Nutritional Management for Glycogen Storage Disease (GSD)

These instructions are for caregivers of Children's Hospital of Philadelphia patients who have glycogen storage disease.

Important information:

Learn more about glycogen storage disease (GSD).

• In glycogen storage disease the simple sugars found in the foods we eat are not broken down properly in the body.

• Because the simple sugars fructose, sucrose and lactose cannot be broken down by the body, they cannot be used as a source of energy.

• Eating simple sugars can also cause other levels such as triglycerides, lactic acid and uric acid to build up in the body. At increased levels, these may be harmful.

Sources of simple sugars:

Fructose

• Fruits and fruit juices

• Foods with high fructose corn syrup

• Soda, candies, cakes, cookies

Sucrose

• Table sugar, brown sugar, raw sugar, honey, molasses, maple syrup

• Also naturally found in some vegetables

Lactose

• Sugar in milk products, yogurt, ice cream

• Also found in lactose-free milk, for example, Lactaid milk-contains galactose

Patient instructions:

Eat often!

• In GSD, the blood sugar does not stay up on its own. People with GSD must have carbohydrates often to keep their blood sugar above 70 mg/dl which is considered in the safe range.

• During the day your child should eat healthy carbs like breads, cereals, whole grains, brown rice, whole wheat pasta, crackers and beans.

• To avoid having to eat often overnight, your child will need cornstarch, overnight tube feedings or dextrose to keep blood sugar above 70 mg/dl.

• Raw, uncooked cornstarch (ARGO) is slowly digested and provides a steady amount of sugar. It can maintain blood sugar for 3-6 hours.

  • Argo cornstarch is the recommended brand

  • Mix with sugar-free fluids such as water, Crystal Light or sugar-free Kool Aid.

  • Add cornstarch to the sugar-free fluid and either shake with the lid on or stir with a spoon.

  • Mix right before your child drinks the cornstarch.

Patient instructions based on type of GSD:

FoodData Central is a website that can help you learn about the ingredients and nutrients in foods.

Type I

• Avoid simple sugars.

• Avoid sorbitol- it turns into fructose.

• Keep the total amount of sugar in each meal to less than 5 grams.

• Limit fructose and galactose to less than 2.5 grams per meal. Take multivitamin and calcium supplement.

Type III

• Eat a high-protein diet. Your healthcare team will recommend how much protein your child should have each day.

• Use protein supplements. Your healthcare provider will help determine which one is best.

• Keep the total amount of sugar per meal to less than 5 grams.

• Do not use sucrose or high-fructose corn syrup.

Type 0, VI, and IX

• Follow the recommendations of your team for how much protein to have each day. Keep the total amount of sugar per meal to less than 5 grams.

• Eat complex carbohydrates like bread, rice, pasta, cereals.

• Do not use sucrose or high-fructose corn syrup.

Multivitamins and Calcium Supplements

• Multivitamins and calcium supplements are important for a child with GSD because of their restricted diet.

• Do not give your child calcium and multivitamin at the same time. Take these with food so that the body absorbs them well.

Suggested multivitamins, your nutritionist will tell you which one to use:

• One A Day (sugar free) or store-brand version

• Flinstones complete (sugar free)

• Centrum or store-brand version

• Poly-Vi-Sol (infants)

Suggested calcium supplements, your nutritionist will tell you which one to use:

• UpCal D (powder)

• Cal-Quick (liquid)

• CalciMix (powered capsule)

• Citra Cal or any Calcium citrate

• Tums (sugar free)

• Calcium carbonate

Patient instructions for foods allowed and foods restricted for GSD:

No fruit allowed

Milk, cheese and dairy

• Foods allowed:

  • Enfamil ProSobee formula

  • Soy milk (unsweetened)

  • Rice milk (unsweetened)

  • Almond milk (unsweetened)

  • Cashew milk (unsweetened)

  • Cheese, 1-2 servings per day (not sweetened with sucrose, fructose or lactose)

• Foods not allowed:

  • All cow's milk

  • All lactose free products (Lactaid)

  • Sweetened soy milks and sweetened soymilk products (Silk brands)

  • Yogurt

  • Ice cream

Bread

• Foods allowed:

  • White

  • Wheat

  • Potato bread

  • English muffins

  • Plain bagels

  • Pitas

  • Tortilla

• Foods not allowed:

  • Any bread that contains fruit or sugar like raisin bread

  • Donuts, muffins, quick breads

  • Pancakes, waffles, that are prepared with large amounts of milk and sugar

Pasta/rice

• Foods allowed:

  • All noodles, pastas, spaghetti, rice

• Foods not allowed:

  • Any pasta, noodle or rice mix made with milk, tomato or cream sauce

Cereal

• Foods allowed:

  • Cheerios

  • Puffed rice

  • Wheat or rice Chex

  • Shredded wheat

  • Fiber one

  • Plain oatmeal

  • Corn flakes

• Foods not allowed:

  • All other cereal (those with more than 5 grams of sugar per serving)

Potatoes

• Foods allowed:

  • Potatoes, baked, boiled, mashed. Mashed should be made with Prosobee or unsweetened soy or rice milk.

• Foods not allowed:

  • Sweet potatoes

  • Potatoes prepared with milk

Vegetables

• Foods allowed:

  • Broccoli, green beans, Brussels sprouts, cabbage, cauliflower, celery, cucumber, eggplant, lettuce, mushrooms, spinach, asparagus, peppers, squash, pea pods, dill pickles

• Limit the following veggies to less than ½-1 cup serving per day:

  • Fresh tomatoes

  • Beets/Sugar beets

  • Carrots

  • Chickpeas

  • Green peas (frozen)

  • Corn

  • Lima beans

  • Turnip

  • Yams

  • Sugar snap peas

• Foods not allowed:

  • Canned vegetables packaged with sugar or milk

  • Pre-packaged frozen vegetables packaged with sugar or milk

  • Tomato sauce with added sugar.

  • Concentrated tomatoes like tomato paste

  • Sweet potato

  • Sweet pickles

Legumes

• Foods allowed:

  • Lentils

  • Dried split peas

  • Beans

  • Soybeans (limit to ½-1 cup per day)

  • Olives

• No legumes are restricted

Soups

• Foods allowed:

  • Broth based soups, bouillon, soups made with allowed foods

  • Beef noodle and chicken noodle are okay

• Foods not allowed:

  • Cream or milk-based soup or soups made with milk

  • Tomato soup

Prepared foods

• Foods allowed:

  • Rice or noodle mixes which contain a broth type sauce or an oil type base like pesto

  • Pizza-limit to 1 slice

• Foods not allowed:

  • Cream or milk-based soup or soups made with milk

  • Tomato, milk/cheese-based sauces, like macaroni and cheese mix or SpaghettiOs, because of added sugar or milk.

Others

• Foods allowed:

  • Broth based soups, bouillon, soups made with allowed foods

  • OK to use non-dairy coffee substitutes (without added sugar), tea, unsweetened pickles, diet drinks

• Foods not allowed:

  • High fructose corn syrup, maple syrup, jelly, molasses, preserves, candy, caramel, honey, regular sodas

Condiments

• Foods allowed:

  • Salt, pepper, spices, herbs, vinegar, soy sauce, lemon and lime juice

  • Go very light with ketchup

• Foods not allowed:

  • Cream-based condiments, sugar

Sweets

• Foods allowed:

  • Crystal Light flavor pops, Rice Dream ice cream (not fruit flavors)

  • Sugar-free Jell-O, liquid or powdered glucose like dextrose or cerelose, Cool Whip

  • Sugar-free sweets with only artificial sweetener such as aspartame, sucralose (Splenda), saccharin, acesulfame K

  • Foods sweetened with rice syrup, dextrose (pixie sticks candy) or glucose

• Foods not allowed:

  • All sweets made with sugar, honey, fruit, fructose, maple syrup, turbinado, or molasses

  • Sweets that contain sugar alcohols (xylitol, mannitol, lactitol, sorbitol, maltitol), limit to serving with less than 4 grams sugar alcohols. Note that sugar alcohols can sometimes have a laxative effect

Snack foods

• Foods allowed:

  • Popcorn (all except caramel), rice cakes, low-fat or reduced fat chips, Doritos, corn chips, cheese curls, tortilla chips, crackers such as saltines, oyster/soup crackers, pretzels and dry cereal are also great snacks

• Foods not allowed:

  • Sweets that contain sugar alcohols (xylitol, mannitol, lactitol, sorbitol, maltitol), limit to serving with less than 4 grams sugar alcohols. Note that sugar alcohols can sometimes have a laxative effect.

Meat and protein

• Foods allowed:

  • Low-fat or lean meats are best

  • Try to pick 98% lean beef and loin cuts of pork, beef

  • Choose lean beef, pork, lamb, veal, chicken, turkey, luncheon meat

  • Limit higher fat meats like hot dogs, sausage to 3 times a week

  • Use egg substitutes or egg whites more often than egg

  • Chicken and turkey breast/wing without skin

• Foods not allowed:

  • Creamed meat, poultry, or fish

  • Eggs prepared with milk or cheese sauces that contain milk or sugar

  • Avoid frying food to reduce unhealthy fat intake. Instead bake, roast, broil, and grill.

  • No glazes, dressings, or sauces on top

Fats

• Foods allowed:

  • Bacon, butter, margarine,

  • Mayonnaise, shortening, all oil

  • Ranch dressing is OK in small portion (1-2 Tbsp.)

• Foods not allowed:

  • Salad dressings made with milk, sugar, high fructose syrup, or honey

Sample snack ideas

Each of these options is about 15-30 grams of carbohydrates.

This amount of carbohydrate should keep blood sugars in the safe range for 1-2 hours. You will still need to check blood sugars frequently.

Toddlers may only need a quarter to half of this serving to maintain their blood sugar.

• 3-6 cups popcorn

• Trail mix: 1/2 cup unsweetened cereal with nuts

• 25-50 Goldfish crackers

• 20-30 Cheez-It crackers

• 1 small frozen pretzel

• 1 cup cereal plain or with 1 cup of plain rice milk

• 2 slices light bread with meat, soy cheese or peanut butter

• 1 small Lenders bagel with peanut butter

• 1 English muffin with butter or margarine

• 8 animal Crackers

• 6 Saltine crackers with soy or veggie cheese

• 2 mini or 1 regular-size frozen waffle with butter or light margarine

• 15 Wheat Thins

• 1 slice of toast

• 30 oyster crackers

• Peanut butter cracker package

• 4 hard bread sticks

• 15 potato chips

• 25 cheese curls

• 10 thin pretzels

• 4-6 mini-rice cakes

 

Reviewed on January 18, 2023, by Heather McKnight, CRNP; Nicole Stewart, RN