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-
galow near Fredericton, Barb Hat-
field marvelled at the bright blue
sky. It was early March 1999, and
the weather was unseasonably fine.
Tomorrow, she thought, she
and her husband, Hal, would go for a
brisk walk. The ringing telephone
broke her reverie.
“Hello, Barb? I’m sorry to bother
you, but there’s something you need to
know.” Immediately Barb knew this
was the call she’d been dreading.
“It’s about Eleanor…”
Barb’s sister Eleanor, a registered
nursing assistant, had worked in a
nursing home—the Wauklehegan
Manor—in McAdam for the last 25
years. She had never married but had
a wide circle of friends. Lately, how-
ever, people had begun noticing
changes in her behaviour.
The caller, a friend of Eleanor’s, had
phoned Barb a few weeks before to
say she was concerned. “She starts a
job and doesn’t finish it. She leaves
early. It’s not like her.”
Now she told Barb that Eleanor’s
electricity had been turned off a few
days ago.
The next morning, Barb and her
brother Laurie met Eleanor at her two-
storey bungalow in McAdam, on the
outskirts of Fredericton. They were
shocked at how cold it was inside.
“What’s wrong?” Barb asked her sis-
ter as the three stood in her living
room, still wearing their overcoats.
“I can’t afford to pay my power bill,”
explained Eleanor, suddenly sound-
ing confused and almost childlike. “I
168
don’t know where all my money goes.”
She confessed that she had not paid
her bill for five months. Then, as she
turned away from Barb, she grabbed
the side of a chair and collapsed into
it. Sobbing, she dropped her head and
said, “I think I have the same disease
Mom had. I think I have Alzheimer’s.”
Eleanor was 45 years old.
Twenty years before, Barb and
Eleanor had watched their mother,
Pauline, fading ever deeper into the
fog of Alzheimer’s. She was in her
early 50s when diagnosed with early-
onset Alzheimer’s, an especially per-
nicious strain of the disease that
typically strikes victims in their 40s
and 50s, and that often runs in families.
It counts for five to seven percent of
Alzheimer’s cases in Canada.
Barb, then 23, knew little about the
disease. Her second cousin, Ed Chris-
tie, told her that many in their ex-
tended family had been ravaged by it.
Ed, a now-retired high-school tech-
nology teacher in nearby Harvey Sta-
tion, took an interest in early-onset
Alzheimer’s when his mother was di-
agnosed with it in her mid-40s, a few
years before. To Ed it seemed there
were just too many relatives who had
suffered from what they called “de-
mentia” or “hardening of the arteries”
and died in their 40s or 50s.
Barb learned that her grandmother,
her uncle and more than three dozen
other extended family members had
fallen victim to early-onset Alzhei-
mer’s. Many viewed it as the “family
curse” and refused to talk about it.
RD I NOVEMBER 2007
“IT WAS BACK THEN THAT
I BEGAN LIVING WITH THIS
TIME BOMB, WONDERING IF
IT WOULD EVER EXPLODE.”
Barb remembers going to the ceme-
tery in Harvey Station with her mother
and seeing her grandmother’s tomb-
stone. When she asked how her grand-
mother had died so young, at 55, her
mother said, “She lost her mind.”
“That’s all she would ever say,” re-
calls Barb. “She felt it was a disgrace
to our family.”
I
n the early 1970s, Ed began as-
sisting the National Institutes
of Health (NIH) in Bethesda,
Md., with an early-onset Alz-
heimer’s research project.
After learning of the high num-
ber of cases in Harvey Station, the
United States-based scientists wanted
to trace the incidence of the disease
among Barb and Ed’s extended fam-
ily. By putting the family under their
microscopes, they hoped to pinpoint
the gene responsible for the “familial”
strain of the disease. Once they found
it, perhaps they could find a prevention
or a cure.
Ed convinced nearly 100 relatives
to donate blood samples for the NIH
researchers. Some started referring to
him as the “Harvey Vampire.” He also
produced a genealogy, discovering that
the first family members had come to
Harvey Station from Northumberland,
170
England, in the early 1800s. (It was
some of these settlers who had
brought with them the gene respon-
sible for early-onset Alzheimer’s.)
Ed, Barb, their siblings and other
relatives realized they too could fall
victim to the family curse in as little as
20 years. Researchers told them that if
one parent had the defective gene, his
or her offspring had a fifty-fifty chance
of getting Alzheimer’s. “It was back
then that I began living with this time
bomb,” Barb remembers, “wondering
if it would ever explode.”
Ed’s mother died in 1976, at 46. Five
years later, Barb’s mom was in rapid
decline. Barb and Hal returned from a
monthlong vacation and were stunned
by how much Pauline had deteriorated
in that short time span. The once-
proud woman, who had raised eight
children, could no longer even hang
her laundry. A lifelong knitter, she
couldn’t manage a stitch.
“I’m just so confused, honey,” she
said, as Barb helped her hang up the
wash. “What’s wrong with me?” As
Barb dusted and vacuumed, Pauline
followed her from room to room; she
seemed afraid to be alone.
Eventually there was no choice but
to move Pauline into a nursing home.
A TICKING TIME BOMB
One day Barb walked into her mom’s
room, and for the first time, Pauline
didn’t know who she was. Barb
watched her mother waste away,
shrinking from over 200 pounds to
less than 80. Pauline died in 1984, at
56, four years after she was diagnosed
with early-onset Alzheimer’s.
Barb’s uncle, Pauline’s brother, died
from the disease in 1995. He was 64
and was diagnosed at 49.
With Alzheimer’s ravaging Barb’s
family, Barb and Hal decided they
wouldn’t have children. Ed Christie and
his wife made the same decision.
Ed had been the family’s point man
with the NIH, collecting blood and tis-
sue samples, but Barb also wanted to
help. It took guts to go public, to be in-
terviewed about the family’s work with
the Alzheimer’s researchers and to risk
someone commenting on their family’s
“curse.” But she and others in the fam-
ily had stopped caring about what any-
one else thought: They had a mission.
Barb and her sister Cheryl both vol-
unteered to go to the NIH in Bethesda
ALZHEIMER’S
LEARN MORE ABOUT
ges of development
symptoms and sta
online this month.
and take a battery of tests. Barb took
a week off from her work as a provin-
cial civil servant, and for seven days
she and her sister were poked, prod-
ded, X-rayed and examined. Ed had
gone through similar tests at the NIH.
Linda Nee, an NIH social-science
analyst, had been working with Har-
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!”
found I had the gene, I don’t think I
could cope.” A cousin of Barb’s adds,
“I’d rather live in hope than in dread.”
In 2001, in her neat nursing-home
room, Eleanor spends her time looking
out her window at the trees behind
Wauklehegan Manor—the same home
she worked in for nearly 25 years.
Sometimes she thinks she’s still on duty
and rushes to “help” another patient.
Her former co-workers pay close at-
tention to her, calming her, helping her
dress, making sure she takes her medi-
cation. She was diagnosed with the dis-
ease two years before.
Barb visits her often. Today she
greets her sister with a hug and a kiss.
Eleanor’s hazel eyes light up when she
tells Barb, “I’m getting married!”
“Really?” says Barb with a wink. “Do
I know him?”
“Yes,” her sister replies, naming an
old childhood friend whom she hasn’t
seen in ages and who is long married.
“That’s nice,” says Barb, knowing
that her sister will forget what she has
said in a matter of minutes.
“Guess what else?” says Eleanor. “I
won a car. It’s being delivered today!”
Barb straightens up her sister’s
room, recalling how she did the same
chores for her mother not so long ago.
174
A
s the years pass, yet another
generation of Barb and Ed’s
relatives are reaching the
target age for early-onset
Alzheimer’s. Barb’s sis-
ter Sheila was diag-
nosed at 39 and joined Eleanor at
Wauklehegan Manor.
“Linda Nee and others at the NIH
say a cure is right around the corner,
but it’s already been ten years,” says
Barb. “We need it now.” She admits
that every time she goes into the
kitchen and forgets what she went
there to get, she thinks, I’m getting
Alzheimer’s.
“Ed reassures me that it’s not a prob-
lem if you forget where you put your
car keys,” says Barb. “It’s a problem if
you find them and don’t know what
they’re for.” She manages a wry smile,
then adds, “I live with this every day.
I am petrified.”
Eleanor died in October 2004, at age
50, followed in 2006 by her sister Sheila
at 43. Ed’s brother, Stewart, 58, is in the
late stages of the disease and several
other relatives are believed to be show-
ing symptoms of early-onset Alzhei-
mer’s. Barb, 51, and Ed, 60, show no
signs. The family, especially Barb, con-
tinues to co-operate with researchers.