Diet in. Chris Smith Senior Paediatric Dietitian

Transcription

1 Diet in Chris Smith Senior Paediatric Dietitian

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3 What do you recommend? How many calories does my child need? How often should I weigh my child and how do I know if the growth is good? What are the answers? What about the ketogenic diet? What about low fibre diets? What supplements should I give?

4 QUALITY NOT QUANTITY

5 KNOW YOUR CHILDS NEEDS

6 What calories does my child need?

7 AGE BOYS GIRLS PWS CALORIE DAILY REQUIRMENT THIS IS A GUIDE ONLY

8 What about adjustments for activity? 20kg, 5 year old boy PWS plays football for 20minutes How many calories will he burn? What would be a good snack? 54kcals 105 calories 183 calories 145 calories

9 ESTABLISH ACTIVITY AS NORMAL PART OF LIFESTYLE TAKE CARE TO PROVIDE APPROPRIATE SNACKS TO PREVENT PROVIDING POSITIVE CALORIE BALANCE

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11 Understand their growth pattern and stages

12 HEIGHT WEIGHT AGE

13 Standardized curves for weight of non growth hormone treated subjects (male subjects [upper] and female subjects [lower]) with PWS (solid lines) and normative percentile ranges (shaded area) with normative 97th to 50th percentiles in dark shading and 50th t... Merlin G. Butler et al. Pediatrics 2015;135:e126-e by American Academy of Pediatrics

14 Standardized curves for height of non growth hormone treated subjects (male subjects [upper] and female subjects [lower]) with PWS (solid lines) and normative percentile ranges (shaded area) with normative 97th to 50th percentiles in dark shading and 50th t... Merlin G. Butler et al. Pediatrics 2015;135:e126-e by American Academy of Pediatrics

15 BMI charts don t account for body composition Don t account for inherent growth pattern difference in PWS Can be used for monitoring but interpretation of results is very difficult Growth charts better

16 Expectation should be for proportional growth along UK centiles Specific PWS charts act as good additional reference Specific PWS charts describe how PWS children grow not how they should grow Due to nature of body composition BMI on standard charts may be misleading

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18 PHASE 1a -Weak suck -Oral feeds very slow -Decreased appetite -Doesn t cry at feeding times -Weak cry PHASE 1b -No longer needs assisted feeding -Grows steadily along curve with N intake -Normal appetite PHASE 2a -No increase in appetite -Appetite for age -Typically needs 60% -Will become obese if typical diet years PHASE 2b -Increased interest in food -Frequent food based questions -Will eat in line of sight years 9-25 months 0-9 months

19 Importance of a team approach

20 ROCKET SCIENCE??

21 PLAN AHEAD BE CONSISTENT Your child is invited to a birthday party at McDonalds. Should they go? PRO Social, inclusive, exposure inevitable CON Exposes child to temptation/ well meaning peers/ represent poor quality food

22 PLAN AHEAD BE CONSISTENT The school class is doing an optional cookery classes. Should your child be involved? PRO CON Great interest, opportunity for education Breaks structure, adds temptation, well meaning friends teachers

23 PLAN AHEAD BE CONSISTENT Allow treats / seconds on special occasions? PRO CON Part of childhood Never forgotten, tightrope of calories

24 Good guys vs bad guys

25 Food Security

26 MISTAKEN BELIEF This is because efforts to limit food, if attempted without establishing FOOD SECURITY,cause increased stress in the form of doubts, hope and disappointments

27 Provide food security Structure Consistent approach Visual menus Portion cups NO DOUBTS about what will be provided and when NO HOPES of obtaining food outside the plan NO DISAPPOINTMENTS concerning food

28 LOCK AWAY TEMPTATION OR DON T HAVE IT

29 BECOME A NUTRITION EXPERT

30 What is a normal portion size?

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32 Segmented lunch boxes Structured, ensures consistent portions

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35 RE THINK HOW YOU THINK ABOUT FOODS

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37 Using food rewards (and withholding them) for behaviors which may be the result of stress or anxiety [e.g. refusals, tantrums, shutdowns] may worsen the situation NON FOOD TREATS FOR PWS CHILDREN LOVE MEANS SAYING NO

38 WHAT EXTRA CAN I DO? Special diets

39 KETOGENIC DIET 108 families express interest 14 families completed requirements 10 families completed 6 months

40 Ketogenic diet CONCLUSION: Despite the burden of managing a structured diet 10 families found it was worthwhile and have continued beyond 9 months. No good quality studies Large amount of work needed Risks of balancing the diet

41 LOW FIBRE Concerns about swallow / food lodging 1. Provide small, frequent meals. Avoid the three LARGE meals per day. Break up meals/snacks to three small meals and two snacks a day (six is only recommended if the person has diabetes). Reduce quantity of food being provided at one time. 2. Include more liquid or semi-liquid food items. Provide liquids during and between meals. Have person drink water or fluids between bites of food. (Helps moisten food and facilitate movement from mouth to the stomach; less work and time with food in stomach). 3. AVOID: RAW vegetables and fruits, nuts and salads. (YES, this is a change) 4. DO PROVIDE: vegetables that have been cooked (softened) and/or mashed, fruits in softer form applesauce, fruits in natural juices, and cooked cerealdont eat too late

42 WHAT EXTRA CAN I DO? Supplements

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44 WHAT DOES IT MEAN? Very small number of PWS patients, also reduced calories, application to the real world not clear At best encouraging and paves the way for more work but insufficient to recommend to all

45 No significant differences in total and free carnitine or CoQ10 levels between individuals with PWS, obese individuals, and sibling control groups. 20 families elected to try carnitine supplementation (50 mg/kg/day divided twice a day) regardless of the serum carnitine profile results. 13 of these twenty families (65%) reported subjective improvement of exercise tolerance and daytime alertness with carnitine supplementation. 7 of the 20 families who tried carnitine supplementation discontinued it due to lack of benefit and/or side effects of therapy. 10 families started CoQ10 supplementation (50 mg/day) 5 (50%) of these families reported benefits in daytime All of the individuals who were reported to benefit from CoQ10 were under 3 years

46 Co enzyme Q10 Carnitine Deficiency rare Associated with increased physical activity, increased muscle tone and strength, increased cognitive ability, less sleeping, increased "energy" Deficiency rare Associated with improved mental and physical lethargy, muscle weakness and hypotonia in the early presentation of PWS No studies in PWS INSUFFICIENT EVIDENCE PARENT CHOICE

47 Vitamins and minerals BIRTH to 5 Vitamin D FROM 3 Consider supplement Iron, Zinc FROM 5 Very likely will require standard multivitamin and mineral iron, zinc, Vit D

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49 Always check the label NO IMPORTANT MINERALS NO IMPORTANT MINERALS UNECESSARY SUGAR

50 >12years Contact producer From birth Prescription only Needs dose calculation

51 TAKE HOME MESSAGES