Coordinated interdisciplinary management of pediatric intestinal failure: a 2-year review

Abstract

Background/purpose: Intestinal failure is a complex metabolic process that results from malabsorption and malnutrition and provides challenges for a variety of pediatric subspecialists. The purpose of this study was to evaluate the effect of coordinated interdisciplinary team management of children with intestinal failure on nutritional outcome measures.

Methods: The Intestinal Care Center (ICC) is staffed with an interdisciplinary team of pediatric specialists including a gastroenterologist, pediatric surgeon, transplant surgeon, clinical dietitians, and a nutrition support nurse. Using an established registry, the authors conducted a comprehensive evaluation of patient data including anthropometric measures, organ system function, and mode of nutrition support. Disease-associated complications including micronutrient deficiencies, growth delay, and death also were monitored. Nutritional outcome was assessed by transition from enteral to oral feeding, cessation of total parenteral nutrition (TPN), and maintenance of linear growth.

Results: Since the inception of the ICC in 1996, 103 patients (69 boys, 34 girls) with intestinal failure have been evaluated with a median age of 2.6 years (range, 0.2 to 21.3 years). Mode of nutritional therapy on initial consultation included TPN (n = 76, 74%), enteral feedings (n = 6, 6%) and oral intake (n = 21, 20%). After intensive management of the 76 patients who were TPN dependent, 22 (29%) subsequently have been weaned from TPN (duration, 0.2 to 17.5 years) to oral (n = 14), oral-enteral (n = 4) or enteral feedings (n = 4). Of the 6 patients who were receiving enteral feedings, 4 (67%) were transitioned to oral feedings. Sixty-eight patients (66%) had evidence of hepatic disease. Of these, 10 underwent transplant, and 23 died (2 posttransplant). Linear growth velocity of neither pre- nor postpubescent patients significantly improved during the 2-year study period.

Conclusion: Data registry establishment and concurrent interdisciplinary team management of children with intestinal failure provides for innovative treatment approaches and a foundation for retrospective or prospective assessment of children with disease.