PMID- 28246220 OWN - NLM STAT- MEDLINE DCOM- 20170912 LR - 20220430 IS - 1468-3296 (Electronic) IS - 0040-6376 (Print) IS - 0040-6376 (Linking) VI - 72 IP - 9 DP - 2017 Sep TI - Validation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD). PG - 832-839 LID - 10.1136/thoraxjnl-2016-209356 [doi] AB - 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: 33 years (18-79 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 10-14 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 alpha: 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. CI - Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/. FAU - Behan, Laura AU - Behan L AD - Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK. AD - NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK. AD - Academic Unit of Clinical and Experimental Sciences Faculty of Medicine, University of Southampton, Southampton, UK. AD - School of Applied Psychology, University College Cork, Cork, Ireland. FAU - Leigh, Margaret W AU - Leigh MW AD - Department of Pediatrics and Marsico Lung Institute, University of North Carolina School of Medicine, Chapel Hill, North Carolina, USA. FAU - Dell, Sharon D AU - Dell SD AD - Division of Respiratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada. AD - Child Health Evaluative Sciences, The Hospital for Sick Children, Toronto, Ontario, Canada. AD - Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada. AD - Institute of Health Policy, Management and Education, University of Toronto, Toronto, Ontario, Canada. FAU - Dunn Galvin, Audrey AU - Dunn Galvin A AD - School of Applied Psychology, University College Cork, Cork, Ireland. FAU - Quittner, Alexandra L AU - Quittner AL AD - Department of Psychology, University of Miami, Coral Gables, Florida, USA. FAU - Lucas, Jane S AU - Lucas JS AD - Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK. AD - NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK. AD - Academic Unit of Clinical and Experimental Sciences Faculty of Medicine, University of Southampton, Southampton, UK. LA - eng GR - WT_/Wellcome Trust/United Kingdom GR - U54 HL096458/HL/NHLBI NIH HHS/United States PT - Journal Article PT - Multicenter Study PT - Validation Study DEP - 20170228 PL - England TA - Thorax JT - Thorax JID - 0417353 SB - IM MH - Adolescent MH - Adult MH - Age Distribution MH - Aged MH - Employment/statistics & numerical data MH - Female MH - Forced Expiratory Volume/physiology MH - Humans MH - Kartagener Syndrome/complications/physiopathology/psychology/*rehabilitation MH - Male MH - Middle Aged MH - Psychometrics MH - *Quality of Life MH - Reproducibility of Results MH - Respiratory Tract Diseases/etiology MH - Sex Distribution MH - Surveys and Questionnaires MH - Young Adult PMC - PMC5738537 OTO - NOTNLM OT - Psychology OT - Rare lung diseases OT - Respiratory Measurement COIS- Competing interests: None declared. EDAT- 2017/03/02 06:00 MHDA- 2017/09/13 06:00 PMCR- 2017/12/21 CRDT- 2017/03/02 06:00 PHST- 2016/08/29 00:00 [received] PHST- 2017/01/26 00:00 [revised] PHST- 2017/01/31 00:00 [accepted] PHST- 2017/03/02 06:00 [pubmed] PHST- 2017/09/13 06:00 [medline] PHST- 2017/03/02 06:00 [entrez] PHST- 2017/12/21 00:00 [pmc-release] AID - thoraxjnl-2016-209356 [pii] AID - 10.1136/thoraxjnl-2016-209356 [doi] PST - ppublish SO - Thorax. 2017 Sep;72(9):832-839. doi: 10.1136/thoraxjnl-2016-209356. Epub 2017 Feb 28.