Nutritional support for children with epidermolysis bullosa

Abstract

Epidermolysis bullosa (EB) comprises a rare group of genetically determined skin blistering disorders characterized by extreme fragility of the skin and mucous membranes, with recurrent blister formation. The cornerstones of management are control of infection, wound management, pain relief, promotion of optimal nutritional status and mobility, surgical intervention and provision of the best possible quality of life. There is currently no cure for EB and, throughout life, those with the more severe types are at risk of significant nutritional compromise which impacts negatively on health and overall quality of life. Nutritional support is an important facet of holistic care and the dietetic challenges can be considerable. This paper describes some of the issues involved in optimizing the nutritional status of children with this disorder.

Nutritional outcome in children with severe generalized recessive dystrophic epidermolysis bullosa: a short- and long-term evaluation of gastrostomy and enteral feeding

“…In our experience, as in previous reports 17,20,23 the nonendoscopic primary insertion of a button device by a trained surgical team or by a nonendoscopic image‐guided technique appears to be the best procedure in patients with RDEB, in order to avoid oesophageal shearing damage due to endoscopy. The rhythm and rate of GTF delivery, volume, energy intake and formula concentration should be adapted on an individual basis.…”

“…Over the past two decades, growth and nutritional condition have become major outcomes in the care of patients with EB. The specific Tool to Help Identify Nutritional Compromise (THINC) in EB has been designed by a reference EB team 17 . The scoring chart of THINC rates growth, digestive state and skin state, and algorithms for a care plan are proposed.…”

“…The scoring chart of THINC rates growth, digestive state and skin state, and algorithms for a care plan are proposed. The suggested nutritional requirements reach about 100–150% of the recommendations for healthy age‐ and sex‐matched children for energy and 115–200% for proteins 17 . In many chronic diseases responsible for malnutrition, gastrostomy (GT) is required, 21 but this therapeutic approach raised obvious concerns of poor healing and infection at the GT insertion site.…”

“…Conventional dietetic approaches using oral high‐energy and high‐protein semiliquid supplements rarely provide sustained improvement of dietary intake 17 . Therefore, malnutrition with growth retardation were accepted until recently as unavoidable consequences of RDEB in children 18–20 .…”

Gastrostomy Tube Feeding in Children with Epidermolysis Bullosa: Consideration of Key Issues

“…First, what are the criteria for, and aims of, G‐tube placement in severe EB? Algorithms (9,10) have been drawn up to aid in the general decision‐making process, but they cannot address specific aspects of individual cases. Is early placement (before growth failure becomes established) likely to be associated with fewer complications, better growth, and greater acceptance of the device?…”