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Brittany Taylor, RD, LDNMetabolic DietitianDuke Pediatrics- Genetics
NUTRITION IN 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
NUTRITION GOALS FOR POMPE DISEASE
Kishnani PS, Steiner RD, Bali D, et al. Pompe disease diagnosis and management guidelines. Genet Med 2006; 8:267-88.
Weight LossModify food/beverage consistency
Formula supplements
Tube feedingsSmall, 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
NUTRITION ISSUES IN POMPE
Slonim AE, Bulone L, Slonim E, Goldberg T, MinikesJ, Galanko J, Martiniuk F. AdultAcid Maltase Defi ciency: Modifi cation 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
THE ROLE OF A HIGH PROTEIN DIET
DIET RECOMMENDATIONS
Protein Carbohydrate Fat
25-30% of total calories
Decrease muscle turnover
Alternative source of energy to replace
carbohydrate
Protein supplements (whey)
30-35% of total calories
Reduce glycogen storage in muscle
Encourage complex carbohydrates vs
Simple Sugars
35-40% of total calories
Alternative source of energy to replace
carbohydrate
Limit sources of saturated and trans
fats
Choose foods high in mono- and
polyunsaturated fats
Lean MeatsFishEggsNuts, nut butters and seedsBeansDairy: 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
SOURCES OF PROTEIN
Complex CarbohydratesRich in fiber, vitamins and minerals
Take longer time to digest Important in the absorption of certain minerals and the formation of fatty acids
COMPLEX CARBOHYDRATES VS SIMPLE SUGARS
VegetablesSpinachBroccoliYamsBeans*ZucchiniLentils
Skim MilkWhole Grains
Brown and wild riceOatmealCorn Whole wheat breads and pastasQuinoa*
* High in protein
Often provide little nutritional value to the body
Digested by the body quickly
Source of simple sugarsWhite flourHoneyCandyChocolateFruit juiceCake JamSodaPackaged cereal
SIMPLE SUGARS
FATS
Decrease saturated and trans- fatsBeef, poultry fatDairy fatButterCheese Ice cream
Increase poly- and monounsaturated fatsOlive oilAvocadosNutsFatty fishSeeds
EXAMPLE MENU
Food/Portion Grams of
Protein
Grams of Carbohydra
te
Grams of Fat
Breakfast2 eggs, scrambled
2 slices canadian bacon1 whole wheat english muffin
1 Tbsp margarine1 cup low fat milk (1%)
SnackWhole wheat saltine crackers (5)1 Tbsp natural peanut butter (low
sugar)
145708
14
3126012
104
151283
18
Food/Portion Grams of
Protein
Grams of Carbohydra
te
Grams of Fat
LunchTurkey 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 yogurt1 cup unsweetened iced tea
SnackHigh Protein, low carbohydrate
shake1 medium apple
28
6110
30
20
6180
5
10
14110
3
EXAMPLE MENU
Food/Portion Grams of
Protein
Grams of Carbohydrat
e
Grams of Fat
Dinner4 oz boneless/skinless chicken
breast, baked with sliced peppers, onions and olive oil
1 whole wheat dinner roll with butter
1/3 cup brown rice½ cup baked beans
1 cup low-fat milk (1%)
254278
018152713
92173
EXAMPLE MENU
TOTAL: Protein Carbohydrate Fat
2234 calories
160 grams
640 calories
29%
178 grams
712 calories
32%
98 grams
882 calories
39%
TOTALS
Brittany Taylor, RD, LDNMetabolic Dietitian
Duke University Medical CenterDivision of Medical Genetics
919-681-1932 (offi ce)919-684-0927 (fax)
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