CHEST Guidelines-Long-term-Assessment-of-Quality-of-Life-in-Primary

Long-term-Assessment-of-Quality-of-Life-in-Primary

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The correspondence discusses the need for new tools to assess the quality of life in patients with Primary Ciliary Dyskinesia (PCD), a genetic disorder causing chronic lung disease due to abnormal mucociliary clearance. The letter commends the work of Quittner et al. on developing the Quality of Life Questionnaire-Bronchiectasis (QOL-B) and reports on a study evaluating the effectiveness of existing patient-reported outcomes (PROs) in PCD. The study tested three common PROs—St. George’s Respiratory Questionnaire (SGRQ), Leicester Cough Questionnaire (LCQ), and Medical Outcomes Study Short Form 36 (SF36)—against spirometry and the 6-minute walk test (6MWT) in 20 PCD patients over a year. The study found no significant correlation between these PROs and spirometry results, nor did they adequately track quality of life changes in PCD.<br /><br />Furthermore, despite small sample size constraints, the study suggests these tools are inadequate for monitoring PCD longitudinally. The letter underscores the lack of a PCD-specific QoL questionnaire and suggests that pending QOL-B validation for PCD, care should be taken when using non-disease specific tools. There's an urging for the development of a PCD-specific instrument to better assess patients' quality of life longitudinally.<br /><br />The authors, affiliated with Federico II University in Naples, Italy, emphasize the importance of disease-specific PRO measures, pointing out that no potential conflicts of interest have been reported. They cite previous research indicating limitations of spirometry and the need for potentially validating QOL-B for PCD, aligning with patients' needs in clinical trials. Validation of such specific tools is crucial for better management and treatment outcomes in PCD.

Keywords

Primary Ciliary Dyskinesia

quality of life

QOL-B

patient-reported outcomes

spirometry

6-minute walk test

disease-specific tools

Federico II University

chronic lung disease

PCD assessment