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BATTLING HUNTINGTON'S DISEASE

As a war reporter, Charles Sabine diced with death. Now he has an incurable brain disorder, and terror is ever-present—yet he has no symptoms. He talks to Laura Spinney ...

From INTELLIGENT LIFE Magazine, Autumn 2009

In 1996, an NBC war reporter and his crew were captured by a renegade platoon of mujahideen guerrillas near the Bosnian town of Doboj. As the sun set and the call to prayer went up, the reporter stared at a blood-spattered wall while a young warrior pulled the pin from a grenade, replaced it with his finger and pressed it to his head. The warrior closed his eyes and prayed.

At that moment a military vehicle appeared, and the Bosnian colonel inside it eventually secured the TV crew’s release, so Charles Sabine lived to tell the tale. These days, when he tells it, his audience tends to consist of doctors, scientists and families affected by Huntington’s disease. That’s because in 2005 Sabine discovered he had the disease, a hereditary brain disorder. Not many people are qualified to compare two of the most terrifying situations known to man, but he is one of them. Of his brush with death in Bosnia, he says, “Not that moment nor any other I’ve experienced instils more dread and terror than this disease.”

Huntington’s has been described as the most disastrous disease known to man because of its peculiarly cruel characteristics. It progressively strips a person of control of his muscles, reason and emotions—though not necessarily in that order. It is caused by a mutation in a single gene, and if you have the mutation, you will develop the disease; the only unknown is when. Typically the first symptoms appear between the ages of 30 and 50, so many people pass the disease on before discovering they have it. The risk of inheriting it from an affected parent is one in two. The unlucky offspring therefore get to watch their sick parent head into a long, slow decline, knowing that the same fate awaits them. And there’s no cure.

Sabine is 49 and a gene carrier, but he doesn’t yet have any symptoms. The first time he heard about Huntington’s disease was in 1994, when he was following the Clintons’ presidential visit to the newly formed Czech Republic. His mother rang to say that his father, who had been acting strangely for some time, had been diagnosed with it. The gene had been identified the previous year, and there was now a blood test available. Sabine’s elder brother, John, who already had four children, rushed to have the test and found that he too had the mutation. Their father died in 2001 and John now has physical symptoms, though cognitively and emotionally he remains relatively intact. An uncle, it emerged belatedly, had died of the disease in 1992. Believing the odds were in his favour, Sabine put off having the test. But the not knowing was worse, and finally, in 2005, he bit the bullet.

His first reaction, on receiving the news, was, “It’s the wrong result.” He maintains that everybody who goes for the test believes they will be one of the lucky ones, or they wouldn’t go, and he still feels angry about the way he was told. “The neurologist effectively said, ‘You’ve got this disease, I’m afraid there’s nothing you can do about it, so live your life the best you can.’” Yet treatments are in the pipeline, and there’s evidence that simple lifestyle changes and keeping one’s brain active can extend a gene carrier’s years of health. To be told the situation is hopeless is no incentive for watching your diet or exercising, Sabine says, yet to those affected, the extra two or three years they might gain from living well would be priceless. As he says, “That’s also an extra two or three years when a new treatment may be found.”

If taking the test was difficult, telling the world the outcome wasn’t any easier, and he waited another two and a half years to do that. Two things drove him out of the closet: the thought of his mother who, he  believes, assumed like him that he had escaped the family curse, and the need to come clean with his employer. It’s hard to think of many professions in which the slightest slowing of the reflexes, or of the ability to make a decision or recognise a face, could have more disastrous consequences, except perhaps soldiering itself, and Sabine hasn’t worked for NBC since he told them in early 2008, though he is still officially under contract to them.

“Most of the time, contact is less awful than the anticipation of it," Peter Beaumont, the Observer’s foreign affairs editor, wrote recently about going into battle. Anticipation is now a constant problem for Sabine, who wakes up every morning thinking this could be his last day of good health. “If I trip on the stairs, if I drop something or break a glass, that’s an early sign of the disease,” he says. “I think about it all the time.”

To turn that preoccupation to constructive ends, and to keep himself busy, he now travels the world, galvanising families to seek help and scientists to seek a cure. The need to feel he is steering his own destiny has had other consequences too. A once-heavy smoker whose every attempt to quit was doomed to failure, upon receiving his diagnosis he offered himself the meagre compensation that he could now give up giving up. Within a month, however, he had smoked his last cigarette. “I needed to take some power back over my life,” he says.

Earlier this year he married his former producer at NBC, Nicole Bakshi, and they now have a one-year-old daughter, Breezy, who does not have the mutation. Bakshi “took him on”, as he puts it, in the full knowledge of what awaited him, which he describes as “extraordinarily brave, or stupid”. She admits that the prospect of looking after him daunts her, as does the thought of the stressful and confusing experiences their daughter might be exposed to as she grows up. But if you’re in love with someone, you don’t desert them when they are diagnosed with cancer or multiple sclerosis, she says. So why would you just because you received the bad news in advance?

For Sabine, she believes, taking the test was a kind of release. “Before I felt that there was a lot of head-in-the-sand activity going on,” she says. “Getting the diagnosis turned that around.” She thinks that he is more content now than he has been since she has known him, which coincides roughly with the time he has known the disease was in his family. Content, and mortally afraid? Sabine struggles to explain. In his quarter-century working with NBC, he witnessed carnage in Rwanda, astonishing acts of bravery in Iraq and the way the Asian tsunami reduced people to indistinguishable mounds of decomposing human flesh, yet he says, “None of those experiences really meant anything until now.”

Now, in every tragedy he sees hope and the capa­city of the human spirit to overcome obstacles. That’s why, in his talks, he tells the tale of the 12-year-old Kurdish girl he saw coming over a mountain pass on the Iran-Iraq border, amid a vast sea of refugees. Shoeless, her face covered with freezing mud, she had already walked 90 miles carrying her sister, a toddler, who was unconscious, and she was determined to get her down the mountain before nightfall. “All humans are capable of far more than you can ever believe,” he tells the scientists he is urging to find a cure.

He met the Kurdish girl in 1991, before he knew the disease was in his family. Twelve years later, he reported on the plight of patients abandoned in the
Al Rashid psychiatric hospital in Baghdad in the wake of the invasion. Did the knowledge that he was at risk steer him towards that story? He wouldn’t go so far as to say that, but he does say that he probably had more empathy with the patients than he might have done had he not seen his father in the final stages of the illness.

The second part of his answer concerns the future. Getting his diagnosis spurred him to get on with things that he had avoided. Having put off fatherhood for a long time, he took the plunge and is now loving it “for all the corny reasons”. He seems almost apologetic as he says it, as if he were listening to an echo. As if, from inside this very modern predicament—knowing his own genetic flaw—he knew he was articulating an age-old truism: that life is most precious when it is under threat.

HUNTINGTON’S DISEASE: THE BACKGROUND
Huntington’s disease is a disorder of the central nervous system affecting an estimated 50,000 people in the European Union today. Previously known as Huntington’s chorea, it causes the gradual loss of physical and then mental and emotional faculties. Early symptoms include involuntary movements, stumbling, clumsiness, memory lapses, depression and mood swings. Although there is no known cure, drugs and exercise can help manage the disease.

Picture credit:  Richard Cannon

(Laura Spinney writes on science for The Economist. She's based in Lausanne, Switzerland and working on her third novel. Her last article for Intelligent Life magazine was about rediscovering Tony Allison.)