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‘Lightning Flowers: My Journey to Uncover the Cost of Saving a Life,’ by Katherine E. Standefer: An Excerpt

PROLOGUE
Tucson, Arizona 2012

Nothing can prepare you for what it feels like to be shocked by an implanted cardioverter defibrillator. Like a badly spliced film reel, my memory of the night is fractured: in one instant, a player on the other intramural soccer team had fallen and the game stopped; he was getting up, brushing his thighs. In the next, my hands became claws. A maul cracked open my chest with a sickening thump, a hot whip tearing through my back. Did somebody kick in my spine? And then I knew. And I was screaming.

“There’s no way you wouldn’t scream if you felt it,” my sister had said.

By then, the defibrillator had been in my body for three silent years, resting loyally above my left breast, keeping watch for the arrhythmia that could send me to the ground unconscious, with a heart quivering rather than pumping blood.

Now, on a crisp November night in Tucson, Arizona, I dropped to my knees in time for the second shock. What if it doesn’t stop? I knew something was wrong, either with my device or with my body, but probably my ICD. If it was an arrhythmia, I should have been collapsed, unconscious—not sharp and alive like this, staring at the backs of houses at the edge of the field, their kitchen lights spilling dully out the windows as I screamed. “Call 911!”

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A third shock. You can either scream or breathe, a voice inside me said, and I began to pull in air, cold heavy breaths, the way I’d learned to breathe into pain in yoga. I am either alive or dead, and I choose which.

The device did not fire again.

“Can I get someone behind me?” I called out. “I don’t trust myself not to fall.” Someone cupped my back immediately, supported me to the ground, and the sky came into view. A ring of faces. The sharp white field lights.

The smell of burning, which was me.

There is a kind of dream state that settles over the body in these moments, a clarity that rarely visits us when our lives are busy unfolding. For lying on my back, looking at the stars, a question lodged itself in my brain, a wild constellation of if-then statements.

If the defibrillator just saved my life. If a defibrillator is just metal. If metal is mined earth. If children sometimes work in mines, if tunnels collapse, if warlords profit, if women are raped, if mountains are dismantled and made toxic.

If mined earth just saved my life: Was it worth it?

The thin, branched burns that uncoil from the heads and necks of lightning-strike victims are sometimes called lightning flowers. Fernlike, following the patterns of rain or sweat, they are rose-colored lightning bolts frozen onto the body, as beautiful as they are terrible.

I will never know what my insides looked like after two thousand volts—if my tissue erupted into lightning flowers of the body cavity, a sudden bloom. What I do know is that the night I took three shocks to the heart I was marked, called into the world in a way I could not turn away from.

What can save us, I would learn, never comes without cost.

Some people say lightning strikes cure blindness; this is my version.

CAUSA FINALIS
CHAPTER 1
Boulder, Colorado 2007

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.”

***

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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.

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.

She was still a freshman in college, facing her second round of finals alongside her second heart surgery. When she went under, her heartbeat was so irregular they had trouble placing the new wire. The surgery went on too long, and this time she woke up, frantically trying to bring her hands to her chest but finding them tied to the bed. “Christine, you have to calm down,” Dr. Oza pleaded. She could see the blue sheet draped at her upper chest, knew somewhere down there she was gaping open. “Christine, don’t move.”

Panic, hot and blue. Then she was out again.

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