Candidacy for Intestinal Transplantation

Parenteral nutrition represents standard therapy for children with short bowel syndrome and other causes of intestinal failure. Most infants with short bowel syndrome eventually wean from parenteral nutrition, and most of those who do not wean tolerate parenteral nutrition for protracted periods. However, a subset of children with intestinal failure remaining dependent on parenteral nutrition will develop life-threatening complications arising from therapy. Intestinal transplantation (Tx) can now be recommended for this select group. Life-threatening complications warranting consideration of intestinal Tx include parenteral nutrition-associated liver disease, recurrent sepsis, and threatened loss of central venous access. Because a critical shortage of donor organs exists, waiting times for intestinal Tx are prolonged. Therefore, it is essential that children with life-threatening complications of intestinal failure and parenteral nutrition therapy be identified comparatively early, i.e. in time to receive suitable donor organs before they become critically ill. Children with liver dysfunction should be considered for isolated intestinal Tx before irreversible, advanced bridging fibrosis or cirrhosis supervenes, for which a combined liver and intestinal transplant is necessary. Irreversible liver disease is suggested by hyperbilirubinemia persisting beyond 3-4 months of age combined with features of portal hypertension such as splenomegaly, thrombocytopenia, or prominent superficial abdominal veins; esophageal varices, ascites, and impaired synthetic function are not always present. Death resulting from complications of liver failure is especially common during the wait for a combined liver and intestinal transplant, and survival following combined liver and intestinal Tx is probably lower than following an isolated intestinal transplant. The incidence of morbidity and mortality following intestinal Tx is greater than that following liver or kidney Tx, but long-term survival following intestinal Tx is now at least 50-60%. It is probable that outcomes shall improve in the future with continued refinements in operative technique and post-operative management, including immunosuppression.

A study was performed to measure the quality of life of patients receiving home parenteral nutrition (HPN). Quality of life is an important determinant of the effectiveness of health technologies, but it has rarely been assessed in patients receiving HPN. To measure quality of life and highlight any moderating factors. Quality of life was measured using two validated instruments (SF 36 and EuroQol) in 51 patients with intestinal failure. All patients had benign disease, the commonest being Crohn's disease (n = 35). HPN patient scores were worse for six of eight SF 36 domains (p 55 years) scored significantly less. Patients addicted to narcotic substances had very low scores. EuroQol utility scores confirmed the SF results. Forty one patients reported that they felt too ill to work and only five were in full time work or education. The health status profile of our young patients on HPN was good compared with the normal population. The poorest scores were in older patients and those dependent on narcotic drugs. This has clinical and economic relevance when considering such patients for HPN.