearly, and each time the minivan turned east again I found myself weeping, leaned over the back seat to watch the mountains recede. All that crisp sun, all those big views, the miles with nothing in sight but a ranch house or two. Afternoons spent in the company of deep snow and pine trees. I had found something there. The first year we drove through the Nebraska panhandle on our way to northern Colorado, crossing the barren stretch of highway from Cheyenne, Wyoming, into the Medicine Bow, I was seven. Those sagebrush steppes, the pulse and push of the wind, the dirt roads that twisted away. I began to say it aloud with the kind of knowing that seven-year-olds can have: I would become a writer in a cabin in Wyoming.

The fall Christine began passing out, I had just graduated from college down in Colorado Springs. By then I'd spent many summers in Jackson Hole and the less-tracked mountains to the south and east—arriving in late spring blizzards, leaving as the aspens turned yellow. I'd been a student, then staff, at a wilderness school. I spent my winters heavy with longing for the place: its silver storms, its antelope, its bear. That first summer out of college, I worked for the camp that owned the Broken Arrow Ranch, and when the place emptied out, I moved into tiny Cabin 2. For the first time, I had an address in Wyoming good for more than a summer. The month Christine first went into cardiac arrest in her dorm room, I was roving the dense woods behind the ranch, stockpiling kindling for the winter. I'd bought a little axe and a sizable maul and a couple of wedges, and under a pine beside my house I was learning to split wood. By the time Dr. Oza leveled his ultimatum at my parents in December, I was training to be a ski instructor—driving seventeen miles north to town each morning before hopping on a bus to the resort, where on the slopes of the Tetons we practiced holding a wedge position on a single strip of icy man-made snow. It was not a job I'd dreamed of consciously, but when a woman leaned over the desk at the climbing gym where I worked to tell me she was applying, it made sense. I was broke, living on ramen and tuna sandwiches, still trying to figure out how things would work for me. And if the land itself had called me all these years, outdoor adventure sports became the paycheck that allowed me to stay. Being a ski instructor would round out the year, allowing me more space to write during mud season—those weeks between summer and snow, snow and summer, when town shut down. Now my winter work would rise with the flow of tourists into town—peaking with required three-week-long, no-days-off stretches around Christmas and spring break—and ebb just when I couldn't take it anymore, leaving me, ideally, with a reasonable bank account and weeks of open space to write.

Over the weeks of our training, the natural snow line crept lower, the wind began to slice. We hobbled out of the locker room with burned cheeks and spasming quads. By the week of solstice, the sun set on the bus ride home, the Snake River steaming by starlight, my Subaru curving those last miles home in the dark. When I got back to the cabin I would loosen my boots at the door and go straight to the stove to blow on the coals, sliding logs in at an angle, waiting for the cabin to warm enough to take off my down coat.

And so I must have gotten the call somewhere between the resort and home, must have arrived into the orange glow of the cabin already afraid for her, because what I remember is using my rickety college printer to print pixelated pictures of my baby sister—three and a half years younger than I—and taping them to my walls, wiping snot on the sleeves of my jacket as I cried. My sister could die. Christine holding our family's new boxer puppy; Christine in her Buffs sweatshirt; all three of us sisters at my college graduation, the previous spring. Just two months earlier I had visited Christine in that dorm room in Colorado, slept on her floor, taken her out for happy hour–priced mac 'n' cheese and pot au chocolat at a fancy Pearl Street restaurant where they served her dessert wine even though she was underage, and we wiggled our eyebrows at each other and didn't say a word. But for much longer than we'd been close, we hadn't been: I was already gone during her high school years, and although we often stayed up late talking when we saw each other, her world then unfolded in a tumble of partying and older guys and emo bands that I, with my unshaven legs and hippie hair and backpacking trips, struggled to relate to. We connected once in a while, but we didn't keep in touch.

That night I cried in the way you do when you understand you have nearly missed something, when you have nearly made a terrible mistake.

The genetic mutation that almost took my sister was one we'd never heard of: congenital long QT syndrome.

If the heart is a muscle, pumping blood by contracting and relaxing, it is also an electrical organ. Each heartbeat unfolds in five separate electrical pulses generated in the sinus node, a patch of tissue in the upper right-hand chamber of the heart, rightfully known as the heart's pacemaker. These electrical pulses crash through the heart like a wave, and to monitor them physicians have labeled each with a letter, P through T. During P, the upper (atrial) chambers act as the little pumps that load the big pumps (the lower chambers of the heart, called ventricles): the right atrium sends oxygen-depleted blood to the right ventricle, and the left atrium sends oxygen-rich blood to the left ventricle. During QRS, the right ventricle pumps out to the lungs for oxygenation, and the left ventricle pumps oxygenated blood out to the body. During the T part of the wave, everything is supposed to electrically reset for the next beat, a process known as repolarization.

If you've ever seen the ziggity-zag of a heartbeat on a monitor in a prime-time hospital drama, you've looked at the PQRST, how it unfolds and unfolds and unfolds as a heart beats onward. An electrocardiogram—or EKG—is the way we capture an image of the heart's electricity, one that allows us to see if electricity is following the right sequence, peaking and dropping in the right places at the right times in the right amount.

We use the same word for hearts and drums; we love a steady beat. A heart is predictable the way the tides are predictable, the way rivers in the desert shrink during the day and expand outward at night.

And yet we cannot take for granted that the heart will ebb and flow at the right time, because sometimes it does not.

In a heart with the type of long QT syndrome my sister has, the physical structure is fine, but repolarization can be prolonged, a problem that becomes exaggerated under certain types of stress. This means the interval from Q to T is—as you might expect from the name—too long. Sometimes when this happens, there are heart cells only half primed at the right moment, so they half fire, triggering other things to half fire. This inconsistency can go unnoticed—a slight palpitation, maybe—or it can cause the rhythm of the heart to spin out of control, unable to pump in the firm, organized manner that gets blood oxygenated and out to the limbs and organs. A heart that quivers instead of pumps fails to get oxygen to the brain.

The lucky faint but wake up when the heart recovers a normal rhythm. The unlucky die of cardiac arrest.

Long QT is most dangerous when you don't know it's there—21 percent of symptomatic patients die within a year if they don't receive treatment. There's a long list of medications to avoid—everything from antihistamines to antimalarials—because they further lengthen the QT interval. Though 90 percent of people with long QT have their first abnormal heart rhythm before age forty, there have been cases of older adults without the genetic mutation who end up with medication-induced long QT syndrome as a side effect of pills they take for other conditions.

There are at least thirteen types of congenital long QT, with three genetic variants accounting for 90 percent of all diagnosed cases. Those with type 1 are most at risk when they exercise, type 3 are most at risk during sleep, and type 2—like my sister—need to avoid being startled. For most types of LQT, taking adrenergic-blocking drugs, known as beta-blockers, can help; these medications decrease the effect of stress hormones in a body, causing the heart to beat more slowly, preventing big spikes in the QT length.

But the heart, sometimes, cannot be controlled. The cardiac defibrillator that my sister had implanted that December was the equivalent of a personal set of emergency-room paddles, a resuscitation device she could carry with her every minute of her life. A small titanium box containing a motherboard, capacitor, and battery, her ICD was connected to her heart by a thin lead wire that ran down her left subclavian vein and screwed into her right ventricle. If she'd had the procedure any later, her surgeon said, she would have received a second wire, screwed into the right atrium, to enable pacing of the heart. These wires contained tiny sensors that could monitor her heartbeat—and if a dangerous arrhythmia were detected, all that quivering instead of pumping, the ICD would shock her heart to disrupt it, delivering between three hundred and eight hundred volts. The heart would flatline. Then, we hoped, the natural rhythm of the heart would kick back in; life would resume.

My mom sent texts when Christine was out of surgery, when she was boarding the plane. In Chicago, my sister spent the month resting. She wore a giant gauze badge over the left side of her chest and followed movement restrictions: Lift nothing over thirty pounds. Do not raise your arms above shoulder height. At a Christmas party, her high school friends shied away from the topic of her heart surgery, her cardiac arrests. No one mentioned the square of gauze or the sling she wore to prevent pulling on the incision. Self-conscious, Christine slipped her arm out of her sling now and again; hurting, she slipped it back in. Beneath the gauze, the device settled into the pocket Dr. Oza had carved for it between the pectoral muscle and skin, because there is no natural space in a body for a titanium box. Years later, Christine told me that she and my mother had huddled together to take off the bandage, audibly gasping with relief when they saw the gentle bump on her chest. "We had no idea what to expect," she said. "We thought it might be huge."

I didn't go home for Christmas that year, working through the holiday rush at the ski mountain, as was required. It was easy not to think about my sister's mortality—the technology had fixed that, right?—and I was busy falling for one of the snowboard instructors, a tall, dangerously charming Hotshot firefighter who came down to my cabin to help split thick rounds of wood, his shirt cast off in a snowbank. It was easy to become absorbed in my life in Wyoming, to ignore what was happening elsewhere, to sneak in ski runs before and after work and stay up late writing angsty love poetry. (That man never did kiss me.) I didn't leave the county for five months.

In the meantime, my family members were slowly filing into doctors' offices to get EKGs, to find out if their QTs were long. My older sister Cindy's was negative. So, too, were my parents', which left the mystery of where the genetic defect came from wide open. They'd call me from their house in Illinois: Kati, you really need to go in. I didn't have insurance. We'll pay for it. I didn't have the time. I didn't know where to go. Twenty-two-year-olds did not just stop into the hospital for EKGs. Just call the hospital. It's important. Whatever, I thought. I was the healthiest person I knew. I taught skiing and rock climbing and ran up mountains. I ate organic food. I lived in the most intact ecosystem in the Lower 48—drank fresh snowmelt, inhaled alpine air.

Christine herself said nothing. She was pretending none of this existed.

The first answers came from Texas, in April. One of my dad's older brothers, Chris, went to the doctor for chest pains and was told he had a long QT. Their eldest brother, Steve—a librarian and the de facto family historian—remembered an old story when he heard this. Their grandfather, a railroad engineer known as Pa Choo-Choo, was an only child. Though he'd grown up in a gaggle of half siblings, his mother had died just five months after he was born, when she was twenty. And, as the story went, it was a heart attack.

This was in early March of 1899, three years before the EKG was invented. Heart attacks occur when blood flow to the heart is blocked, by blood clots or clogged arteries, starving the muscle of oxygen and causing parts of it to die. Cardiac arrest, on the other hand, occurs when the electricity of the heart malfunctions and the heart stops effectively pumping blood. But without the knowledge that the heart is a muscular machine driven by current—without the ability to measure the waves of electricity rolling through the body—there would have been no way to distinguish between the two.

One of the diagnostic criteria for long QT syndrome is a history of unexplained young deaths in the family. It seemed like Lena Proctor Standefer, 109 years back, might hold our answer.

One morning in late April of 2008, my sister was on her way to class when she realized she'd forgotten the paper she needed to hand in. Turning, she quickly power walked back to her dorm. As she slid her key card through the reader, an electric current cut her. Before she could think, she was screaming. Had she been electrocuted by the door? Then she knew. She slipped inside the dorm, clutching her chest, and retrieved her paper. Then she headed back to class. Stunned, she stayed the whole period.

At the hospital that afternoon, they told her one of her wires had moved seven millimeters. Unable to read her heart correctly from its new location, the machine had double-counted her heartbeats. It hadn't been a lifesaving shock; it had been a mistake. Until the wire was removed and a new one placed into the appropriate position, her ICD would be a danger to her. And if they turned it off, she would be without her backup.