What You Need to Know About Frontotemporal Dementia in the Aftermath of Bruce Willis’s Diagnosis
The disclosure of the disease by Willis, seen here in 2019, has brought the little known brain disease into focus. Photo: Angela Weiss/AFP/Getty Images
When Leah Sandals got the news that the family of Die Hard actor Bruce Willis, 67, had chosen to go public with the actor’s diagnosis of frontotemporal dementia (FTD), she was “grateful and relieved.”
“It was so meaningful to me,” says the 48-year-old editor from Toronto who has has been dealing with FTD in her family since she was 17. The disclosure that Willis suffers from the condition has brought the little-known brain disease into focus.
Sandals first noticed it when her mother, in her late 40s, “started to get words wrong.” But because less was known about FTD 30 years ago, it wasn’t until after her mother died that an autopsy confirmed that she’d had the disease, which is characterized by atrophy of certain areas of the brain.
“I think [Willis going public] helps a lot to shine a spotlight on it,” says Dr. Joshua Armstrong, research scientist at the Alzheimer Society of Canada and lead author of the society’s Landmark Study about dementia in this country.
“There’s a lot of interest because he’s such a famous, well-loved actor. People are trying to better understand it, how it comes on, what are the risks,” says Armstrong.
Going public also lessens the stigma while encouraging people to pay more attention to symptoms and more willing to seek a diagnosis, suggests Armstrong. He notes that his conversation with Zoomer is the third media interview he’s done since the news about Willis was announced.
A Genetic Component
Two of Sandals’ aunts also developed FTD and genetic testing revealed that their disease was linked to a genetic variant.
The Landmark study estimates that 30,000 to 60,000 Canadians are living with FTD. FTD is typically an early onset form of dementia, most common among people aged 45 to 64, explains Dr. Freedman. The disease differs from Alzheimer’s in that memory loss is not typically associated with FTD at the beginning of the disease.
“About 40 per cent of people with FTD have a family history of the disease,” says Dr. Morris Freedman, head of neurology at Baycrest Geriatric Centre in Toronto. “There are three major genes we know about that cause it. People can be tested for this. But not all cases are associated with family history.”
Freedman describes FTD and primary progressive aphasia (PPA – a rare neurological syndrome that affects your ability to communicate) that led to Willis’s diagnosis as part of a spectrum of disorders under the umbrella term of frontotemporal lobar degeneration. About 20 per cent of people with FTD have aphasia as a symptom.
“With PPA, there are changes in the ability to use language and when the onset is with language, early on in the disease, that’s all there is. The person doesn’t have other kinds of problems. The difficulties in day-to-day function are all essentially related to language problems,” says Freedman.
Another group of individuals with FTD, characterized with behavioural problems, accounts for 80 per cent of people with the disease. “Patients with the behavioural variant tend to have personality changes. They may become disinterested or inappropriate,” he says.
He gives the example of someone at a restaurant eating spaghetti, noticing a person at the next table having steak and really wanting a steak. But instead of saying, “Next time I’ll order steak,” a person with the behavioural variant of FTD would go to the other person’s table and take the steak. “If a man with the behavioural variant sees someone who’s bald, he might say ‘Hey baldy,’ or he might grab a woman’s breast.”
Freedman cites a classic example of a woman who uses scissors borrowed from a neighbour to trim plants in the garden. She cuts herself badly, bleeding profusely. Her husband (who has FTD) is with her but instead of focusing on her injury, he tells her that he has to return the scissors to the neighbour before he can help her. “He doesn’t recognize that she has a medical emergency. That’s an extreme behavioural example. When it starts, the change in personality or behaviour is not so extreme.”
Even though there is no cure or effective treatment for FTD, Freedman suggests that it’s important to get testing and a diagnosis if someone is experiencing speech or language problems or behavioural or personality changes.
“People can forget words, that happens to everybody,” advises Freedman. “We all forget people’s names, and [difficulty] finding the right word for something [is common], especially as we get older. But if it’s happening more than should occur normally, more frequently and more severely, if someone is worried about it, they should see a doctor and get checked.”
It’s important to understand what’s happening, what the reason is for the changes and what to expect, he says. Although there is no specific treatment or drug that can reverse or slow the disease progression, there are speech and language interventions and medications that can help with behaviour.
As for preventative measures, Freedman suggests the gold standard for keeping the brain healthy is plenty of physical exercise and keeping mentally active. “Exercise is a way of helping to maximize brain function,” he says.
Leah Sandals probably knows more about FTD than most people who are not scientists. Her family’s experience with the disease led her to becoming an editor with the Alzheimer Society.
She also decided to be tested for the genetic variants associated with FTD. “Initially I didn’t want to find out the results,” she says. “Eventually I got to the point where I wanted to know and, if I was positive, to enroll in a drug trial. But I found out that I don’t have the known genes linked with FTD.”
She adds, “I hope this disclosure by Bruce Willis will bring more awareness among doctors, too.”
Sandals suggests these sites for more information about FTD and dementia:
alzheimer.ca/find — find and contact your local team/office to connect with free dementia help for any kind of dementia with cross-Canada support teams
raredementiasupport.ca — offers FTD and some other rarer dementia support, Canada-based at Nipissing University but with virtual support available
theaftd.org — specifically offers FTD info and support, US-based but with some international virtual programs