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

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

The German PWS-Treatment Concept

Ernährungs-

optimierung

Überwachung

der Organfunktionen

Entwicklungs-

überwachung

Wachstums- und

Hormonkontrolle

Ernährungs-

optimierung

Überwachung

der Organfunktionen

Entwicklungs-

überwachung

Wachstums- und

HormonkontrollePWS

Sport

carbohydrates proteins fat

glykogenreserveBody fatthermogenesisactivity

Weight control means balance of energy

Body composition

Blue lean body massWhite body fatweiß: Fettgewebe

PWS

PWS

Energy expenditure depends on muscle mass

• Obese PWS boy with BMI 48 kg/m²

• PWS young adultes with normal BMI but higher % of bodyfat

Basic energy expenditure 1100- 1200 kcal

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

• 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!

PWS weight management means individual size of meals under food

security

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

1 Portion = 35 g

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

Use the knowledge about energy content of food groups

vegetables fruits potatoes ricecooked

noodlescooked

leanmeat

fat meatsausages

cookies chocolate0

100

200

300

400

500

600

3050

70

110130 130

300

400

530

ener

gy in

kca

l/10

0g

PWS food is healthy food for everyone

Lunch: Plate rule

Vegetables

• Low caloric density • Less then 30 kcal/ 100g (expect corn, kidney beans)• Rich on vitamines and minerales• Offer vegetable as a snack• Gives nice colours to meals• Can be given as finger food• Training for chewing muscles

Tipps for people which don‘t like vegetables

• Create a pizza with vegetables instead of salami• Vegetable soups• Pasta with vegetables • sandwiches• „Desensibilisation“ for acceptance of the taste• Offer vegetable positiv• Parents/Caregivers should be model for the person

with PWS

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

 

-Calcium source for bones to prevent osteoporosis

Cheese

70% Fat i d 130kcal/30g40% Fat i d 85kcal/30g10% Fat i d 35kcal/30g

Meat, sausage, fish and eggs

• Important for animal protein• Rich on iron• Don‘t serve meat to often:

– For adultes 3 times a week meat, 2 times a week 3 slices of sausages or lean ham are recommended

• Once a week fish • 2 eggs per week

Conclusion: 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

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)

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“

Short remarks on endocrinologic problems in adultes with PWS

• Diabetes• Hypothyreoidism• Hypogonadism• Growth hormone deficiency

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 weight

come down with diabetes• High bloodsugar doesn‘t hurt, so when

clinical signs occure, diabetes often run more than 5 years

Clinical signs of diabetes

• Frequent general infections• not healing skin infections and mycosis • Reduced appetite• Reduced activity and interest

• Remember diabetes when someone obese lose weight without changing anything

Diabetes

• Diagnostics– Examine blood sugar every year– Test blood sugar bevor and after a meal– Or arrange a glucosetolerancetest– Also check blood pressure and blood lipids

• Therapy– Diet and increase activity to lose weight– Oral Medication– Insulin

Hypothyreoidism

• Clinical signs:– Decreased activity– Weight gain– Increased fatigue– Depression– Increasing Edema– Obstipation– Loss of concentration

Hypothyreoidism

• Diagnostics: – Blood test– Thyroid-Antibodies– Ultrasound

• Therapy: Substitution with L-Thyroxin

Hypogonadism

• Late and/or incomplete pubertal development

• Consequences – Osteoporosis– Infertility– Less self-esteem

Late or incomplete puberty

• Missing signes of puberty after the age of 14 y in boys

• volume of the testes <4 ml

• Missing Breastdevelopment after age of 13,5 y in girls

• No menarche at age of 15 y

Diagnostics: Test the feed-back control system and the function of

the gonades

Therapeutic aimes

• normal puberty• acceptance of the own

body• Better self-esteem• Prevent osteoporosis

• Fertitlity?

Therapy : hormone replacement

• Testosteron (male)– Injections or patches or gel application

• Estrogen (female)– Tablets or patches– Prepare for menarche/ menstruation– Add gestagen, when menarche occur

• Takes the personal situation in account!• Sometimes behavioral problems occure in male• Some women have problems to handle

menstruation every month

Fat

GH reduces body fat

GH increases vein protective HDL-cholesterol

Mind

GH increases activityGH normalizes psycho-social

well-being

Bones

GH increases bone mineral density

Water

GH normalizes fluid balance

Protein

GH transports more protein into cells

GH increases lean body mass

Heart

GH normalizes cardiac muscle mass

and improves cardiac performance

GH

Growth hormone deficiency

• Growth hormone deficiency also occures in adultes with PWS

• It can be tested with standardised tests• Some adultes with PWS fullfill the criteria for

treatment• The Rules for GH treatment in adulthood differ very

much from country to country

Thank you for your attention!