And yet, inevitably, I would find myself filled with rage at times. I thought maybe it was the knowing that made all the difference. I joked about the old Woody Allen lines, from Love and Death: “How I got into this predicament I’ll never know … To be executed for a crime I never committed. Of course, isn’t all mankind in the same boat? Isn’t all mankind ultimately executed for a crime it never committed? The difference is that all men go eventually. But I go at six o’clock tomorrow morning.”
But I wasn’t going tomorrow morning. No one is sure what triggers the onset of Huntington’s. The gene’s interaction with other genes, or environmental factors, or even aging itself may play a role. Heredity and especially gender seem to be very strong factors. The disease could begin to take its toll at almost any time, but because I inherited it from my mother instead of my father, it is more likely to manifest itself around the same age when she got it, at 65 or maybe even older—a very late onset.
“The brain copes, until it can’t anymore” is the exquisite phrase with which Herminia Diana Rosas, an assistant professor of neurology at Mass General and Harvard Medical School, describes the progress of the disease. Huntington’s may well be active in the brain ten, even twenty years before any symptoms begin to show up. We don’t see its effects because the brain rallies to adjust. In a touchingly human response to this threat, the neurons compensate by trying to do less, or by sharing vital information among each other, squirreling away knowledge and memory where they can.
In other words, I had not received some fatal diagnosis, not really. All my Huntington’s gene guaranteed was that I was going to start to die, most likely at the same age that many people start to die, from one thing or another, prostate or heart disease, stroke or diabetes or Alzheimer’s—much as we are all dying, all the time. I might have another good sixteen years ahead of me, maybe even more. I joked that it was like being on death row, only with better company. I joked that it was like being on death row, only with better food. Coming out of my agent’s building on yet another gorgeous summer day—and what a beautiful summer it was—I told myself, “You’ll be doing this ten years from now, and you’ll still have six years to go. Think of how long that is, how much will happen and how much you can do!”
What I really feared was not death but what would precede it. Dr. Hersch’s “coarseness” meant a lack of nuance—a great prescription for a writer. I would be unable to work, to organize my thoughts or comprehend the world around me. I would forget friends, names, faces, facts, memories. I would be unable to control my moods, my movements, my urges, would become a living caricature of my former self—much like what I had seen happen to my mother.
Our efforts to get her to adjust to life at her assisted-living facility were breaking down. She became belligerent if she felt she was being mistreated or thwarted in any way. She kept insisting that she wanted to be married again, kept pursuing men of any age. When someone told her that a 95-year-old fellow resident at Landmark had said she was nice, she harassed him to the point where she had to be forcibly removed from his room, slugging a female staff member in the process.
This time she was ejected from the facility. After a brief and volatile stay in a nearby nursing home, my mother was shipped out to a psychiatric ward, where she was drugged nearly to the point of being insensible. She couldn’t speak, could barely move, and seemed to be experiencing hallucinations. My sister, noticing other inmates walking around dressed in some of her clothes, raised hell, cajoled doctors, and managed to get her transferred to a much better facility, a sprawling state hospital. It was a place with light and space and a dedicated staff that adjusted her medication so that she was alert and talking again. She seemed much happier, the violent rages ebbing away, but all the transfers, and the progress of her disease, had taken its toll. She had trouble completing even a simple sentence, and her gait was so unsteady that she was confined permanently to a wheelchair.
There was no disguising that she was in an institution now. Her ward was all tile and linoleum, and she was surrounded by other inmates suffering from advanced neurological disorders, Huntington’s and Parkinson’s, multiple sclerosis, retardation, and dementia. There was one man, younger looking than my mother, who just sat about with his head crooked permanently to one side, his tongue lolling out of his mouth. Another woman, all but immobile, told us how she had been a nurse for many years but was now suffering from Parkinson’s. She seemed to be alert enough and in her right mind.