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Health-related quality of life and 6 min walk distance in patients with idiopathic pulmonary fibrosis.

ABSTRACT: Few studies have investigated the relationship between physiological outcomes and quality of life in patients with idiopathic pulmonary fibrosis (IPF). Health-related quality of life (HRQL) data may provide a valuable patient-centred outcome for the evaluation of interventions. Determination of which physiological and functional variables are associated with HRQL may facilitate interpretation of disease progression and impacts of therapy.To determine whether an association between Medical Outcomes Short-Form 36 and St George's Respiratory Questionnaire scores and other markers of disease severity exist, and to identify which physiological and functional variables are independently associated with HRQL.In a cross-sectional study, 137 patients with IPF completed HRQL surveys and underwent clinical assessment. Multiple linear regression analysis was used to determine the relationship between age, forced vital capacity, diffusing capacity of carbon monoxide, 6 min walk distance (6MWD) and oxygen requirement, and HRQL scores.6MWD was the only functional measure of disease severity significantly associated with all domain scores of the St George's Respiratory Questionnaire and the Medical Outcomes Short-Form 36 physical component summary score.6MWD was associated with HRQL among patients with IPF. This highlights the importance of 6MWD not only as a prognostic indicator in IPF, but also a predictor of HRQL and a meaningful outcome for patients.

SUBMITTER: Verma G

PROVIDER: S-EPMC3267607 | biostudies-literature | 2011 Sep-Oct

REPOSITORIES: biostudies-literature

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Health-related quality of life and 6 min walk distance in patients with idiopathic pulmonary fibrosis.

Verma Geetika G Marras Theodore T Chowdhury Nouri N Singer Lianne L

Canadian respiratory journal 20110901 5

<h4>Background</h4>Few studies have investigated the relationship between physiological outcomes and quality of life in patients with idiopathic pulmonary fibrosis (IPF). Health-related quality of life (HRQL) data may provide a valuable patient-centred outcome for the evaluation of interventions. Determination of which physiological and functional variables are associated with HRQL may facilitate interpretation of disease progression and impacts of therapy.<h4>Objectives</h4>To determine whether ...[more]

PMID: 21969931

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Project description:BackgroundIdiopathic pulmonary fibrosis (IPF) profoundly affects the quality of patients' lives. A systematic review was performed to evaluate critically the published literature and to examine what is known about health-related quality of life (HRQL) in patients with IPF.MethodsThe MEDLINE, EMBASE, Health and Psychosocial Instruments, and Cochrane Library databases were searched to 1 April 2004. Abstracts and bibliographies of published articles were scanned and contact was made with investigators. Included studies analysed HRQL (or quality of life) in at least 10 patients with IPF. Two reviewers independently selected studies, evaluated their quality according to predetermined criteria, and abstracted data on study design, patients' demographic and clinical characteristics, and quality of life outcome measures.ResultsSeven studies met the inclusion criteria. The studies enrolled 512 patients with IPF and used three different instruments to measure HRQL. All studies had important limitations in methodological quality; none measured longitudinal changes in HRQL over time. Patients reported substantially impaired HRQL, especially in domains that measured physical health and level of independence. Patients with IPF appear to have similar impairments in HRQL to those with chronic obstructive pulmonary disease. Measures of dyspnoea were moderately correlated with scores from domains that measured physical health (R2 = 0.03-0.66) and energy/fatigue/pep (R2 = 0.19-0.55), but measures of pulmonary function and gas exchange did not correlate as strongly with these and other domains.ConclusionStudies of HRQL in patients with IPF suggest that, in addition to the obvious effect on physical health, general health, energy level, respiratory symptoms, and level of independence are also impaired. Variability in HRQL among patients is not fully explained by measures of dyspnoea or pulmonary function, suggesting that HRQL measures provide unique information. More research is needed to identify or design appropriate measurement instruments for patients with IPF and to examine changes in HRQL over time or in response to specific treatments.

Project description:The objective of this study was to examine relationships between pulmonary health and health-related quality of life (HRQOL) in patients with cystic fibrosis (CF) evaluated longitudinally in the Wisconsin Newborn Screening Project.Patients aged 8 to 18 years (mean ± SD, 13.5 ± 2.8) in early diagnosis (n = 45) and control (n = 50) groups completed Cystic Fibrosis Questionnaires (CFQs) to measure HRQOL at three data points over a 2-year period. Pulmonary health was evaluated concurrently by the Wisconsin chest x-ray scoring system (WCXR) and pulmonary function tests (PFTs).WCXR showed significant group differences (P ? .023), with the early diagnosis group showing more-severe lung disease. When adjusted for group differences in mucoid Pseudomonas aeruginosa status and pancreatic status, however, WCXR differences and PFT data were not significant. Most patients (74%) had FEV(1) values ? 80% predicted (within normal range). For patients aged < 14 years, as WCXR scores worsened CFQ respiratory and physical domain scores decreased (both P ? .007). FEV(1)/FVC showed a positive relationship with the respiratory and physical domains (both P ? .006). WCXR scores for patients aged ? 14 years were associated with CFQ weight, respiratory, and health domains (all P ? .011). FEV(1) was associated with CFQ weight, respiratory, health, and physical domains (all P ? .003). Changes in pulmonary health were not associated with changes in CFQ over time. Significant group differences on the CFQ-Child social functioning domain favored the control group.To our knowledge, this study is the first to compare pulmonary outcomes with HRQOL indicators assessed by serial, standardized, patient-reported outcome measures for patients with CF identified either through newborn screening or diagnosed by use of traditional methods. This study found no benefits of newborn screening for pulmonary health or HRQOL after controlling for risk factors. Using WCXR and PFT data collectively helped to identify associations between pulmonary health and HRQOL.

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Health-related quality of life and 6 min walk distance in patients with idiopathic pulmonary fibrosis.

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Health-related quality of life and 6 min walk distance in patients with idiopathic pulmonary fibrosis.

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