Melba Newsome

EXCERPT:

In August, 1996, Christie Kilgore and five of her six siblings, scattered across North America, started phoning one another. The news was bad. Their sister Judy had been diagnosed with a disease that, in time, would destroy her life. Judy was 40 with four children, the youngest, 3 months old.

The entire family was devastated but not surprised, having seen the telltale signs for years. "Judy was clumsy--always falling and tripping, just gangly," says Christie. "She had broken her arm three times in the same place. By her mid-30s, there were too many symptoms to ignore. I used to say, 'Whatever Mom has, Judy has, because they are so much alike.'"

What her mother, Norma Crain, had was Huntington's disease, a debilitating and ultimately fatal genetic illness that crept through her body and mind like a slow, steady fog. It began with forgetfulness and irrational behavior. Next came the clumsiness, followed by the involuntary twitching and jerking and, eventually, the inability to walk, talk, or feed herself.

Huntington's never skips a generation. If a parent has it, the children may as well toss a coin. Their chances of getting it are 50-50. It defied the odds that all of Norma's seven children would escape the family curse, but they'd hoped anyway. Judy's positive diagnosis left no doubt that Huntington's was officially in their generation. But was Judy the only victim? And did anyone else really want to know for sure?

John, Norene, and Charlene, already over 50, chose not to be tested. Still asymptomatic, they figured they were probably okay, since signs of the disease typically show up between 30 and 45. Collene and Christie, who were 43 and 38, respectively wanted to be tested. Dean, 42, did not.

"The minute I heard that Judy tested positive, it put the brakes on any thoughts I had about doing it," says Dean. "Before that it was just theory, but now one of us had it, and I didn't want to know anymore."

Approximately 30,000 Americans have Huntington's disease, and another 200,000 are at risk of inheriting it. Some say it's like being stricken with Alzheimer's and Parkinson's simultaneously. Symptoms may include twitching, clumsiness, lapses in judgment and memory, and behavioral changes. Then it becomes difficult to speak or swallow, and the mental faculties degenerate. Eventually, involuntary movements affect the whole body and make everyday activities such as dressing and eating nearly impossible. Usually 10 to 25 years from the onset of symptoms, Huntington's patients die from complications like infection, choking, or heart failure. The suffering is so prolonged and excruciating that mothers have even been driven to kill their children.

I first met Christie Kilgore on a Saturday afternoon in rune at her home in Goderich, Ontario, almost three hours from Toronto. She bounded down the stairs with all the energy and excitement of a teenager and hugged me as though we were old friends. An hour later, it felt as if we were.

Three weeks later and 2,700 miles away in British Columbia, I recognized Dean Cram the moment he opened his front door. The family resemblance is remarkable: the pale, sandy hair, high cheekbones, pointed chin, and huge almond shaped eyes that occupy more than their fair share of facial real estate. Like most siblings, their similarities reach far beyond their physical resemblance. Besides the legacy of a deadly disease, they also share a painful childhood and far too many family secrets.
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Although the first genetic test for Huntington's became available in 1983, it got much more accurate in 1993, when researchers found the actual gene. More than 5,000 diseases have inherited components: Already genetic tests can predict estimated risk or certainty for some 900 of them, including Tay-Sachs, sicklecell anemia, breast cancer, Alzheimer's, and cystic fibrosis. Medical ethicists such as Arthur Caplan, PhD, director of the Center for Bioethics at the University of Pennsylvania School of Medicine, believe that in the not-too-distant future, such tests will be able to determine whether an unborn child will be tall or smart or even gay.

Every DNA molecule consists of four elements--chemical bases whose names are abbreviated A, T, C, and G. The gene that causes Huntington's involves the CAG repeat. It is the number of repetitions that determines whether a person will develop Huntington's. A normal gene has fewer than 26 repeats. A gene with 27 to 34 repeats means you probably won't develop the disease but your children are at risk. Between 35 and 39 repeats means you may or may not get sick. More than 40 and Huntington's is a given. Judy scored a 38.

By the time Judy was tested, Christie and Dean had each made 180-degree turns in their thinking. Dean, tired of being held hostage by his fears, had two children, and though he'd once been consumed with learning everything he could about Huntington's, he now refused to be tested. "I realized how obsessive I am, and if they'd told me, 'You've got it,' my world would have ended," he says. "I had a choice: Either let Huntington's rule my life or rule my life for as long as I could."

Conversely, Christie felt that regardless of the outcome, knowing was better than not knowing. If she tested negative, she would be free. She could stop worrying every time she stumbled or lost her temper or forgot where she put her keys. If she didn't have the gene, she knew her daughters, Cherish and Kelsey, were safe, too. If she tested positive, at least she could plan ahead and maintain some semblance of control.

In November 1996, shortly after Judy learned of her test results, Christie walked into North York General Hospital in Markham, Ontario, and left with an ounce less of blood. It was such a simple thing, and she expected to feel relieved. She'd made a decision and gone through with it. Instead she panicked. What if she was positive? Then what would she do?

"I never realized how hard it would hit me until I gave my blood," says Christie. 'All the way home, I couldn't stop thinking, Somebody knows my future. It wasn't the same as before, when only God knew. Whatever the answer, it's permanent, and there's no turning back."

Ordinarily, the results would have taken six weeks, but a documentary filmmaker had asked to follow her through the test, the wait, and the results. The entire process would take only a couple of months, he hoped--but the raising of funds for the film dragged on, delaying the release of her tests. Christie was still waiting in June 1997, when she took her girls to British Columbia for a family reunion and came face-to-face with what she might be in for.

Judy's condition was deteriorating quickly. The ticks and movements were constant. Her mother, who had been institutionalized for years, was painfully frail and almost comatose. "The doctor told us that Mom understood everything but just couldn't respond," says Christie. "That's not the quality of life I want. I won't say it's not life, but I wouldn't want it for me. What could I give my family in a state like that?"

Before they married, Christie had extracted a promise from Gary that he would never allow her to end up in such a state. As the possibility became more real, Gary backpedaled. "The first time I met her mother, I saw what Christie feared," Gary recalls. "I promised her I wouldn't let her get to that point, and I meant it. But it's a lot harder when it becomes a reality. Life is a gift, and once a gift is given, it's yours. You can take care of it, ruin it, throw it away. It's stupid to talk about suicide as a sin, but I kept banking on her thinking there was enough reason to go on no matter what the results."