Development and validation of a health-related quality of life questionnaire for pediatric patients with interstitial lung disease

Pediatr Pulmonol. 2018 Jul;53(7):954-963. doi: 10.1002/ppul.24018. Epub 2018 Apr 23.

Abstract

Purpose: Children's interstitial lung disease (chILD) is often associated with multiple burdens and chronic impairment of health-related quality of life. Patient reported outcomes describe the patients' perspective on medical conditions and their treatments. We aimed to develop and evaluate the psychometric properties of a chILD-specific PRO (chILD-QoL) as an instrument for monitoring the patients' health status.

Methods: Items were generated through focus groups with parents, patients, and interviews with pediatric pulmonologists. After a pretest of the German pilot version, the questionnaire was refined and translated into four European languages. Psychometric properties of the questionnaire were analyzed within a multi-center collaborative throughout Europe involving 180 parents of children with an interstitial lung disease and 65 pediatric patients.

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.

Keywords: chILD; health-related quality of life; interstitial lung disease; patient-reported outcome.

MeSH terms

  • Adolescent
  • Caregivers
  • Child
  • Child, Preschool
  • Europe
  • Female
  • Health Status
  • Humans
  • Infant
  • Infant, Newborn
  • Lung Diseases, Interstitial*
  • Male
  • Parents
  • Proxy
  • Psychometrics
  • Quality of Life*
  • Reproducibility of Results
  • Surveys and Questionnaires*