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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

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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

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The German PWS-Treatment Concept Sport

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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.)

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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

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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

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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

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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

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Weight chart

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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

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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

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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

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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

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Nutrition in infancy breast milk or formula vegetable-potato- meat-mash Milk-grain- mash Grain-fruit- mash breakfast Two snacks lunch dinner

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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

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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

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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

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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

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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

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Avoid eating from someone else plate: my food is on my plate Play restaurant: Prepare the plates at the kitchen

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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

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carbohydrates proteins fat glycogenreserve Body fat thermogenesis activity Weight control means balance of energy

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Body composition Blue lean body mass Whitebody fat webe PWS normal

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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

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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)

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Check the results with help of the growth charts

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Use the knowledge about energy content of food groups

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PWS food is healthy food for everyone

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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

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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

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Be a model for your child

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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

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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

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Carbohydrates starch metabolized to glucose EnergyGlykogenFat

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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

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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

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Cereals for Breakfast or Sweets? In 100gFroot Loops SmacksCornflakesToppasHafermsli Carb g8084 7250 Sugar g25438173,9 Children < 5 y : 1 Portion = 20 g

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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

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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

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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

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Meat: hand rule

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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

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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

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Sweets, Cookies, Biscuits, Ice cream Start when the child can understand, that this is something special Max 1 childs hand full

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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

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Reduce energy density - Look for an alternative

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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

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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

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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)

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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

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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

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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

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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

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Have fun with a healthy family diet!