“I didn’t think it ever occurred to me not to get tested,” she said. Often the descendants of F.F.I victims prefer to be left in the dark about whether they, too, carry the mutated gene, but Sonia immediately wanted to know her status. F.F.I is an autosomal dominant disease, which means you have a one-in-two chance of having the disease if your parent has it. The diagnosis was extraordinarily rare-prion diseases, of which F.F.I is one, afflict only one in a million people. Later, an autopsy-her brain had to be shipped to a special lab-showed the cause of death to have been F.F.I. During most of her sickness, her doctors were baffled-she had such terrible dementia that no one focussed on her sleeplessness, a classic symptom of Fatal Familial Insomnia. Vallabh, then twenty-eight years old, softer, brown-eyed, more reposeful, of Indian ancestry, told me her story: Her mother had died in December, 2010, at fifty-two, after a terrifying and confusing illness. Minikel was slender, wearing the smart Cambridge-graduate-school uniform of jeans, a collared shirt, and a down vest (his: golden yellow). We agreed to meet up on a snowy morning this past winter, at the Squeaky Beaker, a coffee shop near M.I.T. The sender of the e-mail was Eric Minikel, and his wife, whose family had the disease-causing mutation, was Sonia Vallabh. I thought of a line I love from a Wallace Stevens poem: “The natives of the rain are rainy men.” It turned out the rain could not be outrun, not even by the canniest of plotters. Maybe that’s how it goes, I remember thinking, maybe that’s life. My book was destined to end in the same dark place where it had begun. But by the time I got to the end of four years of research, it was clear that the realities of their situation-shame, fear, confusion, discord, inertia, all the human verities, not to mention the complexities of their disease-had pretty much stopped their progress. Starting the book, I had expected that the Italian family, with their deep desire to conquer the centuries-old affliction, would play that role. I had always felt like I was missing a hero for my story. The best character may present herself long after publication maybe even after you’ve stopped thinking about a subject that once meant the world to you.
0 kommentar(er)
