Development and validation of a health-related quality of life questionnaire for pediatric patients with interstitial lung disease (ILD)
ILD is an extremely heterogeneous group of diseases with varying degrees of quality of life impairment.
Children's interstitial lung disease (ILD) is an umbrella term used to describe a heterogeneous group of extremely rare pulmonary diseases mainly affecting the lung parenchyma.ILD encompasses more than 200 different diseases with varying morbidity and with a prevalence of 0.36 per 100 000 and an incidence of 1.32 per 1 000 000. The aim of european netwok on ILD (ChILD) project was to develop evidence-based and consensus-agreed clinical guidelines to achieve better care of patients afflicted by chILD and to improve their quality of life (http://www.childeu.net). A5-point rating scale of frequency (from never to almost always) was used. Children, adolescents aged 8-18 years and parents of children aged 0 month-18 years with chILD and sufficient knowledge of the German language were eligible for inclusion. The questionnaires were completed by patients and their caregivers at the clinics. The questionnaires were translated into English, French, Italian, and Turkish.
Results: The final instrument is available in different developmentally adapted versions from infancy to adolescence, comprising between 5 and 11 items. The scales showed high internal consistency (Cronbachs’α between 0.85 and 0.94). Convergent validity was indicated by moderate to high correlations (r = 0.43-0.91) with the Pediatric Quality of Life Questionnaire (PedsQL™ 4.0 Core module). Lower scores were significantly associated with dyspnoea (tproxy = 3.18, P = 0.002), tachypnoea (tproxy = 2.95, P = 0.002), and with worse clinical course of lung disease (tself=3.96, P < 0.05) as reported by the physicians.
Conclusions: The results indicate the reliability and validity of the chILD-QoL for pediatric patients with interstitial lung diseases. It can be used for screening and monitoring subjective health status as perceived by the patients and/or their caregivers, as well as for evaluation of health-related quality of life in clinical trials and intervention research.
Niemitz M, Schwerk N, Goldbeck L, Griese M. Development and validation of a health-related quality of life questionnaire for pediatric patients with interstitial lung disease. Pediatr Pulmonol. 2018 Apr 23.