understanding optimal nutrition in prader-willi syndrome melanie silverman ms, rd, ibclc registered...
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Understanding Optimal Nutrition In Prader-Willi
Syndrome
Melanie Silverman MS, RD, IBCLCRegistered DietitianLactation Consultant
In the office…• 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 “i-anythings” 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
Whole Grains
Vegetables
Fruit (but no fruit juice)
High Quality Protein
Dairy (like yogurt, cheese, milk)
Fats like oils, flax, avocado
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/porkPlant is incomplete protein:
fruits/vegs/grains/nuts• Macronutrient manipulation in weight loss programs• Satiety effect? Maybe.
Harvard School of Public Health, 2012
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 plant-based 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”
• 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?
42LESSON: 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• Dissolves in water to form a
gel-like material. • It can help lower blood
cholesterol and glucose levels.
• EXAMPLES: Oats, peas, beans, apples, citrus fruits, carrots, and barley
Insoluble• Promotes the movement of
material through your digestive system and increases stool bulk
• 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)
Protein(P)
Fat (F)
• Eggs: P, F• 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 Prader-Willi 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 PRO30% 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 CHOPRO: 300/4= 75 grams of PROFAT: 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
• Spaghetti and sauce
• Bagel and cream cheese
• Buttermilk Pancakes, Eggs, Bacon
After• Taco salad
• Spaghetti SQUASH and sauce
• Greek yogurt and granola
• Whole Wheat waffle (>3 grams per waffle) eggs, bacon
How Would You Keep Track
• Myfitnesspal.com• Calorieking.com• Nutrihand.com• Mycaloriecounter.com
How and What To Feed: In Summary
1. Structure in meals/rules2. Physical Activity that is calorie burning and
muscle building3. 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 sweeteners Sugar alcohols Novel Sweeteners Natural sweeteners
Acesulfame potassium (Sunett,
Sweet One)
Erythritol (Z Sweet, Sun Crystals)
Stevia extracts (Pure Via, Truvia, Sweet Leaf, Only
Sweet)
Agave nectar
Aspartame (Equal, NutraSweet)
Hydrogenated starch hydrolysate
Tagatose (Naturlose from dairy products)
Date sugar
Neotame Isomalt Trehalose ( in mushrooms)
Fruit juice concentrate
Saccharin (SugarTwin,
Sweet'N Low)
Lactitol Honey
Sucralose (Splenda) Maltitol Maple syrup
Mannitol Molasses Sorbitol Xylitol (Xylosweet) 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: red blood cell formation, neurologic function and DNA synthesis
• Probiotics: promote gut and immune health
• Harvard School of Public Health, Web MD
Best Food Sources of Supplements
• 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 :
– 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
• 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
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Feeding Philosophies
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Thank you!
EMAIL: [email protected]: 949.607.8248
Melanie R. Silverman MS, RD, IBCLCImages in lecture from GOOGLE IMAGES