Huntington's disease is a genetic disorder caused by a defective gene on chromosome four. Those with Huntington's show no signs of the disease in early life, because symptoms typically appear in adulthood. The progression of Huntington's includes behavior changes, which vary from patient to patient, involuntary spasmodic movements and cerebral degeneration. Rapid tissue degeneration and difficulties with the mechanics of eating are the main nutritional concerns, both of which require a focus on additional calories.
Caloric Needs
Because Huntington's is a wasting disease, the primary goal is to maintain health and prevent weight loss, Huntington's patients need a large number of calories. Although the specific number of calories varies from patient to patient and changes with the progression of the disease, it is often as high as 3,500 to 5,000 calories per day.
Mechanical Difficulties
Because of the progressive loss of control over voluntary movements, Huntington's patients often have difficulty feeding themselves. Those in advanced stages of Huntington's are also likely to have difficulty chewing and swallowing. To counteract the mechanical problems that Huntington's disease presents, you can supplement the diet with thick, high-calorie, high-protein beverages and pureed foods. If swallowing becomes too much of a problem, tube feeding may be necessary.
Small, Frequent Meals
Doctors and dietitians recommend that Huntington's patients be encouraged to maintain as much autonomy as possible -- including feeding themselves. Eating small, manageable meals at frequent intervals can maximize the number of calories while minimizing the fatigue and frustration that may be associated with mealtimes.
Added Fat and Protein
The high calorie needs of Huntington's patients can be partially met by liberally mixing fats and proteins like whey powder or dehydrated milk into other foods. Purees like mashed potatoes are excellent for this type of added calories. You can also make thick soups by pureeing a favorite food and adding liberal amounts of oil and powdered protein.
References
- "Nutrition and Diagnosis-Related Care: Sixth Edition"; Sylvia Escott-Stump; 2008
- Huntington's Disease Society of America; Nutrition and HD: Family Guide Series
- Huntington's Disease Society of America; Coping with Speech and Swallowing Difficulties in Huntington's Disease
