Transplant Center

What is Intestinal Failure?

• Intestinal failure (IF) results from obstruction, dysmotility, surgical resection, congenital defect, or disease-associated loss of absorption. It is characterized by the inability to maintain protein-energy, fluid, electrolyte or micronutrient balance.
• Intestinal failure from loss of intestine (short bowel syndrome) is defined as: “the inability to maintain protein-energy, fluid, electrolyte or micronutrient balances when on a conventionally accepted, normal diet.”

Pediatrics

Most often, the conditions that cause IF occur in the neonatal period as a result of a loss of large portions (i.e., greater than 50%) of the intestine due to surgical resection or congenital defects (e.g., necrotizing enterocolitis, gastroschisis, volvulus). Prior to 1970, the prognosis for survival of these children was dismal; most eventually died as a result of dehydration, malnutrition, and weight loss. The prognosis for children with IF has improved significantly over the past 20 years as a result of improved surgical, pharmacological, and nutritional interventions; most notably with the introduction of total parenteral nutrition (TPN) to provide nutritional support.

Although TPN has reduced the mortality and morbidity associated with IF, maintenance on TPN is associated with extraordinarily high costs, reduced freedom due to needed medical intervention requirement of intensive monitoring, and reduced quality of life. Complications associated with TPN include recurrent central venous catheter infections, dehydration, vascular thrombosis, and/or progressive liver disease.  Decreasing dependence on TPN remains a high priority for children with IF to avoid negative growth, development and long-term health consequences.

Small bowel and/or liver transplantation are viable options when successful TPN weaning is unable to be accomplished. However, a strong multi-disciplinary approach to restoring nutritional autonomy and reduction of infections may allow for the avoidance of transplantation.

Oral feedings help in the recovery from IF by facilitating intestinal adaptation and bowel function. It also improves the social and emotional functioning of the child and family and reduces costs associated with IF. Studies show that children with IF get better faster if they eat and drink by mouth. However, estimates suggest that 77 percent or more of children with IF fail to transition to oral feedings. Systematic feeding therapy is often necessary to transition from TPN and enteral feed nutrition support and an oral diet.

Adults

Intestinal rehabilitation refers to the process of optimizing bowel function to reduce the dependence of TPN. Although outcomes for TPN weaning, despite short bowel syndrome, are highly patient specific based on functional and structural changes of the remaining bowel, all attempts at storing nutritional autonomy should be investigated. In adults with healthy small bowel remaining, it has been estimated the return to nutritional autonomy may be achieved in those with 50-70 cm small bowel if some colon is remaining intact or 110-150 cm if the small bowel ends in a terminal ostomy.

TPN-related complications such as metabolic bone disease, cholestasis and liver failure must be monitored in individuals with short bowel syndrome and TPN dependence. Metabolic bone disease occurs in up to 15 percent of patients within a few months after beginning TPN and can lead to osteomalacia. Osteomalacia causes debilitating bone disease, joint pain, vertebral compression and pathologic fractures. Chronic cholestasis occurs in 15 to 85 percent of patients on home TPN. This problem may be due to the length of time on TPN, the TPN prescription and the possibility of intestinal or systemic conditions associated with intestinal failure.

Comprehensive management with diet modification, patient education, growth factors and surgical intervention for intestinal lengthening (when indicated) may potentially lead to the reduction on TPN dependence and decreased requirement for intestinal transplantation.

“Every night without a night of TPN… is a good night”
-An adult patient from the Intestinal Rehabilitation Program at The Nebraska Medical Center