Prospective evaluation of the family's health-related quality of life in pediatric intestinal failure

Background Due to altered nutrition regimens and complex medical needs, pediatric intestinal failure (IF) may have a powerful impact on health-related quality of life (HRQOL). Studies have shown that children with IF experience lower HRQOL. Data on the HRQOL of families of children with IF are lacking. Methods We performed a prospective analysis of the HRQOL of families of children with IF in a regional intestinal rehabilitation program from 2011 to 2018. The Pediatric Quality of Life Family Impact Module (FIM) was administered annually to parents. FIM scores were regressed on risk factors using linear mixed-effect models that accounted for repeated surveys within families. Results A total of 117 families completed 272 surveys. FIM scores increased with patient age across nearly all survey dimensions. Total FIM scores were lower when compared to families of healthy children (median differences = -5, P = .01) and similar to families of chronically ill children. While IF families reported major deficits in the Communication (-11, P < .001) and Worry (-17, P < .001) dimensions, they also reported higher Family Relationship scores (+7, P < .01). On multivariable regression, presence of a major comorbidity and four or more hospital admissions in the prior year were associated with lower family HRQOL (P < .05). Parenteral nutrition dependence was independently associated with lower scores in the Communication (-7, P = .03) and Daily Activities (-10, P = .02) dimensions. Conclusion Families of children with IF experience a decreased HRQOL that may improve with patient age. Intestinal rehabilitation programs should address the HRQOL of families in addition to patients.