Download Optimal Nutrition in PWS

Understanding Optimal
Nutrition In Prader-Willi
Syndrome
Melanie Silverman MS, RD, IBCLC
Registered Dietitian
Lactation Consultant
In the office…
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Infant Feeding
Picky Eaters
Poor Weight Gain
Weight Management
Food Allergies/Intolerances
Breastfeeding
G-tube Feeding
• PRADER-WILLI SYNDROME
Objectives
Today’s Discussion Is Driven By Your Questions
• Generally…How & What to Feed
• Macronutrient Makeup
– Carbohydrates, Protein and Fat
– Calories
– What is our best in practice recommendation?
• Artificial Sweeteners
– What are they? What are they called?
– What is the recommendation?
• Ketogenic diet
How To Feed
Meal and Snack Structure
• Essential
• Underestimated
Structure
• Separate meals and
snacks by at least 2
½ hours
• Post menus and
rules
• No distractions at
meals (No “ianythings” at the
table)
• Family meals
Physical Activity
• Parks
• Sports
• Family
Participation
What To Feed
Food Guide Pyramid
Prader-Willi Food Pyramid
My Plate (much better)
Prader-Willi Plate
High Quality
Protein
Whole Grains
Fats like
oils, flax,
avocado
Fruit (but no
fruit juice)
Vegetables
Dairy (like
yogurt,
cheese,
milk)
Macronutrient Makeup
Macronutrients
Health, Growth, Healing and Immune Function
PROTEIN: growth and maintenance of the body
FATS: energy, hormones, insulation, Vitamins
ADEK
CARBOHYDRATES: main energy source for
muscles, organs...including the brain
Protein
Protein
Received little attention except in starving
children
Animal is complete protein:
Meat/poultry/fish/pork
Plant is incomplete protein:
fruits/vegs/grains/nuts
• Macronutrient manipulation in weight loss
programs
• Satiety effect? Maybe.
Fat
Fats
• Saturated fat: animal flesh, butter, margarine,
processed/hydrogenated oils, tropical oils, and fried foods
• Polyunsaturated fat: vegetable oils—sunflower, safflower, corn, and
flaxseed oils
• Monounsaturated fats: vegetable oils—olive, peanut, canola, and
many nut oils
The Famous Fats
– Omega-3 fatty acids: seafood such as tuna, mackerel, and salmon, as
well as nuts, soy, canola, and flaxseed oils
– Omega-6 fatty acids : vegetable oils such as soybean, corn, and
safflower oils
Trans Fats
• CHEMISTRY: Adding a
hydrogen to vegetable oil
by process called
hydrogenation
• Freshness
• Commercial baked goods,
crackers, cookies and
cakes, doughnuts and
French fries
• Shortenings and some
margarines
• Stay away
Carbohydrates
CARBOHYDRATES
SIMPLE
• Fruits***** and fruit juice
• Processed foods and
anything with added
refined sugar, such as soft
drinks and some candy.
COMPLEX
• Found in nearly all plantbased foods
• Usually higher in fiber and
take longer for the body to
digest.
• Whole-wheat bread,
whole-grain pasta, brown
rice, and starchy vegetables
(peas, corn, potatoes)
GOOD Carbs vs. BAD Carbs
“GOOD”
• Fruits/Vegetables
• Whole grains (>3 grams per
serving)
• Beans, Peas, Legumes
• Brown rice
• Quinoa
“BAD”
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Candy
Cakes
Cookies
Juice
Frozen desserts
Donuts
Low fiber crackers
White bread, pasta, rice
“Snacky snacks”
So What is the Glycemic Index?
• How quickly and high food causes a boost in
blood sugar compared to pure glucose.
• Foods with a high glycemic index, like white
bread, cause rapid spikes.
• Foods with a low glycemic index, like whole
oats, are digested more slowly, causing a
lower change in blood sugar.
Glycemic Index
70 or higher: high glycemic index
55 or below: low glycemic index
Guess the glycemic index?
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LESSON: Replace highly processed foods with less processed
foods.
Glycemic Load
• Glycemic Index is NOT based on standard serving
sizes
• A low glycemic load is a better indicator that food
won’t have much impact on blood sugar
– i.e. Carrots are good for you. So eat them please.
• Ranking system that measures CHO in serving
– <10 low
– 10-20 moderate
– >20 high
Fiber
• “Roughage or bulk”
• Fiber not digested by
body
• Passes relatively intact
through the stomach,
small intestine, colon
and out of your body.
Fiber Categories
Soluble
Insoluble
• Dissolves in water to form a
gel-like material.
• It can help lower blood
cholesterol and glucose
levels.
• Promotes the movement of
material through your
digestive system and
increases stool bulk
• EXAMPLES: Oats, peas,
beans, apples, citrus fruits,
carrots, and barley
• EXAMPLES: Whole-wheat
flour, wheat bran, nuts and
many vegetables are good
sources of insoluble fiber.
The Fiber on a Food Label
Mixed Macronutrients
Carbohydrate (C) • Eggs: P, F
Protein(P)
Fat (F)
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Yogurt/Milk: C, P, F
Nuts: P, F
Beans: C, P, F
Cottage Cheese: P, F
Cheese: P, F
How Many Calories for PWS?
• For children and adolescents:
– 10-11 calories per centimeter to maintain growth
velocity
– 8-9 calories per centimeter for slow weight loss or
support linear growth
• Adults vary:
– 1,000-1,200 per calories day
– About 60% of a typical person’s diet
ADA Pediatric Nutrition Assessment, 2008
United States Calorie Percentages
USDA
Carbohydrates: 50-70% (+)
Protein: 15-20%
Fat:
30-35%
Calorie Percentages
USDA
CARBS: 50-70%
PRO: 15-20%
FAT:
30-35%
PWS
CARBS: 45%
PRO: 25%
FAT: 30%
FIBER: 20 grams
per day
*Miller, et al. A reduced-energy intake, well
balanced diet improves weight control in children
with Prader-Willi syndrome, J Hum Nutr Diet, 2012
Optimal Diet
2012
“A reduced energy intake, well balanced diet
improves weight control in children with PraderWilli Syndrome”
(J.L. Miller, C.H. Lynn, J. Shuster, D.J. Driscoll)
• Children 2-10
• 30% fat, 45% carbohydrate, 25% protein, 20
grams of fiber
• Energy restricted diet
Results
Balanced macronutrient at 30% fat,
45% carbohydrate and 25% protein
(20 grams of fiber) IMPROVES
weight and body composition in
children with PWS compared to a
simple energy restricted diet
Calorie Calculations
• Carbohydrate: 4 calories per gram
• Protein: 4 calories per gram
• Fat: 9 calories per gram
Calorie Calculations
• Example: 1200 calories
• Percentages: 45% CHO, 25% PRO, 30% FAT
45% of 1200 calories =540 calories of CHO
25% of 1200 calories =300 calories of PRO
30% of 1200 calories=360 calories of FAT
Add this up to 1200 calories
Calorie Calculations
Remember….4 calories per gram for CHO/PRO
and 9 calories per gram for FAT
CHO: 540/4= 135 grams of CHO
PRO: 300/4= 75 grams of PRO
FAT: 360/9= 40 grams of FAT
What does that diet look like?
Before
• Cereal, milk, grapes
• Tuna or Salmon Salad
Sandwich, pretzels, apple
• Pasta and sauce, salad and
garlic bread
• Chicken Noodle Soup with
bread sticks, salad
After
• Oatmeal cooked with ground
flax seeds, roasted pecans and
raisins
• Tuna/Salmon Salad on
cucumber slices, apple
• Eggplant lasagna (roasted
eggplant as the noodles),
berries
• Roasted Chicken, asparagus,
salad
More examples
Before
• Tacos with shell
After
• Taco salad
• Spaghetti and sauce
• Spaghetti SQUASH and
sauce
• Bagel and cream cheese
• Greek yogurt and granola
• Buttermilk Pancakes, Eggs,
Bacon
• Whole Wheat waffle (>3
grams per waffle) eggs,
bacon
How Would You Keep Track
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Myfitnesspal.com
Calorieking.com
Nutrihand.com
Mycaloriecounter.com
How and What To Feed: In Summary
1. Structure in meals/rules
2. Physical Activity that is calorie burning and
muscle building
3. Balanced, Mostly Fresh Food Diet
Sweeteners
Sweeteners
• Artificial: synthetic sugar substitutes (calorie free)
• Sugar Alcohols: hydrogenated form of
carbohydrate (caloric)
– Laxative effect, causing bloating, intestinal gas and
diarrhea.
• Novel Sweeteners: Combinations of new
sweeteners.
• Natural Sweeteners: like agave, date sugar, fruit
juice concentrate (caloric)
Artificial
Sugar alcohols
Novel Sweeteners Natural sweeteners
sweeteners
Acesulfame
Erythritol (Z Sweet,
Stevia extracts
Agave nectar
potassium (Sunett,
Sun Crystals)
(Pure Via, Truvia,
Sweet One)
Sweet Leaf, Only
Sweet)
Aspartame (Equal,
NutraSweet)
Hydrogenated
Tagatose (Naturlose
starch hydrolysate
from dairy
products)
Trehalose ( in
mushrooms)
Date sugar
Neotame
Isomalt
Saccharin
(SugarTwin,
Sweet'N Low)
Lactitol
Fruit juice
concentrate
Honey
Sucralose (Splenda)
Maltitol
Maple syrup
Mannitol
Sorbitol
Xylitol (Xylosweet)
Molasses
Mayo Clinic, 2012
Monk Fruit Sweetener
• Hard-skinned fruit
• Extract is 200-300 times sweeter than sugar
• Monk Fruit In The Raw™ has fewer than 3
calories and zero grams of carbohydrates
– Added to beverages, cereal and fruit
– Used in place of sugar in cooking/baking
• Nectresse™ mixed with erythritol
Risks?
• COMPLEX: Debate for decades
• Cancer?
– Studies dating to the 1970s
– Saccharin carried a warning label
• According to the National Cancer Institute and
other health agencies, there's no sound scientific
evidence that any of the artificial sweeteners
approved for use in the U.S. cause cancer or
other serious health problems.
– Approved for pregnancy
– Label on saccharin dropped
Should We Use Them?
• Personal choice
• Artificial means not real
• Intensify cravings?
Should We Use Them?
Bigger Picture Here
The sweeteners are found in packaged and canned
foods and drinks…not fresh food
Supplementation
Some Supplements in PWS
DISCUSSION WITH PHYSICIAN
• Carnitine—cellular metabolism, hypotonia,
alertness
• Coenzyme Q10—energy levels, muscle
function, metabolism
Some Supplements Seen in PWS
• Multivitamin:
“Insurance plan”
• Omega 3’s: inflammation, blood clotting, cell membranes, brain
development
• Calcium:
bone health (if diet is low in dairy)
• Vitamin D: “sunshine vitamin”, bone health, (lots of discussion
lately with the Institute of Medicine)
• Vitamin B12:
synthesis
red blood cell formation, neurologic function and DNA
• Probiotics: promote gut and immune health
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Harvard School of Public Health, Web MD
Best Food Sources of Supplements
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Carnitine: beef, milk
CoQ10: salmon, tuna, liver, whole grains
Omega 3’s: fish, walnuts, spinach
Calcium: dairy, spinach, kale, beans, cereals
Vitamin D: salmon, egg yolk, fortified milk
and cereal
• Vitamin B12: meat, eggs, dairy
• Probiotics: yogurt, kefir
The Ketogenic Diet
• More than 80 years ago reports that seizures
could be prevented by fasting
Ketogenic diet
• Indications?
– Hard-to-control epilepsy by Johns Hopkins Medical
and a few other specialized medical centers.
The Ketogenic Diet
• Body burns fat for energy instead of glucose
• Strictly limits calories
– 90% of diet comes from fat
– 4 times as much fat as protein and carbohydrate
• Ketones measured in urine is a marker that fat
is being used for energy
The Diet
• Butter, bacon, cream, eggs, and peanut butter
• Foods such as bread, pasta, fruits, and
vegetables are severely limited
• Total calories and fluids are also restricted
• Calcium, vitamins and more minerals are
supplemented
• Toothpaste needs to be sugar-free (and
medications)
• Measured to the gram
*Children’s Healthcare of Atlanta, Johns Hopkins Epilepsy Program
The Ketogenic Diet
I am not endorsing this diet for PWS today
The Ketogenic diet may cause side effects :
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Dehydration
Constipation
Vomiting
High cholesterol level
Kidney stones
Behavior changes
Slower growth rates in children
Vitamin/mineral deficiencies
Requires management by a doctor. Qualified registered dietitian creates the plan
Bottom Line
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Fresh food & better balance of CHO, PRO, FAT
Shop the perimeter of the grocery store
Visit Farmer’s Markets
Cook
Read food labels closely
No juice, soda, sport drinks, etc. (splash for water flavor)
Limit sugar intake
Watch/learn about artificial sweeteners
Supplements per physician recommendation
Structure meals/snacks
Intensive physical activity
Follow Me…
FeedPhilosophy
Feeding
Philosophies
Thank you!
EMAIL: [email protected]
OFFICE: 949.607.8248
Melanie R. Silverman MS, RD, IBCLC
Images in lecture from GOOGLE IMAGES