Skip to main content

Advertisement

Log in

Psychometric evaluation of the Cystic Fibrosis Questionnaire-Revised in a national sample

  • Published:
Quality of Life Research Aims and scope Submit manuscript

An Erratum to this article was published on 13 January 2012

Abstract

Purpose

The psychometric properties of a health-related quality of life (HRQOL) instrument, the Cystic Fibrosis Questionnaire-Revised (CFQ-R), were evaluated in a national sample of patients with cystic fibrosis (CF).

Methods

The Epidemiologic Study of CF is a national, multicenter, longitudinal cohort study containing CFQ-R and health outcomes data. Developmentally appropriate versions of the CFQ-R were available from 7,330 patients aged 6–70 years and a proxy version from 2,728 parents of school-age children. The CFQ-R was completed during a “stable” or “sick” visit before recording health outcomes such as weight, lung function, and pulmonary exacerbations.

Results

There were few floor and ceiling effects and strong internal consistency (Cronbach alpha ≥0.70) for most scales. The CFQ-R consistently discriminated between patients seen for sick-versus-well visits, and among stages of disease severity based on lung function. As predicted, women with CF reported worse HRQOL than men on scales not related to body image and weight. Strong parent–child agreement was found on scales measuring observable behaviors (respiratory symptoms). Convergence between CFQ-R scales and health outcomes provided evidence of construct validity.

Conclusions

The CFQ-R demonstrated robust psychometric properties and consistent associations with health outcomes in a large national sample.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

Abbreviations

ANOVA:

Analysis of variance

BMI:

Body mass index

CF:

Cystic fibrosis

CFQ-R:

Cystic Fibrosis Questionnaire-Revised

ESCF:

Epidemiologic Study of Cystic Fibrosis

FEV1 :

Forced expiratory volume in 1 s

HRQOL:

Health-related quality of life

IV:

Intravenous

MID:

Minimal important difference

PRO:

Patient-reported outcome

SF-36:

36-Item Short Form Health Survey

References

  1. Center for Drug Evaluation and Research (2010) Guidance for industry. Patient-reported outcome measures: Use in medical product development to support labeling claims. US Department of Health and Human Services, Food and Drug Administration. December 2009. http://www.fda.gov/downloads/Drugs/GuidanceComplianceRegulatoryInformation/Guidances/UCM193282.pdf. Accessed July 1, 2010.

  2. Fayers, P., & Hays, R. (Eds.). (2005). Assessing quality of life in clinical trials: Methods and practice (2nd ed.). New York: Oxford University Press.

    Google Scholar 

  3. Retsch-Bogart, G. Z., Quittner, A. L., Gibson, R. L., Oermann, C. M., McCoy, K. S., Montgomery, A. B., et al. (2009). Efficacy and safety of inhaled aztreonam lysine for airway pseudomonas in cystic fibrosis. Chest, 135(5), 1223–1232.

    Article  PubMed  CAS  Google Scholar 

  4. Goss, C. H., & Quittner, A. L. (2007). Patient-reported outcomes in cystic fibrosis. Proceedings of the American Thoracic Society, 4(4), 378–386.

    Article  PubMed  Google Scholar 

  5. Quittner, A. L., Modi, A., & Cruz, I. (2008). Systematic review of health-related quality of life measures for children with respiratory conditions. Paediatric Respiratory Reviews, 9(3), 220–232.

    Article  PubMed  Google Scholar 

  6. Quittner, A. L., Modi, A. C., Wainwright, C., Otto, K., Kirihara, J., & Montgomery, A. B. (2009). Determination of the minimal clinically important difference scores for the Cystic Fibrosis Questionnaire-Revised respiratory symptom scale in two populations of patients with cystic fibrosis and chronic Pseudomonas aeruginosa airway infection. Chest, 135(6), 1610–1618.

    Article  PubMed  Google Scholar 

  7. Quittner, A. L., Buu, A., Messer, M. A., Modi, A. C., & Watrous, M. (2005). Development and validation of The Cystic Fibrosis Questionnaire in the United States: A health-related quality-of-life measure for cystic fibrosis. Chest, 128(4), 2347–2354.

    Article  PubMed  Google Scholar 

  8. Kaplan, R. M., Anderson, J. P., Wu, A. W., Mathews, W. C., Kozin, F., & Orenstein, D. (1989). The Quality of Well-being Scale. Applications in AIDS, cystic fibrosis, and arthritis. Medical Care, 27(3 Suppl), S27–S43.

    Article  PubMed  CAS  Google Scholar 

  9. Ware, J. E., Jr., & Sherbourne, C. D. (1992). The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Medical Care, 30(6), 473–483.

    Article  PubMed  Google Scholar 

  10. Palermo, T. M., Long, A. C., Lewandowski, A. S., Drotar, D., Quittner, A. L., & Walker, L. S. (2008). Evidence-based assessment of health-related quality of life and functional impairment in pediatric psychology. Journal of Pediatric Psychology, 33, 983–996.

    Article  PubMed  Google Scholar 

  11. Scoggins, J. F., & Patrick, D. L. (2009). The use of patient-reported outcomes instruments in registered clinical trials: Evidence from ClinicalTrials.gov. Contemporary Clinical Trials, 30(4), 289–292.

    Article  PubMed  Google Scholar 

  12. Cystic Fibrosis Foundation Patient Registry. (2007). Annual data report 2007. Bethesda, MD: Cystic Fibrosis Foundation.

    Google Scholar 

  13. Quittner, A. L., Barker, D. H., Snell, C., Grimley, M. E., Marciel, K. K., & Cruz, I. (2008). Prevalence and impact of depression in cystic fibrosis. Current Opinion in Pulmonary Medicine, 14(6), 582–588.

    Article  PubMed  Google Scholar 

  14. Smith, B. A., Modi, A. C., Quittner, A. L., & Wood, B. L. (2010). Depressive symptoms in children with cystic fibrosis and parents and its effects on adherence to airway clearance. Pediatric Pulmonology, 45(8), 756–763.

    Article  PubMed  Google Scholar 

  15. Rosenfeld, M., Davis, R., FitzSimmons, S., Pepe, M., & Ramsey, B. (1997). Gender gap in cystic fibrosis mortality. American Journal of Epidemiology, 145(9), 794–803.

    Article  PubMed  CAS  Google Scholar 

  16. Courtney, J. M., Bradley, J., McCaughan, J., O’Connor, T. M., Shortt, C., Bredin, C. P., et al. (2007). Predictors of mortality in adults with cystic fibrosis. Pediatric Pulmonology, 42(6), 525–532.

    Article  PubMed  CAS  Google Scholar 

  17. Briesacher, B.A., Quittner. A.L., Fouayzi, H., Zhang, J., & Swensen, A. (2011). Nationwide trends in the medical care costs of privately-insured patients with cystic fibrosis, 2001–2007. Pediatric Pulmonology, 46(8), 770–776.

    Google Scholar 

  18. Sawicki, G. S., Sellers, D. E., & Robinson, W. M. (2009). High treatment burden in adults with cystic fibrosis: Challenges to disease self-management. Journal of Cystic Fibrosis, 8(2), 91–96.

    Article  PubMed  Google Scholar 

  19. Modi, A. C., & Quittner, A. L. (2003). Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis. Journal of Pediatric Psychology, 28(8), 535–545.

    Article  PubMed  Google Scholar 

  20. Henry, B., Aussage, P., Grosskopf, C., & Goehrs, J. M. (2003). Development of the Cystic Fibrosis Questionnaire (CFQ) for assessing quality of life in pediatric and adult patients. Quality of Life Research, 12(1), 63–76.

    Article  PubMed  Google Scholar 

  21. Quittner, A. L., Sweeny, S., Watrous, M., Munzenberger, P., Bearss, K., Gibson Nitza, A., et al. (2000). Translation and linguistic validation of a disease-specific quality of life measure for cystic fibrosis. Journal of Pediatric Psychology, 25(6), 403–414.

    Article  PubMed  CAS  Google Scholar 

  22. McCoy, K. S., Quittner, A. L., Oermann, C. M., Gibson, R. L., Retsch-Bogart, G. Z., & Montgomery, A. B. (2008). Inhaled aztreonam lysine for chronic airway Pseudomonas aeruginosa in cystic fibrosis. American Journal of Respiratory and Critical Care Medicine, 178(9), 921–928.

    Article  PubMed  CAS  Google Scholar 

  23. Donaldson, S. H., Bennett, W. D., Zeman, K. L., Knowles, M. R., Tarran, R., & Boucher, R. C. (2006). Mucus clearance and lung function in cystic fibrosis with hypertonic saline. New England Journal of Medicine, 354(3), 241–250.

    Article  PubMed  CAS  Google Scholar 

  24. Elkins, M. R., Robinson, M., Rose, B. R., Harbour, C., Moriarty, C. P., Marks, G. B., et al. (2006). A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. New England Journal of Medicine, 354(3), 229–240.

    Article  PubMed  CAS  Google Scholar 

  25. Accurso, F. J., Rowe, S. M., Clancy, J. P., Boyle, M. P., Dunitz, J. M., et al. (2010). Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. New England Journal of Medicine, 363, 1991–2003.

    Article  PubMed  CAS  Google Scholar 

  26. Cystic Fibrosis Foundation. (2010). Cystic Fibrosis Foundation Patient Registry: 2009 annual data report. Bethesda, MD: Cystic Fibrosis Foundation.

    Google Scholar 

  27. Ware, J. E., Brook, R. H., Davies-Avery, A. R., Williams, K. N., Stewart, A., Rogers, W. H., et al. (1980). Conceptualization and measurement of health for adults in the health insurance study: Vol. I, model of health and methodology. Santa Monica: Rand Corporation. Pub. no. R-1987/1-HEW.

  28. Martin, J. L., Ford, C. B., Dyer-Friedman, J., Tang, J., & Huffman, L. C. (2004). Patterns of agreement between parent and child ratings of emotional and behavioral problems in an outpatient clinical setting: When children endorse more problems. Journal of Developmental and Behavioral Pediatrics, 25(3), 150–155.

    Article  PubMed  Google Scholar 

  29. Levi, R., & Drotar, D. (1998). Measuring health-related quality of life in children and adolescents: Implications for research and practice. Mahwah, NJ: Lawrence Erlbaum Associates Publishers.

    Google Scholar 

  30. Havermans, T., Vreys, M., Proesmans, M., & De Boeck, C. (2006). Assessment of agreement between parents and children on health-related quality of life in children with cystic fibrosis. Child: Care, Health and Development, 32(1), 1–7.

    Article  CAS  Google Scholar 

  31. Schmidt, A., Wenninger, K., Niemann, N., Wahn, U., & Staab, D. (2009). Health-related quality of life in children with cystic fibrosis: Validation of the German CFQ-R. Health and Quality of Life Outcomes, 7, 97.

    Article  PubMed  Google Scholar 

  32. Cruz, I., Marciel, K. K., Cheney, J., Wainwright, C., Campbell, M., & Quittner, A. L. (2009). The preschool cystic fibrosis questionnaire-revised: Initial validation results. Pediatric Pulmonology, 32(Suppl), 421 (Abstract).

    Google Scholar 

  33. VanDevanter, D. R., Rasouliyan, L., Murphy, T. M., Morgan, W. J., Ren, C. L., Konstan, M. W., et al. (2005). Trends in the clinical characteristics of the U.S. cystic fibrosis patient population from 1995 to 2005. Pediatric Pulmonology, 43(8), 739–744.

    Article  Google Scholar 

Download references

Acknowledgments

All sources of support for the ESCF in the form of grants, case report forms, and data analysis were provided by Genentech, Inc., South San Francisco, California.

Conflict of interest

This study is sponsored by Genentech, Inc. Alexandra Quittner, Greg Sawicki, Ann McMullen, and Michael Konstan have received honoraria from Genentech for serving as members of the Scientific Advisory Group for the Epidemiologic Study of Cystic Fibrosis (ESCF). Alexandra Quittner and Michael Konstan have served as consultants to Genentech. No compensation was provided to these authors in exchange for the production of this manuscript. Lawrence Rasouliyan and David Pasta are employees of ICON Late Phase & Outcomes Research, which was paid by Genentech for providing analytical services for this study. Ashley Yegin is an employee of Genentech.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Alexandra L. Quittner.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (PDF 95 kb)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Quittner, A.L., Sawicki, G.S., McMullen, A. et al. Psychometric evaluation of the Cystic Fibrosis Questionnaire-Revised in a national sample. Qual Life Res 21, 1267–1278 (2012). https://doi.org/10.1007/s11136-011-0036-z

Download citation

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11136-011-0036-z

Keywords

Navigation