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Development of the Cystic Fibrosis Questionnaire (CFQ) for assessing quality of life in pediatric and adult patients

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Abstract

To assess the impact of cystic fibrosis (CF) and treatment on quality of life (QOL) from childhood throughout adult age, two versions of the Cystic Fibrosis Questionnaire (CFQ), were developed and validated in France: the CFQ 14+ for teenagers and adults, the CFQ Child P, a parent-proxy evaluation for children aged 8–13. They include three modules for assessing QOL, symptoms and health perception. Nine QOL dimensions were identified: physical functioning, energy/well-being, emotions, social limitations, role, embarrassment, body image, eating disturbances and treatment burden. Items were derived from 33 interviews with patients and parents. Item reduction and assessment of internal consistency, convergent and discriminant validity were based on a large cross-sectional survey among 393 patients and parents. A second study was conducted among 124 patients and 85 parents to test reproducibility and responsiveness, confirm the subscale structure and assess scalar properties using Rasch analysis. All psychometric properties were successfully demonstrated and both the CFQ 14+ and the CFQ Child P French questionnaires are now well validated. German and Spanish validated adaptations are available, an English validation is in progress. Conclusion: The CFQ 14+ and CFQ Child P are well validated, multilingual measures which allow QoL assessment in children, teenagers and adults with CF.

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References

  1. Farriaux JP, Dhondt JL. Donneés épidémiologiques-Dépistage néo-et anténatal. Rev Pneumol Clin 1995; 51(3): 109–114.

    Google Scholar 

  2. Delaisi B, Grosskopf C, Reignault E, Goehrs J-M, Navarro J. Registre international sur la mucoviscidose: Comparaison des donneés fran çaises avec les donneées européennes pour 1995. Arch Pediatr 1998; 5: 384–388.

    Google Scholar 

  3. Shwachman H, Kulezycki LL. Long-term study of one hundred five patients with cystic fibrosis. Am J Dis Child 1958; 96: 6–15.

    Google Scholar 

  4. Taussig LM, Kattwinkel J, Friedewald WT, diSant'Agnese PA. A new prognostic score and clinical evaluation system for cystic fibrosis. J Pediatr 1973; 82: 380–390.

    Google Scholar 

  5. Eigen H, Clark NM, Woole JM. National Heart, Lung and Blood Institute workshop summary. Clinical-behavioral aspects of cystic fibrosis: Directions for future research. Am Rev Respir Dis 1987; 136: 1509–1513.

    Google Scholar 

  6. Quittner AL. Measurement of quality of life in cystic fibrosis. Curr Opin Pulmonol Med 1998; 4: 326–331.

    Google Scholar 

  7. Orenstein DM, Nixen PA, Rons EA, Kaplan RM. The quality of well-being in cystic fibrosis. Chest 1989; 95: 344–347.

    Google Scholar 

  8. Tullis E, Guyatt G. Quality of life in cystic fibrosis. PharmacoEconomics 1995; 8: 23–33.

    Google Scholar 

  9. Congleton J, Hodson ME, Duncan-Skingle F. Quality of life in adults withcystic fibrosis. Thorax 1996; 51(9): 936–940.

    Google Scholar 

  10. De Jong W, Kaptein AA, Van der Schans CP, et al. Quality of life in patients withcystic fibrosis. Pediatr Pulmonol 1997; 23(2): 95–100.

    Google Scholar 

  11. Congleton J, Soulsby RE, Jones VS, et al. Quality of life in adults with Cystic Fibrosis [abstract]. Israel J Med Sci, June 1996; 32: S180.

    Google Scholar 

  12. Gee L, Abbott J, Conway S, et al. Testing and validation of the SF-36 as a suitable quality of life measure for adults with CF [abstract]. The Netherlands J Med 1999; 54: S75.

    Google Scholar 

  13. Caine N, Sharples LD, Smyth R, et al. Survival and quality of life of cystic fibrosis patients before and after heart lung transplantation. Transplant Proc 1991; 23(1 Pt 2): 1203–1204.

    Google Scholar 

  14. De Saint Phalle V, Bloch J, Dazord A, Foucaud D, Lenoir G, Navarro J. Preliminary results on quality of life of lung-transplanted CF patients [abstract]. Israel J Med Sci, June 1996; 32: S180.

    Google Scholar 

  15. Orenstein DM, Kaplan RM. Measuring the quality of life of well-being in cystic fibrosis and lung transplantation. Chest 1991; 100: 344–347.

    Google Scholar 

  16. Orenstein DM, Pattishall EN, Nixon PA, Ross EA, Kaplan RM. Quality of well-being before and after antibiotic treatment of pulmonary exacerbation in patients with Cystic Fibrosis. Chest 1990; 98: 1081–1084.

    Google Scholar 

  17. Czyzewski DI, Mariotto MJ, Bartholomew LK, Lecompte SH, Sockrider MM. Measurement of quality of well-being in a child and adolescent cystic fibrosis population. Med Care 1994; 32(9): 965–972.

    Google Scholar 

  18. Munzenberger PJ, Van Wagnen CA, Abdulhamid I, Walker PC. Quality of life as a treatment outcome in patients with cystic fibrosis. Pharmacotherapy 1999; 19(4): 393–398.

    Google Scholar 

  19. Congleton J, Hodson ME, Duncan-Skingle F. Do Nottingham Health Profile change over time in cystic fibrosis? Respir Med 1998; 92(2): 268–272.

    Google Scholar 

  20. Robinson M. Effect of rhDNase on quality of life and spirometry in patients with cystic fibrosis [abstract]. Am Rev Respir Dis 1996; 153(4): A69.

    Google Scholar 

  21. Soulsby et al. Thorax 1995; 50(Suppl 2): A66.

    Google Scholar 

  22. Cappelli M, McGrath PJ, Heick CE, McDonald NE, Feldman W, Rowe P. Chronic disease and its impact: The adolescent's perspective. J Adolesc Health Care 1989; 10: 283–288.

    Google Scholar 

  23. Pearson DA, Pumariega AJ, Seilheimer DK. The development of psychiatric symptomatology in patients with cystic fibrosis. J Am Acad Child Adolesc Psychiatry 1991; 30(2): 290–297.

    Google Scholar 

  24. Shepherd SL, Hovell MF, Slymen DJ, et al. Functional status as an overall measure of health in adults with cystic fibrosis: Further validation of a generic health measure. J Clin Epidemiol 1992; 45(2): 117–125.

    Google Scholar 

  25. Juniper EF, Guyatt GH, Epstein RS, Ferrie PJ, Jaeschke R, Hiller TK. Evaluation of impairment of health related quality of life in asthma: Development of a questionnaire for use in clinical trials. Thorax 1992; 47(2): 76–83.

    Google Scholar 

  26. SPSS version 8.0. User's Guide. SPSS Inc. Marketing Department, Chicago, IL.

  27. Wright BD, Douglas GA. Best procedures for Sample-free item analysis. Appl Psychol Meas 1997; 37: 47–60.

    Google Scholar 

  28. Wright BD, Stone MH. Best Test Design. Chicago: MESA Press, 1979.

    Google Scholar 

  29. Linacre JM, Wright BD. A User's Guide to Bigsteps. Rasch-Model-Computer Program. Chicago: MESA Press, 1997.

    Google Scholar 

  30. Cella DF, Lloyd SR, Wright BD. Cross-cultural instrument equating: Current researchand future directions. In: Spilker B, (ed) Quality of Life and Pharmacoeconomics in Clinical Trials, 2nd ed. Philadelphia: Lippincott-Raven Publishers, 1996; 707–715.

    Google Scholar 

  31. Deyo RA, Diehr P, Patrick DL. Reproducibility and responsiveness of health status measures. Statistics and strategies for evaluation. Control Clin Trials 1991; 12 (4 Suppl): 142S–158S.

    Google Scholar 

  32. Cohen J. Statistical Power Analysis for the Behavioral Sciences, (revised edition) New York: Academic Press, 1977, 41.

    Google Scholar 

  33. Ware JE. Conceptualizing disease impact and treatment outcomes. Cancer 1984; 53: 2316–2326.

    Google Scholar 

  34. Gee L, Abbott J, Conway S, Etherington C, Webb AK. A disease specific health related quality of life measure for adults with CF [abstract]. The Netherlands J Med 1999; 54: S74.

    Google Scholar 

  35. Robinson M, O'Connor R, Bye P, et al. Development of a new quality of life instrument for cystic fibrosis patients and its implementation for assessing the effectiveness of a new therapy (rhDNase)-results of a 12 month study [abstract]. Pediatr Pulmonol 1998; Suppl. 17: 392.

    Google Scholar 

  36. Ware JE Jr, Gandek B. Overview of the SF-36 health survey and the International Quality of Life assessment (IQOLA) Project. J Clin Epidemiol 1998; 51(11): 903–912.

    Google Scholar 

  37. Henry B, Staab D, Prados C, et al. How to measure quality of life in cystic fibrosis (CF) patients across countries and cultures: The Cystic Fibrosis Questionnaire (CFQ) [abstract]. Pediatr Pulmonol 1998; 17(Suppl): 392–393.

    Google Scholar 

  38. Henry B, Aussage P, Staab D, Prados C, Grosskopf C, Goehrs J-M. Assessing cross-cultural validity of the Cystic Fibrosis Questionnaire (CFQ) [abstract]. Qual Life Res 1998; 7(7): 606–607.

    Google Scholar 

  39. Quittner A, Sweeny S, Watrous M, Munzenberger P, Henry B. Initial US validation of a disease-specific quality of life (QOL) measure for cystic fibrosis: The Cystic Fibrosis Questionnaire [abstract]. Pediatr Pulmonol 1998; 17: 393.

    Google Scholar 

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Henry, B., Aussage, P., Grosskopf, C. et al. Development of the Cystic Fibrosis Questionnaire (CFQ) for assessing quality of life in pediatric and adult patients. Qual Life Res 12, 63–76 (2003). https://doi.org/10.1023/A:1022037320039

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