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ATICKING

Many members of a New Brunswick family


carry the gene for early-onset Alzheimer’s,
and many have died from the disease.

TIME BOMB
But their co-operation with researchers is
doing much to help find a prevention
or a cure.
BY ROBERT KIENER

I L LU ST R AT E D BY G É R A R D D U B O I S 167
RD I NOVEMBER 2007

L
ooking out the window of her bun- don’t know where all my money goes.”
galow near Fredericton, Barb Hat- She confessed that she had not paid
field marvelled at the bright blue her bill for five months. Then, as she
sky. It was early March 1999, and turned away from Barb, she grabbed
the weather was unseasonably fine. the side of a chair and collapsed into
Tomorrow, she thought, she it. Sobbing, she dropped her head and
and her husband, Hal, would go for a said, “I think I have the same disease
brisk walk. The ringing telephone Mom had. I think I have Alzheimer’s.”
broke her reverie. Eleanor was 45 years old.
“Hello, Barb? I’m sorry to bother
you, but there’s something you need to Twenty years before, Barb and
know.” Immediately Barb knew this Eleanor had watched their mother,
was the call she’d been dreading. Pauline, fading ever deeper into the
“It’s about Eleanor…” fog of Alzheimer’s. She was in her
Barb’s sister Eleanor, a registered early 50s when diagnosed with early-
nursing assistant, had worked in a onset Alzheimer’s, an especially per-
nursing home—the Wauklehegan nicious strain of the disease that
Manor—in McAdam for the last 25 typically strikes victims in their 40s
years. She had never married but had and 50s, and that often runs in families.
a wide circle of friends. Lately, how- It counts for five to seven percent of
ever, people had begun noticing Alzheimer’s cases in Canada.
changes in her behaviour. Barb, then 23, knew little about the
The caller, a friend of Eleanor’s, had disease. Her second cousin, Ed Chris-
phoned Barb a few weeks before to tie, told her that many in their ex-
say she was concerned. “She starts a tended family had been ravaged by it.
job and doesn’t finish it. She leaves Ed, a now-retired high-school tech-
early. It’s not like her.” nology teacher in nearby Harvey Sta-
Now she told Barb that Eleanor’s tion, took an interest in early-onset
electricity had been turned off a few Alzheimer’s when his mother was di-
days ago. agnosed with it in her mid-40s, a few
The next morning, Barb and her years before. To Ed it seemed there
brother Laurie met Eleanor at her two- were just too many relatives who had
storey bungalow in McAdam, on the suffered from what they called “de-
outskirts of Fredericton. They were mentia” or “hardening of the arteries”
shocked at how cold it was inside. and died in their 40s or 50s.
“What’s wrong?” Barb asked her sis- Barb learned that her grandmother,
ter as the three stood in her living her uncle and more than three dozen
room, still wearing their overcoats. other extended family members had
“I can’t afford to pay my power bill,” fallen victim to early-onset Alzhei-
explained Eleanor, suddenly sound- mer’s. Many viewed it as the “family
ing confused and almost childlike. “I curse” and refused to talk about it.
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RD I NOVEMBER 2007 A TICKING TIME BOMB

“IT WAS BACK THEN THAT One day Barb walked into her mom’s
room, and for the first time, Pauline
I BEGAN LIVING WITH THIS didn’t know who she was. Barb
TIME BOMB, WONDERING IF watched her mother waste away,
shrinking from over 200 pounds to
IT WOULD EVER EXPLODE.” less than 80. Pauline died in 1984, at
56, four years after she was diagnosed
with early-onset Alzheimer’s.
Barb remembers going to the ceme- England, in the early 1800s. (It was Barb’s uncle, Pauline’s brother, died
tery in Harvey Station with her mother some of these settlers who had from the disease in 1995. He was 64
and seeing her grandmother’s tomb- brought with them the gene respon- and was diagnosed at 49.
stone. When she asked how her grand- sible for early-onset Alzheimer’s.) With Alzheimer’s ravaging Barb’s
mother had died so young, at 55, her Ed, Barb, their siblings and other family, Barb and Hal decided they
mother said, “She lost her mind.” relatives realized they too could fall wouldn’t have children. Ed Christie and
“That’s all she would ever say,” re- victim to the family curse in as little as his wife made the same decision.
calls Barb. “She felt it was a disgrace 20 years. Researchers told them that if Ed had been the family’s point man
to our family.” one parent had the defective gene, his with the NIH, collecting blood and tis-
or her offspring had a fifty-fifty chance sue samples, but Barb also wanted to

I
n the early 1970s, Ed began as- of getting Alzheimer’s. “It was back help. It took guts to go public, to be in-
sisting the National Institutes then that I began living with this time terviewed about the family’s work with
of Health (NIH) in Bethesda, bomb,” Barb remembers, “wondering the Alzheimer’s researchers and to risk
Md., with an early-onset Alz- if it would ever explode.” someone commenting on their family’s
heimer’s research project. “curse.” But she and others in the fam-
After learning of the high num- Ed’s mother died in 1976, at 46. Five ily had stopped caring about what any-
ber of cases in Harvey Station, the years later, Barb’s mom was in rapid one else thought: They had a mission.
United States-based scientists wanted decline. Barb and Hal returned from a Barb and her sister Cheryl both vol-
to trace the incidence of the disease monthlong vacation and were stunned unteered to go to the NIH in Bethesda
among Barb and Ed’s extended fam- by how much Pauline had deteriorated
ily. By putting the family under their in that short time span. The once-
ALZHEIMER’S
microscopes, they hoped to pinpoint proud woman, who had raised eight LEARN MORE ABOUT
ges of development
the gene responsible for the “familial” children, could no longer even hang symptoms and sta
strain of the disease. Once they found her laundry. A lifelong knitter, she online this month.
it, perhaps they could find a prevention couldn’t manage a stitch.
or a cure. “I’m just so confused, honey,” she and take a battery of tests. Barb took
Ed convinced nearly 100 relatives said, as Barb helped her hang up the a week off from her work as a provin-
to donate blood samples for the NIH wash. “What’s wrong with me?” As cial civil servant, and for seven days
researchers. Some started referring to Barb dusted and vacuumed, Pauline she and her sister were poked, prod-
him as the “Harvey Vampire.” He also followed her from room to room; she ded, X-rayed and examined. Ed had
produced a genealogy, discovering that seemed afraid to be alone. gone through similar tests at the NIH.
the first family members had come to Eventually there was no choice but Linda Nee, an NIH social-science
Harvey Station from Northumberland, to move Pauline into a nursing home. analyst, had been working with Har-
170 171
A TICKING TIME BOMB

vey Station family members since


1977 and had formed friendships with
many of them. One afternoon, after
an especially gruelling round of test-
ing, Barb asked her, “Linda, do you
think there will ever be a cure for us?”
“The cure is coming,” said Nee.
“You just have to wait.”

“Ed! ed! ed!” Nee shouted into the tele-


phone. “Got some great news!” It was
June 1995. After nearly two decades of
research, and thanks in large part to
the co-operation of Ed and Barb’s ex-
tended family, scientists had isolated
the gene that causes early-onset Alz-
heimer’s. Dr. Peter St George-Hyslop
and his team at the University of
Toronto had just announced their dis-
covery. “Identifying the gene is huge,
Ed. Now we can use it as a model and
see if we can find a cure.”
Ed could hardly believe his ears.
The work was beginning to pay off.
While the 1995 discovery was the
first giant step towards developing
a prevention or cure for early-onset
Alzheimer’s, researchers believed
it could take decades to accomplish
that. However, it was now possible for
anyone to be tested to see if they had
the mutant gene responsible for the
disease. But there was a catch: If they
had the gene, there was a 99 percent
chance they would develop early-onset
Alzheimer’s.
Barb, Ed—virtually everyone in the
family—decided not to be tested. “If I
were showing signs of Alzheimer’s,”
says Barb, “I would be tested. But I
won’t before that. If I were tested and
173
“ED! ED! ED!” LINDA NEE
SHOUTED INTO THE
TELEPHONE. “GOT
SOME GREAT NEWS!”

A
found I had the gene, I don’t think I s the years pass, yet another
could cope.” A cousin of Barb’s adds, generation of Barb and Ed’s
“I’d rather live in hope than in dread.” relatives are reaching the
target age for early-onset
In 2001, in her neat nursing-home Alzheimer’s. Barb’s sis-
room, Eleanor spends her time looking ter Sheila was diag-
out her window at the trees behind nosed at 39 and joined Eleanor at
Wauklehegan Manor—the same home Wauklehegan Manor.
she worked in for nearly 25 years. “Linda Nee and others at the NIH
Sometimes she thinks she’s still on duty say a cure is right around the corner,
and rushes to “help” another patient. but it’s already been ten years,” says
Her former co-workers pay close at- Barb. “We need it now.” She admits
tention to her, calming her, helping her that every time she goes into the
dress, making sure she takes her medi- kitchen and forgets what she went
cation. She was diagnosed with the dis- there to get, she thinks, I’m getting
ease two years before. Alzheimer’s.
Barb visits her often. Today she “Ed reassures me that it’s not a prob-
greets her sister with a hug and a kiss. lem if you forget where you put your
Eleanor’s hazel eyes light up when she car keys,” says Barb. “It’s a problem if
tells Barb, “I’m getting married!” you find them and don’t know what
“Really?” says Barb with a wink. “Do they’re for.” She manages a wry smile,
I know him?” then adds, “I live with this every day.
“Yes,” her sister replies, naming an I am petrified.”
old childhood friend whom she hasn’t
seen in ages and who is long married. Eleanor died in October 2004, at age
“That’s nice,” says Barb, knowing 50, followed in 2006 by her sister Sheila
that her sister will forget what she has at 43. Ed’s brother, Stewart, 58, is in the
said in a matter of minutes. late stages of the disease and several
“Guess what else?” says Eleanor. “I other relatives are believed to be show-
won a car. It’s being delivered today!” ing symptoms of early-onset Alzhei-
Barb straightens up her sister’s mer’s. Barb, 51, and Ed, 60, show no
room, recalling how she did the same signs. The family, especially Barb, con-
chores for her mother not so long ago. tinues to co-operate with researchers.
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