Post by workingmom on Dec 24, 2007 10:26:30 GMT -5
2007-12-23
A disease that steals the self
By Joseph Hall
Health Reporter
As always, Diane Schaef is impeccably turned out.
Her blue-grey cashmere sweater is accented by a string of pearls, which circle a neck that would surely have been described as regal in earlier times. Her greying hair is stylishly short and beauty-salon neat. Her skin – a genetic gift from her Aunt Doey, they say – is creamy and, at 58, almost flawless.
But it's her eyes that stop you. Black, black eyes. Eyes once incandescent with intelligence, authority and sometimes mirth – eyes now flat and eerily still.
Those disturbing eyes tell her story.
It's a story of descent from happy confidence to crippling self-doubt, of profound personality change, of people stricken in the prime of life, and of a little-known brain disorder called frontotemporal dementia.
"Isn't she beautiful," says her husband, Bruce Schaef, 65, a baritone with the Canadian Opera Company and the artist behind his wife's daily glamour.
"I do it to honour her ways ..." Then tears well up and he turns his gaze away.
Diane Schaef is in an advanced stage of frontotemporal dementia, commonly known as FTD. It's a degenerative neurological ailment that attacks the brain's frontal and temporal lobes, where personality traits and language control largely reside.
It's a disease, says Dr. Tiffany Chow, that attacks men and women equally and can replace a loved-one with a doppelganger stranger. Unlike Alzheimer's disease – which steals memory yet can leave personality intact for lengthy periods – FTD is a body snatcher.
"So you no longer have a partner helping you run a household and parent children," says Chow, a clinician and scientist with the Baycrest Centre's Rotman Research Institute in Toronto. "You've got another person who is an unknown quantity."
FTD is not a new disease. But only recently has its brain pathology been identified and studied, and have physicians begun to diagnose it consistently.
Previously blamed on everything from bipolar affective disorder to depression to a bad mid-life crisis, FTD was often well entrenched before it was actually identified in patients.
"So people didn't come to attention for the disorder appropriately oftentimes until four to six years of having the symptoms," says Chow.
She stresses that FTD is "a totally different animal" from Alzheimer's.
The telltale plaque deposits and tangled fibres that mar the brains of Alzheimer's sufferers are absent in FTD patients.
And while its cause is still unclear, one theory gaining wide acceptance is that many cases of the ailment involve a genetic shortage of the healing protein progranulin. "It's a protein that's actually involved in tissue healing and some of the immunology related to making sure that the body starts to repair itself," Chow says.
A gene mutation that limits progranulin production may allow neurological damage to go unrepaired over time, she adds.
And in many cases, a lack of progranulin may allow build-up of a misshapen protein known as TDP-43 in parts of the brain.
"One way to put those things together," says Chow, "would be to say the TDP-43 is building up, but the progranulin that is somehow responsible for clearing it out isn't doing so."
"With the (progranulin gene) mutation you're not as able to clear it out, therefore you get a bigger build-up of TDP-43 ... and then that person has FTD."
Imaging scans of FTD brains show significant and progressive degeneration of the affected areas. The disease ultimately leads to death within six to 10 years of onset.
Where Alzheimer's attacks deep areas of the brain – like the memory-storing hippocampus region – FTD targets the frontal lobes, which store key parts of personality.
Indeed, FTD can shift a victim's personality 180 degrees, Chow says, producing a complete stranger behind a dear and familiar face.
"So a person ... who seems like a cold, selfish person may actually become warmer and more sappy, " she says.
"Of course, the cases that make it into the news are the people who have the tragic story of the warm humanitarian who is now really horrible to be around."
Twice a week, Peter participates in Baycrest's unique day program for FTD patients.
Begun last year, the program gives patients structured and therapeutic programming while allowing family members a welcome break from their caregiver roles.
It is believed to be the first in Ontario that specifically caters to the needs of FTD patients and their families.
Often disruptive and nearly always younger than the other dementia or stroke patients who typically attend such programs, FTD sufferers can be downright scary in these settings, Chow says. They are usually stricken in their 50s, when careers and family life are still in full swing.
"These people are young, robust, and even if they don't mean to, are able to wreak quite a lot of havoc in a daycare."
But the Baycrest experiment has successfully used extra nurses, social workers and an increased care staff to integrate the FTD clients into a more elderly and sedate population.
Peter, whose family requested that his real name not be used, was by all accounts a gentle and highly respected swim coach whose career, until a few years ago, was marked by his insistence that all his charges be treated as winners.
Today, he's often a glint-eyed lech.
"I like to do the breast stroke," he says as he grabs for a nurses bosom. The nurse calmly but firmly tuts him away, and leads him to a group exercise session.
Others in the program exhibit drastically different personality alterations.
Luna, whose family also requested her real name not be used, was once elegant and weight-obsessed, but has gained 40 pounds since the disease took hold a few years ago. As well, she's convinced herself she's fluent in both French and Hebrew, though neither language is remotely in her verbal repertoire.
"She was a beautiful woman, quite young, around 55, and she always looked after herself," says Anna Grinberg, the nurse in charge of the centre's daycare program.
"Now she's excessively eating, singing, using inappropriate language. She'll ask you 100 times about speaking these other languages, and she can't really comprehend any of it."
Coquettish, blonde and dressed neatly by her octogenarian mother, Luna sits happily spouting her singsong gibberish throughout the day.
It's hard to gauge how many people suffer from FTD. Chow says that according to the best estimates, FTD accounts for about 16 per cent of all people who succumb to dementia.
This would put the number of people with FTD at about 15,000 across Canada, she adds.
Because parts of FTD pathology have only recently been explained, there are no brain-altering drugs in the pipeline that might one day change the course of the disease.
Unlike Alzheimer's, for which many experts predict there will be effective neurological treatments in the next three to seven years, FTD won't likely have any drug therapies for at least 10 years, Chow says.
Currently, treatments for FTD patients consist largely of anti-anxiety medications and a careful manipulation of their physical environments to make them safer and more comfortable.
Bruce Schaef gave up a splendidly gardened home in Riverdale to provide such an environment for his wife.
Now living in an elegantly refurbished apartment in a large, west-end retirement home, the couple exist in a melancholy postscript to their fairy-tale marriage.
An elementary school principal in Toronto, Diane Schaef was just finishing off her career and planning to travel in 2002 when her mind started to slip.
"Diane was a very quiet and reserved person, but she had a wicked sense of humour," her husband recalls, sitting on his living room floor beside his wife's favourite chair.
"She was measured about things, and we were a great complement to each other. We were joined at the hip, we always have been," he says.
Solid, intelligent – a woman who kept a copy of New York Times Sunday Book Review section in her purse – Schaef had an eye for organized detail that astonished her husband.
So when "Di" began to exhibit apathy, stopped talking and needed long periods of rest, the couple knew something was very wrong.
First diagnosed as depression, Diane's FTD was detected through MRI scans that noted shrinkage in her frontal lobes.
And since then, she has gradually become less and less "there" says her husband.
"To lose someone slowly, insidiously, gradually is tough."
On one level, however, he hasn't accepted the ailment's insidious sentence.
"We still have a great life, and we really, really focus on that," he says. "We have an incredible love."
"This is still Diane to me, in all aspects of our relationship. I just adore her."
And as a testament to that enduring love, he leans back on an old endearment: "Hey, you old bag of bones."
And as a reward, almost miraculously, she looks back with a beaming smile.
Torstar Syndication Services
Reprinted from Toronto Star, in the "News" section.
Copyright © 2007 Torstar Syndication Services. Displayed by permission. All rights reserved. Toronto Star logo is a registered trademark of Torstar Syndication Services. The iCopyright logo is a registered trademark of iCopyright, Inc.
A disease that steals the self
By Joseph Hall
Health Reporter
As always, Diane Schaef is impeccably turned out.
Her blue-grey cashmere sweater is accented by a string of pearls, which circle a neck that would surely have been described as regal in earlier times. Her greying hair is stylishly short and beauty-salon neat. Her skin – a genetic gift from her Aunt Doey, they say – is creamy and, at 58, almost flawless.
But it's her eyes that stop you. Black, black eyes. Eyes once incandescent with intelligence, authority and sometimes mirth – eyes now flat and eerily still.
Those disturbing eyes tell her story.
It's a story of descent from happy confidence to crippling self-doubt, of profound personality change, of people stricken in the prime of life, and of a little-known brain disorder called frontotemporal dementia.
"Isn't she beautiful," says her husband, Bruce Schaef, 65, a baritone with the Canadian Opera Company and the artist behind his wife's daily glamour.
"I do it to honour her ways ..." Then tears well up and he turns his gaze away.
Diane Schaef is in an advanced stage of frontotemporal dementia, commonly known as FTD. It's a degenerative neurological ailment that attacks the brain's frontal and temporal lobes, where personality traits and language control largely reside.
It's a disease, says Dr. Tiffany Chow, that attacks men and women equally and can replace a loved-one with a doppelganger stranger. Unlike Alzheimer's disease – which steals memory yet can leave personality intact for lengthy periods – FTD is a body snatcher.
"So you no longer have a partner helping you run a household and parent children," says Chow, a clinician and scientist with the Baycrest Centre's Rotman Research Institute in Toronto. "You've got another person who is an unknown quantity."
FTD is not a new disease. But only recently has its brain pathology been identified and studied, and have physicians begun to diagnose it consistently.
Previously blamed on everything from bipolar affective disorder to depression to a bad mid-life crisis, FTD was often well entrenched before it was actually identified in patients.
"So people didn't come to attention for the disorder appropriately oftentimes until four to six years of having the symptoms," says Chow.
She stresses that FTD is "a totally different animal" from Alzheimer's.
The telltale plaque deposits and tangled fibres that mar the brains of Alzheimer's sufferers are absent in FTD patients.
And while its cause is still unclear, one theory gaining wide acceptance is that many cases of the ailment involve a genetic shortage of the healing protein progranulin. "It's a protein that's actually involved in tissue healing and some of the immunology related to making sure that the body starts to repair itself," Chow says.
A gene mutation that limits progranulin production may allow neurological damage to go unrepaired over time, she adds.
And in many cases, a lack of progranulin may allow build-up of a misshapen protein known as TDP-43 in parts of the brain.
"One way to put those things together," says Chow, "would be to say the TDP-43 is building up, but the progranulin that is somehow responsible for clearing it out isn't doing so."
"With the (progranulin gene) mutation you're not as able to clear it out, therefore you get a bigger build-up of TDP-43 ... and then that person has FTD."
Imaging scans of FTD brains show significant and progressive degeneration of the affected areas. The disease ultimately leads to death within six to 10 years of onset.
Where Alzheimer's attacks deep areas of the brain – like the memory-storing hippocampus region – FTD targets the frontal lobes, which store key parts of personality.
Indeed, FTD can shift a victim's personality 180 degrees, Chow says, producing a complete stranger behind a dear and familiar face.
"So a person ... who seems like a cold, selfish person may actually become warmer and more sappy, " she says.
"Of course, the cases that make it into the news are the people who have the tragic story of the warm humanitarian who is now really horrible to be around."
Twice a week, Peter participates in Baycrest's unique day program for FTD patients.
Begun last year, the program gives patients structured and therapeutic programming while allowing family members a welcome break from their caregiver roles.
It is believed to be the first in Ontario that specifically caters to the needs of FTD patients and their families.
Often disruptive and nearly always younger than the other dementia or stroke patients who typically attend such programs, FTD sufferers can be downright scary in these settings, Chow says. They are usually stricken in their 50s, when careers and family life are still in full swing.
"These people are young, robust, and even if they don't mean to, are able to wreak quite a lot of havoc in a daycare."
But the Baycrest experiment has successfully used extra nurses, social workers and an increased care staff to integrate the FTD clients into a more elderly and sedate population.
Peter, whose family requested that his real name not be used, was by all accounts a gentle and highly respected swim coach whose career, until a few years ago, was marked by his insistence that all his charges be treated as winners.
Today, he's often a glint-eyed lech.
"I like to do the breast stroke," he says as he grabs for a nurses bosom. The nurse calmly but firmly tuts him away, and leads him to a group exercise session.
Others in the program exhibit drastically different personality alterations.
Luna, whose family also requested her real name not be used, was once elegant and weight-obsessed, but has gained 40 pounds since the disease took hold a few years ago. As well, she's convinced herself she's fluent in both French and Hebrew, though neither language is remotely in her verbal repertoire.
"She was a beautiful woman, quite young, around 55, and she always looked after herself," says Anna Grinberg, the nurse in charge of the centre's daycare program.
"Now she's excessively eating, singing, using inappropriate language. She'll ask you 100 times about speaking these other languages, and she can't really comprehend any of it."
Coquettish, blonde and dressed neatly by her octogenarian mother, Luna sits happily spouting her singsong gibberish throughout the day.
It's hard to gauge how many people suffer from FTD. Chow says that according to the best estimates, FTD accounts for about 16 per cent of all people who succumb to dementia.
This would put the number of people with FTD at about 15,000 across Canada, she adds.
Because parts of FTD pathology have only recently been explained, there are no brain-altering drugs in the pipeline that might one day change the course of the disease.
Unlike Alzheimer's, for which many experts predict there will be effective neurological treatments in the next three to seven years, FTD won't likely have any drug therapies for at least 10 years, Chow says.
Currently, treatments for FTD patients consist largely of anti-anxiety medications and a careful manipulation of their physical environments to make them safer and more comfortable.
Bruce Schaef gave up a splendidly gardened home in Riverdale to provide such an environment for his wife.
Now living in an elegantly refurbished apartment in a large, west-end retirement home, the couple exist in a melancholy postscript to their fairy-tale marriage.
An elementary school principal in Toronto, Diane Schaef was just finishing off her career and planning to travel in 2002 when her mind started to slip.
"Diane was a very quiet and reserved person, but she had a wicked sense of humour," her husband recalls, sitting on his living room floor beside his wife's favourite chair.
"She was measured about things, and we were a great complement to each other. We were joined at the hip, we always have been," he says.
Solid, intelligent – a woman who kept a copy of New York Times Sunday Book Review section in her purse – Schaef had an eye for organized detail that astonished her husband.
So when "Di" began to exhibit apathy, stopped talking and needed long periods of rest, the couple knew something was very wrong.
First diagnosed as depression, Diane's FTD was detected through MRI scans that noted shrinkage in her frontal lobes.
And since then, she has gradually become less and less "there" says her husband.
"To lose someone slowly, insidiously, gradually is tough."
On one level, however, he hasn't accepted the ailment's insidious sentence.
"We still have a great life, and we really, really focus on that," he says. "We have an incredible love."
"This is still Diane to me, in all aspects of our relationship. I just adore her."
And as a testament to that enduring love, he leans back on an old endearment: "Hey, you old bag of bones."
And as a reward, almost miraculously, she looks back with a beaming smile.
Torstar Syndication Services
Reprinted from Toronto Star, in the "News" section.
Copyright © 2007 Torstar Syndication Services. Displayed by permission. All rights reserved. Toronto Star logo is a registered trademark of Torstar Syndication Services. The iCopyright logo is a registered trademark of iCopyright, Inc.