Transcript Geschlechtersdeterminierung bei Hermaphroditismus

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
Constanze Lämmer
Dr. med.
Zentrum
ChildrensPädiatrisches
Hospital St Bernward
Krankenhaus
Hildesheim/Krankenhaus
Germany
St. Bernward
Hildesheim
Quality of Life and Life expectancy in
PWS is hardly related with the bodyweight
Prader Oslo 1995
„if we don‘t feed them dead, children
with PWS will live to same age as we“
The German PWS-Treatment Concept
Sport
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.)
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
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
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
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
Weight chart
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
Energy content
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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
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
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
Nutrition in infancy
breakfast
Grain-fruitmash
breast milk
or
formula
Milk-grainmash
vegetable-potatomeat-mash
Two
snacks
lunch
dinner
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
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
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
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
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
Avoid eating from someone else plate: my
food is on my plate
Play restaurant: Prepare the plates at the kitchen
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-glycemicindex-carbohydrates
– Prefer unsaturated fatty acids (oil)
– Add free-caloric drinks
• Add vitamins and minerals if necessary
Weight control means balance of energy
carbohydrates
proteins
fat
glycogenreserve
Body fat
thermogenesis
activity
Body composition
PWS
normal
Blue
White
lean body mass
body fatwebe
PWS
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
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)
Check the results with help of the growth
charts
Use the knowledge about energy content
of food groups
600
530
500
400
energy in kcal/100g
400
300
300
200
130
130
noodles
cooked
lean
meat
110
100
70
50
30
0
vegetables
fruits
potatoes
rice
cooked
fat meat
sausages
cookies
chocolate
PWS food is healthy food for everyone
Vegetables
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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
Hints for children which don‘t like
vegetables
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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
Be a model for your child
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
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
Carbohydrates
starch
metabolized to
glucose
Energy
Glykogen
Fat
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
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
Cereals for Breakfast or Sweets?
In 100g
Froot
Loops
Smacks
Cornflakes
Toppas
Hafermüsli
Carb g
80
84
84
72
50
Sugar g
25
43
8
17
3,9
Children < 5 y : 1 Portion = 20 g
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
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
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
Meat: hand rule
Avoid hidden fat
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1 Pizza Salami
fried sausage
100 g potatochips
Salami 30 g
44 g Fat
43 g Fat
40 g Fat
15 g Fett
• If you see meat in the sausage – it is fine
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
Sweets, Cookies, Biscuits, Ice cream
Start when the child can understand, that
this is something special
Max 1 child‘s
hand full
Ten rules for healthy food
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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
Reduce energy density - Look for an
alternative
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
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
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)
Nutrition for PWS Toddlers
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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
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
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
Quality of Life and Life expectancy in
PWS is hardly related with the bodyweight
Prader Oslo 1995
„if we don‘t feed them dead, children
with PWS will live to same age as we“
Have fun with a healthy family diet!