MIND
BOMB
Left:
Baker at the age of 1, with his mother at home in Tenafly, New
Jersey. Right: Claire Baker, thirteen years after her Huntington’s
disease manifested itself.
(Photo: Courtesy of Kevin Baker; Michal Chelbin for New York Magazine)
Every
step of the way, meanwhile, my mother’s denial only made
everything worse for her. It now meant the dissolution of the
life she had made with Larry—the quiet days in retirement
they loved to spend fishing or reading and watching the news together
in the evening; going to their favorite bakery every Sunday morning.
None of it was enough to appease what was going on inside her
brain. She wouldn’t stop sneaking drinks, wouldn’t
keep taking the medications the doctors prescribed to control
her moods. We would have been happy to let her drink if that had
helped her, but the alcohol only stressed the brain and served
to dilute the effects of the drugs. My mother now demanded almost
constant attention to keep her entertained, to keep her from wandering
into disaster. Eventually Larry told us he wanted out. They had
been married for 21 years, and my mother had nursed him through
a heart attack, cancer, and occasional bouts of epilepsy. But
Larry, an émigré from Middle Europe, had seen both
the Nazis and the Communists march into his life, and he knew
when to head for cover. I couldn’t blame him. It’s
an enormous burden for any one person to take care of a Huntington’s
victim, particularly someone who is a senior citizen himself,
and he had been dealing with it for a decade. Pam and I hired
more lawyers to negotiate the divorce, the sale of their neat
little house, the overwhelming thicket of bureaucracy that determined
where and how she might live now.
Pam
managed to find her a nice studio apartment in an assisted-living
facility in Beverly, Massachusetts; a converted high school with
the pretentious name of “Landmark at Oceanview.” (Just
what was the “landmark”? The last leg of the voyage
before you ease into the good harbor of death?) There were big
school windows that brought in lots of light, and bright, cheery
carpeting, and a diligent staff to make sure that she took her
pills and to cook her meals and do her laundry—all tasks
that she was having increasing difficulty performing.
My
mother’s denial tormented those of us who loved her. But
now I found her desire to cling to the life she had known understandable,
even admirable.
Personally,
I wanted to shoot myself every time I set foot inside the place.
My mother, on the other hand, thought it was unbelievably “posh,”
the sort of home where she had always dreamed of living.
Meanwhile,
my father, whom she had divorced nearly 30 years ago, came east
with cinematic notions of getting remarried and taking care of
her. He disregarded almost everything we told him about Huntington’s
and instructed her that she should just try to concentrate on
holding her limbs still. Before one ghastly family dinner in a
restaurant, he let her have a drink, then tried to cut her off.
When his back was turned for a moment, she calmly snatched up
his full wineglass and downed the contents in one swallow. I saw
his eyes widen. My mother spent the rest of the meal barely able
to speak, rolling her head back to try to get food down, until
we made her stop out of fear she would choke to death in front
of our eyes. My father fled back to his apartment in Hollywood.
Good-bye, Golden Pond.
I
was determined by this time to face the disease head-on. If my
mother had made everything worse for herself by remaining in denial,
I would throw it off. I would take whatever medications were necessary,
volunteer for whatever experimental programs there must surely
be. I convinced myself that this was a purely practical idea.
Why go about looking for cures or ways to ameliorate the effects
of the disease if I didn’t have the gene? Looking back now,
I think my decision may have been more emotional than anything
else, a desire to know this and be done with the uncertainty.
I told myself I would be stronger than my mom, and take whatever
I was given. Early in 2007, I set up an appointment at Columbia
University’s HDSA Center for Excellence, located up in Washington
Heights.
The Columbia
Center deals with every aspect of the disease, both for those
already suffering from HD and for those at risk, and it endeavors
to help patients through each stage. It’s unique in that
the care it offers is free, although there are nominal fees for
some parts of the gene test. The center’s testing protocol,
established and refined over the fifteen years since the Huntington’s
gene was first identified, is actually an involved process, one
that takes place over a few months—and is an infinitely
better one than what my mother went through. One of its chief
purposes was to slow me down—to let me think over what I
was doing. As the center’s genetic counselor warned me at
the start, “Once you know, you can’t not know.”
There was
the session with the genetic counselor, a visit to a psychiatrist,
neurological and neuropsychological exams. If after all that I
still wanted to know, they would draw my blood and tell me the
result of the gene test. That result would be a number—the
number of times a particular string of glutamine DNA, known as
CAG, repeated itself at the beginning of my Huntington’s
gene. If that number was 27 or below, I was in the clear. If it
was 36–39, I would fall into a tiny percentage of the population
that might, or might not, develop the disease. If it was 40 or
more, it meant that I had inherited the defective gene—and
that I would get the disease.
I threw myself
eagerly into the testing process, glad now to be doing this, to
be confronting these phantom fears. The not knowing by now had
become as bad as knowing the worst could possibly be, I told myself.
I disavowed everything I had told people before. Best to look
this fate in the eye, to see if it really was waiting for me.
The initial
exams went well. The doctor found no signs that I had the disease
already (my twitching left arm promptly stopped twitching so much).
But the doctor seemed less than pleased that I was taking the
test at all. The genetic counselor had insisted that there was
no right or wrong reason for wanting to take the gene test, but
it seemed clear that I had made the wrong choice.
Later, I found
that I had misinterpreted the doctor’s attitude. There really
was no right or wrong reason to get the test, she was simply a
little surprised by my motives. Most of the people they saw came
because of some life trigger. They were making career decisions,
getting married, thinking of having children—or wanting
to spare their children, now marriage age, from having to take
the test. Or they had just learned that Huntington’s was
in the family, or that a genetic test existed.
Getting tested
so that you could see what you could do about the disease was
unusual … in part because there is currently nothing you
can do to cure the disease or even curb its progress.
Huntington’s
is what the federal Food and Drug Administration (FDA) officially
calls an “orphan disease”—it affects too few
people to make it worth the drug companies’ while to develop
a drug for it on their own. The official cutoff number is 200,000
people; Huntington’s currently afflicts only 30,000 to 35,000
people in the United States, with perhaps another 200,000 to 250,000
at risk of getting the disease.
The National
Institutes of Health (NIH) provide subsidies in such cases, but
the total amount spent in the U.S. on finding a cure this year
will probably be no more than $120 million to $130 million. A
substantial sum, to be sure—but not so much when you consider
that it can easily cost drug companies $1 billion to come up with
an effective drug. As it stands now, a cure is not on the horizon.
The most promising idea seems to be “turning off”
the defective Huntington’s gene, but discovering how to
do that presents a host of technical problems that remain far
from solved.
The
doctor’s surprise at my motives drew me up a little short—not
a bad thing. Getting tested for the gene of a hereditary disease
with no cure is and should be a hugely complicated decision, one
with implications beyond one’s own self. It can mean “outing”
other family members, who may have no desire to learn if they
will get the disease. It can mean any number of problems with
one’s health insurance (no doubt a big reason why fewer
than 3 percent of at-risk Americans get tested for HD, as opposed
to 20 percent of Canadians who might have the gene). It can mean
dealing with unanticipated feelings of guilt, dread, despair.
It all made me think again. Was I engaging in a reckless act of
bravado, moving into a realm that I was not psychologically or
emotionally prepared for, just to show that I could do it?
“No
matter what the result is,” my genetic counselor warned
me, “nobody is the same person they were when they walked
in here.”
I was pretty
sure that if the results were negative I would be the same person
I was in about five minutes. On the other hand, the 50-50 chance
that I had the gene had already begun to unravel any peace of
mind about my future. Bravado or not, I had to know. I had them
draw the blood. They told me it would take two to four weeks for
a result, depending on how crowded the lab was. No matter what
the verdict was, I would have to come back to the clinic for the
counselor to tell me in person.
During my
trips to the Columbia Center, Ellen and I would sit in the plain,
institutional waiting room and watch other outpatients coming
and going. Some displayed no outward signs of having the disease;
others clearly had the telltale movements. Some of these people
carried themselves with remarkable bravery, others were so young
that it was almost unbearable to watch them. I knew that, in a
very short time, I would either walk away and never see them,
never see this place again, or that I would join their small fraternity.
The two to
four weeks that I had to wait seemed to stretch out like a lifetime—in
the best sense of the words. I put the possibility of having the
gene out of my head again, and was more sanguine about my chances
than I had been in months, still buoyed by the revelation that
all my twitching had been, so to speak, in my head.
Only one afternoon,
while I was working in a library, did the full understanding of
what I was doing sneak up on me. What if it really is positive?
I thought to myself, out of the blue, and I realized that I had
no answer. I was stepping over a cliff, into a state of mind that
I had no real way of even imagining. I quickly went back to my
research, shutting this idea safely away again behind my own walls.
But it was still there.
“Once
you know,” the clinic’s counselor told me before I
was tested for the mutant gene, “you can’t not know.”
When
the call came two weeks later to set up my appointment, I wished
for more time. I had two days to wait. I joked with my wife that
our appointment was on an auspicious day—the anniversary
of Hitler’s invading Russia. But I also couldn’t help
wondering, “If it’s negative, wouldn’t they
tell you over the phone? I mean, even if they say they won’t?
Just to give you peace of mind, as soon as they can?”
“No, they have a whole procedure,”
my wife insisted. “It’s going to be fine, you don’t
have this.”
“But I’m just saying. Wouldn’t
you tell the person? Wouldn’t it be sadistic to let you
wonder for the next two days if the news was good?”
“It’s a procedure!”
We took the subway up to the center and got there
a little early, seating ourselves in the waiting room again. Almost
as soon as we arrived, my genetic counselor came out to see us—and
we had our answer. When she walked into the waiting room we could
both see that tears were welling up in her eyes, and that her
mouth was set in a tight little smile, like someone trying to
pretend there’s nothing wrong. It was like being on trial
and having a jury come back that won’t look you in the face.
After that, it was all over very quickly. The counselor sat us
down in her windowless office and told us at once that the number
of my CAG repetitions was 41—one number higher than my mother’s.
I had the defective gene, and my brain, too, would begin to die.
I
can’t say that I was immediately stricken or horrified.
I didn’t even feel as upset as I have sometimes when an
editor hasn’t liked a manuscript. It felt, as bad news often
does, as if I’d known what it was going to be all along.
Outside, it was still a sunny early-summer day. We got back on
the subway. Ellen tried to be consoling, but I wasn’t in
need of any, not just then. What was there to say? When we got
home, I got a call from a podiatrist wanting to move up an appointment
for a minor foot problem I was having. I hustled over to the East
Side and there I sat, in another doctor’s waiting room,
not an hour later. It all seemed unreal, like some weird simulation
of what I had just been through. I thought about how giddy I would
have been feeling if the results had been negative. I felt like
blurting out the news to anyone I encountered, I just found
out I will get a fatal disease. But I didn’t. The doctor
prescribed some egg cups for my shoes, and I went home.
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