Dysphagia is, directly or indirectly, the most common cause of death in people with late
stage HD, whether through choking, aspiration, or malnutrition.
Dysphagia results from impaired voluntary control of the mouth and tongue, impaired respiratory
control due to chorea, and impaired judgment, resulting in eating too rapidly, or taking overly large bites of food and gulps
Dry mouth, which can be brought on by neuroleptics, antidepressants, and anticholinergics,
may worsen the problem.
TABLE 4: Swallowing Tips|
- Eat slowly and without distractions.
- Prepare foods with appropriate size and texture.
- Eating may need to be supervised.
- Caretakers should know the Heimlich maneuver
No medications are known to improve swallowing directly. Early referral to a speech-language
pathologist will help identify swallowing difficulties, and periodic reassessment can identify changes in swallowing ability
and suggest appropriate non-pharmacologic interventions such as a change in food consistency.
Devices such as enlarged grips tor silverware and nonslip plates with raised edges to
prevent spilling may prolong independent eating.
HD affected individuals should be instructed early in the disease, before the onset of
dysphagia, to eat slowly and deliberately, to sit in an upright position during and after meals, to take small bites, and
to clear the mouth of food after each bite by taking sips of liquid.
Individuals with dysphagia should avoid doing other activities while eating, in order
to concentrate on chewing and swallowing. For instance, patients should not talk while eating, nor be distracted by television
or ambient noise. Those who tend to hyperextend the neck due to chorea or dystonia should be encouraged and reminded to use
a "chin-tuck" position.
Drinking fluid through a straw may be easier than drinking directly from a cup, and the
use of a covered cup or mug, like a "sippy cup" used by young children, may prevent spillage due to chorea.
Grainy items, such as ground beef or rice, may irritate the pharynx and cause choking.
Foods such as steak, which are hard to chew, should also be avoided, or ground to a puree.
Patients may have difficulty adjusting to different textures of food, and may do better if they finish each item on the
plate in turn.
In late HD, when even liquids may.be difficult to swallow, the texture of food should be soft and smooth, and liquids may
be thickened with an additive (see Appendix 3).
For those patients who may be unable to follow instructions reliably, a caregiver can cut the food in advance, and ensure
that each mouthful has been completely chewed and swallowed before the next bite is begun.
Supervision throughout the meal may be necessary, and the family or caregiver should be taught to perform the Heimlich
In some cases, eating eventually requires so much energy and concentration that the patient becomes tired and frustrated
before consuming adequate amounts of food.
Weight loss, very prolonged mealtimes or an inability to handle utensils may be the signal that he will need to be fed
for at least part of the meal. Self-feeding may be prolonged by having the patient eat more frequent, but smaller meals, and
by using "finger foods."
The transition to assisted feeding does not have to be all or nothing, as patients may still be able to eat unassisted
at certain times and be fed at other times.
Choking may decrease once self-feeding is stopped, because the caregiver will have greater control over the size and frequency
of the bites. The caregiver should still promote eating slowly, and not talking while eating, and should make sure the mouth
is empty before each bite.
With supervision, most patients are able to assist with feeding and to take adequate amounts of food by mouth quite far
into the illness.
However, before dysphagia and communication difficulties become severe, the issue of feeding tubes should be discussed
with the patient and family, to ensure that appropriate nutrition can be maintained throughout the illness.
A gastrostomy tube can clearly improve nutritional status in a debilitated person with severe dysphagia, and may prolong
life. However, patients and families may not desire this intervention late in the course of HD. The question of whether to
use a gastrostomy tube, and other end of life issues are discussed in the final section of chapter 6.