Huntington’s is a “profound” disease, one of the few neurological disorders that attacks nearly every area of the brain, says Steven Hersch, an associate professor of neurology at Harvard Medical School and Massachusetts General Hospital, and my mother’s neurologist. It affects the cerebral cortex, where thought, perception, and memory are stored. It also shrinks the basal ganglia, which serves as a sort of supercomputer for the rest of the brain, regulating almost everything from movement to the input and output of thoughts, feelings, emotions, behavior. The result is what Hersch terms a loss of “modulation” and “a coarsening” of how we do just about everything—move, think, react. Huntington’s sufferers have trouble correctly reading emotions in others or even recognizing familiar faces. They no longer understand when their behavior is inappropriate, and have difficulty planning, organizing, and prioritizing. They can become both intensely angry and apathetic and indecisive.
“They’re losing possibilities,” says Hersch. “They’re losing the possibilities of things they could do, or think of, or want to do.”
Not the least of Huntington’s effects is what the knowledge of the disease does to its victims. There is no cure. Adult- onset Huntington’s usually afflicts individuals sometime between 35 and 55, although early-onset, or juvenile, Huntington’s can manifest itself before the age of 20. The disease commonly takes ten to thirty years to run its course, as body and mind slowly shut down and leave the sufferer all but inert. Before that happens, victims commonly die from major infections, pneumonia, choking, or “silent pneumonia,” as food goes down the wrong pipe.
Suicides are not uncommon, even among “at-risk” patients who have yet to actually ascertain that they have the disease. Depression is also frequent among such individuals. If one is faced with such a fate, denial can be a survival strategy.
“Often the people who do best are those who can wall it off and go on with their lives,” acknowledges Hersch. “It’s a very good approach in a lot of ways. The trick for people sometimes is to figure out when it’s in their best interest to drop the denial and gain knowledge that will help them.”
The test results devastated my mother. Before long she reverted to denial. She insisted that her test had produced “a false positive.” She told us that she had “the syndrome of the disease, but not the disease itself.” She told us that, somehow, she was “the control” in the test. But as her brain continued to shrink, she began to lose even these words. It became a little joke between us.
“Dearest, I don’t have this, you know,” she would tell me, very seriously, out of the blue. “I’m the—the … ”
“Yes, that’s it!” she would say, delighted.
“Mom, you can’t be the control if you can’t remember the word control,” I would tell her, and she would laugh, and I would laugh. But a few minutes later she would tell me again that she didn’t really have this, you know.
The fact that my mother had Huntington’s meant that I had a fifty-fifty chance of inheriting the gene—and thus the disease—myself. I didn’t like to look at this too directly. That was my own form of denial. I told friends and relatives that I would not take the genetic test that my mother had taken. What was the point, without a cure? It could only screw up my health insurance, and who wanted to live with such a fate hanging over their head?
But those 50-50 odds worked their own havoc. Eventually I found that I couldn’t help thinking about the disease whenever I had trouble coming up with a word or organizing an article. I noticed whenever my body flinched involuntarily. I became especially aware of how often I would drag one of my feet along the sidewalk, or how frequently my left arm would twitch. I took to holding my left hand out in front of me, trying to reassure myself by seeing how long I could keep it still—a better test for delirium tremens, I suppose, than Huntington’s disease.
“Would you stop doing that? You don’t have it!” Ellen, my wife, would tell me.
Yet I was sure that I could feel something deep inside me, something that I came to think of, a little melodramatically, as a stirring. Sometimes, lying awake in bed late at night, I was sure that I was only holding it back by force of will. I was certain that if I let go, I would begin to move compulsively, uncontrollably, just as my mother did now. The dance.
“I think I might have this,” I told Ellen.