Dataset Information

Health-related quality of life in children with cystic fibrosis: validation of the German CFQ-R.

ABSTRACT:

Background

This study evaluates the psychometric properties of the Child and Parent versions of the German CFQ-R (Cystic Fibrosis Questionnaire Revised), a disease-specific measure of Health-Related Quality of Life (HRQoL) in children with cystic fibrosis (CF). Self-Rating is combined with proxy-rating by parents in the use of the questionnaire.

Methods

136 children with CF (6 - 13 years) and their parents were recruited to evaluate internal consistency (Cronbach's alpha) and validity, 20 children and parents to examine reproducibility (ICC).

Results

Cronbach's alpha is high in all but two dimensions of the Child version (alpha = 0.23-0.77) and for all dimensions of the Parent version (alpha = 0.69-0.89). For both questionnaires, reproducibility is moderate to high (ICC = 0.50-0.94). Factor analysis shows loadings of >0.4 in the majority of items. Higher HRQoL is reported by children with mild disease compared to those with moderate/severe disease and by boys compared to girls. Convergence between self-rating and proxy-rating depends on the dimension.

Conclusion

The German CFQ-R, Child and Parent versions, are reliable and valid measures of HRQoL. They should be administered in combination as both, child and parent, provide important information. The measure offers a new patient-reported outcome for clinical purposes as well as for national and international studies in schoolchildren.

SUBMITTER: Schmidt A

PROVIDER: S-EPMC2794264 | biostudies-literature | 2009 Dec

REPOSITORIES: biostudies-literature

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Publications

Health-related quality of life in children with cystic fibrosis: validation of the German CFQ-R.

Schmidt Anne A Wenninger Kerstin K Niemann Nadja N Wahn Ulrich U Staab Doris D

Health and quality of life outcomes 20091202

<h4>Background</h4>This study evaluates the psychometric properties of the Child and Parent versions of the German CFQ-R (Cystic Fibrosis Questionnaire Revised), a disease-specific measure of Health-Related Quality of Life (HRQoL) in children with cystic fibrosis (CF). Self-Rating is combined with proxy-rating by parents in the use of the questionnaire.<h4>Methods</h4>136 children with CF (6 - 13 years) and their parents were recruited to evaluate internal consistency (Cronbach's alpha) and vali ...[more]

PMID: 19954541

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Project description:ObjectiveAirway clearance physiotherapy is recommended in cystic fibrosis, but limited evidence exists to suggest how much treatment is enough. As a secondary analysis of a prior study investigating the safety, efficacy, and participants' perceptions of a novel airway clearance technique, specific cough technique (SCT) compared to forced expiration technique (FET), we aimed to evaluate whether the intervention was associated with changes in health-related quality of life (HRQoL).MethodsWe conducted randomised, controlled individual trials with six adults (N-of-1 RCTs). Each trial included eight weeks of treatment, twice a week, using saline inhalation in horizontal positions, one with SCT and one with FET, in random order. Efficacy was measured by sputum wet weight (g) after each session. Perceived usefulness and preference were self-reported at the end of the study. Lung function was assessed at baseline and at the end of study. HRQoL was measured using the Cystic Fibrosis Questionnaire-Revised (CFQ-R) at baseline (week 1) and at completion of the study (week 8). Individual HRQoL scores (0-100) were coded and analysed using CFQ-R Software Program, version 2.0.ResultsPatient-reported outcomes were completed by all subjects. Individual CFQ-R-Respiratory Symptoms Scores (CFQ-R-RSS) showed a positive change, meeting the minimal important difference (MID) ≥ 4 points in five participants and a negative change in one individual. A strong correlation (r = 0.94 (p<0.01) was found between total sputum weight (g) and the positive changes in CFQ-R-RSS, and between changes in lung function and CFQ-R-RSS (r = 0.84 (p = 0.04).ConclusionThe airway clearance intervention was associated with clinically meaningful changes in patient-reported symptoms on the CFQ-R in the majority of the participants. This finding warrants further investigation regarding treatment, duration and frequency. A long-term study may reveal beneficial effects on other clinically meaningful endpoints, such as pulmonary exacerbations, high-resolution computed tomography scores and HRQoL.Trial registrationThe study was registered in ClinicalTrials.gov, under the number NCT0 1266473.

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.

Project description:BackgroundHealth-related quality of life (HRQOL) has received much attention in patients with cystic fibrosis (CF). The goal of this study was to evaluate the association between clinical, lung function, sleep quality, and polysomnographic variables with 2 HRQOL questionnaires, the shorter-version World Health Organization Quality of Life (WHOQOL-BREF) and Cystic Fibrosis Quality of Life (CFQOL) questionnaires, in adult subjects with CF.MethodsIn a cross-sectional study, 51 subjects underwent clinical evaluation and overnight polysomnography and answered WHOQOL-BREF, CFQOL, Pittsburgh Sleep Quality Index, and Epworth Sleepiness Scale questionnaires. In addition, pulmonary function tests, 6-min walk tests, and echocardiography were performed.ResultsFor WHOQOL-BREF scores, the sleep quality index was associated with the physical domain; the percent-of-predicted 6-min walk distance (6MWD) and sleepiness scale were associated with the psychological domain; the percent-of-predicted FEV1 and sleep quality index were associated with the social relationship domain; and the sleep quality index was associated with the environment domain. For CFQOL scores, age at diagnosis, clinical score, and sleep quality index were associated with the physical functioning domain; the percent-of-predicted 6MWD and pulmonary arterial systolic pressure were associated with the role domain; sex and sleep quality index were associated with the vitality domain; the apnea-hypopnea index was associated with the emotional functioning domain; sex and body mass index (BMI) were associated with the body image domain; the percent-of-predicted 6MWD and sleep quality index were associated with the health perception domain; age, sex, BMI, and arousal index were associated with the weight domain; age, sex, percent-of-predicted FEV1, percent-of-predicted 6MWD, and pulmonary arterial systolic pressure were associated with the respiratory symptom domain; and the clinical score was associated with the digestive symptom domain.ConclusionsThe sleep quality index score, 6MWD, sleepiness scale score, and FEV1 were predictors of WHOQOL-BREF scores. Age at diagnosis, clinical score, sleep quality score, 6MWD, sex, apnea-hypopnea index, BMI, current age, arousal index, FEV1, and pulmonary arterial systolic pressure were predictors of CFQOL scores.

Dataset Information

Health-related quality of life in children with cystic fibrosis: validation of the German CFQ-R.

Background

Methods

Results

Conclusion

Publications

Health-related quality of life in children with cystic fibrosis: validation of the German CFQ-R.

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