Background

Childhood interstitial lung disease (chILD) is a heterogeneous group of mostly chronic respiratory disorders. Assessment of health related quality of life (HrQoL) in chILD has become increasingly important in clinical routine care and research. The aim of this study was to assess factors influencing HrQoL.

Methods

This cross-sectional study used data obtained from the Kids Lung Register, a web-based management platform that collects data of children diagnosed with chILD. Validated HrQoL questionnaires (PedsQL? 4.0) were filled by caregivers entered into the register by participating centres. HrQoL scores (Total Score) were calculated. Group differences were analyzed using Mann-Whitney-U test. For correlation, linear regression models were used and tested for significance by ANOVA.

Results

A total of 438 children (median age 4.9 (IQR 1.0 ? 11.5) years; 52.5% male) were included in this study. Median Total Score was 68.6% (IQR 52.8 ? 85.4). HrQoL scores were influenced by presence of respiratory symptoms (65.3% vs 77.8%; p<0.001), malnutrition (62.5% vs 71.7%; p=0.004), having a PEG (60.5 vs 69.9%; p=0.007), pulmonary hypertension (57.6% vs 68.8; p=0.003), and need for ventilatory support (no additional support 76.1% vs oxygen supplementation 65.0% vs invasive ventilation 50.0%; p<0.001). Total score correlated moderately with FEV1 z-score (r=0.340; p<0.001) and FVC z-score (r=0.345; p<0.001). No influence had disease category (p=0.076) or sex (p=0.165). Total Score did not correlate with age (p=0.999).

Conclusions

Multiple factors impair HrQoL in chILD. Further studies are needed to assess how these factors can be influenced and HrQoL improved.