Health-related quality of life in childhood interstitial lung disease.

BACKGROUND

Childhood interstitial lung diseases (chILD) encompass a diverse group of rare, chronic respiratory disorders. While treatment options are limited, evaluating health-related quality of life (HRQoL) has become a vital approach for clinical monitoring. This study aims to enhance our understanding of the medical and sociodemographic factors influencing HRQoL in chILD.

METHODS

This study used prospectively obtained data from the chILD-EU Register. Caregivers were handed validated, age-specific HRQoL questionnaires (the chILD-specific questionnaire and the generic Pediatric Quality of Life Questionnaire (PedsQL) 4.0 questionnaire). The scores were linearly transformed from a 5-point scale to a percentage scale ranging from 0% to 100%. We used exploratory univariate analyses and multivariable ordinary least squares (OLS) regression models on cross-sectional data to investigate the associations between subjects' clinical features and HRQoL scores.

RESULTS

424 patients from 10 European countries and 48 study sites were included in the final analysis. The most relevant factors associated with lower HRQoL scores for the chILD-specific questionnaire and the PedsQL 4.0 Total Score, as identified in the univariate analysis and confirmed in the multivariable OLS regression model, were "inpatient medical care in the last 3 months" (-13.6%; p<0.0001 and -11.4%; p<0.0001, respectively) and "failure to thrive" (-9.1%; p=0.0014 and -12.1%; p<0.0001, respectively). Among subjects old enough for pulmonary function testing (PFT) (n=162), we observed only a weak correlation between PFT results and different HRQoL scores.

CONCLUSIONS

The main factors linked to reduced HRQoL scores were prior inpatient treatment and failure to thrive. Further interventional studies should determine whether addressing these factors can enhance HRQoL scores and potentially improve quality of life in chILD.