Part 2

A family
secret

Even before Kim’s death wish, life was hard for the Teskes. Gwen and her husband, Larry, were from hard-working stock – she from Saskatchewan, he from Ontario. Larry was 42 when he died of testicular cancer, leaving a genetic secret that lay buried for 25 years: Huntington’s disease. Because Larry’s symptoms were masked by cancer, nobody suspected that he may have passed on a neurological time bomb to his children.

Back then, Gwen was more concerned about the present than the future. “I looked into the coffin and said, ‘How do I go on from here?’ ” she remembered thinking. “But the good Lord gives you strength,” she said. “I had to be there for my kids.” At 39, she had six children to support. The eldest, Brian, was 16. The youngest, Deanna, was 9. Gwen put in long hours running a restaurant at a truck stop to put food on the table. As soon as they were old enough, the kids worked there on weekends and after school.

Adversity was only part of the glue bonding the family, whose warmth and unpretentiousness remain obvious today. Hugging is endemic, humour is rarely barbed, no visit is intrusive and nobody is a stranger for more than a minute. “It’s all about my mother,” Stuart explained. “She’s the one who held us together. She’s a rock.”

None of the kids gave Gwen any trouble. “Not real trouble,” said Lynn Teske, who married Brian in 1978, when he was 21 and she was 19.

They had met a few years earlier at a high-school dance, but he had disappeared with a pal when the music stopped. “A girlfriend and I used to drive around Orangeville looking for him, until I found him,” she said, laughing at her sweet-sixteen boldness. Nearly 40 years later, she still won’t let him go.

Brian had gone to high school in nearby Mayfield, but he switched to Orangeville District Secondary School for Grade 12, “and then we were in the same school.” For nearly 20 years, life was busy but good. They had two children – a son, Jason, and a daughter, Sarah – and eventually Brian ran his own construction company with brother Stuart, digging basements and installing septic systems.

Everything changed as Brian approached 40. The easygoing, laid-back guy who never swore, who never spoke harshly or critically of others, became unpredictably belligerent. “There was no filter,” his wife said. “If he was thinking it, it came out.” Brian developed strange tics, like repeatedly shrugging his shoulders, and opening and closing his hands. One day, when he was sitting at the kitchen table, he picked up a glass and banged it down. After a few crashes on the tabletop, his wife asked him to stop. Bewildered, he asked, “Stop what?”

He also began making strange decisions, according to Stuart, a taciturn guy who fails to hide his tender heart and strong emotions. After 10 years in business with his brother, Stuart quit and moved to Florida to work with standard-bred horses. That’s where he met his wife, Rebecca, and their teenaged daughters, Victoria and Gabriella, were born.

Brian’s daughter tells much more graphic tales of the changes in her funny and generous father. As a teenager, Sarah lost count of the number of times he backed his Dodge Ram pickup into her Mazda, a car she was buying with her own money. “He’d put a hole right through the hood and I would be on my knees crying and he had no empathy at all.”

Eventually, things got so bad that the once-profitable business went broke, the Teskes lost their home and had to move in with relatives. Lynn finally persuaded her husband to go to a doctor, who prescribed antidepressants. “He walked around like he was drunk,” she said. “It was awful.” After repeated trips to the doctor, Brian was finally referred to a neurologist. Having checked his reflexes and asked him to do simple things like stick out his tongue, the specialist looked at Brian and said, “I think you have Huntington’s disease. I want you to consider going for a DNA test.”

Of course, Lynn immediately searched the Internet. “The more I read, the more I knew in my heart that that was what he had.” But denial came easily because nobody else in the family had Huntington’s – his mother was robust, and all his younger siblings seemed fine. Brian finally agreed to go for DNA testing in 1997, when he was 40. But it was another five years before he was willing to ask for the results of the simple blood test that would explain his increasingly bizarre physical and emotional behaviour.

“He didn’t want to know,” Lynn said.

This is a common reaction. At best, one in five of those at risk for Huntington’s are tested, because knowing doesn’t help when you have an incurable and largely untreatable disease. Lynn, nevertheless, tried to persuade him. “We can’t deal with what we don’t know,” she told Brian.

The entire family went to hear the results in July, 2002. By then, Brian was 45, Lynn was 43, Jason was 25 and Sarah was 22. Lynn said she remembers the doctor saying: “You know, Brian, when I open this envelope, I could be handing you a death sentence.” Even before the doctor read the diagnosis, Lynn knew that it would be positive because Brian had become that much worse.

Still, the confirmation was a shock. It took a year for everyone in the Teske clan to accept the news and to suspect that the disease had probably come from their long-dead father. Five of the six Teskes already had children; if they had Huntington’s, there was a 50-50 chance they had unwittingly passed it on – a horrifying thought for any parent. Brian’s daughter spent years obsessing about the disease, but refused to be tested. “I didn’t want a death sentence. I didn’t want to know at 24, 25 or 26 that I was going to be dying of Huntington’s,” Sarah said.

But that didn’t mean she didn’t worry: “If my arm had a twitch, I would think that was it.” She reached a point where the tension was so grinding that she realized knowing was better than not knowing. By then, she had decided that, if she were positive, she wouldn’t have children, although there is now a complicated and costly in vitro fertilization procedure that tests embryos and implants only those that are free of Huntington’s. Finally, in July, 2008, on a day Sarah could only describe in superlatives as “the best of her life,” she learned she was negative. Now, she is married and the mother of two small children.

Part 3

Genetic
reckoning

On the same day Sarah found out she had won the genetic lottery, her aunt phoned home from Regina to say she had lost. Deanna, the youngest of the Teske siblings, had tested positive. Her daughters, Chantelle and Shelby, were in Ontario visiting their grandmother and extended family. But before they even had a chance to celebrate with their older cousin, they had to grapple with the horror of their mother’s diagnosis, which was potentially their own. That is how Huntington’s works: It is a capricious disease, sparing some and skewering others, like a fickle finger of fate.

Deanna will tell you now that she wasn’t surprised to learn she had it. The year before, when she had gone back to Ontario to celebrate Brian’s 50th birthday, she already had a strange tingling in her fingers and toes, and some jerky movements.

Her brother pressed her to find out for certain, so she went for testing after she returned to Saskatchewan. The process was gruelling, partly because her marriage was going through a rough patch. Her husband, Mike Smith, another salt-of-the-earth type who’s a carpenter and a partner in a small construction firm, was “grumpy,” she confided to me on speaker phone from her kitchen in Regina. “I didn’t need that when I was going through getting the results.” So she kicked him out. They went for marriage counselling and eventually reconciled. “He is very supportive and he loves me a lot,” she said. “I am happy.”

Deanna can no longer work as a hairdresser, manage simple tasks like holding a telephone receiver or speak without slurring some of her words. Yet, she radiates cheerfulness, even as her arms flail about like an aberrant windmill. It’s as though she got an overdose of chorea (the involuntary movements that typically accompany Huntington’s) and escaped the depression and belligerence that are also characteristics of the disease.

She’s “good” with older sister Kim wanting to end her life, but that’s not for her. “I want to live till my dying day,” she said, even if that means being incapacitated in a nursing home. “I am happy and I am in love with my husband and I could never imagine ending my life, ever.”

Most of the Teskes long suspected that Kim also had Huntington’s. Deanna said that Brian kept “bugging Kim to get tested” because there were doctors and programs that could monitor the symptoms and ease some of them. But Kim was in denial and, Deanna added, may have gone west a decade ago to escape family pressure. After working for close to 20 years as a barber not far from Orangeville in Alton, she settled near an aunt in Invermere, B.C.

A few years later, though, Kim could no longer cut hair and was working behind the counter in a delicatessen – until even that job became too stressful. “There were times when she was overhearing customers say, ‘Oh, my gosh, I think she is drunk,’ ” younger sister Marlene recalled.

“Brian had it and so did Deanna, and I was hoping and praying I didn’t have it, too,” Kim told me the first time we met. She was finally tested in 2008, the summer of reckoning for the Teske family. She was 47. Sisters Dawn and Marlene flew out to B.C. to bring her back to Ontario. A year later, she could no longer drive or handle simple tasks. Though she technically lived independently, her mother and Ontario sisters hovered lovingly.

Of the six siblings, only Dawn and Marlene haven’t been tested. They believe they have been spared because, in their 50s, they are still symptom-free. Stuart was at risk of losing his workplace life insurance until he tested negative. “I knew I didn’t have it because I had no symptoms, but I did the test for my kids.” Does he feel bad that he and his family are safe, while three of his siblings are doomed? “No,” he said. “It bothers me that they’ve got it, but I’m glad that I don’t.” As for Dawn and Marlene, they don’t have time to feel guilty. Besides full-time jobs and their own families, they are at ground zero, helping to care for their afflicted siblings, including Brian.

At 57, Brian lives in a long-term-care facility. He can’t walk or feed himself, his speech is almost incomprehensible and he frequently chokes on his puréed food. Lynn drives to the nursing home on her way from work, bringing him an Iced Capp from Tim Hortons as a treat before she spoonfeeds him dinner.

Kim, who visits once a week, has watched her brother deteriorate. “I don’t want to live like that. It is very important for me not to do that,” she said on the second day of her fast. “I’ve never married, so I am making the decision for myself. It would be harder if I had children.”

Part 4

A cruel
inheritance

No matter what Kim does, the disease won’t stop with this generation of Teskes. Deanna’s daughter Chantelle tested positive last year, at 24. “I think deep down inside herself, she knew,” her mother said. “At least that is what she told the counsellor.” Deanna felt worse for her daughter than she does for herself. “I cried and I cried and I cried and I felt guilty,” she admitted. “And then I decided that’s not fair,” she said, her sunny disposition rising to the surface. “I shouldn’t feel that way. It is a fifty-fifty situation. I hope she can be like me, or maybe there will be something to stop the movements or cure the disease when her signs show up.”

Chantelle tried for a brave face when I met her and younger sister Shelby at the Smiths’ bungalow in Regina. The two girls were nestled like cats on their parents’ chesterfield. Outside the picture window, a city crew was cleaning up fallen trees from a brutal winter.

“When you don’t know,” Chantelle said, “it is always on your mind.” At the time of her diagnosis, she was married and debating whether to have children. “He really wanted kids,” she recalled, as tears began to flow. While they were trying, she tried to rationalize the decision: “If I do have a kid and he has Huntington’s,” she told herself, “there will be a cure by then.” Ultimately, she realized she could not knowingly pass the disease on to a child; she went for testing. Her husband supported her decision, she said, insisting that Huntington’s didn’t break up her marriage, which ended earlier this year. Now, she is between jobs and living at home. “My life is kind of upside down.”

Bizarrely, a municipal wood chopper began to devour a downed tree outside the window, and we all started to laugh. The tension broken, Chantelle summoned her bravado. “I just want to be happy more than anything else in the whole entire world,” she said, her chin rising. “In my family, it has been around 40 when people start showing signs. I’m 25 now, so I figure I have about 15 years.” That’s not to say she was giving up. “I hope and pray every day that they can find something to stop it, so at least I can have a chance to live.”

Both young women look to their mother for inspiration. “The best person in the world as a role model,” Chantelle said. “My mother is dealing with it amazingly,” Shelby added. “She is always happy and ready to throw optimism your way.”

By contrast, Shelby said, her aunt took the diagnosis “really hard.” She understands Kim’s choice and said she “would probably do the same thing.” That may be why Shelby has refused so far to be tested. “I would dwell on it a lot more than I do not knowing,” she predicted. “I don’t want to feel sorry for myself, right?”

Brian’s son, Jason, hasn’t been tested, either. He is married and living in Calgary, but has no children. Some family members – all of them adept at recognizing telltale signs – fear he has inherited the genetic short straw. His mother is waiting it out. “It preys on my mind a lot,” she said. “He is 36 years old and, in four years, I am going to know just by looking at him.”

The pain in her voice was almost audible as she described the possibility of her son having the same disease as his father. For anyone whose family has been plagued by Huntington’s, watching for symptoms is almost as harrowing as the fate they herald.

“Between 10 and 20 years to complete the course,” novelist Ian McEwan writes of the disease’s inexorable march in Saturday, “from the first small alterations of character, tremors in the hands and face, emotional disturbance, including – most notably – sudden, uncontrollable alterations of mood, the helpless jerky dance-like movements, intellectual dilapidation, memory failure, agnosia, apraxia, dementia, total loss of muscular control rigidity sometimes, nightmarish hallucinations and a meaningless end. This is how the brilliant machinery of being is undone by the tiniest of faulty cogs, the insidious whisper of ruin, a single bad idea lodged in every cell, on every chromosome four.”

Part 5

Brian’s way

There was a time when Brian wanted to end his life. “He always said he was not going to live in a nursing home,” Lynn said, “that when it got to the point where he needed someone to wipe his bum, he didn’t want to be here.” But somehow, he never found the means or the opportunity. He waited too long, some of his siblings will say quietly.

“I’m here to help you through the disease but not to end your life,” Lynn remembered the doctor saying in response to Brian’s entreaties. Although grateful for the medical care, the treatment programs and the help from family – for years, Gwen spent one day a week with Brian, feeding him lunch, taking him for drives and doing the family laundry – she wishes there was more home care available. “If you have lots of money to pay somebody to come in, you are fine. But if you don’t, you are euchred,” she said.

There was a crisis in the summer of 2012. They had a hospital bed in the kitchen, but the bathroom was upstairs and Brian was falling a lot. Sometimes, so was Lynn, trying to keep him balanced as she propelled him up and down the stairs. That October, Brian moved to the nursing home, where he is by far the youngest male patient. Eventually, he won’t be able to swallow mush and he will either choke to death or he will be put on a feeding tube. Lynn doesn’t think he wants that, but she isn’t certain. “He has a son and a daughter and he has grandchildren,” she said. “And then there is his mom and his sisters. He has a lot of family around him.” What she doesn’t say, but which is obvious to anybody who has seen them together, is that he is still gobsmacked in love with the girl he met at a high-school dance.

Part 6

Kim’s way

Kim picked April 25 to start her fast. She figured that spring would have arrived, not a certainty given the hard Ontario winter, and that she would be done by her birthday, May 9. “I will take the movement drugs,” she told me in February. The medication helps to ease her chorea, allowing her to sleep at night. “So we will take them and I will sleep and we are going to have fun at 18 Faulkner,” she said, referring to her apartment. “Marlene and Dawn are going to be here, and I told God I would jump out of the window and meet him.”

Two months later, she sits on the edge of her bed, smoking cigarettes – the Teskes are like chimneys when it comes to nicotine – and looks out the second-floor window at the oak tree in the front yard. It is her talisman. Kim believes she will be resurrected after her ordeal and come back as part of the tree’s towering presence. Whether she is saying that to reassure herself or her family is moot. It is the mantra that sustains her day after day of refusing food and sipping only enough water to swallow her medication.

Both Ontario sisters have taken time off work, made sure that Kim’s do-not-resuscitate form is prominently displayed on the refrigerator, gone with her to a local general practitioner (who has refused to help Kim die) and applied for palliative and personal care for their sister.

Then Gwen, the matriarch, who has been watching and smoking for months, intervenes. She can’t stop Kim from killing herself, but she can gather all her children together one last time. She summons her younger son first. “It was an awful expense, but it was worth it,” she tells me later. Stuart is the catalyst who makes the reunion happen. He comes twice from Saskatchewan, once by himself and again with Deanna. Nobody has told Lynn or Brian what Kim is doing because they fear it may upset him, but Stuart breaks that well-meaning code soon after he and Deanna land in Toronto, on day seven of Kim’s fast. Lynn is glad he did, saying her husband knew something was up. “He has Huntington’s, but he’s not stupid.”

For the first time since they celebrated their mother’s 75th birthday five years earlier, the Teske clan, including Brian in his chair, congregate at Dawn’s house on the outskirts of Orangeville. The place is a multi-generational jumble of kids, friends, food, instant coffee (with an occasional shot of Bailey’s) and frequent trips to the garage, where the smokers gather around an old wood stove to trade anecdotes and josh Dawn’s partner, Paul Omrode, about the two gleaming Harleys parked in front of the door, patiently anticipating a summer romp.

The fast could stop if Kim changes her mind, or if the authorities step in. But nobody makes a move, waiting like bystanders at a traffic accident. It is horrible to watch, especially seeing the suffering in Gwen’s face as she chain smokes in the garage.

Before Stuart and Deanna head back to the airport, knowing they will probably not see their sister again, the siblings gather to plant a burning bush outside Kim’s apartment. She is now too weak to walk to the window. A visiting palliative-care nurse thinks it will be several more days, but Kim has other plans. She dies peacefully, with none of the delirium or agitation that some watchers had anticipated, around noon on day 12 of the fast, with Dawn by her side. When the police are summoned, along with emergency services, they decline to lay charges. It is three days before Kim’s target date.