Looking back, Amanda Wootten can clearly identify the first signs that her 36-year-old mother, Susan, was succumbing to the same disease that had killed her great-grandfather, her grandmother, her maternal great-aunts and -uncles, and two aunts: early onset Alzheimer’s.
"My mother’s vocabulary was never really wide, but she began having trouble when it came to identifying objects and other things," recalls Amanda, who was 16 when her mother was diagnosed in 1996. "She spoke as a child would. Instead of saying ‘I have a pain in my stomach’ she’d say ‘I have a tummy ache.’"
Alzheimer’s specialist Dr. Sandra Black, of Sunnybrook Health Sciences Centre in Toronto, notes that the disease first attacks areas in the brain that affect language, memory, and attention span. Sufferers routinely grope for familiar words, repeat themselves, can’t recall recent events, and are easily distracted.
Two years after the diagnosis and those first signs, Susan’s emotional controls slackened, too. "When my mother got excited, she got really excited," says Amanda, now 27, and began using broad, exaggerated gestures to colour her conversation. "When trying to remember the word volcano, she’d say, ‘Volc… volc… it explodes!!" throwing both arms up."
Within two more years, Susan’s child-like tendencies extended to her behaviour. She lost the ability to judge when it was appropriate to speak. "You don’t start talking to someone as they’re walking out the door," says Amanda, "but children do. Like them, my mother spoke when she had the urge."
At that point, in 2000, Susan was hospitalized. "When my mother was hospitalized, I had to dress her and brush her hair," recalls Amanda. "She didn’t care about her appearance. I had to remind her to bathe."
"The disease robs people of the ability to plan, think, be rational and organize themselves," says Dr. Black. Goal-directed planning becomes difficult, she adds. It is at this point in the progression that round the clock supervision becomes necessary, and home caregivers realize they are out of their depth.
Compared to the more common form of the disease, which strikes those in their 70s, the advancing symptoms of early onset Alzheimer’s can travel at a galloping pace. Within three to five years, if not sooner, says Dr. Black, those with early onset often become totally dependent on others for the most basic of needs, like going to the bathroom. Amanda’s mother took a drug that successfully slowed her symptoms for a couple of years. But shortly before she died in 2006 at age 46, virtually all motor function ceased. Susan could neither stand, speak nor swallow.
In that last stage, when sufferers have trouble clearing their throats, swallowing or otherwise protecting the airway, pneumonia often occurs, says Dr. Black. And pneumonia commonly leads to death.
One thing Susan never lost, even at the end, was her sunny disposition, says Amanda. "Her facial expression never changed – she was an easygoing person who always had a smile. But the glimmer in her eye faded away."
New research is focusing on reducing buildup of abnormal amyloid proteins that are a hallmark of Alzheimer’s. "Researchers are also working to develop antibodies against the amyloid protein, or vaccines and drugs that slow down amyloid production," says Dr. Black. "One study in Ontario is testing old-fashioned antibiotics thought to alter the tendency to overproduce the protein. Researchers are working in whole new ways to slow the disease down, not just reduce symptoms."
After two years of agonizing reflection, Amanda decided she needed to know if she carries the gene that condemns her to early onset Alzheimer’s. The test, which was positive, changed her life: she elected to be sterilized (she already had one child), has become an Alzheimer’s activist, and is completing an intensive Person Support Worker course. When she graduates in spring, "I want to work in people’s homes, giving care to people with medical needs. I won’t go looking for people with Alzheimer’s, but I’d prefer it."
To find out how a New Brunswick family was key in unlocking the mystery of early onset Alzheimer’s, read "A Ticking Time Bomb" by Robert Kiener in the November issue of Reader’s Digest.