Island Practice (22 page)

In 2002, though, Xyrem was approved for narcolepsy with cataplexy, the first FDA-approved treatment for cataplectic patients. Later it was approved for excessive daytime sleepiness in patients with narcolepsy.

A doctor prescribing Xyrem to McLaughlin would have to use a centralized pharmacy that tracks patients’ drug use. McLaughlin would take it at bedtime, and it would knock her out cold for four hours, after which she would wake abruptly, take another dose, and sleep another four hours. There would be no possibility of waking her during those
stretches, under the theory that uninterrupted sleep would make her less likely to need short, sudden naps during the day.

McLaughlin was convinced she should try Xyrem. For one thing, in early 2011, she found a fellow traveler with narcolepsy plus cataplexy, Justin Curry, who lives in Scituate, Massachusetts, southeast of Boston. He was taking Xyrem. McLaughlin invited Curry, who was twenty-five then, to Nantucket, relieved to have someone who shared her hair-raising, discombobulating symptoms. Curry, who’d been diagnosed as a teenager, knew the ignominy of it all: he had even survived passing out face-first in a bowl of chicken noodle soup in his high school cafeteria.

Curry’s Nantucket cataplexies rivaled her own. Once, Curry recalls, he collapsed face down in front of the Ralph Lauren boutique, one of the only chains that has been allowed to set up shop on Nantucket. “Of all stores,” Curry moans in recollection. “I just planked,” part of him landing on the brick sidewalk, part on the island’s quaint cobblestones. Good food, like a big plate of baby-back ribs, made him so happy that McLaughlin remembers it triggering a collapse, even if “I sat there talking about the Holocaust through the whole meal.”

And while they love going to the beach because, with all the sunbathers, “everybody looks narcoleptic,” they’ve found they have to be wary of other triggers: excessive heat and, especially for men, arousal. McLaughlin plays defense for Curry “when I see a really hot chick is coming his way,” telling him, “Look at me, look at me, think of something awful.” Once while they were eating on the pavilion at Jetties Beach, though, she stepped away briefly to return a tray and realized, “I just left Justin surrounded by hot women in bikinis. Sure enough, he went down.” He landed under a table, and during the twenty minutes it took him to revive, McLaughlin had to calm down the “bikini girls” with a “stand-up comedy routine: ‘Where are you folks from?’”

At Surfside Beach, he fell while waiting for McLaughlin outside the ladies room, and a police officer ran toward him, hands cupped, ready to do CPR. McLaughlin had to body block the cop because, for someone
who doesn’t need it, CPR can be painful and potentially harmful. “For men,” she adds, “half the time when they collapse, they look like they’re unconscious on the ground, with an erection. It’s the most socially inappropriate illness.”

One stormy night, McLaughlin and Curry were leaving the emergency room after McLaughlin had been taken there during an episode. McLaughlin was still in what she calls a hallucinatory “autonomic state,” imagining that she was in Boston trying to hail a cab. Suddenly there was a big clap of thunder, and, right outside the hospital, they both went down. For a short time, hospital staff, perplexed about which medical personnel should bring patients back into the hospital after discharge, “left us in our little narcolepsy pile in the rain,” McLaughlin recalls.

McLaughlin brought Curry to meet Lepore, “so if Justin went down,” Lepore “would know what his face looked like.” He sat in the waiting room during her appointment, unnerved at first by the large furry chair, the Picasso-esque hybrid of animal parts: black hooves, red tongue, goat-like horns. “What the fuck is this?” he asked. “Alexandra, do you see what I see? Am I having a hypnagogic hallucination?” Nar-coleptics can have those before falling asleep.

“I swear to God, it’s a real thing,” McLaughlin told him. Curry couldn’t avoid the psychedelic chair: “Even if I was looking in the glass cases with the bones and things in them, you can still see it in the reflection.” And Lepore’s office was full of Curry’s triggers. “That door slams like the dickens every few seconds. Justin was bored, the room was cold—he didn’t stand a chance.”

Suddenly, Curry fell, “face down, straight as an arrow. You had the nurses freaking out, trying to figure out what is wrong.” Other patients in the waiting room were perturbed. “I was just terrified,” one told McLaughlin. “I really thought he was dead.”

Before scooping Curry off the floor, Lepore, sensing a teachable moment, summoned the hospital’s head of emergency nursing, telling her enthusiastically, “This is what it looks like!”

To try to acclimate Nantucket’s emergency responders to their condition, Curry and McLaughlin printed a flyer with their smiling pictures and an explanation of their disorders. Rather than take her to the hospital, McLaughlin wanted emergency personnel to know, “it’s much easier to drive me home and put me in bed.”

They struck a deal: “If I cataplexy in front of a huge crowd, the EMTs have to bring me in to the ER because otherwise they look like incompetent jerks, and they can’t just stay there until I wake up.” But if she collapsed off the beaten path, they could leave her or drive her home. In return, when she and Curry wanted to attend an event, they would check with EMTs first.

Fourth of July fireworks were a challenge they wanted to tackle, even though the loud, startling booms would cause them to collapse, probably repeatedly. When McLaughlin asked Lepore about watching the display in their car with the windows rolled up, Lepore said, “Oh no. If somebody sees two unconscious people in a car with the windows rolled up, they’re going to think it’s a suicide pact.” People invited them to fireworks-watching parties, then disinvited them when they realized they might make a scene. “How do you find a spot where two people can be unconscious-looking on Nantucket and nobody’s going to notice it?” McLaughlin puzzled. Finally, they snuck into a yacht club, plunked down on the beach, and told EMTs where they were.

Another time, they wanted to attend the annual demolition derby, although “watching cars crash together is going to stress me out,” McLaughlin knew. To be safe, she and Curry sat near the EMTs, where “I cataplexied nine times in front of everyone. I was part of the show.”

Despite these accommodations, McLaughlin continued to have episodes that unnerved her. After her collapse while walking her dog, she called Lepore distraught, desperate to try Xyrem: “I know that this diagnosis is me. This is fitting the things that are just so private I never told anybody. Please, Dr. Lepore, let’s try this new medicine. I’ll pay for it out of pocket—I don’t care.”

While Lepore began researching Xyrem, McLaughlin had another episode on her doorstep and was taken to the ER. Lepore swung by and said: “Absolutely, I’ll write the prescription.”

McLaughlin felt a swell of gratitude. “He didn’t care about the perfect diagnostic criteria. It looks like this. It fits me perfectly. Wait-and-see is what’s been the nightmare of my life. He’s just saved my life for me.”

In August 2011, she began taking Xyrem, a foul-tasting liquid she mixes with water. McLaughlin expected to pay for the Xyrem herself, but somehow Lepore’s office persuaded her insurance carrier to cover it.

So far, McLaughlin is napping less and “having fewer cataplexies.” During a two-hour visit with McLaughlin in January 2012, for example, she knocked out only once, after being startled by the ring of her mother calling her on her cell phone. But only a few minutes after her head dropped to the table, she awoke. (Curry, who was with her, cataplexied briefly soon afterward, during a discussion of a fatal accident that he found too distressing.)

McLaughlin finds she can drive if she hasn’t eaten recently, if there’s no chance of thunder, if she isn’t tired, and if she sings along with the radio. She is aware that for brief moments after taking Xyrem, her brain thinks “whatever I’m doing is fascinating,” prompting her to order ridiculous merchandise like a dog wig from late-night TV. And she has taken precautions, telling the fire department, for example, that in the event of a nighttime fire, she won’t be rousable. She is getting a service dog that will be trained to catch her when she falls.

She even got a job: office manager for a theater company. “Who would want to hire this?” she’d thought. And it was a close call. During her interview, the interviewer, upset about the recent drowning death of a child at a Nantucket summer camp beach program, cried and hugged McLaughlin. McLaughlin couldn’t allow herself to feel sadness; already her arms felt paralyzed. “I knew I had twenty seconds before I was going to collapse. I was trying to think of something humorous.”

But when McLaughlin explained why she could not return the hug, the woman was very understanding. The job was short-lived, as it turned out. Still, McLaughlin considered it progress. Lepore is trying to find her another job or some volunteer work, and has jotted a note on his prescription pad, saying that she has narcolepsy and that concerned parties should call him. She carries it in her pocket, but it doesn’t work all the time.

People still misunderstand and overreact, or they think her symptoms are figments of her imagination. But the message Lepore hopes to convey is clear, McLaughlin says: “It’s Nantucket—we can handle weird.”

Lepore and his neighbor, Chris Fraker, were outside in the small compound that contains their houses when they noticed something glinting in a patch of woods. Moving closer, they saw a bottle of yellowish liquid: Captain Morgan rum.

They realized the bottle must belong to Lepore’s son Nick and Fraker’s son Porter, who were both sixteen and fast friends. Other parents might confront the kids with the seriousness of their behavior or let it slide, thinking that confrontation would make things worse. Most would confiscate the bottle, to at least temporarily deprive the boys of booze and let them know they’d been caught.

Not Lepore. He poured out some rum, unzipped his fly, and without ceremony pissed into the bottle, refilling it with his urine. Then he put it back in the bushes.

Soon after, Nick, Porter, and another friend grabbed for the bottle and drank. Lepore waited a few months before telling them what they had swallowed, impishly asking them “if it tasted a little salty.”

Nick, who swears he was drinking only beers that night, was mortified. “My friends said that didn’t happen, and then they never talked about it again.”

But Lepore savored his victory. How many parents, especially how many parents who are doctors, would divest of their own bodily fluids in the distinct hope that their children would drink the waste, be disgusted, and sober up?

“He’s insane” is Nick’s verdict. “If he was living on the street, they’d call him crazy. But because he has a house and family, he’s eccentric.”

Growing up as one of Lepore’s children was always an adventure. He could be unpredictable, given to unusual hobbies, competitive flights of fancy, and off-the-cuff comments steeped in shock value. But he was also a strong, dependable presence, someone they could see was vital to their island. It’s probably no accident that all three Lepore children’s jobs relate to health care, but also no accident that they are not trying to do the work their father does.

“My father’s a big personality—he casts a very large kind of shadow,” says T.J., the middle child. “He and Nantucket have been a little like Kurtz up the river,” he adds, referring to Joseph Conrad’s
Heart of Darkness
.

Meredith, the oldest, often called Meri, did not realize until she attended nursing school how “different he was from your typical physician. He goes above and beyond. And he gave up a lot to move to Nantucket. I was like, ‘Why did you move here? You could have been a big-time surgeon. You picked up and moved to this teeny little island?’”

The Lepores had been on Nantucket only a few years when they came face to face with the island’s medical limitations. It was shortly before Christmas, and Lepore was working the emergency room. Suddenly, Cathy called to say that something was wrong with T.J., who was seven. He had been sitting on a couch complaining of a headache, and when he stood up, he went limp on his left side. “I crashed to the floor,” T.J. recalls.

Lepore bolted home, but when he arrived, “I had everything back,” T.J. remembers. “He didn’t know what to think. He walked me around in circles, and he left. And I went limp and crashed to the floor again.”

Lepore rushed back, scooped up his son, and headed to the hospital. T.J. had had a stroke, and his left side was paralyzed.

Lepore felt T.J. should get to a bigger hospital where state-of-the-art diagnostics could determine exactly what damage occurred. But the weather was dicey, and boats had not been running regularly for days. “The hell with it,” Lepore decided. “We’re going to Boston.”

After three hours of wrangling, Lepore got T.J. on a small plane, so tiny he had to fly with only a nurse. He remembers being wrapped “like a potato in this tinfoil warming blanket.” A brain scan showed an infarction, an area of dead tissue, in his right parietal lobe, which helps coordinate spatial and mapping abilities.

Even for Lepore, the experience at Children’s Hospital Boston was frightening. “No terror like a sick kid,” as he puts it. “Your kid is totally paralyzed on his left side, yet he’s the best kid on the floor, because all these other kids got tumors.”

Despite an intensive diagnostic workup, the stroke’s cause remained a mystery. Lepore, the master diagnostician, couldn’t solve the riddle of his own child’s condition. His impulse to fix things took hold: he planned a ramp to their house and obsessed about helping T.J. learn to walk again.

Late Christmas Day, the hospital discharged T.J., and for months he underwent physical therapy. That summer, Lepore devised an additional workout regimen, taking T.J. to Mount Katahdin, Maine’s highest peak, where Lepore and his limping son hiked the Knife Edge trail, a narrow ridge with a dauntingly shear drop-off. “I was terrified,” T.J. recalls. “It was not the best parenting decision. But he got me out there.”