Features of Life Quality in Long-Term Survivors of Intestinal Transplantation in Childhood

Abstract

Background: Improving outcomes after intestinal transplantation have produced an expanding cohort of long-term survivors. Aim: In order to gain insight into life quality of this group, we reviewed the status of 48 infants and children who received an intestinal transplant at least 5 years before analysis and who possessed a fully functional graft, i.e. receiving total enteral nutrition with no central vein access. Results: Pre-transplant diagnosis was short bowel in 88% while the remainder had a functional form of intestinal failure. Median age at intestinal transplantation was 1.8 years (0.5 to 16.5) and included a liver graft in 75%, a multi-visceral graft in 10%, and a colon graft in 25%. Mean age at analysis was 12.5±4.7 years, giving a post-transplant survival of 8.7±2.4 years. Primary immunosuppression was tacrolimus in 66%, tacrolimus-sirolimus in 22%, and tacrolimus-mycophenolate in 10% with 1 fully tolerant patient taking no immunosuppression. Mean daily drugs consumed was 10±5. Anti-diarrheal agents were used regularly by 55% and anti-hypertensive drugs by 28%. Estimated GFR (cystatin C-sCr) was 70±19 mL/min/1.73 M2. Dietary restriction based on food allergy was present in 33% while supplemental feeding, generally via gastrostomy, continued in 38%. Mean height and weight z-scores were −1.24±1.83 and −1.10±1.45, respectively. Significant developmental delays were present in 29%, generally recognized before transplant and most often associated with marked prematurity. Of the remaining patients, 60% had special schooling arrangements, most notably individual educational programs. Conclusion: Although intestinal transplantation is a life-saving operation for patients experiencing life-threatening complications of intestinal failure, numerous threats to life-quality persist despite total independence from parenteral nutrition. Some of these threats originate before transplant and are inherent to the underlying condition whereas others are more directly related to the transplant itself.