Validation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD)

Laura Behan, Margaret W. Leigh, Sharon D. Dell, Audrey Dunn Galvin, Alexandra L. Quittner, Jane S. Lucas

Research output: Contribution to journalArticlepeer-review

27 Scopus citations


Background Quality of life (QOL)-primary ciliary dyskinesia (PCD) is the first disease-specific, health-related QOL instrument for PCD. Psychometric validation of QOL-PCD assesses the performance of this measure in adults, including its reliability, validity and responsiveness to change. Methods Seventy-Two adults (mean (range) age: 33â €..years (18-79â €..years); mean (range) FEV 1 % predicted: 68 (26-115)) with PCD completed the 49-item QOL-PCD and generic QOL measures: Short-Form 36 Health Survey, Sino-Nasal Outcome Test 20 (SNOT-20) and St George Respiratory Questionnaire (SGRQ)-C. Thirty-five participants repeated QOL-PCD 10-14â €..days later to measure stability or reproducibility of the measure. Results Multitrait analysis was used to evaluate how the items loaded on 10 hypothesised scales: physical, emotional, role and social functioning, treatment burden, vitality, health perceptions, upper respiratory symptoms, lower respiratory symptoms and ears and hearing symptoms. This analysis of item-To-Total correlations led to 9 items being dropped; the validated measure now comprises 40 items. Each scale had excellent internal consistency (Cronbach's α: 0.74 to 0.94). Two-week test-retest demonstrated stability for all scales (intraclass coefficients 0.73 to 0.96). Significant correlations were obtained between QOL-PCD scores and age and FEV 1. Strong relationships were also found between QOL-PCD scales and similar constructs on generic questionnaires, for example, lower respiratory symptoms and SGRQ-C (r=0.72, p<0.001), while weak correlations were found between measures of different constructs. Conclusions QOL-PCD has demonstrated good internal consistency, test-retest reliability, convergent and divergent validity. QOL-PCD offers a promising tool for evaluating new therapies and for measuring symptoms, functioning and QOL during routine care.

Original languageEnglish (US)
Pages (from-to)832-839
Number of pages8
Issue number9
StatePublished - Sep 1 2017


  • Psychology
  • Rare lung diseases
  • Respiratory Measurement

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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