Please note: While some information will still be current in a year, other information may already be out of date in three months time. If you are in any doubt, please feel free to ask.


Please could you advise me which are the best validated & accepted survey questionnaires designed for adult and adolescent CF patients to:
ascertain their knowledge of CF,
quality of life
satisfaction with the CF services they access.


Thank you for your question asking about outcome measures to assess a patient’s knowledge of CF, quality of life and satisfaction with CF services. I will try to answer each of your queries separately.

Knowledge of Cystic Fibrosis
To my knowledge there is no universal fully validated questionnaire to ascertain patient knowledge of their CF disease. However, some groups have developed there own questionnaire to assess knowledge, for example a questionnaire was developed in Leeds (UK) to assess patient knowledge. This questionnaire was a multiple choice questionnaire covering the following areas of CF: lung infection, gastrointestinal problems, reproduction and sexuality, lung complications, general and miscellaneous, diabetes, liver problems, nasal polyps, lung transplant and genetics. In this study patients were aged from 15-40 years and so included both adolescents and adults. The full questionnaire is available from the group on request and the contact details are in the reference below.

Conway SP, Pond MN, Watson A, Hamnett T (1996). Knowledge of adult patients with cystic fibrosis about their illness. Thorax;51:34-38.
Contact details: Regional Adult Cystic Fibrosis Unit, Seacroft Hospital, York Road, Leeds, LS14 6UH, UK.

Quality of Life
In a review of patient-reported outcomes in cystic fibrosis, two different disease specific health related quality of life (HRQOL) instruments have been developed specifically for CF. They are the Cystic Fibrosis Questionnaire-revised (CFQ-R) and the Cystic Fibrosis Quality of Life (CFQoL) questionnaire. The CFQ is available in an American or UK format and include several versions: an interview format for children aged from 6-11 years, a self-report for children aged 12-13 years, a self report for adolescents and adults aged 14 years and older and a caregiver version. The CFQoL questionnaire consists of 52 items across nine domains of functioning which have been identified by and are of importance to adolescents and adults with cystic fibrosis (Gee et al, 2000). Both of these instruments are considered valid instruments with demonstrated reliability, internal validity and sensitivity (Goss and Quittner, 2007). Additionally there has been an excellent review of quality of life just published that considers various instruments to measure quality of life, the factors that influence how people report quality of life, the monitoring of quality of life in clinical practice and whether quality of life can predict survival (Abbot, 2009).

Gee L, Abbott J, Conway SP, Etherington C, Webb AK (2000). Development of a disease specific health related quality of life measure for adults and adolescents with cystic fibrosis. Thorax;55:946-954.

Goss CH, Quittner AL (2007). Patient reported outcomes in cystic fibrosis. Proc Am Thorac Soc;Vol 4 pp378-386.

Abbott J (2009) Health related quality of life measurement in cystic fibrosis: advances and limitations. Chronic Repsiratory disease. 6; 31-41.

Satisfaction with CF Services
Many hospitals use generic patient satisfaction questionnaires but to my knowledge there is no universal fully validated questionnaire to assess level of satisfaction specifically with the CF services they access. That said, in the UK the Cystic Fibrosis Trust started a process of reviewing CF Centres several years ago and these are still ongoing today. The aim of these peer reviews is to help improve the level of care that specialist CF Centres and networked clinics in the UK can offer to their patients. As part of peer review all patients attending a centre due to be peer reviewed are sent a questionnaire which asks them about satisfaction with different aspects of the CF service. For more detail on this questionnaire please contact the CF Trust directly.

Best wishes,

Judy Bradley
Lauren Wilson
The answer is edited by: Prof Judy Bradley