A change in behavior is often noted in people with Down syndrome (DS) who have Alzheimer’s disease (AD). For some individuals, this is a natural part of the progression of AD. However, for others, the change may have other reversible causes, and careful assessment is important. An evaluation for medical problems (including pain), environmental factors, mental health changes, and other issues may find a cause that is not directly related to AD. Because of the person’s reduced ability to understand or inform others of their discomfort or ill-feeling due to these factors, the person may be using behavioral changes to communicate the discomfort.
Some individuals may display behavior that is more withdrawn or passive. Others may seem to be upset, voice displeasure, or even display behavior that is aggressive. Particularly in people with DS, some may display increased compulsive behavior, repetitively doing an action or behavior and not being able to participate in activities that they would typically enjoy.
Assessing a change in behavior
If the person is displaying a change in behavior, the first step is to assess what problems the behaviors are causing. Is the behavior disturbing to the individual, is it causing challenging situations for those around the individual, or is there a safety concern? For example, if a person develops a compulsive need to go into their room at 3 PM sharp to watch a certain program, it is unlikely it would be a behavior that would need to be addressed or changed. However, if the person is getting aggressive with people they live with, that will need some form of intervention for the person’s safety and the safety of the people they live with.
The second step is to assess for possible contributing causes. These include caregiver, environmental, physical, and mental health triggers.
Caregiver triggers
Caregivers, even with the best intentions, can be a trigger to a behavioral change. For example, reasoning with the person who has a declining ability to reason often results in little success in helping the person understand and can result in a change in behavior. Short of an immediate safety issue (the person is doing or about to do something that is dangerous), in most situations, backing off, waiting a few minutes, and trying again is more likely to be successful and contribute to fewer behavioral changes.
Environmental triggers
Environmental factors, like too much noise, cold or hot temperatures, too much or too little activity, bad smells, too much or too little light, and other factors can be stressful and result in a change in behavior. Also, going to a new environment can be challenging. For example, are we expecting that an individual can successfully participate in a gathering at a new setting in the presence of many relatives the individual has not seen for years? These events and even events or settings that are much less hectic can be challenging for the individual.
For individuals who do not live with family, sometimes even going back to the family home is too overwhelming. Their present home/residence is now familiar, and the family home - no matter how long the person lived there - may no longer be “familiar” due to memory loss. Visiting the family home may become too stressful. The family may need to visit the person in his or her home rather than bring the person to the family home. Leaving his or her environment, going into the community, or visiting with less familiar people, especially in larger groups, may be frightening or upsetting to the individual and/or may contribute to a change in behavior.
There are ways to use the environment in positive ways. Highlighting and using things in the environment that the individual did not find stressful prior to developing AD can be comforting and beneficial. For example, posters of favorite singers, a favorite pillowcase, or a preferred snack may all be comforting. Adding things that make the environment more manageable can also be helpful. For example, putting the person’s picture on their bedroom door, putting a picture of a toilet on the bathroom door, and similar interventions can help the individual feel less confused and reduce behavioral changes.
Wandering can sometimes be a problem that requires environmental changes to prevent the person from wandering unsafely. If the person is just wandering around the house, no intervention may be needed, but if they are wandering outside, some means of preventing that will be important for safety. Good resources are available from Down’s Syndrome Scotland (Living with Dementia) and the National Down Syndrome Society (Alzheimer’s Disease and Down Syndrome).
Physical triggers
Pain is a common contributor to a change in behavior. Also, a variety of medical conditions can contribute to behavioral changes. A few examples include infections (e.g., a bladder infection or pneumonia), metabolic conditions (e.g., diabetes, thyroid conditions), dehydration, and/or inadequate sleep. Something as simple (and as common in people with DS) as ear wax blocking the ear canal causing reduced hearing and/or pain can have a significant impact on behavior.
Mental health triggers
Alzheimer’s disease is often accompanied by mental health changes in people with DS. Depression, anxiety, obsessive-compulsive disorder, and others can lead to behavioral changes. Often these can be treated with non-medicinal approaches. However, if these approaches do not adequately address the problems, additional therapies can be considered. Further information follows.
Treatment
Review of the “need to treat/address” criteria outlined above is important as treatment is considered. Once it is clear intervention is needed, the next step is to recognize that the treatment may require multiple approaches. Addressing caregiver and environmental triggers as well as using techniques to make the environment more “friendly” to the individual should be reviewed and used appropriately for all individuals. This is true even when other underlying physical and mental health conditions are found.
In those with contributing physical health issues including pain, these factors need to be addressed to optimize the person’s comfort as well as reduce the impact of their behavior on those around them.
For mental health conditions and symptoms, a similar approach is recommended. The primary purpose is to reduce the person’s suffering and to optimize their enjoyment and participation in life activities. Various therapies may be considered. For example, pet therapy, music therapy, or art therapy may be helpful. Sometimes it is also necessary to consider medication. If medication is being considered, review of the mental illness diagnosis is key as well as choosing medications that will optimize the person’s comfort and safety, reduce the negative impact of the behaviors on others, and minimize potential side effects.
There are a variety of medications available to treat conditions that are common in people with DS and AD including depression, anxiety, and obsessive-compulsive disorder. For more information on these conditions, please see the Mental Health section of our Resource Library.
In addition, AD may be associated with hallucinatory behavior, paranoia, or psychoses. Often this is not really a problem for the individual or those around the individual and no treatment is needed. However, if this is disturbing to the person or is a safety issue, medications can be considered. Atypical anti-psychotics are sometimes prescribed. However, these medications have a “black box warning” from the Food and Drug Administration (FDA) and are not approved for dementia-associated psychoses. These medications are associated with increased mortality mostly related to cardiovascular or infectious events when used in people with dementia. If they are considered, a careful discussion about the risks and possible benefits should be undertaken. More common side effects include unsteadiness, sedation, confusion, and incontinence. If used, starting with small doses may reduce side effects. A newer atypical anti-psychotic medication, brexpiprazole (Rexulti), has an FDA-approved indication for “dementia-associated agitation in patients with Alzheimer’s disease” but still has a black box warning against using it for dementia-related psychosis (as noted above).
If the change in behavior is displayed with the person getting quite upset or even aggressive, a seizure (anti-epileptic) medication may also be helpful. Since many people with DS who have AD develop seizures, these medications may treat both seizures and these behavioral changes. We have found valproic acid (Depakote), carbamazepine (Tegretol) or lamotrigine (Lamictal) to be effective in some individuals. Levetiracetam (Keppra) works well for seizures in many individuals with DS and AD. Unfortunately, an occasional side effect is that the individual displays behavioral changes of being upset, with fluctuating mood, or even aggression. If these behavior changes occur while on levetiracetam, we consider changing to a different anti-epileptic medication.
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, from the Alzheimer's Association.
More information on AD in people with DS can be found in the Alzheimer's Disease and Dementia section of our Resource Library and in our Mental Wellness book.