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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
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

focus on six topics
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
weight control means balance of energy
Weight control means balance of energy

carbohydrates

proteins

fat

glykogenreserve

Body fat

thermogenesis

activity

body composition
Body composition

PWS

PWS

Blue lean body mass

White body fat

weiß: Fettgewebe

energy expenditure depends on muscle mass
Obese PWS boywith BMI 48 kg/m²

PWS young adultes with normal BMI but higher % ofbodyfat

Energyexpendituredepends on musclemass

Basic energyexpenditure1100- 1200 kcal

recommended caloric intake
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)

energy intake
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!
slide11

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
hyperinsulinemia
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
focus on five topics
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
vegetables
Vegetables
  • Low caloric density
  • Lessthen 30 kcal/ 100g (expectcorn, kidneybeans)
  • Rich on vitaminesandminerales
  • Offervegetableas a snack
  • Givesnicecolourstomeals
  • Can begivenas finger food
  • Training forchewingmuscles
tipps for people which don t like vegetables
Tipps forpeoplewhichdon‘tlikevegetables
  • Create a pizzawithvegetablesinsteadofsalami
  • Vegetablesoups
  • Pasta withvegetables
  • sandwiches
  • „Desensibilisation“ foracceptanceofthe taste
  • Offervegetable positiv
  • Parents/Caregiversshouldbemodelforthepersonwith PWS
fruits 2x a day
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

milkproductes choose the right cheese
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

meat sausage fish and eggs
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
conclusion ten rules for healthy food
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
everyday nutrition sunday nutrition
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
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)

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“

Quality of Life and Life expectancy in PWS is hardly related with the bodyweight
short r emarks on endocrinologic problems in adultes with pws
Short remarks on endocrinologicproblems in adultes with PWS
  • Diabetes
  • Hypothyreoidism
  • Hypogonadism
  • Growth hormonedeficiency
diabetes
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
clinical signs of diabetes
Clinical signsofdiabetes
  • Frequentgeneralinfections
  • not healingskininfectionsandmycosis
  • Reducedappetite
  • Reducedactivityandinterest
  • Rememberdiabeteswhensomeoneobese lose weightwithoutchanginganything
diabetes1
Diabetes
  • Diagnostics
    • Examinebloodsugareveryyear
    • Test bloodsugar bevor and after a meal
    • Orarrange a glucosetolerancetest
    • Also check bloodpressureandbloodlipids
  • Therapy
    • Dietandincreaseactivityto lose weight
    • Oral Medication
    • Insulin
hypothyreoidism
Hypothyreoidism
  • Clinical signs:
    • Decreasedactivity
    • Weightgain
    • Increasedfatigue
    • Depression
    • IncreasingEdema
    • Obstipation
    • Loss ofconcentration
hypothyreoidism1
Hypothyreoidism
  • Diagnostics:
    • Blood test
    • Thyroid-Antibodies
    • Ultrasound
  • Therapy: Substitution with L-Thyroxin
hypogonadism
Hypogonadism
  • Lateand/orincompletepubertaldevelopment
  • Consequences
    • Osteoporosis
    • Infertility
    • Lessself-esteem
late or incomplete puberty
Lateorincompletepuberty
  • Missingsignesofpuberty after theageof 14 y in boys
  • volumeofthetestes<4 ml

MissingBreastdevelopment after ageof 13,5 y in girls

Nomenarcheatageof15 y

diagnostics test the feed back control system and the function of the gonades
Diagnostics: Test thefeed-backcontrolsystemandthefunctionofthegonades
therapeutic aimes
Therapeuticaimes
  • normal puberty
  • acceptanceoftheownbody
  • Betterself-esteem
  • Preventosteoporosis
  • Fertitlity?
therapy hormone replacement
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
slide38

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

growth hormone deficiency
Growth hormonedeficiency
  • Growth hormonedeficiency also occures in adultes with PWS
  • Itcanbetestedwithstandardisedtests
  • Some adultes with PWS fullfillthecriteriafortreatment
  • The Rules for GH treatment in adulthooddifferverymuchfromcountrytocountry