Quality of life in Turkish children with interstitial lung disease: insights from the National ChILD Registry

Abstract

This study, the first of its kind in this population, evaluates health-related quality of life (HRQoL) in children with childhood interstitial lung disease (ChILD) and their families using data from the Turkish National ChILD Registry. Methods: As of January 2024, data from 17 centers were analyzed. Patients aged 1 month to 18 years and their families completed the Pediatric Quality of Life Inventory (PedsQL™ 4.0 Generic Core Scales) and PedsQL™ 2.0 Family Impact Module through face-to-face interviews. Higher scores indicate better HRQoL. The patients' PFT and Fan scores were recorded. Results: The study included 175 patients (90 girls), with a median age of 93 (13–214) months. FEV₁% positively correlated with the PedsQL™ Physical Health Summary Score, Parent-Reported Physical Health Summary Score, Psychosocial Health Summary Score, and total score (p=0.007, r=0.608; p=0.025, r=0.527; p=0.032, r=0.522; p=0.009, r=0.612). The Fan score negatively correlated with PedsQL™ scores (p<0.001). No significant correlation was found between FEV₁% and the Family Impact Module total score, though a weak negative correlation with the Fan score was noted (p<0.001–0.01). Conclusion: Lung function strongly correlates with HRQoL, emphasizing the impact of disease severity on physical and psychosocial well-being. These findings highlight the need for multidisciplinary care and psychosocial support to enhance the quality of life in children with ChILD and their families.