Impact Feature Issue on Aging and People with Intellectual and Developmental Disabilities

There are an estimated 5.3 million people in the U.S. with Alzheimer's disease. By the year 2030, that number is expected to increase to 7.7 million. Alzheimer's disease is the most common form of dementia, a group of symptoms characterized by loss in memory and at least one other cognitive skill. Sixty to 80% of people with dementia have Alzheimer's disease (Alzheimer's Association, 2009). The most significant risk factor for Alzheimer's is age. For most people the disease begins with difficulty remembering new or current information, and other cognitive abilities such as the ability to plan, use language, and understand time and spatial relations deteriorate as the disease progresses.

Alzheimer's Disease and Intellectual/Developmental Disabilities

People with intellectual and developmental disabilities develop Alzheimer's disease at rates similar to older adults in the general population. However, adults with Down syndrome develop Alzheimer's disease at greater rates. Recent studies for adults with Down syndrome show that 10-25% of individuals between ages 40-49, 20-50% of individuals between ages 50-59, and 60-75% of individuals with Down syndrome older than age 60 have Alzheimer's disease (Alvarez, 2008). Because people with Down syndrome experience accelerated aging, they are at greater risk for this disease of the aging brain. Additionally, the amyloid precursor protein gene sits on chromosome 21; most people with Down syndrome have two copies of this chromosome in every cell and therefore double the risk of having one of the genes implicated in the brain degeneration associated with Alzheimer's. Clinical studies have also noted that the volume of the hippocampus, the area of the brain responsible for beginning the process of memory storage, is reduced in people with Down syndrome (Piner, et al., 2001).

Signs of Alzheimer's Disease

For years, the Alzheimer's Association has provided the public with a list of the "10 Warning Signs" of Alzheimer's disease. These include memory changes significant enough to impact daily life, challenges in planning or problem solving, difficulty completing familiar tasks and confusion with time or place. (For a complete list of the warning signs seewww.alz.org ). The warning signs for people with intellectual and developmental disabilities focus on functional capabilities (see Figure 1). Most often, the first change that family members or professionals notice is a change in activity of daily living skills. For example, if an individual has worked independently in a restaurant for many years she may begin to lose attention for the task, make many errors or become frustrated with the task. A man who has enjoyed going out with others in his group home may seem reluctant to do this and want to stay home. Families and staff will most often notice that the individual loses the ability to perform skills he or she has been able to do for many years.

Warning Signs of Alzheimer's Disease for Adults with Intellectual and Developmental Disabilities 

• Loss of activity of daily living skills, difficulty with well-learned abilities
• Changes in personality; more withdrawn, frustration
• Periods of inactivity or apathy, disinterest in activities the individual previously enjoyed
• Development of seizures not previously seen
• Disorientation to time and place
• Increase in stereotyped behaviors
• Hyperactive reflexes
• Visual retention deficits
• Speech difficulties, not able to use words or speech that is not clear

They may notice other changes as well. She may ask the same question repeatedly. An individual who has been outgoing may seem more withdrawn. She may seem less alert and move more slowly. He may cry easily and appear frustrated when completing tasks that have been part of his daily routine in the past. He may be confused and get lost in familiar places. These changes will not happen quickly, but will be noticeable when compared to previous abilities.

With individuals who have severe developmental disabilities it may be more difficult to identify changes in behavior. However, among those changes that may appear are becoming more socially withdrawn, being less responsive to others, seeming easily frustrated even in familiar surroundings, appearing much less interested in activities that engaged them in the past, and having shortened attention.

Having a clear and specific understanding of the individual's skills, abilities, and interests is important for comparing current behaviors to those from the past and noticing changes. A baseline evaluation of skills at work, abilities at home, and interests will help caregivers to notice possible signs of Alzheimer's disease, and share changes with medical professionals.

Diagnosing Alzheimer's Disease

There is no one test for the diagnosis of Alzheimer's disease. Physicians may suggest several tests as a way to rule out other issues. There are many reasons why an individual may experience difficulties with activities of daily living or show changes in his or her personality. If caregivers notice changes and losses in skills, it is important to seek a medical evaluation. A medical professional will want to rule out issues which can be corrected such as thyroid disorders, depression, physical health problems, and medication side effects. Clear documentation as to current abilities is important so that staff and medical professionals can compare current abilities to past.

Progression

Alzheimer's is a progressive disease, and while it does not progress at the same rate in every person, there are some general stages of the disease. For individuals with mild to moderate developmental disabilities the changes first noticed in the early stages of Alzheimer's disease include loss of activity of daily living skills, personality changes, and disorientation to time and place.

As the disease progresses the symptoms seen in the early stage increase. Individuals become increasingly confused and their frustration may increase. Many individuals will experience disruptions in their sleep schedule and become very confused about day and night. Some individuals may attempt to leave their residence and become lost.

Late in the disease process individuals are very disoriented to time and place and exhibit physical issues. They may have difficulty swallowing, be incontinent, and have difficulty walking. They will need total care for survival.

Medications for Treating Cognitive Symptoms

The FDA has approved four medications for treating the cognitive symptoms of Alzheimer's disease. The approved medications are three cholinesterase inhibitors -- donepezil (Aricept), rivastigmine (Exelon), and galantamine (Razadyne) -- and memantine (Namenda), a glutamate receptor antagonist. While these have been tested in older adults without intellectual or developmental disabilities, there have been no large randomized controlled studies in adults with intellectual or developmental disabilities. It is important for caregivers to seek advice from a medical professional about the use of these medications.

Care Principles

The University of Stirling (Scotland), the University at Albany, and the University of Illinois at Chicago called a group of international experts together in 2001 to define internationally applicable working practices for the community supports of adults with intellectual and developmental disabilities who have Alzheimer's disease. Known as "The Edinburgh Principles," these guidelines focus on quality of life and promote care management. The group proposed that "governments, organizations, and providers adopt these and promote their use in aiding those adults with intellectual disabilities affected by Alzheimer's disease and other similar conditions resulting in dementia"

The Principles are as follows:

1. Adopt an operational philosophy that promotes the utmost quality of life of persons with intellectual disabilities affected by dementia and, whenever possible, base services and support practices on a person-centered approach.
2. Affirm that individual strengths, capabilities, skills, and wishes should be the overriding consideration in any decision-making for and by persons with intellectual disabilities affected by dementia.
3. Involve the individual, her or his family, and other close supports in all phases of assessment and services planning and provision for the person with an intellectual disability affected with dementia.
4. Ensure that appropriate diagnostic, assessment and intervention services and resources are available to meet the individual needs, and support healthy ageing of persons with intellectual disabilities affected by dementia.
5. Plan and provide supports and services that optimize remaining in the chosen home and community of adults with intellectual disabilities affected by dementia.
6. Ensure that persons with intellectual disabilities affected by dementia have the same access to appropriate services and supports as afforded to other persons in the general population affected by dementia.
7. Ensure that generic, cooperative, and proactive strategic planning across relevant policy, provider and advocacy groups involves consideration of the current and future needs of adults with intellectual disabilities affected by dementia.

The Edinburgh Principles are the foundation of the caregiving suggestions in the following section.

Caregiving Suggestions

There are a number of things caregivers can do to support quality of life, as well as safety and well-being of individuals with intellectual or developmental disabilities who have Alzheimer's disease. Among them are the following:

• Caregivers can support adults with intellectual and developmental disabilities who have Alzheimer's disease by helping the person to feel safe and secure, emphasizing maintaining abilities rather than teaching new skills, and simplifying routines and reducing choices.
• Early in the disease process, make minimal changes to the home or work environment or schedule, maintain the routine and familiar, and continue regular activities with structure and added supervision. Respond to confusion and repeated questions with patience. Because of changes to the brain, he may not recognize familiar places or caregivers and will not know he has asked when lunch is multiple times. Simplify directions and offer several clues so that the individual knows exactly what you mean.
• As the disease progresses, it is important for caregivers to anticipate the individual's physical and emotional needs. To prevent bathroom accidents, use a scheduled toileting time. Continue to offer nutritious food and adequate fluids; she may not ask for either. Respond to the feelings behind the confusion. If she insists it is time to go home in the middle of the day, spend some time talking with her, look at familiar photographs or just sit together with a glass of juice. Create a calm environment, allow for rest, and simplify tasks.
• Modify work and home environments as the disease progresses. Attend to his physical needs and support with gentle comfort. Allow the person to "age in place" as he is no longer able to be active and needs longer rest times. Consider palliative/comfort care and hospice care to support his physical needs.

Conclusion

As people with intellectual and developmental disabilities continue to age more successfully and live longer, the numbers with Alzheimer's disease and other dementias will increase. To promote quality of life it is important that caregivers understand the early warning signs of dementia. Being aware of changes individuals experience will allow individuals and their caregivers to seek an evaluation by a medical professional to diagnose dementia or treat another medical condition. Having a diagnosis and understanding the progress of dementia will allow caregivers to adjust supports and services to meet the new and changing needs of the individual.