Health Related Quality of Life in Patients with Biliary Atresia Surviving with their Native Liver

Abstract

Objectives To quantify health related quality of life (HRQOL) of patients with biliary atresia with their native livers and compare them with healthy children and patients with biliary atresia post-liver transplant (LT) and to examine the relationship between HRQOL and medical variables. Study design A cross-sectional HRQOL study of patients with biliary atresia with their native livers (ages 2-25 years) was conducted and compared with healthy and post-LT biliary atresia samples using PedsQL™ 4.0 child self and parent proxy reports, a validated measure of physical/psychosocial functioning. Results 221 patients with biliary atresia with native livers (54% female, 67% white) were studied. patient self and parent proxy reports showed significantly poorer HRQOL than healthy children across all domains (p < 0.001), particularly in emotional and psychosocial functioning. Child self and parent proxy HRQOL scores from patients with biliary atresia with their native livers and post-LT biliary atresia were similar across all domains (p=NS). Child self and parent proxy reports showed moderate agreement across all scales, except social functioning (poor to fair agreement). On multivariate regression analysis, black race and elevated total bilirubin were associated with lower Total and Psychosocial HRQOL summary scores. Conclusions HRQOL in patients with biliary atresia with their native livers is significantly poorer than healthy and similar to post-LT biliary atresia children. These findings identify significant opportunities to optimize the overall health of patients with biliary atresia.

Medical Status of 219 Children with Biliary Atresia Surviving Long-Term with Their Native Livers: Results from a North American Multicenter Consortium

“…Although 98% of five-year native livers survivors who were not listed for LT had clinical or biochemical evidence of chronic liver disease, it was intriguing that over half of these subjects still reported normal HRQOL, emphasizing the need to consider patient-reported subjective outcome variables in assessing clinical outcomes. Our group recently reported HRQOL in subjects with BA surviving with their native livers was similar to subjects with BA post-LT, although psychosocial and school functioning were significantly impaired in both groups compared with healthy children 48 . The similarities observed between the subjects with BA with their native livers and pediatric LT recipients were mainly driven by psychosocial problems rather than issues with physical health.…”

Quality of life after living donor liver transplant for biliary atresia in Japan

“…To compare the total scores and those of the subscales, effect sizes (Cohen's d ) were used; 0.20–0.49, 0.50–0.79, and >0.80 indicated a small, moderate, and large difference, respectively. The generic HRQOL scores were compared with those obtained from the following US samples: US I, mean age at survey, 7.2 ± 3.8 years; female, 64%; white, 58%; and US II, LT, 53.8%; kidney transplant, 26.3%; heart transplant, 9.6%; small bowel transplant, 1.8%; and multiple organ transplants, 8.5%; mean age at survey, 11.3 ± 5.0 years; mean interval from transplant to survey, 7.0 ± 4.8 years; female, 44%; white, 73% . Data on the US pediatric and adolescent solid organ transplant patients were used to compare scores on transplant‐specific HRQOL.…”

Maternal coping with the prospect of liver transplant among their school‐age children

“…Unfortunately, the pretransplant issues faced by this population have often been overlooked and more studies have focused on just the child following liver transplantation (Lind et al, ; Sundaram, Alonso, Haber, et al, ), whereas daily lives (Fujita, ) or experiences of children and adolescents who had undergone living‐donor liver transplantation have been described in the recent year. Research has yet to provide a detailed description of maternal coping challenges or the daily lives of school age children who are afflicted with this disease.…”