Health-related quality of life in pre-adolescent liver transplant recipients with biliary atresia: A cross-sectional study

Clin Res Hepatol Gastroenterol. 2019 Aug;43(4):427-435. doi: 10.1016/j.clinre.2018.10.018. Epub 2018 Dec 6.

Abstract

Objective: Pediatric recipients of liver transplantation (LT) often report lower Health-Related Quality of Life (HRQOL) than healthy controls when assessed on generic HRQOL measurement tools. The recent addition of the Pediatric Liver Transplant Quality of Life (PeLTQL), a novel disease-specific HRQOL instrument for pediatric LT recipients, into the clinical armamentarium of tools now routinely available to clinical care teams, provides the unique opportunity to identify disease-related challenges in children who have undergone this life-saving intervention. This study assesses HRQOL in pre-adolescent aged patients with a primary diagnosis of biliary atresia (BA) who underwent LT as an infant, using both generic and disease-specific HRQOL instruments validated for children. We also examined modifiable factors associated with HRQOL after pediatric LT.

Methods: HRQOL was the primary outcome of this study assessed using the disease-specific PeLTQL and the generic Pediatric Quality of Life Inventory 4.0 (PedsQL). Exposure variables of interest included medication status (e.g., monotherapy, dual therapy) and participation in sports.

Results: A total of 70 (56% female, mean age 9.89 ± 1.25 years) pediatric LT recipients (mean interval since LT was 9.0 ± 1.26 years) comprised the study cohort. LT recipients reported significantly lower PedsQL Scores relative to the general population. Immunosuppression monotherapy was associated with higher patient-reported PeLTQL Scores, and sports participation was associated with higher parent-reported PedsQL Scores.

Conclusions: Pre-adolescents who underwent LT as an infant with BA, self-report low HRQOL on both disease-specific and generic HRQOL tools. Further research targeting sports participation and simplifying immunosuppression may further optimize quality of life years restored by life-saving LT.

Keywords: Children; Immunosuppression; Patient-reported outcome; Sports and adherence.

Publication types

  • Multicenter Study

MeSH terms

  • Biliary Atresia / surgery*
  • Canada
  • Child
  • Cross-Sectional Studies
  • Europe
  • Female
  • Health Surveys*
  • Humans
  • Immunosuppression Therapy / psychology*
  • Infant
  • Liver
  • Liver Transplantation / psychology*
  • Male
  • Patient Reported Outcome Measures
  • Quality of Life / legislation & jurisprudence*
  • Sports / psychology
  • Survivorship
  • Transplant Recipients / psychology*