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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EMAIL: melanie@feedingphilosophies.comOFFICE: 949.607.8248

Melanie R. Silverman MS, RD, IBCLCImages in lecture from GOOGLE IMAGES