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Mind Bomb

If you had a fifty-fifty chance of carrying a mutant gene that causes a fatal brain disease, would you want to know?


Kevin Baker and his mother, Claire, at the hospital in Massachusetts where she lives.  

Sometime about thirteen years ago, my mother’s brain began to shrink. The signs that something was wrong proliferated slowly, but as ominously as something out of a science-fiction movie.

My mother became unaccountably restless and unable to concentrate. She complained constantly that Larry, my stepfather, didn’t take her out anywhere, that they never did anything—although she could no longer follow the conversation at a dinner party and said things that other people found incomprehensible. She tried going back to work in some of the little tourist shops where she lived, in Rockport, Massachusetts. My mother worked most of her adult life, but now she couldn’t figure out how to run a cash register or follow the simplest directions. Each time, she was fired after a few days, returning home baffled and indignant.

She was becoming indignant a lot. She began to fly into a rage at the smallest frustrations, cursing at herself and at other people in words we never heard her use before. She started to drink as well, something else she had never done much before. My mother had been a classic half–a–glass–of–Champagne–on–New Year’s Eve drinker. Now she had to have wine every night, and even a little made her garrulous and belligerent.

It was as if every part of her personality was being slowly stripped away, layer by layer. The loving, gentle woman my sisters and I had known was being replaced by someone we did not recognize.

There were other things going on as well, physical changes. Her speech was often slurred, even when she was not drinking. Her movements became jerky and exaggerated, as if she could no longer fully control her limbs. She could not abide any restraints. During a drive one Christmas afternoon, I watched while she sat in the passenger’s seat, compulsively buckling and unbuckling her seat belt, again and again, for over an hour. She kept complaining about how it stuck against her body, as if she had never seen or used a seat belt before.

My youngest sister, Pam, and I persuaded her to see a doctor. Her primary-care physician thought she knew what was wrong and sent her to a neurologist to confirm it—but at the last minute my mother refused to keep the appointment. Instead she went on drinking and growing steadily more impatient with everything and everyone around her. She shoved a woman she thought was crowding her at an airport baggage carousel, swore at people over parking spaces.

Mostly she fought with Larry. When he tried to stop her from drinking, she became incensed, threatening to burn down the house they owned together or to blacken over the paintings he sold at a local gallery. Their fights became wild and vituperative. She accused him of being the one with a problem, of being a depressive, of stifling her. A certain mania seemed to come over her during these arguments. Once she did a sort of Indian war dance around him, challenging him to fight.

“I told him, if you want to fight, my family knows how to fight!” she told me. “You ain’t seen nothin’ yet!”

Around that time, I went to Boston to try to intervene. I remember it was a beautiful spring day and we were walking around Beacon Hill. She seemed delighted to see me, but all I could notice was how strange she acted. She hadn’t had a drink, but her gait was wobbly and her eyes looked glazed. She no longer seemed to understand how the stoplights worked. Repeatedly, I had to reach out and pull her back from walking straight into traffic. We strolled down into Boston Common, where a homeless guy on a bench made some innocuous passing comment to her. My mother, always the most private, the most dignified of people, stopped and twirled around, doing a little pirouette for the homeless man, beaming the whole time.

Soon afterward, Larry confronted her with an empty wine bottle she had secreted in their closet. She wrenched it from his hand and hit him across the face with it, cutting his nose. He needed stitches to close the wound, and while he didn’t press charges, my mother had to appear in court under the domestic-abuse laws. I hired a defense lawyer, who got her off with a warning, and finally we prevailed on her to go to a neurologist. This was a full eight years after we first noticed dramatic changes in her behavior. The tests revealed what her primary-care doctor thought all along: My mother was suffering from Huntington’s disease.

Huntington’s (HD) is a hereditary disease, its most illustrious victim the folk- singer Woody Guthrie. It’s caused by a defective gene that produces a mutant “huntingtin” protein. The protein is necessary to human development, but its mutant version produces an excess of glutamines, amino acids that begin to stress, then eventually kill the brain’s neurons. Huntington’s is also known as Huntington’s chorea, or “dance,” for the florid movements that are its most obvious symptom. But such movements do not afflict all sufferers, nor are they the illness’s most destructive characteristic.

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