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NUTRITION IN
POMPE DISEASE
Brittany Tayl or,
RD, LDN
Metabol i c
Dietitian
Duke
Pediatri cs Geneti cs
NUTRITION GOALS FOR
POMPE DISEASE
 Decrease glycogen deposition in the lysosomes
 Increase amino acid utilization
 Maximize overall nutritional health
 Meet macro and micronutrient requirements (DRI’s ) to
provide optimal nutrition
Kishnani PS, Steiner RD, Bali D, et al. Pompe disease diagnosis and
management guidelines. Genet Med 2006; 8:267-88.
NUTRITION ISSUES IN POMPE
Weight Loss
 Modify
food/beverage
consistency
 Formula
supplements
 Tube feedings
 Small, frequent
meals
Weight Gain
 Decreased activity
 Calorie
requirements may
be decreased
 High protein diet
NUTRITION ISSUES IN POMPE
 Osteoporosis
 Chronic limited mobility and weakness
 Poor nutrition
 Not well understood in Pompe
 Optimize nutrition for bone health
 Meet calcium and vitamin D requirements
 May require additional supplementation
 Citracal, Viactiv, Caltrate
 Vitamin D supplementation, monitoring blood levels
THE ROLE OF A HIGH PROTEIN DIET
 Slonim AE, Bulone L, Slonim E, Goldberg T, MinikesJ,
Galanko J, Martiniuk F. AdultAcid Maltase Deficiency:
Modification of natural history by Nutrition & Exercise
Therapy. Muscle and Nerve 2007; 35: 70 -77.
 34 patients treated with nutrition and exercise therapy
(NET) for periods of 2-10 years
 High protein, low carbohydrate diet with L-Alanine
supplementation
 Aerobic exercise
 Concluded NET compliance can slow deterioration of
muscle function, improve natural history of adult -onset
Pompe Disease
DIET RECOMMENDATIONS
Protein
Carbohydrate
Fat
25-30% of total calories
30-35% of total calories
35-40% of total calories
Decrease muscle
turnover
Reduce glycogen
storage in muscle
Alternative source of
energy to replace
carbohydrate
Encourage complex
carbohydrates vs
Simple Sugars
Alternative source of
energy to replace
carbohydrate
Protein supplements
(whey)
Limit sources of
saturated and trans
fats
Choose foods high in
mono- and
polyunsaturated fats
SOURCES OF PROTEIN
Lean Meats
Fish
Eggs
Nuts, nut butters
and seeds
Beans
Dairy: milk, yogurt,
cheeses
SOURCES OF PROTEIN
 100% whey protein powders (little to no carbohydrate)
 Unjury
 Beneprotein
 GNC
 High protein, low carbohydrate snacks**




Atkins, South Beach shakes, bars, snacks
Advantedge bars and shakes
PureProtein or Premier Protein powders, shakes and bars
ThinkThin bars
** some of these products contain sugar alcohols
COMPLEX CARBOHYDRATES VS
SIMPLE SUGARS
Complex Carbohydrates
 Rich in fiber, vitamins and
minerals
 Take longer time to digest
 Important in the absorption
of certain minerals and the
formation of fatty acids
* High in protein
Vegetables
Spinach
Broccoli
Yams
Beans*
Zucchini
Lentils
Skim Milk
Whole Grains
Brown and wild
rice
Oatmeal
Corn
Whole wheat
breads and
pastas
Quinoa*
SIMPLE SUGARS
Often provide
little nutritional
value to the body
Digested by the
body quickly
Source of simple
sugars
 White flour
 Honey
 Candy
 Chocolate
 Fruit juice
 Cake
 Jam
 Soda
 Packaged cereal
FATS
Decrease
saturated and
trans- fats
 Beef, poultry fat
 Dairy fat
 Butter
 Cheese
 Ice cream
Increase poly- and
monounsaturated
fats
 Olive oil
 Avocados
 Nuts
 Fatty fish
 Seeds
EXAMPLE MENU
Food/Portion
Breakfast
2 eggs, scrambled
2 slices canadian bacon
1 whole wheat english muffin
1 Tbsp margarine
1 cup low fat milk (1%)
Snack
Whole wheat saltine crackers (5)
1 Tbsp natural peanut butter (low
sugar)
Grams of
Grams of
Protein Carbohydrate
Grams
of Fat
14
5
7
0
8
3
1
26
0
12
15
1
2
8
3
1
4
10
4
1
8
EXAMPLE MENU
Food/Portion
Lunch
Turkey sandwhich on whole wheat
bread (3 slices oven-roasted turkey, 1
slice tomato, 1 Tbsp mustard and 1
leaf lettuce)
1 oz roasted almonds (low salt)
5 oz Greek yogurt
1 cup unsweetened iced tea
Snack
High Protein, low carbohydrate shake
1 medium apple
Grams of
Grams of
Protein Carbohydrate
Grams
of Fat
28
20
10
6
11
0
6
18
0
14
11
0
30
5
3
EXAMPLE MENU
Food/Portion
Dinner
4 oz boneless/skinless chicken breast,
baked with sliced peppers, onions
and olive oil
1 whole wheat dinner roll with butter
1/3 cup brown rice
½ cup baked beans
1 cup low-fat milk (1%)
Grams of
Protein
Grams of
Carbohydrate
Grams
of Fat
25
4
2
7
8
0
18
15
27
13
9
2
1
7
3
TOTALS
TOTAL:
2234 calories
Protein
Carbohydrate
Fat
160 grams
178 grams
98 grams
640 calories
712 calories
882 calories
29%
32%
39%
FOR MORE INFORMATION
Brittany Taylor, RD, LDN
Metabolic Dietitian
Duke University Medical Center
Division of Medical Genetics
919-681-1932 (office)
919-684-0927 (fax)
[email protected]