Laurie Scott stands at the portable keyboard in her living room, singing. Her support worker, Laurie McAndrew, is behind her, and stretches her arms around her to pick out a tune on the keys.
“You are my sunshine, my only sunshine,” the two sing, swaying together in an embrace.
This used to be Ms. Scott's favourite song, one she loved to belt out.
But she doesn't sing much any more, and even today, one of her good days, she can't summon many of the words. She warbles a few lines in a rough, weak voice, then wanders away.
Ms. Scott, 55, was born with Down syndrome but is slowly dying from Alzheimer's disease, a progressive, degenerative illness that is destroying her brain. The two conditions are tragically linked; most people with Down syndrome are afflicted with Alzheimer's in middle age, and their final years are marked by a profound intellectual, emotional and physical decline.
Ms. Scott used to love travelling, singing and all things Scottish, including the Highland fling. Now she hates leaving home and spends her days sleeping and wandering in circles.
The culprit appears to be chromosome 21. Most people with Down syndrome have an extra copy, and it carries the instructions for a protein believed to play an important role in Alzheimer's. That extra chromosome shaped their brains and their bodies in the womb, and now it is shaping their final years, robbing them of the skills they worked so hard to learn, along with their memories and their personalities.
“People with Down syndrome are living much longer than they ever used to, and one of the consequences as they get older is this very high risk of Alzheimer's disease. It is really very tragic,” says Nicole Schupf, a researcher at Columbia University in New York.
She is part of a team in the United States that studies dementia in people with Down syndrome. Their goal is to understand why some people get it so early, in their 40s, while others aren't afflicted until their 60s.
While their work is focused on a unique group, Dr. Schupf believes their findings may lead to a better understanding of the pathology of Alzheimer's and the complex interplay between the environment and genes that triggers the disease.
They hope, one day, to find a way to slow the onset.
A common disability
Down syndrome is a relatively common developmental disability that affects roughly one in 900 babies born in Canada, and Alzheimer's hits at a younger age and moves much faster in people who have it.
In 1920, a baby with Down syndrome was expected to live only nine years. Improved medical care – including cardiac surgery and better antibiotics – means individuals with the syndrome are living much longer, to 55 or more.
But studies show that by 40 their brains are becoming clogged by the plaques and tangles characteristic of Alzheimer's. By 65, three-quarters of them have dementia, Dr. Schupf says. That compares with roughly one in 10 people over 65 in the general population.
Living with someone descending into dementia can be both heartbreaking and backbreaking. In the case of people with Down syndrome, their parents are often elderly themselves, and find they can no longer look after adult children who now need help dressing, bathing and eating, and who sometimes no longer recognize them.
Group homes, where many people with Down syndrome live, are working to accommodate their changing needs.
Ms. Scott lives in a group home on Dissing Crescent in London, Ont., perhaps the first in the country built specifically for people with Down syndrome and dementia. She was one of the first to move in seven years ago, shortly after it opened.
Her generation is the first to have lived so long, but lost so much.
