Nutrition and Growth Lesley Foster & Jacqueline Denyer

FACTORS AFFECTING NUTRITION Oral hygiene difficulties / sore mouth/ reduced mouth and tongue movement Narrowing of oesophagus in RDEB, and difficulty swallowing Gastro –oesophageal reflux Constipation Gut inflammation Lack of hand dexterity Poor general heath (anaemia, pain) Mood

Growth charts Traditionally used to measure height and weight Designed for the healthy child Are these relevant for children with a severe form of EB? Is there an alternative, more accurate way of measuring growth in our EB population?

OPTIONS Oral supplements Oesophageal dilatation Short term gastrostomy feeding Long term gastrostomy feeding Bolus or overnight feeds

Gastrostomy feeding The pros: Supplementary feeding when growth poor and swallowing difficult Weight and height increase A route for giving medication The cons: Decreased appetite Leakage and excoriation ? Link with colitis Increased gastro oesophageal reflux Bedwetting and soiling

Abdominal distension and excoriation

Abdominal Distension

Points for discussion The relevance of use of growth charts for children with severe EB? What practical alternatives are there? Should gastrostomy placement be a short term / long term/ or ‘last resort’ option? Should we try to maintain nutrition and growth with oral supplements and oesophageal dilatation alone?

CONCLUSION Gastrostomy feeding - life saving at times but may not be the answer in all cases. How do other centres monitor growth? How do you manage feeding difficulties? What problems do you encounter?