A cruel, dual blow for special patients
Down syndrome and Alzheimer's go hand in hand. As lifespans rise, so do problems.
The Atlanta Journal-Constitution
Published on: 03/11/07
It's a good day for Jesse Zanca.
He sits silently on the couch in his mother's living room, staring straight ahead through big glasses. When addressed directly, he grins shyly and gives brief answers to questions. He likes his group home. It's in Stone Mountain. He likes to eat hamburgers.
Jesse, 37, was once garrulous and sociable, said his mother, Jane. He worked in the kitchen of a nursing home and was engaged to be married.
"Jesse, in his mid-20s, had really come a long way," Jane Zanca said.
Then, at about 26, "some things started to slip," she said.
Jesse lost interest in social activities. He wouldn't talk on the phone. He would start making a snack, abandon it, then start another and another. He couldn't sleep.
Like most people with Down syndrome who live long enough, Jesse was developing Alzheimer's disease.
Scientists have long known of a connection between the two conditions without knowing the specific reasons, said Arthur Dalton, director of the Center for Aging Studies at the New York State Institute for Basic Search in Developmental Disabilities.
Until recently, people with Down syndrome usually died young, before exhibiting the symptoms of dementia. In 1983, the life expectancy of someone with Down syndrome was just 25, according to the National Down Syndrome Society.
Now, better overall health and advanced medical care have more than doubled that life expectancy, keeping many people with Down syndrome alive into their 50s, 60s and even longer. As they grow older, more of the 350,000 Americans with Down syndrome are developing Alzheimer's — and at a younger age and faster acceleration than the general population.
The Down-Alzheimer's combination presents their families with a daunting challenge.
"You go to elder care people, and they don't do developmental disabilities," said Jane Zanca. "You go to developmental disabilities people, and they don't do elder care."
Genetic link established
In their exploration of human genetics over the last few years, researchers believe they have identified the link between Down syndrome, a disorder that is the most common genetic cause of mental retardation, and Alzheimer's, a progressive brain disease that destroys a person's memory and mental ability.
Down syndrome occurs when a person's DNA has an extra copy of a particular chromosome known as 21. The abnormality causes the body to produce an excessive amount of a substance that accumulates as plaque in the brains of people with Alzheimer's.
By the time people with Down syndrome are in their 40s, almost all their brains will exhibit some of the changes associated with Alzheimer's, said neurologist Jim Lah of Emory University School of Medicine. That's about 20 years earlier than in the general population.
About 85 percent of autopsies of adults with Down syndrome show the plaque associated with Alzheimer's, said Dalton of the New York research institute.
But people with Down syndrome "end up developing Alzheimer's with virtually 100 percent certainty if they live long enough," said Emory's Lah. "If all Down syndrome victims lived to be age 80, I think you could say with confidence that all of them would develop outward signs of dementia."
Alzheimer's often seems to strike when someone with Down syndrome is operating at peak performance.
Louise Maier got her first job at age 50 in the bakery of a grocery store. She did so well putting bread into bags that she was promoted to pizza-making. After the store closed, she went to work in a restaurant, folding napkins and rolling up silverware.
She never missed a day, said her sister, Betty Didicher, 65.
Then, a little over a year ago, Maier started crying frequently at work. One day she wandered out into the parking lot and co-workers didn't know where she was.
She began frequently calling her sister from Just People, the Roswell group home where she lives, hysterical that someone might try to hurt her.
Now 59, she is on medication to control anxiety. The crying and wandering have stopped, but Maier is no longer able to work, and she is reluctant to participate in activities at Just People.
Becky Dowling, director of Just People, researches resources for Maier as she seeks assistance for her own mother, who has Alzheimer's.
Some days Maier seems lucid and engaged, Dowling said. Other times, she doesn't seem to know where she is.
"Our program is not designed for Alzheimer's," Dowling said. "It's not designed for people with Downs and Alzheimer's."
Eventually, her sister fears, Maier will have to go into a nursing home at a cost of nearly $5,000 a month — if her family can find one that will accept her.
Despite the challenges, Didicher sees a bit of good fortune.
"I think we were very blessed that she was 57 before it kicked in," she said.
Early symptoms
The first symptoms of Alzheimer's in developmentally disabled people are often behavioral, such as increased stubbornness, said Janice Nodvin, executive director of the Atlanta-based Institute for the Study of Disadvantage and Disability.
"They often stop talking, exhibit extreme anxiety and stop their daily functions," she said.
The extent of dementia can be hard to assess in people with developmental disorders, she said. Because retardation in people with Down syndrome can vary from mild to profound, health care professionals need to do "baseline" mental examinations of people in their 30s, she said.
When an older adult with Down syndrome is developing dementia, an accident or illness can seem to accelerate the symptoms, said Adam Pomeranz, executive director of Annandale Village, a not-for-profit community in Suwanee for adults with disabilities.
The fear of undergoing treatment and the confusion of being in different surroundings can cause erratic behavior, he said. "It's very scary for them."
Amy Somers, 44, an Annandale resident, was showing signs of Alzheimer's when she broke her leg and had to have surgery. Afterward, she went into the nursing home on the Annandale grounds for rehabilitation.
"That's where the symptoms of Alzheimer's seemed to become worse and worse," said Pomeranz. Somers went to Ridgeview Institute in Cobb County to be stabilized, and is now back at Annandale with 24-hour supervision.
"She really just wants to sit and do nothing," said her sister, Lisa Jackson, 41. "She used to be involved in everything. Now it's unbelievable the care she needs. She's withdrawn. She sleeps a lot. She needs someone to help her with her hygiene. There are toileting issues. It's too hard for me to take her anywhere."
Jackson said she is grateful that her sister moved to Annandale four years ago when their mother died and was there when she started to decline.
"The people at Annandale have been wonderful," she said.
But Pomeranz admits, "Amy's been a huge challenge for us, and it's really sad."
Struggle for family members
Jane Zanca, Jesse's mother, has tried the range of support systems.
She gave up her job as a writer for nine months to take care of him, paying personal caregivers to help. Then, after looking at 19 different nursing homes, she found one that agreed to take her son. She visited daily to help him bathe and shave.
Three years ago he went into a Stone Mountain home run by Jewish Family and Career Services of Atlanta.
Jane Zanca now works as a writer with the federal Centers for Disease Control and Prevention. At 60, she has severe arthritis.
"I have to limit my time with Jesse to what I know I can handle," she said.
Her son comes home most weekends, and enjoys visits from his sister and 4-year-old nephew.
"They're pretty much on the same level," his mother said.
Zanca said she knows many parents and siblings of aging disabled adults are struggling as she is.
"We're not ready in this country for the elders who are coming," she said. "We're certainly not ready for people in Jesse's situation."
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