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Caregivers ‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS

Caregivers ‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS . Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward Krankenhaus Hildesheim. Dr. med. Constanze Lämmer Childrens Hospital St Bernward Krankenhaus Hildesheim/ Germany.

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Caregivers ‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS

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  1. Caregivers‘ Programme IPWSO Conference in Cambridge 2013Nutrition andEndocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward Krankenhaus Hildesheim Dr. med. Constanze Lämmer Childrens Hospital St Bernward Krankenhaus Hildesheim/ Germany

  2. Focus on six topics • energy intake- body composition • Need to individualize the nutrition in the setting of food security • Role of insulin • Possibility to low caloric density of meals • Rules for everyday and Sunday nutrition • Endocrine problems in PWS adults

  3. TheGerman PWS-Treatment Concept Sport

  4. Weight control means balance of energy carbohydrates proteins fat glykogenreserve Body fat thermogenesis activity

  5. Body composition PWS PWS Blue lean body mass White body fat weiß: Fettgewebe

  6. Obese PWS boywith BMI 48 kg/m² PWS young adultes with normal BMI but higher % ofbodyfat Energyexpendituredepends on musclemass Basic energyexpenditure1100- 1200 kcal

  7. Recommended caloric intake PWSA guidelines: • To maintain weight: 8-11 kcal/cm/day • To reduce weight 7 kcal/cm/ day German Recommendations: • To maintain weight: 2/3 of a normal weight person of the same age 12-14 y 1100-1300 kcal/day > 15 y 1200-1600 kcal No diet under 1000 kcal/day (cave lack of vitamins and minerales)

  8. Energy intake • Has to be individualized in PWS • Weekly weighing controls the success • An individual weight/BMI aim (= BMI 20-25 kg/m²) has to be defined • Adapt the energy intake to weight development • If the energy intake of the served meals and weight development doesn’t fit together, search for gaps in the fence!

  9. PWS weight management means individual size of meals under food security

  10. 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

  11. 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

  12. Cerealsfor Breakfast or Sweets? 1 Portion = 35 g

  13. Focus on five topics • energy intake- body composition • Need to individualize the nutrition in the setting of food security • Role of insulin • How to lower caloric density of meals with easy rules • Nutrition every days and on Sundays

  14. Usetheknowledgeaboutenergycontentoffoodgroups

  15. PWS food is healthy food for everyone

  16. Lunch: Plate rule

  17. Vegetables • Low caloric density • Lessthen 30 kcal/ 100g (expectcorn, kidneybeans) • Rich on vitaminesandminerales • Offervegetableas a snack • Givesnicecolourstomeals • Can begivenas finger food • Training forchewingmuscles

  18. Tipps forpeoplewhichdon‘tlikevegetables • Create a pizzawithvegetablesinsteadofsalami • Vegetablesoups • Pasta withvegetables • sandwiches • „Desensibilisation“ foracceptanceofthe taste • Offervegetable positiv • Parents/Caregiversshouldbemodelforthepersonwith PWS

  19. Fruits – 2x a day • Have vitamines and minerales 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

  20. Milkproductes: choosetherightcheese -Calcium source for bones to prevent osteoporosis Cheese 70%Fat i d 130kcal/30g 40% Fat i d 85kcal/30g 10% Fat i d35kcal/30g

  21. Meat, sausage, fishandeggs • Importantforanimalprotein • Rich on iron • Don‘tservemeattooften: • For adultes 3 times a weekmeat, 2 times a week 3 slicesofsausagesorleanhamarerecommended • Once a weekfish • 2 eggs per week

  22. Conclusion: Tenrulesforhealthyfood • 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

  23. 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

  24. 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)

  25. 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

  26. Short remarks on endocrinologicproblems in adultes with PWS • Diabetes • Hypothyreoidism • Hypogonadism • Growth hormonedeficiency

  27. Diabetes • High insulin levels in the blood will be followed by insulin resistance and later diabetes • Obesity is a high risk factor for diabetes • but also PWS adultes with normal weightcome down withdiabetes • High bloodsugardoesn‘t hurt, so whenclinicalsignsoccure, diabetesoftenrunmorethan 5 years

  28. Clinical signsofdiabetes • Frequentgeneralinfections • not healingskininfectionsandmycosis • Reducedappetite • Reducedactivityandinterest • Rememberdiabeteswhensomeoneobese lose weightwithoutchanginganything

  29. Diabetes • Diagnostics • Examinebloodsugareveryyear • Test bloodsugar bevor and after a meal • Orarrange a glucosetolerancetest • Also check bloodpressureandbloodlipids • Therapy • Dietandincreaseactivityto lose weight • Oral Medication • Insulin

  30. Hypothyreoidism • Clinical signs: • Decreasedactivity • Weightgain • Increasedfatigue • Depression • IncreasingEdema • Obstipation • Loss ofconcentration

  31. Hypothyreoidism • Diagnostics: • Blood test • Thyroid-Antibodies • Ultrasound • Therapy: Substitution with L-Thyroxin

  32. Hypogonadism • Lateand/orincompletepubertaldevelopment • Consequences • Osteoporosis • Infertility • Lessself-esteem

  33. Lateorincompletepuberty • Missingsignesofpuberty after theageof 14 y in boys • volumeofthetestes<4 ml MissingBreastdevelopment after ageof 13,5 y in girls Nomenarcheatageof15 y

  34. Diagnostics: Test thefeed-backcontrolsystemandthefunctionofthegonades

  35. Therapeuticaimes • normal puberty • acceptanceoftheownbody • Betterself-esteem • Preventosteoporosis • Fertitlity?

  36. Therapy : hormonereplacement • Testosteron (male) • Injectionsorpatchesorgelapplication • Estrogen (female) • Tabletsorpatches • Prepareformenarche/ menstruation • Add gestagen, whenmenarcheoccur • Takes the personal situation in account! • Sometimesbehavioralproblemsoccure in male • Somewomenhaveproblemsto handle menstruationeverymonth

  37. Fat GH reduces body fat GH increases vein protective HDL-cholesterol Mind GH increases activity GH normalizes psycho-social well-being Heart GH normalizes cardiac muscle mass and improves cardiac performance Bones GH increases bone mineral density Protein GH transports more protein into cells GH increases lean body mass Water GH normalizes fluid balance GH

  38. Growth hormonedeficiency • Growth hormonedeficiency also occures in adultes with PWS • Itcanbetestedwithstandardisedtests • Some adultes with PWS fullfillthecriteriafortreatment • The Rules for GH treatment in adulthooddifferverymuchfromcountrytocountry

  39. Thankyouforyourattention!

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