Health-Related Quality of Life in Children with Intestinal Failure

Abstract

Introduction: The life expectancy of infants with intestinal failure (IF) has increased in the past decade. Now children with IF survive well into their childhood years with a strong potential for long-term survival. However, data on their health-related quality of life (HRQOL) are lacking. We hypothesized that HRQOL would be lower among children with IF and their families compared to that of healthy children. Methods: We performed a cross-sectional study of HRQOL of children and their parents enrolled in the outpatient intestinal rehabilitation program at Seattle Children's Hospital using the PedsQLTM 4.0 Generic Core Scales parent proxy-report and the Family Impact Module questionnaires. We compared PedsQLTM scores with previously published normative data and examined associations between PedsQLTM scores and patient demographic and clinical characteristics. Parents were asked two open-ended questions pertaining to the suitability and completeness of the PedsQLTM to assess their and their child's HRQOL. Results: Parents of 23 children with IF completed the questionnaires. Compared to norms for healthy children, parents reported significantly lower total PedsQLTM scores for children ages 1-2 years (mean difference= 13.16, p=0.003) and 3-4 years (mean difference= -17.63, p=0.001). Scores were also lower for children younger than 1 year (mean difference= -6.43) and children aged 2-3 years (mean difference= -11.96), though neither test reached statistical significance. The Family Impact Module scores of parents were similar to previous scores of parents caring for children with chronic illnesses (mean difference= -1.19, p=0.852). No measured demographic or clinical characteristics were associated with HRQOL in this sample. The majority of parents (65%) said the PedsQLTM failed to address important impacts of IF on children and their families. In particular, the PedsQLTM did not address challenges parents face nurturing their child or supporting their child's attainment of developmental milestones. Discussion: Children with IF and their parents have a decreased HRQOL compared to healthy children as measured by the PedsQLTM. The majority of parents identified additional challenges associated with IF that affect their child's and family's HRQOL. A disease-specific module or separate HRQOL questionnaire is needed for a more comprehensive assessment of patient and family HRQOL of children with IF.