Prader-Willi

Patient, Parent and Caregiver Guide

to Nutrition
 INTRODUCTION TO
PRADER-WILLI

Prader-Willi is a rare genetic condition that

occurs in about 1 out of every 15,000 births.
Prader-Willi symptoms may present in six
different phases that we will review in this
guide. It is important to note that these six
different phases are characterized by different
eating behaviors that must be attended to in
order to have the best possible outcome.

PHASES OF PRADER-WILLI
Phase 1a: Hypotonia and feeding problems
Phase 1b: Improved feeding and growth
Phase 2a: Weight gain without increase in appetite
Phase 2b: Weight gain with increase in appetite and calories
Phase 3: Hyperphagia
Phase 4: Appetite is no longer insatiable
NUTRITIONAL PHASE 1A
hypotonia and feeding problems

Stage 1a usually occurs in infants until

9 months old and is characterized by Phase 1a
hypotonia (decreased muscle tone) and Your child may need to be
difficulty feeding. on tube feeds after 9
months depending on their
growth and progress.
Difficulty feeding and characteristics
may include: Your dietitian and patient
care team will lead you
through the tube feeding
Trouble breastfeeding process.
Needs assistance with feeding tube
Decreased appetite
Does not cry or show interest in food
Weak cry
Failure to thrive

During this stage, many infants utilize

tube feeding in order to receive proper
nutrition. Tube feeding should always
be supervised by a dietitian and the
patient's care team.
NUTRITIONAL PHASE 1B
improved feeding and appropriate growth

Stage 1b usually occurs between 9 Phase 1b

months old and 25 months old and is This is an important time to
establish a meal and snack
characterized by improved feeding and
schedule.
appropriate growth.
Be sure to include a wide
variety of foods on your
Characteristics of nutritional phase 1b
child's plate; vegetables,
may include: fruits, whole grains, protein.

Experiment with different

No longer needs assistance feeding
foods so your child becomes
Grows along growth curve accustomed to new flavors
Normal appetite and textures.

Always consult your

dietitian when necessary.
NUTRITIONAL PHASE 2A
weight increases without an increase in appetite

Stage 2a usually occurs between the Phase 2a

ages of 2 and 4.5 years old among Continue to utilize the meal
Prader-Willi patients. During this and snack schedule so your
child is accustomed to their
nutritional phase, children will gain schedule.
weight, however without an overt
Experiment with different
increase in appetite or caloric intake.
foods during this time. Be
sure to incorporate fruits,
Characteristics of nutritional phase vegetables, whole grains
and lean proteins in their
2a may include:
meals.

No increase in appetite Encourage movement

through play and physical
Weight gain
activity that your child will
Normal appetite enjoy.
Rapid weight gain is possible if
food not restricted
 NUTRITIONAL PHASE 2B
weight gain, increased appetite and interest in food

Stage 2b usually occurs between 4.5 to 8

Phase 2b
years old among children with Prader-Willi.
Be sure to follow a strict
Nutritional phase 2b shows an increase in
meal and snack schedule so
weight, as well as more interest in food and your child knows when to
food-related things. expect their next meal.

Continue to include a
Characteristics of nutritional phase 2b may variety of food on their
include: plates to allow for
exploration.

Increased interest in food Consult with your dietitian

Concerned about when next mea/snack is regarding how many
calories should be in your
Will eat more food than a typical child if
child's day.
allowed
Can sense fullness Begin measuring food with
a food scale.
Will stop eating voluntarily
Weight gain
NUTRITIONAL PHASE 3
hyperphagia

Stage 3 usually begins around 8 years old Phase 3

and can last until or through much of Continue following the
adulthood. Nutritional phase 3 is meal and snack schedule.

characterized by hyperphagia.
Use a food scale to measure
Hyperphagia is an insatiable appetite and a foods.
constant desire to consume food. This can
It may be helpful to add
cause rapid weight gain among patients
locks to the fridge or pantry
with Prader Willi. to avoid excess eating
during non-meal times.

Characteristics of nutritional phase 3 may Consult with your dietitian

include: regarding how many
calories your child should
be consuming.
Always thinking about food
Rapid weight gain
Rarely feels full after eating
May eat food from the garbage, others
plates or raw food
May steal food
Temper tantrums related to food
Consume non-food items
Sneak food from cabinets and fridge
 NUTRITIONAL PHASE 4
appetite is no longer insatiable

Stage 4 can occur in some Prader-Willi

patients. In some cases, Prader-Willi Phase 4
patients in adulthood will be able to Continue to eat a balanced

feel full and food-seeking behaviors diet filled with fruits,

veggies, lean protein and
will decrease. whole grains.

Characteristics of this phase may It is important to keep up

with an exercise routine in
include: adulthood.

Increased satiety from meals Consult your dietitian about

calorie recommendations as
Decreased food-seeking behaviors they may change
Ability to stop eating throughout the different
phases.

It is important to note that not all

patients with Prader-Willi reach this
nutritional phase. Each patient with
Prader-Willi will have a different
experience depending on a myriad of
factors.
 Helpful
Resources
Exercise guide, Meal plan ideas, Food scale tips,
Helpful kitchen tools and Low-calorie alternatives
 FOOD SCALE HOW-TO

Since your child will likely be on a restricted calorie diet, a food scale is a very important
tool to measure their portions and keep their calories at the level directed by their dietitian.
Each food scale is slightly different, however, here is a basic run-down of how to use one.

Step one Measuring Tips

Place your bowl or container that you Be sure to look at food labels
want to put the food in on the scale to know how many calories
(the container should not have food in are in a serving size in grams.
it yet)
If your food does not have a
food label, use the internet to

Step two look up the nutrition facts.

If you are making a meal or

Zero out the scale by pressing
snack with more than one
the "tare" button on the scale ingredient, be sure to measure
them separately.

Step three

Add the food to the container

that you would like to measure.
The scale will show the weight
in grams.
 HELPFUL KITCHEN TOOLS

In order to accurately measure food, here are some important tools that will be helpful.
Patients with Prader-Willi may also benefit from using different utensils and plates to make
mealtime less stressful for your family.
Measuring Cups
Food Scale

Sectioned Plates Food Picks

Water Bottle
Chopsticks

Patients with Prader-Willi may have trouble with

water intake. It may help to purchase a fun and
colorful water bottle or cup that your child enjoys
drinking out of.
MEAL AND SNACK IDEAS

Patients with Prader-Willi disease must consume a low-calorie

diet due to weight gain and baseline decreased energy needs. In
order to keep weight under control, it is necessary to consult
your dietitian to determine how many calories is sufficient for
your child. The following meal and snack ideas are low-calorie
suggestions to complement your dietitian meetings.

Breakfast

1 egg - 70 kcal 2 hard boiled eggs (100 g) - 155 kcal

1/2 banana - 65 kcal Fresh or frozen blueberries (100g) - 60 kcal
1 tbsp peanut butter - 94 kcal Total calories - 215 calories
Total calories - 229 calories

Scrambled egg white (200g) - 104 kcal

Fat-free shredded cheese (28g) - 45 kcal
Fresh or frozen blueberries (100g) - 60 kcal
Low fat greek yogurt (100g) - 95kcal
Total calories - 149 calories
Fresh or frozen blueberries (100 g) - 60 kcal
Plain cheerios (28g) - 101 kcal
Raspberries (100g) - 57 kcal
Total calories - 256 calories
Low fat greek yogurt (100g) - 95 kcal
Plain cheerios (28g) - 101 kcal
Total calories - 253 calories

Multigrain bread (36g) - 100 kcal

Low fat cottage cheese (113 g) - 80 kcal 2 hard boiled eggs (100 g) - 155 kcal
Fresh or frozen strawberries (100g) - 35 kcal Fresh or frozen blueberries (100g) - 60 kcal
Total calories - 215 calories Total calories - 215 calories
Lunch

Turkey Wrap w/ side of strawberries Turkey rice cake w/ a side of carrots +

Low carb tortilla (43g) - 70 kcal hummus
3 slices of turkey (48g) - 31 kcal 2 rice cakes (18 g) - 70 kcal
1 tbsp plain hummus (15g) - 40 kcal 6 slices of turkey (98 g) - 63 kcal
Sliced tomato (50g) - 11 kcal 2 tbsp light mayo (30 g) - 70 kcal
Romaine lettuce leaves (20g) - 5 kcal 20 baby carrots (200 g) - 80 kcal
Fresh or frozen strawberries (100g) - 35 kcal 2 tbsp plain hummus (30 g) - 80 kcal
Total calories - 192 calories Total calories - 363 calories

Tuna salad toast w/ side of almonds Turkey lettuce wrap w/ a side of blueberries
Canned tuna, in water (85g) - 109 kcal 6 leaves of romaine lettuce (78 g) - 12 kcal
1 tbsp light mayo (15g) - 35 kcal 6 slices of turkey (98 g) - 63 kcal
1 slice multigrain bread (36g) - 100 kcal 2 tbsp light mayo (30 g) - 70 kcal
Almonds, no salt (15g) - 82 kcal Fresh or frozen blueberries (100g) - 60 kcal
Total calories - 329 calories Total calories - 205 calories

Greek salad w/ a side of apple Hummus + Cheese roll-ups w/ a side of

3 cups mixed salad greens (105 g) - 18 kcal almonds
1/2 cup feta (75g) - 41 kcal 2 slices reduced-fat cheese (40 g) - 120 kcal
1 cup cucumber (120g) - 21 kcal 2 tbsp hummus (30g) - 70 kcal
1 cup grape tomatoes (152 g) - 50 kcal Almonds, no salt (15g) - 82 kcal
1/4 cup chickpeas (47 g) - 97 kcal Total calories - 272 calories
2 tbsp fat free Greek dressing (30g) - 15 kcal
1/2 small apple (85 g) - 42 kcal
Total calories - 284 kcal
Dinner

Chicken caesar salad Grilled chicken + veggies

3 cups mixed salad greens (105 g) - 18 kcal Grilled chicken (96 g) - 142 kcal
2 tsp reduced-fat parmesan (5 g) 25 kcal Zucchini (196 g) - 33 kcal
2 tbsp light caesar dressing (30 g) - 60 kcal 1/2 bell pepper (75 g) - 30 kcal
Grilled chicken (96 g) - 142 kcal Total calories - 205 kcal
Total calories - 245 calories

Grilled Salmon + veggies Quinoa power bowl

Grilled salmon (114 g) - 234 kcal Quinoa (100 g) - 120 kcal
Zucchini (196 g) - 33 kcal Zucchini (196 g) - 33 kcal
1/2 bell pepper (75 g) - 30 kcal 1/2 bell pepper (75 g) - 30 kcal
Total calories - 297 calories Sliced tomato (50g) - 11 kcal
Grilled chicken (96 g) - 142 kcal
Total calories - 336 calories

Turkey Wrap w/ side of carrots Hummus + Cheese roll-ups w/ a side of

Low carb tortilla (43g) - 70 kcal almonds
3 slices of turkey (48g) - 31 kcal 2 slices reduced-fat cheese (40 g) - 120 kcal
1 tbsp plain hummus (15g) - 40 kcal 2 tbsp hummus (30g) - 70 kcal
Sliced tomato (50g) - 11 kcal Almonds, no salt (15g) - 82 kcal
Romaine lettuce leaves (20g) - 5 kcal Total calories - 272 kcal
20 baby carrots (200 g) - 80 kcal
Total calories - 237 calories
Snacks

Cottage cheese + berries String cheese + carrots

1/2 cup cottage cheese (113g) - 90 kcal Light string cheese stick (24g) - 50kcal
1/2 cup raspberries (75g) - 40 kcal 20 baby carrots (200 g) - 80 kcal
Total calories - 130 kcal Total calories - 130 kcal

Apple w/ yogurt peanut butter dip Celery + peanut butter

1 small apple , sliced (165 g) - 86 kcal 4 pieces of celery (16 g) - 5 kcal
1 tbsp peanut butter (16 g) - 95 kcal 1 tbsp peanut butter (16 g) - 95 kcal
3/4 cup fat-free greek yogurt (170 g) - 90 kcal Total calories - 100 kcal
Sprinkle of cinnamon
Total calories - 271 kcal

Greek yogurt w/ berries Hard-boiled egg + strawberries

3/4 cup fat-free greek yogurt (170 g) - 90 kcal 1 hard-boiled egg (50 g) - 72 kcal
1/2 cup raspberries (75 g) - 40 kcal 1 cup strawberries, sliced (166 g) - 55 kcal
Total calories - 130 kcal Total calories - 127 kcal
 EXERCISE GUIDE

Exercise and regular physical activity for patients with Prader-Willi disease is very
important for bones, health, muscle retention and keeping weight under control.
Exercise can also aid in emotional regulation and stress relief for children with
Prader-Willi. Your child may also benefit from working with a physical therapist.

When first beginning exercise, it is important to start small. Maybe set one small
exercise goal for the first few weeks. As exercise becomes more and more a part of
your routine, begin to make the goals a little more challenging.

Exercise Goal Ideas

Try to find fun ways to
incorporate activity into your
3x a week we will walk
child's day. It may be helpful to
outside for 15 minutes include the whole family for
some motivation!
3x a week we will swim in the
pool for 30 minutes

3x a week we will incorporate

muscle strengthening
exercises for 15 minutes
 EXERCISE GUIDE

Both aerobic and strength exercises are important for patients with Prader-Willi. It is
important to try and incorporate each of these kinds of exercises into their routine. Aerobic
exercises increase our heart rate and work on our cardiovascular fitness while strength
exercises work our muscles to make them stronger.

Aerobic Exercise Ideas Exercise Tips

Establish an exercise schedule
1. Walking that you can stick to.

2. Cycling
Make exercise fun and involve
3. Skipping the whole family.

4. Swimming
Competition may help
5. Dancing motivate them to move their
6. Roller skating bodies more.

Ensure a variety of physical

Strength Exercise Ideas activity options for your child.

1. Squats
2. Lunges
3. Stretching
4. Resistance band
exercises
5. Yoga
6. Stretching
LOW-CALORIE FOOD
ALTERNATIVES

1. Healthy Life 35 Calorie Wheat Bread

2. Aunt Millie Live Light 40 Calorie Whole Low-calorie Tips
Grain Bread To find low-calorie packaged
3. Rice Cakes foods in the grocery store,
4. Sugar-free jelly look for low-carb, sugar-free
5. Plain greek yogurt, low-fat or fat-free or reduced-fat foods
6. Popcorn (without butter)
7. Egg whites Be sure to check the nutrition
8. Shirataki Noodles labels before purchasing a
9. Cottage cheese, low-fat or fat-free new food from the store. Pay
10. Sugar-free jello careful attention to the
11. Kraft Fat-free mayo serving size, calories and
12. Palmini pasta sugar content.
13. Outer Aisle cauliflower pizza crust and
wrap Fruits and vegetables are the
14. Simple Truth freeze-dried smoothie best low-calorie option to
melts purchase for snacks and meals
15. Gourmet Basics Smart Fries
16. Maple Grove Farms sugar free syrup
17. I Can't Believe It's Not Butter