Diet in

Chris Smith

Senior Paediatric Dietitian

• 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?

QUALITY NOT QUANTITY

KNOW YOUR CHILDS NEEDS

What calories does my child need ?

AGE BOYS GIRLS

2 600 558

3 702 648

4 834 774

5 888 816

6 936 888

7 990 918

8 1050 978

9 1104 1032

10 1218 1164

11 1278 1212

12 1350 1260

13 1446 1332

14 1578 1404

15 1692 1434

16 1782 1446

17 1848 1476

18 1896 1476

PWS CALORIE DAILY REQUIRMENT

THIS IS A GUIDE ONLY

• 20kg , 5 year old boy PWS plays football for 20minutes

• How many calories will he burn?

• What would be a good snack ?

105 calories 183 calories 145 calories

What about adjustments for activity ?

54kcals

ESTABLISH ACTIVITY AS NORMAL PART OF LIFESTYLE

TAKE CARE TO PROVIDE APPROPRIATE SNACKS TO PREVENT PROVIDING POSITIVE CALORIE BALANCE

Understand their growth pattern and stages

HEIGHT

WEIGHT

AGE

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-e135

©2015 by American Academy of Pediatrics

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-e135

©2015 by American Academy of Pediatrics

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

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

PHASE 2b -Increased interest in food-Frequent food based questions-Will eat in line of sight

PHASE 2a-No increase in appetite-Appetite for age-Typically needs 60% -Will become obese if typical diet

PHASE 1b-No longer needs assisted feeding-Grows steadily along curve with N intake-Normal appetite

PHASE 1a-Weak suck-Oral feeds very slow-Decreased appetite -Doesn’t cry at feeding times-Weak cry

0-9 months

9-25 months

2.1-4.5years

4.5-8 years

Importance of a team approach

ROCKET SCIENCE ??

• 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

PLAN AHEAD BE CONSISTENT

• The school class is doing an optional cookery classes. Should your child be involved?

• PRO Great interest, opportunity for education

• CON Breaks structure, adds temptation, well meaning friends teachers

PLAN AHEAD BE CONSISTENT

• Allow treats / seconds on special occasions?

• PRO Part of childhood

• CON Never forgotten, tightrope of calories

PLAN AHEAD BE CONSISTENT

Good guys vs bad guys

Food Security

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

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

LOCK AWAY TEMPTATIONOR

DON’T HAVE IT

BECOME A NUTRITION EXPERT

What is a normal portion size?

Segmented lunch boxes

Structured, ensures consistent portions

RE THINK HOW YOU THINK ABOUT FOODS

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”

WHAT EXTRA CAN I DO ?Special diets

KETOGENIC DIET

• 108 families express interest

• 14 families completed requirements

• 10 families completed 6 months

Ketogenic diet

• No good quality studies

• Large amount of work needed

• Risks of balancing the diet

CONCLUSION:Despite the burden of managing a structured diet 10 families found it was worthwhile and have continued beyond 9 months.

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

WHAT EXTRA CAN I DO ?Supplements

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

• 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

Co enzyme Q10

• 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

Carnitine

Vitamins and minerals

BIRTH to 5 Vitamin D

FROM 3 Consider supplementIron, Zinc

FROM 5 Very likely will require standard multivitamin and mineral – iron, zinc, Vit D

Always check the label

• NO IMPORTANT MINERALS

• NO IMPORTANT MINERALS

• UNECESSARY SUGAR

>12years

From birthPrescription only Needs dose calculation

Contact producer

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