Other genetic mutations have been associated with profound behavioral changes. Rett syndrome, which affects mostly girls, is caused by a mutation in a gene that codes for the MeCP2 protein. People with the syndrome compulsively wring their hands and rub them together as if they were washing them. Children with Williams syndrome have an elfin appearance, an affinity for music and language, an extreme sensitivity for sound, and are very sociable. Williams syndrome is caused by a deletion of a bit of code from chromosome 7.
There is still great uncertainty about how much of a role genes play in major, common conditions such as depression or bipolar disorder. One wonders where obsessive-compulsive disorders come from, or such behaviors as compulsive hand-washing, compulsive neatness. Do some people suffer from OCDs that are caused by misspellings in their code? What about borderline personality disorder? How many mental illnesses are the result of errors in the code or certain combinations of errors? No one knows. It seems quite evident that a lot of human behavior is affected or governed by the blueprint of a person’s DNA. Even where there is evidence of a family history of disease, scientists are unsure how a single gene could choreograph a suite of behaviors. There are roughly twenty-five thousand active genes in the human genome, each with about a thousand to fifteen hundred letters of code. The human genome could be thought of as a kind of piano with twenty-five thousand keys. In some cases, a few keys may be out of tune, which can cause the music to sound wrong. In other cases, if one key goes dead the music turns into a cacophony or the whole piano self-destructs.
The havoc that the Lesch-Nyhan mutation causes cannot easily be undone. Early on, Nyhan tried giving his Lesch-Nyhan patients allopurinol, a drug that inhibits the production of uric acid. The drug is effective with gout. It lowered the concentration of uric acid in Lesch-Nyhan patients, but it didn’t reduce their self-injurious actions. The uric acid, it seemed, was another symptom, not a cause of the behavior.
Nyhan experimented with simple treatments, such as soft restraints, which seemed to relax patients and made them feel safer from themselves. Matthew Morlen had frequently asked to be tied into his stringlyjack. Nyhan also began recommending that Lesch-Nyhan children have their upper teeth removed, so that they couldn’t bite off their lips and tongues as easily. “I’m profligate with those upper teeth,” he said. This led to arguments with dentists. Some dentists would refuse to extract healthy teeth, even when the Lesch-Nyhan syndrome was explained to them.
I was visiting Nyhan in his office, and there was a lull in the conversation. He sat back in his chair and looked at me. I couldn’t tell what he was thinking. Outside the window, the hawks were still riding thermals over the canyon. We had come to the end of knowledge about Lesch-Nyhan. The disease remained as mysterious and frightening as it had seemed on the day when Nyhan and Lesch had first seen it, nearly forty years earlier. But I had never seen it. “I can’t imagine what it’s like to have Lesch-Nyhan,” I finally said to him.
“You could ask someone who has it,” he replied.
I MET JAMES ELROD and Jim Murphy one winter day not long afterward. They were living next to each other in rented bungalows in a somewhat marginal neighborhood in Santa Cruz, California. James Elrod was then in his early forties, and Jim Murphy was just over thirty. They were close friends, except when their Lesch-Nyhan symptoms annoyed each other. (Murphy died in 2004; Elrod, who is fifty as I write this, became one of the oldest living people with Lesch-Nyhan.) The men were clients of Mainstream Support, a private company contracted by the state of California to help people with developmental disabilities live in community settings. Before he came to to Santa Cruz, James Elrod lived for eighteen years in a state institution in San Jose called the Agnews Developmental Center. Jim Murphy had spent all of his adult life in a California state institution in Sonoma. Mainstream employees, called direct-care staff, stayed with Elrod and Murphy around the clock, to help them with daily tasks and to make sure they didn’t harm themselves. Elrod and Murphy had the authority to hire and fire their assistants and direct their work, though an assistant could refuse an order if he thought it would put the client in danger.
At the time, Mainstream was run by two business partners, Andy Pereira and Steve Glenn. “James and Jimmy are real down and gritty guys,” Pereira said. “They are not sweet types. They’re into fast cars and women.” Steve Glenn confessed that he still had difficulty seeing into the labyrinth of Lesch-Nyhan. “There are these Lesch-Nyhan moments when you feel like you’ve kind of got it,” he said. “James and Jimmy are pretty good at telling you when they think they’re in danger of hurting themselves, but whenever they’re doing something, you always have to ask, Is this James or Jimmy, or is it Lesch-Nyhan?”
James Elrod had a square, good-looking face, which was marked with scars, and he had brown, hyperalert eyes. His shoulders and arms were large and powerful, but the rest of his body seemed slightly diminished. One day at the Agnews Developmental Center, before he was with Mainstream, an attendant left him alone at a dining table for a few minutes. To his horror, his left hand picked up a fork and used it to stab his nose and gouge it out, removing most of his nose and permanently mutilating his face. “My left side is my devil side,” he told me. When I met him, he wore black leather motorcycle gloves that had been reinforced with Kevlar. If he thought his left hand was threatening him or someone else, he would grab it or swat it with his right hand. He owned a pickup truck, and his assistants drove him around in it. He had a job working at a recycling facility. He also used to sell flowers on the Santa Cruz pier. He carried business cards explaining that he had a rare disease that compelled him to hurt himself. “I have injured myself in many ways including my nose, as you can see,” the card said. “I will even try to hurt myself by getting into trouble with others.” One day, a man bought some flowers from Elrod and said, “God bless you.” “Eat shit,” Elrod replied, and handed the man his business card. While crossing a street in his wheelchair, Elrod had been known to suddenly roll himself straight into oncoming traffic, yelling, “Slow down, you morons! Don’t you know it’s Lesch-Nyhan?” His assistants wrestled him to safety.
Elrod was sitting in front of his house in his wheelchair when I arrived. It was a sunny day. He offered me his right hand to shake. When I gripped his glove, the right index finger collapsed. “You broke my finger!” he gasped. Then he grinned and explained that he didn’t have that finger, as he had bitten it off some time ago.
I started laughing, but then regretted it. “I’m sorry to laugh,” I said, imagining what he had done to himself.
No worries. He had given me a test, and I had passed it: I had laughed. “A lot of people get uptight when I do that,” he said. “Kids love it. They want to break my finger again.” We chatted for a while. “Hey, Richard—danger,” he said.
“What’s wrong?”
He was staring at my notes. I had been taking notes, as usual, in a little notebook. He cautiously pointed his finger at the mechanical pencil I was using. “Hey, Richard. Your pencil is scaring me.” It had a sharp metal tip. “My hand could grab it and put it in my eye. Please step away from me and put your pencil down. Just listen.”
I backed away from him, putting my notes and pencil in my pocket. But then he said, “You’d better go see my neighbor. He’s waiting for you.”
It only occurred to me later that James Elrod might have entangled me in an act of self-sabotage. He had been looking forward to meeting a writer and describing his disease. He had been waiting in his driveway for me. Because he wanted very much to tell me about his disease, the Lesch-Nyhan part of him had threatened to grab my pencil and puncture his eye with it, had ordered me to put down my notes, and finally had sent me away to interview another man with Lesch-Nyhan syndrome, instead of himself. Was I reading too much into it? It was hard to say. It seemed as if he might be playing a chess game with himself in which he was doing everything he could to put his desires in checkmate.
JIM MURPHY was sitting in his wheelchair
at a table in the living room of his house. He had been waiting for me, too. An assistant named Michael Roth was cutting up pancakes and feeding them to him with a spoon. Murphy was a bony man with dark hair and a lean, handsome face. He had a neatly trimmed goatee and a crew cut, and his eyes were mobile and sensitive-looking. His lips were missing. Two of his brothers had also had Lesch-Nyhan; they had died when they were young. “Jimmy will be shy when you first meet him,” one of his sisters told me on the phone. I could expect to hear a lot of swearing, though. “He doesn’t mean it,” she said. “When he swears at me, I just say, ‘I love you, too.’”
That day in Santa Cruz, Murphy stared at me out of the corners of his eyes, with his head involuntarily thrown back and turned away, braced against a headboard. His hands were stuffed into many pairs of white socks, and he wore soft, lace-up wrestler’s shoes. His chest heaved against a rubber strap that held him in place. He started throwing punches at me, and he kicked at me. He seemed to be enduring his disease like a man riding a wild horse. The wheelchair shook.
I kept back. “It’s nice to meet you,” I said.
“Fuck you. Nice to meet you.” Jim Murphy had a fuzzy but pleasant-sounding voice. His speech was very hard to understand. He looked at Michael Roth. “I’m nervous.”
“Do you want your restraints?” Roth asked.
“Yeah.”
Roth placed Murphy’s wrists in soft cuffs fastened with Velcro, and he placed his legs in cuffs, as well. The wheelchair trembled and rattled as his limbs fought against the cuffs.
“I’m a little nervous, too,” I said, sitting down on the couch.
“I don’t care. Good-bye.”
I stood up to leave.
Roth explained, however, that this was one of those Lesch-Nyhan situations where words mean their opposite.
I sat down again. “Do you want me to call you Jim or Jimmy?” I asked.
His answer was blurry.
“I’m having a little trouble understanding you,” I said.
“I duhcuh…”
“You don’t care?”
He repeated his words several times until he saw that I understood. He was saying: “I don’t care between Jim or Jimmy. Either’s fine.”
“I sort of like Jim better, myself.”
He said something I couldn’t understand.
“What’s that?”
He repeated his words. He was saying, “Do you want me to call you Richard or Dick?”
“Oh, I don’t care. Either is fine,” I said.
An impolite grin spread across Murphy’s face. “I’m going to call you Dickhead. That’s your new dickname.”
I burst out laughing.
Meanwhile Roth, the assistant, seemed not to be hearing a word of our conversation.
Later, Jim Murphy explained what his disease was like. “You try to tick everybody off, and then you feel bad when you do it,” he said. Slowly I became better able to identify his words. “If you get too close to me, I could—” He said something indecipherable.
“I’m sorry, what?”
“Coldcock you, Richard. I’ll say, ‘Get my water,’ and I’ll give you a sucker punch.”
A pair of red boxing gloves was hanging on the wall. Every day, his assistants placed him on a wrestling mat on the floor, where he rolled around and did stretches and then boxed with them. “I could definitely whip you,” he told me.
Later, Jim Murphy asked if I would like to go for a walk with him around Santa Cruz, alone, without his assistant. I said sure. Then he asked me to take off his restraints. “Don’t worry, Richard,” he said.
Feeling nervous about the situation, I opened his cuffs. His arms flew out and started waving around, but he didn’t throw any punches at me. I pushed him out the door. We went down a driveway and came to a cul-de-sac, where we had to make a decision, to turn either left or right.
“Go right,” he said. I started to turn him to the right “No! Left,” he said. So I turned left. “No! No! Right!”
We were trapped in a Lesch-Nyhan hall of mirrors, filled with reflections of desire and repulsion. “Which way do you really want to go, Jim?”
“Left.”
“Are you sure?”
“Left! Left!”
The leftward path led through a gate. As we passed through the gate, one of his sock-covered hands shot out and struck the gate, hard. He had compulsively hurt himself. I apologized to him and said, “I guess I really should have gone the other way.”
“Not your fault.”
I began pushing him along in the street, keeping away from mailboxes. (I was afraid he’d try to hit one.) I was beginning to be able to figure out his speech.
A young woman driving a vintage Ford Mustang convertible passed us.
He waved to her and called out a greeting, and she waved back; they apparently knew each other.
Murphy seemed entranced. “Did you see that? She waved at me. She’s beautiful,” he said in a slushy voice. “She likes me. I love Mustangs,” he added.
“I used to have a ’65 Ford Falcon when I was in college,” I said as I pushed him along. “It was a ’65 Mustang under the skin. It had the same engine and interior as the Mustang, but the body had this weird shape.”
“Yeah!” He grinned. “I love those old Falcons. Do you still have it?”
“I gave it to one of my brothers.”
“Does he like it?”
“Well, he sold it to some kid for fifty dollars.”
“Aw, no!” Murphy said. “I bet you want it right now.”
“I sure do.”
We ended up at a corner grocery store, and while Murphy chatted with a woman at the cash register, who was a friend of his, I went to the cooler case to buy him a Coke. “Get whatever you want. It’s my treat,” he said. “Take my wallet.” He used his eyes to indicate a pocket in the wheelchair where he kept his wallet.
“Thanks,” I said.
“Fuck you. You’re welcome.”
Later, at James Elrod’s house, I was sitting on his back porch and chatting with him, and he pointed out various plants he was cultivating in pots. The pots sat on shelves where he could reach them from his wheelchair. Elrod was a passionate gardener. He dug in the soil with his gloved hands; he didn’t dare hold a tool of any sort. His assistants often did gardening tasks for him, while he told them what to do.
“Jim gave me a new nickname,” I remarked to James Elrod.
“Yeah? What is it?”
“It’s ‘Dickhead,’” I said.
A glow of delight lit up Elrod’s face. “Hey, guess what. I’m going to call you…it’s…” He couldn’t get the word out. “Jack—” he mumbled.
“My name is Jack?” I asked.
“Eee! Aww!”
“What?”
“It’s Jackass, Dickhead!” He laughed uproariously.
JAMES ELROD was born in 1957 in a small town in northern California, where his father worked as a laborer in a rice-drying warehouse. As a child, he was never able to walk, and doctors diagnosed him with “cerebral palsy.” He had an older brother, Robert, who also had “cerebral palsy,” but, unlike James, Robert was considered to be mentally retarded. (Robert Elrod died in 1998.) James attended regular elementary school until he was in fifth grade, when his parents put him in a special education program.
“I couldn’t walk, but I could scoot,” James explained. Scooting meant crab-walking on his hands and feet with his stomach facing up and his rear end bumping along the ground. James and his older sister, who here will be called Marjorie, were very close as children. When they were children, Marjorie was James’s steadfast companion. She pulled James around in a wagon. Marjorie towed James to school every morning in the wagon, and she towed him home in the afternoon. James and his sister have remained close.
Their father drank heavily. He would come home drunk and become enraged with James. “My dad used to hit me with a belt on my bare back,” he said. “I’ll forgive him for it. But he never forgave me for
being what I am.”
The family went camping in the Sierra Nevada, and James fished with his father and helped him hunt deer; he learned how to dress a deer. He hauled wood for the campfire, scooting around on the ground while balancing pieces of wood on his stomach. He was well-liked pretty much wherever he went.
His grandparents lived nearby in the same town, in a farmhouse near the railroad tracks. James was fond of his grandparents and spent much of his free time visiting with them. He liked to work in his grandmother’s garden with her, scooting up and down the rows of tomatoes, pulling weeds and helping out. Hoboes drifted by on the railroad tracks, walking along the back side of James’s grandparents’ property. James’s grandmother would hire the hoboes to pull weeds, and her payment was a sandwich. James pulled weeds along with the hoboes. He also delivered milk and cookies to the hoboes, perched on his stomach, and he hung out by the railroad tracks for hours, talking with the hoboes. Eventually, James’s grandmother invited a drifter named Herbie to come stay with the family and work for them, in exchange for room and board. For the rest of his life, Herbie made his home in a gardening shed behind James’s grandparents’ house.
James Elrod.
Christopher Reeves
Things were not so smooth at home, where his father became increasingly violent. “My father was drinking a lot,” James recalled. “He forced booze down my throat and then he lunged at my mother.” This terrified and enraged the boy, but he was helpless, and all he could do was scuttle around on the floor; he couldn’t protect his mother from the violence. Until then, he had not been known to engage in self-injury. “The first Lesch-Nyhan episode I remember was when I was about ten,” he said. “My mother was taking me home from school in the wagon and I jumped out the wagon and tried to hurt myself. Afterward, the school nurse called up Child Protection Services and told them that I was getting hurt a lot when I was around my mother.” These official suspicions of his mother tormented him as a child, because he knew very well that she wasn’t hurting him, he was hurting himself.
Panic in Level 4: Cannibals, Killer Viruses, and Other Journeys to the Edge of Science Page 21
