Robert Venick^1,2, Laura Wozniak¹, Kanela Artavia³, Yvonne Esmailian², Vilayphone Hwang², Susan Ponthieux², Sue McDiarmid^1,2, Ronald Busuttil², Douglas Farmer²

¹Department of Pediatrics, UCLA , Los Angeles, CA, United States; ²Department of Surgery, UCLA, Los Angeles, CA, United States; ³Department of Social Work, UCLA, Los Angeles, CA, United States

Background: Knowledge of long-term pediatric intestinal transplant (pITx) recipients is limited.This cross-sectional study aimed to describe nutritional, developmental and psychosocial outcomes at the time of most recent outpatient visit for children >5 years from primary ITx.

Methods: We conducted a retrospective, IRB-approved study using a prospectively maintained database of all pITxs between November 1990 and May 2011. Inclusion criteria were age <18 yrs at ITx, survival >5 yrs post-pITx, and follow-up care provided by our center. By protocol, pts >5 yrs post-pITx are followed in clinic 4-12 times per year.  Of 85 pITx performed, our analysis included 25 patients who received 29 grafts.

Results: Mean follow-up time =7.9±2.9 years. Median age at pITx =2.8 years. The majority of children were male (72%), Latino (56%), transplanted for gastroschisis or NEC (60%) and received liver-inclusive grafts (84%).  64% had ≥1 episode of biopsy proven ACR. 20% had PTLD. A mean of 4.1±4.7 episodes of infectious enteritis per patient were documented. 76% did not have an ostomy with surgical takedown occurring at a median of 489 days. Recipients had a median of 21 total readmissions. One-third of patients had not been hospitalized in >1 year. Median number of outpatient clinic visits in the prior year= 7.

Median Ht and Wt Z-scores improved from pre-ITx to most recent follow-up (Ht: -3.4 vs. -1.7, p<0.001 and Wt -1.5 vs. -0.7, p=0.17).  92% are off PN. 40% receive supplemental G-tube feeds.  2/3 are on Tac + Rapa and off steroids. Median # of medications =11 /pt/day. All children are in school; 68% receive special education. 71% have 2 parents involved in their care; 20% are in foster or adoptive care. 36% of primary caregivers are working outside the home. Mean # of siblings in the household  =1.8±1.8. 60% of young adult pITx recipients are working or in school. 88% are on state Medicaid.

Conclusions: This observational study provides an in depth view of long-term pITx recipients.  Growth and weight gain are sustained although oral aversion and dependence of supplemental tube feeds remains an issue. Most recipients are successfully weaned off steroids and managed as outpatients, on multiple medications. Though the majority of children require special education, most are high-functioning with intact families.