and Huntington's Disease
MS, RD, EdM
and opinions in this book are not necessarily those of the Huntington's Disease Society of America, nor does HDSA promote,
endorse, or recommend any treatment or therapy mentioned herein. The reader should
consult a physician or other appropriate health care professional concerning any advice, treatment or therapy set forth in
1. Nutrition and Huntington's Disease
2. Nutritional Needs in Early, Middle and Advanced Stages of HD
3. The Eating Environment
4. Tips for Increasing Calorie Intake
5. Tips for Coping with Swallowing Problems
6. Tube Feeding
7. Selected High Calorie Recipes
More and more people are coming to realize the importance of good nutrition in maintaining health
and preventing disease. Advice has been issued by many organizations about diet as it relates to the major killer diseases:
heart attacks, strokes and cancer. This advice focuses on reduction of fat and cholesterol, and increased consumption of fruits,
vegetables, and grains. While this is sound advice for the majority of the population, there are come situations in which
other factors take priority in the maintenance of optimal nutritional status.
People with Huntington's Disease may have higher calorie needs than the average person, possibly
due to chorea, metabolic changes, or a combination of both. There is also some
anecdotal evidence that maintaining a body weight slightly above "desirable" weight will facilitate control of the disease.
This would place an even greater emphasis on maintaining a high caloric intake. Of course, along with increased calories,
people with HD need to maintain an adequate intake of all nutrients. They can easily meet the Required Daily Allowances (RDA's)
for vitamins and minerals with a little planning, attention to a varied intake, and appropriate use of supplemental feedings.
The purpose of this booklet is to provide information about how accomplish these goals. It is hoped that by making practical information about how to apply this dietary guidance accessible to
patients and caregivers, the lives of people with HD can be enhanced.
Nutrition and Huntington's Disease
People with increased caloric needs must often be encouraged to eat even when they are not hungry
in order to meet their nutritional requirements. This is usually not true of people with HD. They often have excellent appetites
and sometimes eat very quickly. Frustration at being unable to get sufficient
food down quickly enough without choking on it can exacerbate the psychological problems associated with food and eating in
One approach to this problem is to provide six to eight smaller meals per day instead of the usual
three. Frequent feeding can stave off the most high-level hunger and diminish eating urgency. Another approach is to offer
frequent snacks or liquid supplements between meals.
As the disease progresses, and various activities of daily living become impossible, maintenance
of independence in self-feeding becomes increasingly important. Foods should be selected with this capacity in mind. Items
that are easier to manipulate may be the most appropriate. Also, with diminished capabilities in other areas, catering to
food preferences provides an important psychological boost.
When it is no longer for the HD patient to meet his or her nutritional needs with an oral intake,
feedings may be initiated. Tube feedings may be given either as a supplement to an oral diet, or as a sole means of nutritional
support. This type of feeding is discussed further in Section 6.
Nutritional Needs in Early, Middle, and Advanced Stages of HD
While nutritional issues and eating difficulties may vary during the course of HD, maintaining quality
of life and maximum functional ability is paramount throughout.
In the early stages, depression resulting from the diagnosis can cause decreased appetite and/or
changes in usual eating habits. Some people have reported food "cravings," especially for high carbohydrate foods. There is
no harm in indulging these cravings, as long as other foods are eaten to maintain a balanced diet. A multi-vitamin supplement
providing 100% of the RDA can offer some nutritional insurance. However, there is no evidence that excessive amounts of any
nutrient are of benefit in HD.
Moving into middle-stage HD, dysphagia (swallowing difficulties), choking, difficulty with self-feeding
due to choreic (dance-like) movements, and additional difficulties due to psychological issues may appear. Other sections of this booklet offer some suggestions, and health professionals such as registered dieticians,
speech-language pathologists, and occupational therapists can also be helpful. Many techniques and tools are available to
help maintain independence in eating.
As the transition is made to advanced HD, dependence on others increases. Feeding another person
is a loving and intimate activity that can be enjoyable or stressful depending on how it is approached by the individuals
involved. It takes patience, and sometimes a sense of humor, to cope with this aspect of care. Social and cultural factors
can also play a large role.
When it is impossible to get an adequate intake from an oral diet, tube feeding may be considered.
It is possible to do this at home, given proper training. Tube feedings may be used to supplement an oral intake, or they
may become the primary source of nutrition.
The Eating Environment
A five-star restaurant is likely to be as well-known for its "ambience" as for its food. Where we
eat and what is going on around us have a major influence on our eating experience. This is just as true with people with
HD as anyone else. When eating problems arise, surroundings can be a crucial factor in helping to overcome them. It is important
to keep distractions to a minimum so that the person with HD can concentrate on the task at hand-eating. Certain things may
be more distracting to some individuals than others, so experimentation may be needed to come up with the ideal arrangements
for eating. Avoiding potentially stressful conversations, turning off the TV, having appropriate lighting and using proper
positioning (when necessary) can all contribute to ease and enjoyment of eating. Needs will change with various stages of
the disease, so flexibility is key.
The HD Kitchen
Having the right equipment can make preparing and serving meals for the person with HD much easier.
Of course, what becomes "essential" is a matter of individual needs and preferences, as well as budget.
A blender and/or food
processor can be very helpful in preparing shakes, soups and sauces, as well as pureeing favorite foods when a very soft consistency
is needed. If possible, get one that has more than one container, to allow for preparation of multiple foods quickly.
A juicer can be useful
for a person with dental problems, who cannot chew fruits and vegetables well, If swallowing is a problem, try adding thickening
powder, or mix the juice into a thick shake for soup for some needed calories
A cappuccino maker
may seem like a luxury, but switching from regular coffee to cappuccino made with whole milk and added cream (or vanilla ice
cream!) can add some needed calories.
A pastry cutter or
potato masher can serve many purposes in mixing and mashing foods. These are also useful for adding "extras" like butter or
sour cream into a dish.
Dishes with sides,
also called "soup plates" or "pasta dishes" can make picking up food much easier and less messy. They are readily available
in most department, house ware or medical supply stores.
"Sports" cups with
a cover and straw attached can be helpful in preventing spills.
Spoons or forks with
larger handles can make picking up food easier. Rubber sleeves for utensils can be bought at a medical supply store.
A heated "baby dish"
for a warming tray can keep food warm throughout a meal for a slow eater.
Tips for Increasing Calorie Intake
Maintaining weight is important for people with HD. Keeping weight up is easier if you include high-calorie
foods as much as possible and maximize the calories in a favorite dish. Dig out grandma's old "fattening" recipes. The following
are some high-calorie food ideas to get started:
Premium ice cream-choose
plain flavors, not one with "chunks" or "mix-ins" which could cause choking
or sour cream-can be added as a supplement or topping.
Butter-can be melted
and mixed with many foods such as vegetables, mashed potatoes or yams, or hot cereal.
Creamed soups, chowders,
or bisques-mix in blender for food processor to get the best consistency.
to tuna, chicken or egg salad salads.
Peanut butter, tahini,
or other "smooth" nut butters-to avoid choking think smooth-NOT chunky.
Cheese sauce-as a fondue
to dunk pieces of soft bread, or as a topping for vegetables or potatoes.
Blue cheese or ranch
style dressing-as a dipping sauce for "chicken fingers."
Refried beans-as a
side dish or in a soft tortilla.
Avocado-slices to dunk
in salsa, or mashed as a side dish.
When you are too tired to prepare meals:
When you have the energy
and are able to cook, make double the entire recipe or amount of your favorite foods. Freeze the leftovers in single servings,
or whatever size you will need, to heat up and eat another day when you don't feel like cooking.
Use convenience foods
for quick and easy meals. Try frozen dinners, especially those with extra portions included, canned soups, chili, spaghetti,
or quick cooking macaroni & cheese. Instant breakfast drinks can be blended with milk, ice cream and fruit. Be sure to
choose the foods with the right consistency.
Order out occasionally
from a nearby restaurant. Save a file of menus from restraints that deliver, and keep it near your phone. Put one or more
stars next to foods that you have tried before that were especially good.
Keep lots of sandwich
"fixings" on hand for a quick and easy meal or snack. Some high-calorie choices would be: bologna, liverwurst, or most types
of luncheon meat, and cheddar cheese, Swiss cheese or most other types of hard cheese. Don't forget to include a spread such
as mayonnaise or Russian dressing.
Ask friends and family
to bring food or to help you cook meals.
See if any commercial
meal service plans are available in your community. Such plans usually run by prescription, and will provide a package of
re-heatable foods for a specified number of meals per week. Some plans include delivery to your home.
Tips for Coping with Swallowing Problems
Coping with Speech and Swallowing Difficulties in Huntington's Disease by Lynn Rhodes, MS, CCC-SLP, HDSA, 1996.)
Pay attention to which
food and/or beverages seem to cause coughing and choking. Identifying "problem foods" can save further trouble later on. Dry,
"puffed" flaky or crumbly foods are often the culprits.
Cut meat and vegetables
into small pieces, and take small amounts in each bite.
Choose soft, moist
foods. Try mashed potatoes, noodles, soups, casseroles, yogurt, puddings, gelatins, soft cheeses, ice cream, milk shakes,
creamed cereals, and macaroni and cheese.
Add moisture to your
foods with gravies, cream sauces, salad dressings, broth, sour cream, mayonnaise, or butter.
Chew your food well.
Sip liquids with a straw.
Stay away from very
acidic for spicy foods that may burn your throat if irritation is a problem. These include: citrus fruits and their juices,
tomatoes and tomato juice, and spices like red pepper, black pepper, chili powder and hot curry powder.
Try adding a thickener
(like Thickit or Thicken-Up) or cornstarch to liquids to make them easier to swallow.
Tube feedings are given when a person is unable to eat or tolerate enough food and/or oral supplements
to meet his/her nutritional needs. Specific feeding instructions depend on the amount and type of tube being used. If you
are taking care of someone who is on tube feeding, you will need specific directions from your healthcare providers (dietitian,
doctor, and/or nurse).
There are three basic kinds of feeding tubes:
A naso-gastric tube
(NGT) is threaded through a nostril, down the throat and into the stomach. This type of feeding is usually temporary and does
not require surgery to be put in. One disadvantage is that it can be pulled out accidentally.
A naso-gastric tube also interferes with swallowing, which is a disadvantage is the person is able to eat.
A peri-epigastric tube
(PEG), or gastrostomy tube (G-tube), is a tube that is implanted through the abdomen into the stomach. It functions in essentially
the same way as an NGT. Formula flows through the tube into the stomach. It is usually for long-term use. As it does not pass
down the throat, the patient can receive both tube feedings and an oral diet. It is a good idea to continue oral feeding as
long as possible, even if the quantities given are not nutritionally significant, as this can provide significant psychological
A new type of tube in this category is called a "button" tube. It is a very short tube attached to the
stomach with a longer "snap on" tube for use during feedings. When the tube is not in use, a plastic cap covers the opening.
This can be useful for people who do not receive a feeding constantly over 24 hours (e.g., bolus feeding, feedings run only
at night, etc.).
A jejunostomy tube
(J-tube) is implanted below the stomach, directly into the small intestine. It functions similarly to tubes leading into the
stomach, but with several differences. The advantage of a J-tube is that it reduces the risk that formula will back up into
the esophagus into the trachea and lungs. This is called "aspiration," and for someone at high risk of aspiration, the J-tube
might be the preferred option. Increased probability of diarrhea, and increased probability of the (very narrow) tube getting
clogged, are some disadvantages.
Feedings are either "continuous" or "bolus" servings. As the name implies, continuous feedings run
down the feeding tube at a fixed rate throughout the day and/or night. Tolerance problems are usually minimized when feedings
are given in this way. A very weak or debilitated patient may need to be fed continuously in order to tolerate enough intake
to meet his or her needs. Patients at risk for regurgitation because of limited stomach capacity need continuous feeding.
Most patients who are just starting out on tube feedings are given continuous feedings and then gradually changed over to
Bolus feedings are essentially the equivalent of a meal, consisting entirely of formula. A "bolus"
is a set amount of formula run down the feeding tube at specific times during the day. These usually, but not always, correspond
to breakfast, lunch and dinner times. Some regimens include one or more bolus "snacks" as well, for a total of four to six
feedings per day. Bolus feedings are usually more convenient for caregivers,
since feedings are administered only at specific times, and larger amounts are given at each sitting.
It should be noted that for some people, the weight gain achieved after the gastric tube insertion
leads to an improvement in swallowing ability. This improvement may allow a person to resume eating.
Some Additional Tips About Tube Feedings:
Position the person
so that he or she is sitting up, or at least so the upper-body is above the level of the stomach.
Practice good sanitation.
Wash your hands before handling the feeding equipment. Wash feeding bags with water. Do not use soap, as it will stick to
the inside of the bag and get into the formula. This can cause diarrhea and other unpleasant consequences.
Feedings should be
given at room temperature to minimize risk of cramping and/or diarrhea. Open cans of formula could be kept in the refrigerator,
and discarded if not used within 24 hours. They should be taken out 15-20 minutes before a feeding and allowed to warm up
to room temperature.
Always flush the feeding
tube with water after a feeding. This will help to prevent the patient from getting dehydrated. It will also prevent the food
from getting clogged.
If medications are
to be run down the feeding tube, always be sure they are finely crushed. Flush the tube with water to wash them down.
If the tube seems to
be clogged and a small flush of water isn't effective, a flush of about 100cc of cola may do the trick. You can also try dissolving
a quarter teaspoon of meat tenderizer in a teaspoon of water and placing it into the tube. Wait five minutes before flushing
again. If none of these strategies work, contact your health care provider(s) for advice.
Selected High-Calorie Recipes
4 cups diced cooked
1 1/2 cups cooked rice
1 can cream of chicken or cream of mushroom soup
Salt and pepper to taste
1 cup shredded cheese
Mix chicken, rice soup,
mayonnaise, salt and pepper together and place in a shallow baking pan. Top with shredded cheese. Bake at 350 degrees for
about 30 minutes. Makes four servings. Each serving provides 794 calories.
are: 8 meat, 1 bread and 5 fat
1 pound ground beef
1/2 teaspoon Worcestershire sauce
3 tablespoons flour
1 cup sour cream
1 teaspoon salt
1/4 chopped onion
1/4 teaspoon pepper
pound fresh mushroom, chopped
Melt butter in a large
skillet and brown ground beef. Slowly mix in flour. Add chopped onions, mushrooms and seasonings. Cover and let simmer for
about five minutes or until mushrooms and onions are soft. Add sour cream and mix well. Serve over soft noodles.
NOTE: This recipe also
works well in a crockpot (slow cooker). Brown all the ground beef first. Then add all the other ingredients and cook on "medium"
heat for 8010 hours. Makes four servings. Each serving provides 581 calories.
are 5 meat, 1/2 bread and 7 fat.
a large pie pan and sprinkle with the following:
12 slices of bacon, fried and crumbled
shredded Swiss cheese
1/2 cup finely chopped onion
Place the following
in a blender or food processor and blend until well mixed:
2 cups milk
1/2 cup Bisquick
1/4 teaspoon salt
1/4 teaspoon pepper
Pour blended mixture
over the bacon, cheese and onion. Bake at 350 degrees for 50-55 minutes until well-set and golden brown. Allow quiche to cool
for five minutes before serving.
NOTE: This recipe can
be made in many variations depending on what you like and happen to have on hand. Shredded carrots, broccoli, spinach, mushrooms,
shrimp, crabmeat and different kinds of cheeses can be substituted for the items listed above. Makes 4 servings. Each serving
provides 597 calories.
are: 6 meat, 1 bread, 6 fat and 1/2 milk
8 oz. (about 4 cups)
cooked egg noodles
1/2 cup butter
1/2 cup heavy cream
1/2 cup grates Parmesan
1/4 cup shredded Swiss cheese
Salt and pepper to taste
Cook noodles in boiling
water until soft and drain. In small saucepan, melt butter. Add cream and heat through. Pour over noodles. Add cheeses, salt
and pepper. Toss lightly until noodles are coated. Makes four servings. Each provides 671 calories.
are 2 meat, 2 bread and 9 fat
1 cup all-purpose flour
1 cup whole wheat flour
1/2 teaspoon salt
2/3 cup brown sugar
apples, peeled, cored and quartered
1/2 teaspoon nutmeg
1/2 cup chopped walnuts
2 eggs, beaten
1 cup sour cream
Sift flours and salt
into large bowl. Reserve two tablespoons brown sugar. Set aside. Add remainder of sugar to flour mixture and mix well. Add
butter and walnuts to mix. Pack into 9 inch round cake pan. Arrange apple sections over mixture and sprinkle with two tablespoons
brown sugar. Bake at 375 degrees for about 15 minutes or until apples are soft. While the above is baking, combine eggs, sour
cream, cinnamon and nutmeg. Remove aple and crust mixture from oven and pour sour cream mixture evenly over the top. Sprinkle
a little more brown sugar. Return the kuchen to the oven and bake for an additional 30-40 minutes. Serve warm or cooled. Makes
4 servings. Each serving provides 898 calories.
are: 1 meat, 3 bread, 12 fat and 1/2 fruit
5 slices of "day old"
3 cups milk, scalded
2 tablespoons soft butter
1/4 teaspoon salt
1/2 cup sugar
1/2 teaspoon vanilla
Preheat oven to 350
degrees. Butter a medium sized baking dish. Toast bread lightly, then spread with butter while hot. Cut slices into quarters.
Fit into prepared pan, overlapping slightly. Sprinkle raisins between slices, not over the top. Scald milk. Beat eggs slightly.
Add salt to eggs, then all but two teaspoons of the sugar, then the hot milk and vanilla. Pour over toast and let stand 10
minutes, pressing down toast slightly once or twice to soak up milk mixture. Sprinkle cinnamon and remaining sugar over the
top. Bake 25 to 30 minutes, or until a knife inserted in the center comes out clean. Serve warm or cold, plain or with whipped
cream on top.
NOTE: For a finer texture,
rip up toast into very small pieces instead of cutting it into quarters. Makes four servings. Each serving provides 450 calories.
are: 1 meat, 1 bread, 3 fat, 1 milk and 1 fruit.
6 8 oz. Packages of
cream cheese (3 pounds)
2 cups sugar
1 pint heavy cream
7 whole eggs
1 pint sour cream
2 teaspoons vanilla extract
1 1/2 cups crushed graham
1/2 cup soft butter
Mix graham cracker
crumbs and butter together and divide mixture in half. Press half of crumb mixture into the bottom of each of two 9 inch round
cake pans. Cream together cream cheese, sugar, eggs and egg yolks. Add sour cream, heavy cream and vanilla. Mix well. Pour
mixture into pans over the cracker crust. Bake at 350 degrees until set. Makes two cakes, eight servings each. Each serving
provides 620 calories.
are: 1/2 meat, 13 fat and 1/2 bread
18 vanilla wafer cookies
6 oz cream cheese
2 teaspoons vanilla
1 can cherry pie filling
Put paper cupcake liners
into muffin tins. Place one vanilla wafer in each liner. Beat cream cheese, sugar, eggs an vanilla eat medium speed. Pour
into papers, on top of vanilla wafers. Bake 12-15 minutes in a 350 degree oven. When cool, spoon cherry pie filling on top.
Keep tars in the refrigerator until ready to serve. Tarts may also be garnished with whipped cream. Makes 18 servings. Each
serving (one tart) provides 133 calories.
are: 1/2 bread and 3 fat
Chocolate Chip Bread**
2/3 cup butter
2/3 cup brown sugar
1 pound canned pumpkin
2/3 cup water
3 1/3 cup flour
2 teaspoons baking soda
1 1/2 teaspoons salt
1 teaspoon cinnamon
1 teaspoon ground cloves
1 teaspoon nutmeg
2 8 oz. packages semi-sweet chocolate chips
Preheat over to 350
degrees. Grease two 9" x 5" x 3" loaf pans. Cream together butter and sugar. Add eggs, pumpkin and water. Add dry ingredients
and mix well. Stir in chocolate chips. Pour into loaf pans. Bake 65-75 minutes. Makes 2 loaves, about six servings each. Each
serving provides 586 calories.
are: 2 bread and 8 fat.
recipes, which may be appropriate, may be found in:
The Non-Chew Cookbook
By J. Randi Wilson
P.O. Box 2190
order also see HDSF http://endoflifecare.tripod.com/huntdisscholarshipfund/id14.html
The Dysphagia Challenge
By Pam Womack, RD
1904 East 1223rd Street
Olathe, KS 66061-5886
Catalog # 2459
contributed by HD families at http://www.hdac.org/recipe/recipes.php