Health-related quality of life in children interstitial lung disease

Introduction: Health-related quality of life (HR-QoL) is an understudied area in rare diseases. Interstitial lung diseases in children (chILD) are heterogeneous disorders that are associated with a high morbidity and mortality. The impaired lung function, the nutritional impact and the burden of the treatments can impair the HR-QoL of these children. The aim of this study was to compare the HR-QoL in chILD to an aged-matched healthy population and to identify the factors that were associated to an altered QoL. Methods: Patients were recruited in the French RespiRare network. Patients with chILD of all causes, aged 1 month to 18 years were prospectively included. The children over 8 years old and the 2 parents of all children were asked to complete the PedsQLTM 4.0 Generic Core Scale (0 to 100 points). Results: A total of 78 children were included in the RespiRare centres. Total mean scores appeared to be significantly reduced in child self-report and in parent proxy-report (11.94 points (p=0.0003) and 14.08 points (p Conclusion: The study shows that HR-QoL is significantly impaired in chILD compared to a healthy population, using a validated generic QoL scale. Factors altering QoL score are easy to recognize and could help identify children at a heightened risk of low QoL. A longitudinal follow-up of HR-QoL in chILD is necessary to follow the impact of the disease and the treatments. To better appreciate chILD QoL, a specific scale is currently under study by the chILD-EU group.