When an adult with Down syndrome is diagnosed with Alzheimer's disease, we
focus on "the four S's": Safety, Stability (of the environment), Social (emotional)
issues, and Symptoms. As the adult with Down syndrome experiences greater
decline, family or caregivers will often have difficulty maintaining a safe
environment. Falling because of gait apraxia, wandering from home, and touching
or using objects in the home that can be harmful if used inappropriately
(e.g., the stove) can all be problems that the family or caregiver cannot
prevent. In addition, when the adults become bed-bound, frequent turning
or changing the position to prevent bedsores is necessary and may be more
than the family can provide. Safety is frequently the concern that requires
a change in living arrangements and, if no specialty care programs are available,
may require admission to a nursing facility.
As the person declines, stability of the environment helps reduce confusion.
Persons with Down syndrome often have difficulty with change in environment
including changes in caregiver and location. This is no less true for persons
with Down syndrome who develop Alzheimer's disease. Negotiating a new environment
is more difficult and increases confusion and limits the remaining skills.
This frequently presents a dilemma because the present environment may no
longer be safe, but a new environment may increase confusion. We recommend
maintaining the individual in the present environment as long as it is safely
possible, making only minor changes as necessary and using canes, walkers,
handrails, or other devices to prolong the length of time that the adult
remains ambulatory and is able to remain in his or her present environment.
Emotional supports are also important. These include supporting the family
and caregivers who are dealing with their own grieving issues of loss of
the individual with Down syndrome. The rate of decline for a person with
Down syndrome who develops Alzheimer's disease may be more rapid than in
the general population, and family or caregivers may require a great deal
of emotional support to deal with the change.
Another important social issue involves keeping the individual with Down
syndrome as much involved in activities as possible. Keeping the adult involved
in activities reflects the goal of maximizing function by stimulating the
person at a level that is challenging but not overwhelming. Regular assessment
is needed to determine the level of stimulation of activities to meet the
ever-changing level of skill. Regular small changes seem to be beneficial
so as not to confuse the patient but at the same time meeting the changing
needs.
Incontinence is a problem and is even seen in some adults with Down syndrome
who have no other decline in function. Therefore, evaluation for other causes
can help reduce or delay this problem. Obstruction from benign prostatic
hypertrophy or other causes, detrusor dysfunction, infections, and urinary
dysfunction secondary to atlanto-axial subluxation should also be considered.
For those whom no reversible cause is found, toileting regimens may be helpful.
Seizures will generally respond to standard medications. Tonic-clonic seizures
are frequently seen but adults may also have petit mal seizures as well as
less pronounced "jerking" type movements. These abnormal movements have also
tended to respond to antiseizure medications. One woman [in our clinic] had
jerking or twitching movements of her arms and her body that actually caused
her to fall or flip out of bed. These momentary abnormal movements responded
nicely to phenytoin and helped prevent further injuries.
Many adults will also have psychological symptoms such as depression, psychotic
features and anxiety. Sleep disturbance and changes in eating are common.
An evaluation for other causes will sometimes find a problem in addition
to the Alzheimer's disease. Medical problems such as hypothyroidism, infections,
peptic ulcers, and others may alter sleep, appetite or behavior. Environmental
issues may also contribute to these symptoms. Again, a careful evaluation
of the environment is necessary to maximize function without overwhelming
the individual's declining abilities. Antidepressants or anti-psychotic medications
are sometimes necessary to improve the function or reduce the symptoms. Using
the adverse-effect profile of each [medication] to guide the selection of
medication reduces the symptoms while minimizing additional problems. For
example, for adults who develop depression and have problems with sleep,
use of amitriptyline or other sedating antidepressants at night may benefit
the sleep disturbance directly as well as treat the depression. However,
another factor in the selection of the drug is that adults with Down syndrome
do seem to be particularly sensitive to the anticholinergic side effects
of medications, and many of the antidepressants and antipsychotics have adverse
effects that could further reduce skills.
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