by Masha Gessen
“We wandered around, the two of us, for the next couple of days, in a haze of...” Rob trailed off. “After we left the hospital, we went to a park for a walk, and I can remember being there but I can’t remember anything about what we talked about, and I didn’t know how to be supportive except just to say that things will be okay and everything else, and I think what I needed really from Bill was a direct statement like, ‘Don’t worry, Rob, I will look after you.’ Or something like that. Some kind of leadership, or at least a willingness to take part in the whole process and say, ‘We’ll figure it out.’ But Bill, he was floundering even worse than I was.”
Rob’s father had died by this point, relieving Rob of the temptation to say, “I told you so!” Rob’s mother was in a nursing home, bedridden and essentially nonverbal. Rob went to the nursing home every day, to sit by her bed and read to her. Sometimes he thought he saw “a flicker of a smile” on her face; that was the extent of her ability for interpersonal contact. She would live for another two years, but her son was all alone with their common illness.
Rob got his test results on a Friday. The weekend passed in a haze. On his way out the door Monday morning, he found a Post-it note from Bill: “Don’t forget your lunch!” He was not in the habit of leaving his lunch at home any more than other people, but he was a marked man now. Although nothing in his physical or mental health had actually changed, Rob felt like he now stood at the threshold of his life as a man sick with Huntington’s. “If I had known that in twelve years’ time I would be talking to you, living in the country alone, and not too greatly changed from—you know—then I might have made different decisions. I started to sort of rush into doing things.”
Rob quit his job. He did all the things genetic counselors had warned him against: He quickly made life-changing decisions, and he got ready to get sick tomorrow. At the same time, of course, he did exactly what the genetic counselors advised him to do: He prepared for the rest of his life, and he made plans and decisions in accordance with his new knowledge. The line dividing these two approaches is as obvious and as elusive as the line dividing the behavior of a man reevaluating his priorities in his forties from that of a man who has been told he faces a certain future of torment and death. At root it is the same thing, just much, much starker. One man, upon reaching midlife, has realized he will eventually die; the other has learned he will die a protracted, horrible death.
Rob had been working as an assistant at a Toronto reference library. He had taken the job intending to stay there “only long enough to figure out what I was going to do with my life”—that had been twenty years earlier, when he dropped out of college. He had grown to like his job, but had for years thought of photography as his true vocation. So he quit—only to discover the curse of all freelancers and artists: Time expanded and shrank at the same time, and he never seemed to get much done. He went back to work part-time for a while. Then an opportunity to house-sit for friends in the countryside came up. Rob moved, the arrangement dragged on, Rob got used to country living, and finally bought a place of his own: the house where I visited him.
He had some regrets. He would be better off financially if he had stayed in his job longer. Drifting into the country life might not have been the best idea either: “It stretched out without really knowing where it was going, it allowed me to get into that sort of—I didn’t have any plan. And my relationship with Bill was falling apart, partially because I was spending so much time out here on my own. Anyway. Where was this going?”
I reminded Rob that we had started out talking about regrets. He said perhaps he should have stayed in Toronto. Later in the conversation, though, he said that going through with the genetic test “worked out okay. I wouldn’t have done anything else.” There was a slight possibility that Rob was exhibiting a behavior I had been told was characteristic of Huntington’s patients: answering the same question differently each time, depending on how it was phrased or how the neurons happened to fire. But more likely, he was doing what we all do: continuously reevaluating his less-than-perfect predicament, now thinking he took a wrong turn somewhere, now thinking he was just where he should be anyway. “I’m prepared to stay here as long as I—obviously, I can’t survive here if I can’t drive. As soon as I can’t drive, I’ll have to do something else. And I should start making the sort of end-of-life things I want to do before I—while I still can, like go down an Arctic river in a rubber raft.”
“It’s a little cold, a rubber raft,” I observed.
“I want to see the North.”
“How will you know when you can no longer drive?”
“Oh, yeah. I’m very careful with driving. But so far, um, yeah, I’m very self-observant. I guess there will be a close call someday. Something will knock me for a loop, and then I’ll have to sort of say, ‘Well, it’s time to give up.’ I don’t know.”
“But you might just have an accident.”
“Some jerk could have caused an accident. Oh, is today Thursday?”
It was Tuesday. This was not, however, a Huntington’s moment. It was just that a garbage truck showed up on the wrong day for some reason. But it gave Rob a momentary scare—forgetting to put his garbage out might also be an early sign of the disease. He fretted for a minute, then observed that someone could walk through the door at that moment and he might forget how to introduce me. My saying that this sort of thing happened to me, too, was cold comfort. “The thing about Huntington’s that’s so horrible,” he said, “is the mental stuff and the emotional stuff. I could accept any amount of physical disability and pain, but not being able to trust your own emotions and perceptions and your own awareness of reality—that terrifies me more than anything else.” Imagine living with that fear every day. Now imagine living with the certainty.
***
In the early 1980s, when the possibility of developing a predictive test for Huntington’s disease began to seem real, a number of studies attempted to predict how many people at risk for the disease would actually take advantage of the test. Every study showed that at least half and perhaps as many as two-thirds of those who were aware of their 50 percent risk of Huntington’s would seek genetic testing once it became available.
“I believed all those people,” said Barbara Handelin, a geneticist who had worked at the Massachusetts Institute of Technology lab that ultimately found the gene and who had gone on to run the first commercial laboratory to offer testing for Huntington’s. “We all believed them. And so we got busy dedicating ourselves to coming up with these very—we hoped—very thoughtful protocols for how you go through testing.” These were the protocols that required talking to family, coming with a partner, and sitting out a waiting period before the lab work ensued.
Nobody came. By early 1992—more than eight years after testing became available—only three hundred people in the United States and fourteen hundred people worldwide had completed the process. Even the Wexler sisters—neuropsychologist Nancy, who managed the campaign to find the gene, and Alice, an English professor who wrote a book about the quest, which began soon after their mother was diagnosed—never had their blood drawn. In April 1986 Nancy Wexler appeared on 60 Minutes to talk about Huntington’s. “I’ve always believed in knowledge for its own sake,” she said. “And it is ironic that after working for precisely that, I’m now finding it much more complex than I ever thought it would be.” Diane Sawyer asked her if she had believed that she would take the test once it was discovered. “Absolutely,” Nancy Wexler responded. “Yes. I never doubted it. And now I’m not so sure.”
As of this writing, Nancy Wexler, now a professor at Columbia University and still active in Huntington’s research, still had not been tested and had even become something of a campaigner for moderation in genetic testing. Speaking once again on CBS, in 2004, she said, “I think there’s a huge amount of social pressure on people to get tested. I know that with me, if I were to go to bed every night thinking, I’m going to die of Huntington’s, you know, w
hy should I bother getting up?”
By the early 1990s researchers started looking at why people were not choosing to be tested. The following list of reasons emerged: increased risk to children if one was found to be a gene carrier, absence of an effective cure, potential loss of health insurance, financial costs of testing, and the inability to “undo” the knowledge. In other words, some people did not get tested because a positive result would raise their children’s theoretical risk from 25 percent to 50 percent. Conversely, of course, a negative result would drop the children’s risk to zero. Some people did not get tested because the result was pure knowledge: There was nothing one could do to postpone the onset of symptoms or to treat the disease itself. Some people did not get tested because they feared losing their health insurance or because the test itself cost too much, which is why I did the research for this chapter in Canada, which has universal health insurance. And, finally, some people were too afraid of what their own reaction might be, whatever the result.
There was another possible reason, the most universal human decision-making factor of all: habit. “Huntington’s families grow up knowing, ‘I’ve got a fifty-fifty chance of having gotten that trait,’” said Barbara Handelin, who by the time we met had spent twenty years thinking about this. “It’s a sense of having lived with uncertainty. And it turns out that most people feel a lot more comfortable with a state of uncertainty than certainty.” I later found a study showing that people who chose to be tested were more likely to have learned of their risk status as adults, rather than as adolescents. So it may not be that most people prefer uncertainty—some just have had to make lifelong partners with it.
***
In Sudbury (population 155,000), the largest town in northern Ontario, I did my interviews at a long-term care facility. I went there with Julie Denomme, the social worker who ran the Northern Ontario Huntington Disease Resource Centre chapter and who had arranged for the interviews. The long-term care facility was simply a convenient, quiet location, but the choice of it was obviously ominous. Like most long-term care facilities in the area, this one housed a couple of Julie’s clients.
Julie wanted to introduce me to Abbee, a woman in her late thirties who was something of a success story. She had grown incapable of taking care of herself out in the world. Julie’s description was stark and unappetizing: spoiled food in the refrigerator, a bathtub full of dirty dishes, life-threatening weight loss. Placing Abbee in a long-term care facility returned her to the world of the living, but the fact that this world was populated with frail octogenarians served to amplify the sense of unfairness and insult that went along with Huntington’s. Her disease, together with her situation, made her prone to tantrums, sulks, and even hunger strikes. Eating is one of those nightmarish Huntington’s conundrums: It becomes more and more difficult as the symptoms progress, but also more and more necessary because the involuntary movements that are among those symptoms burn up far more calories than a normal adult generally uses.
One of the nurses told Julie that Abbee had not been at supper. Julie frowned: “This means she is upset.” We walked—nearly jogged—along long gray corridors, past doors through which I could glimpse beds and televisions, two to a room, through heavy waves of smells, institutional food mixing with medication, in search of Abbee. We finally found her in one of the smoking areas—not the one Abbee liked, explained Julie, and this might well be the thing that upset her. Abbee preferred to assert her separation from the other residents of the facility by using the other, more remote smoking area. Now that area had been closed.
Another of Julie’s clients, Abbee’s aunt Colette, was also in the smoking area, sitting in a chair, her knees propped against her walker. Colette’s fingers were covered with cigarette burns, red and blistered and raw. Julie asked to see Colette’s fingers. Colette, simultaneously surprised and embarrassed, asked for time to finish her cigarette. She had smoked it almost down to its filter, and now she tried to take one last drag, which proved impossible: She kept missing her mouth. Her hand slapped against her face, her mouth opening out of sync as her cigarette burned into her fingers, which had lost all sensation. Julie calmly asked why Colette did not use her “smoking robot,” a mouthpiece with a long rubber tube that attached to a cigarette that was supposed to sit in an ashtray affixed to Colette’s walker. Julie did not get an answer. Colette had large cigarette burns on her purple fleece sweatshirt.
Abbee, an average-build woman with long, not particularly clean, dark hair, watched the scene with evident disgust. As we walked back down the hall, Abbee explained that she was upset with Colette over something Colette had done—apparently hit someone during an aggressive outburst, but ages ago—and this was the source of her current state and her refusal to eat.
“It’s not good, eh?” asked Julie.
“I don’t know. I’d rather go to the other place. I’ve been getting a walker to sit on so I could smoke in the other place. But I can’t go there now because they say they have too many siblings about smoking, so I’m angry at Theresa now.”
“Oh, shnucks. Shnucks, shnucks, shnucks. So you are angry at something they did, and you are punishing yourself.”
“Yeah, I know, but she is the one that’s making me go here. I gotta go into my room.”
“Because you are pissed off now. I’ll come and talk to you tomorrow again, okay?”
“Mmmmmm.”
“Is that okay? Because I don’t want you to stop eating for a long time.”
“I’ve only been eating one meal a day. Because in the other place I was so happy.”
“Don’t you hate change?”
“Yes, I know. That way I don’t have to deal with Colette.”
And so on. It was like talking to a four-year-old trapped in the body of a forty-year-old trapped in a place for eighty-year-olds. I got a chance to ask Abbee why she was there.
“I got diagnosed for Huntington’s there and I had somebody come to my home but I had my slippers on so I slipped on my butt there so she said I can’t be alone.”
“Is that why you got diagnosed? Because you slipped?”
“I don’t know.”
“How old were you when you got tested?”
“I never was tested. No. I was going there, to do it, with my aunt, when she was going, but I chickened out of the testing.”
“You didn’t want to know?”
“I guess I was moving like it so I knew. When I was being tested with her, the doctor ask me, ‘How do you move?’ I say, ‘I move fine, I feel fine.’ Diane [Abbee’s sister] said, ‘I find Abbee moves a bit like my mother.’ I said, ‘Oh boy. I didn’t want to know if I was being tested or not.’”
“Your mom had Huntington’s?”
“Oh yes. Died at fifty. She was in a nursing home because she kept running away from home. Dad had to call the police to go find her. My cousin Susie didn’t know about it, because we didn’t talk to anybody about anything.”
“Does your son want to know?”
“Oh no.”
“How old is he now?”
“Ah, he is twenty-one. He right away wanted to know about Huntington’s. But they are saying you can’t do it for kids, eh? My brother went and got tested too, he lives with him.”
“And what’s his result?”
“Mmm, negative, yeah. He wanted to get tested, he says so.”
“Your son wants to get tested?”
“Oh, he does. But they say he is too young, remember, I said?”
“But now that he is twenty-one, he can.”
“Yes, I know. So he is going to college. Scholarship he got, you know what that is? They given him money for school, so it’s free for him.”
In the end we seemed to leave Abbee in a better mood. She even said that she liked Julie’s pink leather jacket, and Julie promised to give it to Abbee once she stopped wearing it. I found Julie’s way of handling the conversation extraordinary: She was caring and insistent without being either condescending or disresp
ectful of Abbee’s privacy. She spoke the way a very good teacher speaks to an adolescent, that human creature who combines an adult appearance with childish fears and a sort of innocent self-destructiveness. Julie had been on the job for about four years. She had seen forty-five of her clients die. She had withstood battles none of us would want to witness—like when a nursing-home staffer started to feed a Huntington’s patient who could no longer swallow but had signed a no-tube-feeding order. She had had her most firmly held beliefs turned upside down: She had even had second thoughts about her opposition to euthanasia. She expected this to be another death-heavy year: These things tend to go in cycles. She said she was “finding it extremely difficult to remain as good as I can be.”
Wendy and I sat down for our interview in a conference room of the long-term care facility. Wendy was used to these sorts of places. Her mother, who had Huntington’s, was in one. Her father, an alcoholic who had lost the ability to live independently, was in another one. Wendy was the assistant director of care at a third one. She spent all her time in them, working during the week and visiting her parents on the weekends. And she had made a very conscious decision not to find out whether she would also one day live in one.
Right around the time Wendy was learning about Huntington’s disease in nursing school, she found out that it was the disease that had killed her grandfather. He had died at fifty-five. “That it was hereditary—that was the big one. And that it was the dominant gene. At that point my risk status was 25 percent, because my grandfather had had it and my mom had not been diagnosed. Plus, I was in a serious relationship that I’d just started, and I thought, What is he going to think? Are we going to have kids? And blahblahblah.” It does get predictable after a while: Wendy’s mother had certainly gone through all the same thought processes. She was twenty-five and pregnant with Wendy when her father died. Her sister had already had her children and attempted to talk Wendy’s mother out of starting her own family, but Wendy’s mother said, “You can’t live forty years up the road” and went on to have two children. Out of five siblings, Wendy’s mother turned out to be the only one who inherited the faulty huntingtin gene.