Cystic Fibrosis

Cystic fibrosis is a genetic disease that frequently has its first health impact in infancy. The disease often progresses through childhood into young adulthood, with death usually occurring as a result of pulmonary complications. The average lifespan has been steadily increasing, with a mean life expectancy of 30 years by 1995. While cystic fibrosis may be viewed as similar to COPD in its chronicity and pulmonary disability, the younger age distribution that influences expectations about functional status and quality of life and characteristics may affect how measures perform in this disease.

Characteristics that may contribute to variability of QOL impact in cross-sectional and longitudinal studies include:

• Variable progression of disease
• Multiple body systems affected to varying degrees, with predominance of pulmonary and gastrointestinal complications
• Unpredictable occurrences of acute exacerbations, particularly of respiratory disease, which may have particular salience compared to the chronic symptoms of the disease
• Need for complex treatment regimen, particularly as the disease severity increases

Both generic and respiratory condition-specific instruments have been used to measure QOL in cystic fibrosis.

Generic Health-Related Quality of Life Instruments Used in Cystic Fibrosis

Cystic Fibrosis Questionnaire (CFQ); Cystic Fibrosis Questionnaire Revised (CFQ-R)

• Henry B, Aussage P, Grosskopf C, Goehrs JM. Development of the Cystic Fibrosis Questionnaire (CFQ) for assessing quality of life in pediatric and adult patients. Qual Life Res. 2003 Feb;12(1):63-76.
• Wenninger K, Aussage P, Wahn U, Staab D; German Cystic Fibrosis Questionnaire study group. The revised German Cystic Fibrosis Questionnaire: validation of a disease-specific health-related quality of life instrument. Qual Life Res. 2003 Feb;12(1):77-85.
• Modi AC, Quittner AL. Validation of a disease-specific measure of health-related quality of life for children with cystic fibrosis. J Pediatr Psychol. 2003 Dec;28(8):535-45.
• Quittner AL, Sweeny S, Watrous M, Munzenberger P, Bearss K, Gibson Nitza A, Fisher LA, Henry B. Translation and linguistic validation of a disease-specific quality of life measure for cystic fibrosis. J Pediatr Psychol. 2000 Sep;25(6):403-14.
• Saiman L, Marshall BC, Mayer-Hamblett N, Burns JL, Quittner AL, Cibene DA, Coquillette S, Fieberg AY, Accurso FJ, Campbell PW 3rd; Macrolide Study Group. Azithromycin in patients with cystic fibrosis chronically infected with Pseudomonas aeruginosa: a randomized controlled trial. JAMA. 2003 Oct 1;290(13):1749-56.
• Wenninger K, Weiss C, Wahn U, Staab D. Body image in cystic fibrosis – development of a brief diagnostic scale. J Behav Med 2003;26:81-94.
• Klijn, P.H., van Stel, H.F, Quittner, A.L, van der Net, J., Doeleman, W., van der Schans, C.P, van der Ent, C.K. Validation of the dutch cystic fibrosis questionnaire (CFQ) in adolescents and adults. J Cyst Fibros. 2004 Mar;3(1):29-36.

Child Health Questionnaire - Parent Form 50

• Britto MT, Kotagal UR, Hornung RW, Atherton HD, Tsevat J, Wilmott RW. Impact of recent pulmonary exacerbations on quality of life in patients with cystic fibrosis. Chest. Jan 2002;121(1):64-72.
• Britto MT, Kotagal UR , Chenier T, Tsevat J, Atherton HD, Wilmott RW. Differences between adolescents' and parents' reports of health-related quality of life in cystic fibrosis. Pediatr Pulmonol. 2004; 37(2):165-71.
• Sawyer MG, Reynolds KE, Couper JJ, et al. Health-related quality of life of children and adolescents with chronic illness--a two year prospective study. Qual Life Res. Sep 2004;13(7):1309-1319.
• Yi MS, Tsevat J, Wilmott RW, Kotagal UR, Britto MT. “The impact of treatment of pulmonary exacerbations on the health-related quality of life of patients with cystic fibrosis: does hospitalization make a difference?” J Pediatr. 144(6):711-8.

Stein Functional Status Scale

• Bartholomew LK, Czyzewski DI, Parcel GS, Swank PR, Sockrider MM, Mariotto MJ, Schidlow DV, Fink RJ, Seilheimer DK. Self-management of cystic fibrosis: short-term outcomes of the cystic fibrosis family education program. Health Educ Behav 1997;24(5):652-66.

Nottingham Health Profile

• Antonelli M, Midulla F, Tancredi G, et al. Bronchial artery embolization for the management of nonmassive hemoptysis in cystic fibrosis. Chest. Mar 2002;121(3):796-801.
• Busschbach JJ, Horikx PE, van den Bosch JM, Brutel de la Riviere A, de Charro FT. Measuring the quality of life before and after bilateral lung transplantation in patients with cystic fibrosis. Chest 1994;105(3):911-917.
• Caine N, Sharples LD, Smyth R, Scott J, Hathawah T, Higenbottam TW, Wallwork J. Survival and quality of life of cystic fibrosis patients before and after heart-lung transplantation. Transplant Proc 1991;23(1):1203-1204.
• Congleton J, Hodson ME, Duncan-Single F. Do Nottingham Health Profile scores change over time in cystic fibrosis? Respir Med 1998;92:268-72.
• Congleton J, Hodson ME, Duncan-Single F. Quality of life in adults with cystic fibrosis. Thorax 1996;51:936-40.
• Dennis C, Caine N, Sharples L, et al. Heart-lung transplantation for end-stage respiratory disease in patients with cystic fibrosis at Papworth Hospital. J Heart Lung Transpl 1993;12:893-902.

Quality of Well Being

• Bartholomew LK, Czyzewski DI, Parcel GS, Swank PR, Sockrider MM, Mariotto MJ, Schidlow DV, Fink RJ, Seilheimer DK. Self-management of cystic fibrosis: short-term outcomes of the cystic fibrosis family education program. Health Educ Behav 1997;24(5):652-666.
• Czyzewski DI, Mariotto MH, 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.
• 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):393-8.
• Orenstein DM, Hovell MF, Mulvihill M, et al. Strength vs aerobic training in children with cystic fibrosis: a randomized controlled trial. Chest. Oct 2004;126(4):1204-1214.
• Orenstein DM, Kaplan RM. Measuring the Quality of Well-Being in cystic fibrosis and lung transplantation: the importance of the area under the curve. Chest 1991;100:1016-1018.
• Orenstein DM, Nixon PA, Ross EA, Kaplan RM. The quality of well-being in cystic fibrosis. Chest 1989-95:344-347.
• Orenstein DM, Pattishall EN, Ross EA, Kaplan RM. Quality of well-being before and after antibiotic treatment of pulmonary exacerbation in cystic fibrosis. Chest 1990;98:1081-1084.
• Selvadurai HC, Blimkie CJ, Cooper PJ, Mellis CM, Van Asperen PP. Gender differences in habitual activity in children with cystic fibrosis. Arch Dis Child. Oct 2004;89(10):928-933.
• Selvadurai HC, Blimkie CJ, Meyers N, Mellis CM, Cooper PJ, Van Asperen PP. Randomized controlled study of in-hospital exercise training programs in children with cystic fibrosis. Pediatr Pulmonol. Mar 2002;33(3):194-200.
• Squier HC, Ries AL, Kaplan RM, Prewitt LM, Smith CM, Kriett JM, Jamieson SW. Quality of Well-being predicts survival in lung transplantation candidates. Am J Resp Crit Care Med 1995; 152:2032-2036.

Self-Administered Dependency Questionnaire

• Drotar D, Ievers C. Age differences in parent and child responsibilities for management of cystic fibrosis and insulin-dependent diabetes mellitus. Dev Behav Pediatr 1994;15(4):265-272.

Medical Outcomes Study SF-36

• Abbott J, Baumann U, et al. Cross cultural differences in health related quality of life in adolescents with cystic fibrosis. Disabil Rehabil 2001;23(18): 837-44.
• Blair C, Kacser E, Porteous D. Gene therapy for cystic fibrosis: a psychosocial study of trial participants. Gene Ther 1998;5:218-22.
• Boling W, Macrina DM, Clancy JP. The Caregiver Quality of Life Cystic Fibrosis (CQOLCF) scale: modification and validation of an instrument to measure quality of life in cystic fibrosis family caregivers. Qual Life Res. 2003;12(8):1119-26.
• Britto MT, Kotagal UR, Hornung RW, Atherton HD, Tsevat J, Wilmott RW. Impact of recent pulmonary exacerbations on quality of life in patients with cystic fibrosis. Chest. Jan 2002;121(1):64-72.
• Goldbeck L, Schmitz TG. Comparison of three generic questionnaires measuring quality of life in adolescents and adults with cystic fibrosis: the 36-item short form health survey, the quality of life profile for chronic diseases, and the questions on life satisfaction. Qual Life Res. 2001;10(1):23-36.
• Yi MS, Tsevat J, Wilmott RW, Kotagal UR, Britto MT. “The impact of treatment of pulmonary exacerbations on the health-related quality of life of patients with cystic fibrosis: does hospitalization make a difference?” J Pediatr. 144(6):711-8.

Sickness Impact Profile

• deJong JJ, Kaptein AA, van der Schans CP, Mannes GP, van Aalderen WM, Grevink RG, Koeter GH. Quality of life in patients with cystic fibrosis. Ped Pulmonol 1997;23(2):95-100

Functional Status II-R (FS II-R)

• Bartholomew LK, Czyzewski DI, Parcel GS, Swank PR, Sockrider MM, Mariotto MJ, Schidlow DV, Fink RJ, Seilheimer DK. Self-management of cystic fibrosis: short-term outcomes of the cystic fibrosis family education program. Health Education & Behavior 1997;24(5):652-666.

Condition-Specific Health-Related Quality of Life Instruments Used in Cystic Fibrosis

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Asthma Quality of Life Questionnaire [Juniper's]

• Granton JT, Shapiro C, Kesten S. Noninvasive nocturnal ventilatory support in advanced lung disease from cystic fibrosis. Respir Care. Jun 2002;47(6):675-681.

Caregiver Quality of Life in Cystic Fibrosis (CQOLCF)

• Boling W, Macrina DM, Clancy JP. The Caregiver Quality of Life Cystic Fibrosis (CQOLCF) scale: modification and validation of an instrument to measure quality of life in cystic fibrosis family caregivers. Qual Life Res. 2003;12(8):1119-26.

Chronic Respiratory Disease Questionnaire-CRQ

• Bradley J, Dempster M, Wallace E, Elborn S. The adaptations of a quality of life qu estionnaire for routine use in clinical practice: the Chronic Respiratory Disease Questionnaire in cystic fibrosis. Qual Life Res 1999;8:65-71.
• Granton JT, Shapiro C, Kesten S. Noninvasive nocturnal ventilatory support in advanced lung disease from cystic fibrosis. Respir Care. Jun 2002;47(6):675-681.
• Wolter J, Seeney S, Bell S, Bowler S, Masel P, McCormack J. Effect of long term treatment with azithromycin on disease parameters in cystic fibrosis: a randomised trial. Thorax. Mar 2002;57(3):212-216.
• Wolter JM, Bowler SD, Nolan PJ, McCormack JG. Home intravenous therapy in cystic fibrosis: a prospective randomized trial examining clinical, quality of life, and cost aspects. Eur Respir J 1997;10:896-900.

Cystic Fibrosis Quality of Life Questionnaire

• Henry B, Aussage P, Grosskopf C, Goehrs JM. Development of the Cystic Fibrosis Questionnaire (CFQ) for assessing quality of life in pediatric and adult patients. Qual Life Res. 2003;12(1):63-76.
• Klijn PH, Oudshoorn A, van der Ent CK, van der Net J, Kimpen JL, Helders PJ. (2004) “Effects of anaerobic training in children with cystic fibrosis: a randomized controlled study.” Chest. 125(4):1299-305
• Sawyer MG, Reynolds KE, Couper JJ, et al. Health-related quality of life of children and adolescents with chronic illness--a two year prospective study. Qual Life Res. 2004;13(7):1309-1319.
• Wenninger K, Weiss C, Wahn U, Staab D. Body image in cystic fibrosis--development of a brief diagnostic scale. J Behav Med. 2003;26(1):81-94.
• Wenninger K, Aussage P, Wahn U, Staab D. The revised German Cystic Fibrosis Questionnaire: validation of a disease-specific health-related quality of life instrument. Qual Life Res. 2003;12(1):77-85.

Questions on Life Satisfaction - Cystic Fibrosis (FLZM -CF)

• Goldbeck L, Schmitz T.G., Henrich G, Herschbach P. Questions on life satisfaction for adolescents and adults with cystic fibrosis. Chest. 2003 Jan;123(1):42-8.