Parents ‘ Programme IPWSO Conference in Cambridge 2013 Healthy eating and nutrition for infants and toddlers and young

1. Parents‘ ProgrammeIPWSO Conference in Cambridge 2013Healthy eating and nutrition for infants and toddlers and young children until 5 years with PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward Krankenhaus Hildesheim Dr. med. Constanze Lämmer Childrens Hospital St Bernward Krankenhaus Hildesheim/ Germany

3. Prader Oslo 1995 „if we don‘t feed them dead, children with PWS will live to same age as we“ Quality of Life and Life expectancy in PWS is hardly related with the bodyweight

4. TheGerman PWS-Treatment Concept Sport

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 havean insatiable appetite and are able to feel full. (Miller J.)

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

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

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

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

10. Weight chart

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

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

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

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

15. Nutrition in infancy breakfast Twosnacks Grain-fruit- mash breast milk orformula Milk-grain- mash lunch vegetable-potato- meat-mash dinner

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

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

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

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

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

21. Avoid eating from someone else plate: my food is on my plate Play restaurant: Preparetheplatesatthekitchen

22. Nutrition in young children with PWS • Balanced diet “OptimierteMischkost” • 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

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

24. Body composition PWS PWS normal Blue leanbodymass White bodyfatwebe

25. 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 Energy expenditure depends on muscle mass

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)

27. Check the results with help of the growth charts

28. Use the knowledge about energy content of food groups

29. PWS food is healthy food for everyone

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

31. Hints for children which don‘t 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

32. Be a model for your child

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

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

35. starch metabolized to glucose Carbohydrates Energy Glykogen Fat

36. Rememberthespecialroleof Insulin whenyouchoosefoodandarrangemeals • 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

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

38. Cereals for Breakfast or Sweets? Children < 5 y : 1 Portion = 20 g

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

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 d35kcal/30g

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

42. Meat: hand rule

43. Avoid hidden fat • 1 Pizza Salami 44 g Fat • fried sausage 43 g Fat • 100 g potatochips 40 g Fat • Salami 30 g 15 g Fett • If you see meat in the sausage – it is fine

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

45. Sweets, Cookies, Biscuits, Ice cream Start when the child can understand, that this is something special Max 1 child‘s handfull

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

47. Reduce energy density - Look for an alternative

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

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

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)