These resources include the best available evidence about the course of Rett syndrome and its management as well as practical information about family associations, specialist clinical centres and links to relevant websites.
Assessment and management of nutrition and growth in Rett syndrome
Leonard, H., Ravikumara, M., Baikie, G., Naseem, N., Ellaway, C., Percy, A., Abraham, S., Geerts, S., Lane, J., Jones, M., Bathgate, K., & Downs, J. (2013). Assessment and management of nutrition and growth in Rett syndrome. Journal of Pediatric Gastroenterology and Nutrition, 57(4), 451-60. Download snapshot, see article abstract.
Many girls with Rett syndrome have problems with their growth and this may continue into adulthood. Several factors may be contributing to poor growth, including feeding difficulties such as difficulties with chewing and swallowing. We developed recommendations to assess and manage issues relating to poor growth and weight gain in Rett syndrome, including consideration of gastrostomy.
What we did
Through evidence review and the consensus of a multidisciplinary panel of clinicians, recommendations were created to assist families and clinicians. Items included clinical assessment of growth, anthropometry (measuring body size), feeding difficulties and management to increase energy intake, decrease feeding difficulties, and consideration of gastrostomy.
What we found
Using a comprehensive approach to medical health issues is recommended to manage poor growth in Rett syndrome, taking into account factors such as feeding difficulties and nutritional needs.
Increasing energy and reducing difficulties
Energy-dense foods were identified as the most appropriate way of increasing energy intake. A diet containing the recommended daily allowance for essential nutrients was optimal, administered during frequent small feeds offered throughout the day. Optimal participation in feeding activities is encouraged; for example, it may be worth a trial of intensive therapy to optimize abilities for some girls because new skills can be learned, and physical positioning and postural supports should also be considered. Prompting and socializing during meals may also be important.
Consideration of gastrostomy
Gastrostomy should be considered where there is failure to thrive despite efforts to increase energy intake, where the girls experienced difficulties swallowing, or where the length of the feeding time is causing undue stress for both caregiver and child. Gastrostomy may assist with growth. Other benefits of gastrostomy might include shorter mealtimes, less risk of aspiration and chest infections.
What it means
We combined medical literature with parental input and expert clinician consensus in order to develop recommendations to assist management for feeding and growth. The recommendations have the potential to improve care of nutrition and growth in Rett syndrome by promoting discussion between caregivers and clinicians and to stimulate further research into health and development. We have developed the recommendations in to a booklet for families which is available on our website.
For further reports and findings, see our publications list.