Parents Programme IPWSO Conference in Cambridge 2013 Healthy
eating and nutrition for infants and toddlers and young children
until 5 years with PWS Dr. med. Constanze Lmmer Pdiatrisches
Zentrum St. Bernward Krankenhaus Hildesheim Dr. med. Constanze
Lmmer Childrens Hospital St Bernward Krankenhaus Hildesheim/
Germany
Slide 2
Slide 3
Quality of Life and Life expectancy in PWS is hardly related
with the bodyweight Prader Oslo 1995 if we dont feed them dead,
children with PWS will live to same age as we
Slide 4
The German PWS-Treatment Concept Sport
Slide 5
Nutritional phases of untreated people with PWS Stage 1 poor
feeding, frequently with failure to thrive (age 0-15 mo, mean 9
mo)) Stage 2 hyperphagia leading to obesity in later childhood 2A)
12-3 y, mean 2,5 y: weight increases without a significant change
in appetite or caloric intake 2B) 3-5,25 y, mean 4,5 y: increased
interest in food Stage 3 is characterized by hyperphagia, typically
accompanied by food-seeking and lack of satiety (age 5-13 y, mean
8y) Some PWS adults reach Stage 4 adults no longer have an
insatiable appetite and are able to feel full. (Miller J.)
Slide 6
Stage I: Nutritional situation in the first year of life
Feeding problems caused by muscular hypotonia Sucking difficulties
Failure to thrive-infants do not gain weight Get tired before
finishing feedings Do not progress to solid food by 7 month and
soft table food at age of 10-12 month
Slide 7
Therapeutic aspects for PWS infants < 6 mo If weight gain is
impossible a tube feeding is necessary Continue feeding training
beside the tube is important Add vegetable oil (Rapeoil) if
necessary Do not add sugar or carbohydrates
Slide 8
Nutritional Therapy for PWS infants Nutrition starts with
physiotherapy Special feeding training for example Castillo Morales
Wake for feedings every 4 hour 2/3 of the energy of healthy infants
of the same age needed to gain weight because of the reduced muscle
mass
Slide 9
Nutritional Therapy for PWS infants Adequate nutrition is
necessary for good growth Adequate energy intake Adequate food
composition Weight every week Define energy intake at the weigh
gain compare result with the chart Adapt the energy amount so that
growth along the 3.-10. 25.) percentile is possible
Slide 10
Weight chart
Slide 11
Nutritional supplements Vitamin D is always added during the
first two years, in some countries longer. Often Iron has to be
added In preterm also other vitamins and minerals have to be
supplemented
Slide 12
Energy content 100 g breast milk/ Formula - 68 kcal 100 g
cooked potatoes 70 kcal 100 g vegetable 30 kcal 100 g vegetable+
meet (4:1) 50 kcal 100 g apple 52 kcal 100 g =3 oz
Slide 13
Energy intake If caloric intake is to low, oil can be add
Rapeoil has a excellent composition of the necessary essential
unsaturated fatty acids and is good for brain development 1
teaspoon rapeoil has 40 kcal, the same energy than 60 ml (2 fl. oz)
breast milk or formula
Slide 14
Introduction of food > 6 month The right moment is
individual it depends from the neuromotor development Head control
in an upright position is necessary Pureed food can be started
after 6 month Thick pureed with mall lumps after 8 month Soft
smashed table food after 9 month Soft, bite sized table food
(bread) after 9-12 month
Slide 15
Nutrition in infancy breast milk or formula vegetable-potato-
meat-mash Milk-grain- mash Grain-fruit- mash breakfast Two snacks
lunch dinner
Slide 16
Nutritional Practice in Infancy Educate family in nutrition:
Target: Taking part at the family meals when be able to sit at the
table Give more and more solid food for learning to chew and moving
the tongue Chewing is a good training for speaking Give a piece of
bread instead of cookies No sweet drinks Meals and snacks should be
served at scheduled times
Slide 17
Stage 2: PWS Toddlers between 1-5 years 2 A) 20-31 mo, mean age
2 y: weight increases without a significant change in appetite or
caloric intake 2 B) 3- 5,25 y mean 4,5 y: increasing interest in
food
Slide 18
Nutritional Practice in Toddlers Healthy food means back to the
routes: Cook yourself No Fast food Avoid ready to serve meals Use
vegetables and potatoes Accept the natural tasted of food, avoid
adding sugar Use herbs and spices for better taste Learn to eat
with a spoon
Slide 19
Nutrition for PWS Toddlers Time for shaping taste and learning
to accept healthy food as way of life Children eat the food witch
is often presented on the table Learn to drink water or sugar free
tea, no soft drinks Milk is not a beverage, it is an important
source for Calcium, but it is not made against thirst Watch the
refrigerator carefully, because now the children is able to open
the door
Slide 20
Nutrition for PWS toddlers Clearly defined eating rules Where
are places to eat? When is the right time to eat? What is to be
eaten? No eating in front of the TV Learn the difference between
everyday food and Sunday food Make agreements about nutrition with
grandparents, family, nursery school Nutritional control have to be
in the hands of the parents
Slide 21
Avoid eating from someone else plate: my food is on my plate
Play restaurant: Prepare the plates at the kitchen
Slide 22
Nutrition in young children with PWS Balanced diet Optimierte
Mischkost Energy relations: 50.-55% energy from carbohydrates,
15-18% proteins, 35% fat until age of 2y, than 25-30% Restricted
use of sugar and high-glycemic- index-carbohydrates Prefer
unsaturated fatty acids (oil) Add free-caloric drinks Add vitamins
and minerals if necessary
Slide 23
carbohydrates proteins fat glycogenreserve Body fat
thermogenesis activity Weight control means balance of energy
Slide 24
Body composition Blue lean body mass Whitebody fat webe PWS
normal
Slide 25
Energy expenditure depends on muscle mass Even PWS children
with normal weight and BMI have a higher % of bodyfat PWS children
need 2/3 of the energy of healthy toddlers of the same age to
grow
Slide 26
Write a nutrition plan 5-(7) meals breakfast and snack 1/3
lunch 1/3 snack and dinner 1/3 ( incl bed hop) Calculate the
necessary energy intake 1.-3. y 500-700 kcal (ca 60 kcal/kg)
Slide 27
Check the results with help of the growth charts
Slide 28
Use the knowledge about energy content of food groups
Slide 29
PWS food is healthy food for everyone
Slide 30
Vegetables Low caloric density Less then 30 kcal/ 100g (expect
corn, kidney beans) Rich on vitamins and minerals Offer vegetable
as a snack Gives nice colors to meals Can be given as finger food
Training for chewing muscles
Slide 31
Hints for children which dont like vegetables Create a pizza
with vegetables instead of salami Vegetable soups Desensibilisation
for acceptance of the taste Parents should be model for their child
Pasta with vegetables sandwiches
Slide 32
Be a model for your child
Slide 33
Fruits 2x a day Have vitamins and minerals like vegetables Main
energy source: carbohydrates mostly with less glycemic index Free
of fat Suitable as a snack Avoid bananas and grapes because of the
higher sugar content Prefer apples, cherries, berries, orange,
plums, grapefruits pears
Slide 34
Products from grain, pasta, potatoes Main component: starch
Beside proteins Vitamins, minerals Less fat, no cholesterol Choose
the right amount: 15 g cereals + 75 ml low fat milk 110 kcal
(weight!) the cereals
Slide 35
Carbohydrates starch metabolized to glucose
EnergyGlykogenFat
Slide 36
Remember the special role of Insulin when you choose food and
arrange meals Insulin has the task to transport glucose in the
muscles Stops Neogenesis of glucose in the liver Build up fat
reserves Stops fat reduction High insulin levels in the blood will
be followed by insulin resistance and later diabetes
index-carbohydrates weight reduction possible
Slide 37
Hyperinsulinemia Insulin anabolic hormone Makes feeling hungry
Low-glycemic-index-carbohydrates prevent hyperinsulinemia and make
weight reduction possible It is important which kind of snack we
offer, although it is on the same amount of energy
Slide 38
Cereals for Breakfast or Sweets? In 100gFroot Loops
SmacksCornflakesToppasHafermsli Carb g8084 7250 Sugar g25438173,9
Children < 5 y : 1 Portion = 20 g
Slide 39
Milk and milk products Important source for Calcium Energy from
protein, milk fat and milk sugar Children age 1-2 y need 250-300 ml
(8,5-10,5 oz) 3-6 y 400 ml (13,5 oz) 7-10 y 500 ml >10 y 600 ml
100 ml milk = 15 g cheese
Slide 40
Milk products: choose the right cheese Calcium source for bones
to prevent osteoporosis Chose low fat milk and milk products Cheese
70% Fat i d 130kcal/30g 40% Fat i d 85kcal/30g 10% Fat i d
35kcal/30g
Slide 41
Meat, sausage, fish and eggs Important for animal protein Rich
on iron For toddlers and young children 3 times a week meat, 3
times a week 2 slices of sausages or lean ham are recommended Once
a week fish 2 eggs per week
Slide 42
Meat: hand rule
Slide 43
Avoid hidden fat 1 Pizza Salami44 g Fat fried sausage43 g Fat
100 g potatochips 40 g Fat Salami30 g 15 g Fett If you see meat in
the sausage it is fine
Slide 44
But give healthy fat Is obligate in the daily nutrition To less
fat will stop the growth of your child 35% of energy from fat until
age of 2y, than 25-30% Oil is better than butter Unsaturated fatty
acids are important Rapeoil and Fishoil contain the important PUVAs
for better brain development
Slide 45
Sweets, Cookies, Biscuits, Ice cream Start when the child can
understand, that this is something special Max 1 childs hand
full
Slide 46
Ten rules for healthy food Varied, but to much can scare Enough
grain and potatoes Plenty of vegetables Daily low fat milk and
milkproductes Less fat and no high caloric food Avoid sugar and
high-glycemic- index-carbohydrates 2 2.5 l of free caloric drinks
per day Cook tasty and with care on the vitamins Avoid fried food
Take your time when you are eating Take 45 min for your daily
activity
Slide 47
Reduce energy density - Look for an alternative
Slide 48
Nutrition for PWS Clearly defined eating rules Where are places
to eat? When is the right time to eat? What is to be eat? No eating
in front of the TV Learn the difference between everyday food and
Sunday food Make agreements with grandparents, family, nursery
school, school Nutritional control have to be in the hands of the
parents
Slide 49
Everyday nutrition- Sunday nutrition People with PWS like their
daily routine Routines give them safety Changes irritated them and
can trigger behavioral problems Nutrition is the most sensitive
point Discuss special situations and exceptions hardly in advance
Adultes with PWS can understand the difference between everyday and
Sunday/holiday
Slide 50
Psychological Food Security Controlled food access means No
doubt when, what, and how much the person with PWS will eat. No
hope of receiving any more No disappointment due to false
expectations (L.M: Gourash. J.Forster)
Slide 51
Nutrition for PWS Toddlers Be strong! Learn to say no Avoid
boredom Keep the child busy between the meals Put portion on
seperate plates for each person. Put only things in the middle of
the table, everyone can add (vegetable) Clear table, talk
after
Slide 52
Everyday nutrition- Sunday nutrition Children with PWS like
their daily routine Routines give them safety Changes irritated
them and can trigger behavioral problems Nutrition is the most
sensitive point When PWS children in the age of visiting a nursery
school they can understand the difference between everyday and
Sunday/holidays, than you can start with exceptions Explain the
exceptions hardly in advance
Slide 53
There is no successful weight management without physical
activity Daily 30-45 min for everybody Take into account individual
increased risk for osteoporosis and other handicaps Spark interest
in doing exercises Increase energy expenditure Build lean body mass
Prevent obesity
Slide 54
Quality of Life and Life expectancy in PWS is hardly related
with the bodyweight Prader Oslo 1995 if we dont feed them dead,
children with PWS will live to same age as we