Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr....
-
Upload
austen-martin -
Category
Documents
-
view
214 -
download
2
Transcript of Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr....
![Page 1: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/1.jpg)
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
![Page 2: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/2.jpg)
![Page 3: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/3.jpg)
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
![Page 4: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/4.jpg)
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
![Page 5: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/5.jpg)
carbohydrates proteins fat
glykogenreserveBody fatthermogenesisactivity
Weight control means balance of energy
![Page 6: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/6.jpg)
Body composition
Blue lean body massWhite body fatweiß: Fettgewebe
PWS
PWS
![Page 7: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/7.jpg)
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
![Page 8: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/8.jpg)
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)
![Page 9: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/9.jpg)
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!
![Page 10: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/10.jpg)
PWS weight management means individual size of meals under food
security
![Page 11: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/11.jpg)
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
![Page 12: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/12.jpg)
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
![Page 13: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/13.jpg)
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
![Page 14: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/14.jpg)
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
![Page 15: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/15.jpg)
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
![Page 16: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/16.jpg)
PWS food is healthy food for everyone
![Page 17: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/17.jpg)
Lunch: Plate rule
![Page 18: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/18.jpg)
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
![Page 19: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/19.jpg)
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
![Page 20: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/20.jpg)
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
![Page 21: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/21.jpg)
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
![Page 22: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/22.jpg)
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
![Page 23: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/23.jpg)
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
![Page 24: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/24.jpg)
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
![Page 25: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/25.jpg)
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)
![Page 26: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/26.jpg)
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“
![Page 27: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/27.jpg)
Short remarks on endocrinologic problems in adultes with PWS
• Diabetes• Hypothyreoidism• Hypogonadism• Growth hormone deficiency
![Page 28: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/28.jpg)
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
![Page 29: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/29.jpg)
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
![Page 30: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/30.jpg)
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
![Page 31: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/31.jpg)
Hypothyreoidism
• Clinical signs:– Decreased activity– Weight gain– Increased fatigue– Depression– Increasing Edema– Obstipation– Loss of concentration
![Page 32: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/32.jpg)
Hypothyreoidism
• Diagnostics: – Blood test– Thyroid-Antibodies– Ultrasound
• Therapy: Substitution with L-Thyroxin
![Page 33: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/33.jpg)
Hypogonadism
• Late and/or incomplete pubertal development
• Consequences – Osteoporosis– Infertility– Less self-esteem
![Page 34: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/34.jpg)
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
![Page 35: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/35.jpg)
Diagnostics: Test the feed-back control system and the function of
the gonades
![Page 36: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/36.jpg)
Therapeutic aimes
• normal puberty• acceptance of the own
body• Better self-esteem• Prevent osteoporosis
• Fertitlity?
![Page 37: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/37.jpg)
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
![Page 38: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/38.jpg)
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
![Page 39: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/39.jpg)
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
![Page 40: Caregivers‘ Programme IPWSO Conference in Cambridge 2013 Nutrition and Endocrinology in PWS Dr. med. Constanze Lämmer Pädiatrisches Zentrum St. Bernward.](https://reader031.fdocuments.in/reader031/viewer/2022020320/56649e205503460f94b0ba17/html5/thumbnails/40.jpg)
Thank you for your attention!