Quality of life matters

Abstract

Children with chronic medical health problems are likely to have lower health-related quality of life (QOL) scores than their healthy peers. Those with more complex medical conditions involving multiple body systems are at greatest risk for having a poor QOL. Improving the lives of these children requires a better understanding of the factors that adversely impact QOL. Without such knowledge our ability to improve their QOL is limited. Alagille syndrome (ALGS) affects multiple systems including liver, heart, eyes, face, skeleton, kidneys, and vasculature. Each system disorder can impair health-related QOL, and the combined effects are potentially devastating. Surprisingly, there are little data on health-related QOL in children with ALGS. The study by Kamath et al in this issue of The Journal attempts to correct this deficiency. Using the Pediatric Quality of Life Inventory, children with ALGS and liver disease were compared with published normative data, as well as with two groups of children with other chronic liver diseases (alpha-1antitrypsin deficiency [A1ATD] and chronic intrahepatic cholestasis [IHC]). Within the group with ALGS, various determinants of health-related QOL were analyzed for effect. Not unexpectedly, QOL scores in patients with ALGS, A1ATD, and IHC were lower than the healthy population. There were some differences between patients with ALGS, A1ATD, and IHC, those with ALGS having the lowest scores. Within the ALGS group, weight and height z-scores exerted a positive effect with better growth associated with higher QOL scores. Overall, this study confirms the need to identify more precisely what impacts QOL in children with chronic disorders. The somewhat surprising finding that better growth positively impacts QOL scores in ALGS emphasizes this fact and provides one opportunity for a therapeutic intervention. Article page 390▶ Quality of Life and Its Determinants in a Multicenter Cohort of Children with Alagille SyndromeThe Journal of PediatricsVol. 167Issue 2PreviewTo assess health-related quality of life (HRQOL) in children with Alagille syndrome (ALGS) in comparison with healthy and other liver disease cohorts, and to identify determinants of HRQOL in patients with ALGS. Full-Text PDF