1. Vanessa Dourass Paediatric Dietitian Birmingham Children’s Hospital

2. * Importance of nutrition * Impact of poor nutrition * Characteristics of children with RTS and effects on nutrition * Nutritional treatment options and pathways * Changing nutritional needs as we grow.

3. * The first year of life, nutritional requirements are at their highest: * Supports development * Supports adequate growth * Reduces risks of serious illness * Decreases risk of contracting infections * Increases ability to heal and fight infections * Good nutrition from the beginning can determine future health * Social aspect and development of relationships

4. * Characteristics: Normal birth weight Often poor feeding skills recognised at birth Subsequent poor growth/faltering growth Reflux Constipation Dystonia Features including small stature and small head size

5. http:// www.rubinstein-taybi.org/html/female.html

6. http://www.rubinstein-taybi.org/html/male.html

7. * Increased nutritional requirements: Cardiac abnormalities Seizures Dystonia/Swallowing difficulties Recurrent infections Gastro-oesophageal reflux disease (GORD) Vomiting

8. * Support from health care professionals * Support with breast feeding and positioning from the beginning * Support from Speech and Language therapists regarding feeding and swallowing difficulties * Thickened feeds- reflux/swallowing difficulties * Introduction of formula feeds to support growth * Specialised feeds to support feed tolerance

10. * The option of tube feeding may need to be considered - To help increase energy intake, reduce reflux symptoms, increase intake where feed aversions are apparent etc - Can be a short term and long term option - Needs to be an informed decision and supported by a multi-disciplinary team - Takes the pressure off difficult feeding times

11. * Nasogastric tube * Nasojejunal tube * Gastrostomy * Feeding into the Jejunum http://www.cfmedicine.com/htmldocs/CFText/dietarysupplements. htm

12. * Often the feeding difficulties experienced will improve in time * Weaning onto solids foods can help to relieve the symptoms of reflux * Support from speech and language is important when swallowing difficulties have been apparent * Food texture modification may be required * Weaning off tube feeds may be required * There are no specific guidelines for RTS and weaning

13. * Often during adolescence increased weight gain becomes an issue * Is this a result of over feeding and increased energy intakes as an infant? Research supports this suggestion. * Is it just part of the condition- Low muscle tone * How can we tackle this?........

14. * Prevention is key * Healthy food choices at weaning age * Continuing healthy eating patterns into adolescence * Healthy lifestyle choices * Involving the whole family in positive lifestyle choices * Behavioural change tools- (NICE guidelines) * Support

15. www.food.gov.uk

17. * Alucas A, MB, MD1,(2005) Long term programming effects of early nutrition – Inplications of preterm infant: Journal of Perinatology 25:S2–S6. * Hennekam RCM (2006)Pratical Genetics-Rubinstein Taybi Syndrome: European Journal of Human Genetics: 14, 981–985 * Wiley s, Swayne s, Rubinstein J,Lanphear N,3 & Stevens C (2003)Research Review- Rubinstein-Taybi Syndrome Medical Guidelines: American Journal of Medical Genetics 119A:101–110 * Sawaya A, Roberts s (2003) Stunting and future risk of obesity-principal physiological mechanism: Cad. Saúde Pública, Rio de Janeiro, 19(Sup. 1):S21-S2 (http://www.scielosp.org/pdf/csp/v19s1/a03v19s1.pdf)http://www.scielosp.org/pdf/csp/v19s1/a03v19s1.pdf * Special need growth charts: https://www.waisman.wisc.edu/wic/pdf/08-charts.pdfhttps://www.waisman.wisc.edu/wic/pdf/08-charts.pdf * Mcdonald S (2015) Gastroenterology: Clinical Paediatric Dietitics, fourth edition : John wiley & Sons Ltd. * NHS Choices(2016)The Eat well Guide.Available from: http://www.nhs.uk/Livewell/Goodfood/Pages/the-eatwell- guide.aspxhttp://www.nhs.uk/Livewell/Goodfood/Pages/the-eatwell- guide.aspx