GI and Nutrition

Infants and children with EB, in particular recessive dystrophic EB and junctional EB, are at high risk for gastrointestinal complications and poor nutrition. Anticipation of potential problems, frequent monitoring through the use of laboratory studies and clinical examination, and intervention before the development of overt complications are the best ways to ensure optimal gastrointestinal health and nutrition in children with EB.  In addition, infants with EBS-pyloric atresia and JEB-pyloric atresia may develop severe vomiting and hyponatremic dehydration if this complication is not quickly diagnosed and treated appropriately.

Gastrointestinal complications include the following:

Poor nutrition in children with EB results from a combination of factors, including intake of insufficient calories and nutrients to meet metabolic demands, which are increased due to chronic inflammation and wound healing, and loss of nutrients though open wounds. Use of high-calorie liquid nutritional supplements and specific micronutrient supplements are often recommended. Children at risk for severe malnutrition may require placement of a gastrostomy tube to facilitate supplemental feeding.
Complications of inadequate nutrition include the following: