One positive result of the advances in modern medicine is the vastly increased lifespan of people with Down syndrome. Yet with the lifespan increase has come a new worry: People with Down syndrome appear to have an elevated risk of developing Alzheimer’s and often will at a much earlier age than people in the general population.
Down syndrome is a genetic disorder that results from an extra gene copy found in chromosome 21. Chromosomes, as you may recall from science class, are those bundles of DNA, proteins and RNA found in cells controlling how we are built. Down syndrome is the most common genetic disorder, affecting about 1 in 1,000 births (though the prevalence rises with maternal age – 55 in 1,000 for mothers aged 45 to 49).
The common effects of Down syndrome have been known for over a hundred years – weak immune system, small stature, developmental delays, impairments in cognitive functioning, distinctive physical characteristics and increased risk for numerous other health problems. Now we know one of those increased risks includes Alzheimer’s.
For people with Down syndrome, the Alzheimer’s-related symptoms of frontal-lobe dementia and cognitive decline can start as early as age 30. (Typical “late onset” Alzheimer’s, which includes about 95 percent of patients, starts after age 60.)
One of the complications for people with Down syndrome is they often already have mild or moderate impairments in intellectual functioning (an IQ of 70 or below), which means the usual signs of cognitive decline can be difficult for doctors and even primary caregivers to see. Plus, people with Down syndrome frequently have decline in verbal skills and long-term memory after the age of 50 without dementia.
Yet, there are certain symptoms that are signs of possible dementia in people with Down syndrome: loss of previous skills, geographical disorientation, confusion, low mood, aggression, change in sleep patterns, fearfulness, changes in personality, urinary incontinence, hallucinations, loss of speech or change in ability to speak or walk.
Onset of one or more of these symptoms doesn’t necessarily equate to the onset of Alzheimer’s but should lead to consultation with a medical specialist who can rule out other causes of the symptoms.
With the increased prevalence of Alzheimer’s and other dementias in people with Down syndrome, more research is needed into the effects of dementia treatments. People with Down syndrome often have a different response to medications than the people who participate in the studies.
As with most dementia patients, efforts are put into treating and mitigating the behavioral symptoms, focusing on making life easier for the person and his or her caregivers. The good news is people with Down syndrome have access to developmental-disability services other folks with dementia struggle to find – including respite, behavioral supports and even community residential care. And as more people with Down syndrome and other developmental disabilities live longer, healthier lives, the more we will have learned about Alzheimer’s and its treatment.
Tara Kiene is the director of case management with Community Connections Inc.