Health-related quality of life of patients with cystic fibrosis assessed by the SF-36 questionnaire.

INTRODUCTION
Cystic fibrosis (CF) is a genetic disorder, which is most common among Caucasians. There are about 100,000 people suffering from this disorder in the world, including 25 000 in Europe. Although the first mention of cystic fibrosis is thought to have occurred in 1595, recognition of the entire clinical spectrum of CF and the resultant development of contemporary knowledge occurred in the 20th century. In the past, CF was considered a fatal childhood disorder; however, contemporary statistical data shows that 50% of people with cystic fibrosis have a chance to live up to 30 years of age, and the lifespan of children born in the 1990s is projected to be at least 40 years. Consequently, the number of adults with cystic fibrosis is increasing, making it necessary for multidisciplinary actions aimed at the improvement of clinical management of the condition as well as minimizing the influence of CF and its treatment on the quality of continually extending life of patients. Since cystic fibrosis interferes with almost all important aspects of human functioning, quality of life (QoL) of individuals with CF should be constantly and closely monitored, thus allowing for consideration of their needs and providing an opportunity to modify the therapeutic approach if necessary. The aim of this study was to visualize the QoL of people with cystic fibrosis, to identify differences in their assessment of QoL depending on sex and age, as well as to compare the QoL of patients with CF with a control group of people without CF.


MATERIAL AND METHODS
The study was conducted among patients hospitalized at the Clinic of Pulmonology and Cystic Fibrosis at the Institute of Tuberculosis and Lung Diseases in Rabka-Zdrój from February to April 2012. The study encompassed 30 patients (19 women and 11 men), aged between 16 and 42 years, with acute episodes of bronchopulmonary disease. A Short Form-36 (SF-36) questionnaire was used to evaluate the health-related quality of life. The control group encompassed 30 healthy individuals with the same sex ratio and similar age ratio as in the group of patients.


RESULTS
The analysis of quality of life with the use of the SF-36 questionnaire showed that in general the quality of life of the assessed patients was low. The patients scored on average below 50 in the following subscales: Role Physical (RP), General Health (GP), Vitality (VT), Social Functioning (SF), Physical Component Summary (PCS) and Mental Component Summary (MCS). In other subscales the results slightly exceeded 50. In women, low quality of life was found in the following subscales: Role Physical, Bodily Pain (BP), General Health (GH), Vitality (VT), Social Functioning (SF), Physical Component Summary (PCS) and Mental Component Summary (MCS). In men, average results below 50 were found in the following subscales: General Health (GH) and Vitality (VT). Moreover, in all subscales, the quality of life assessed by women was lower than that reported by men.Patients > 25 years assessed their quality of life as lower in all subscales except for the Role Emotional (RE) subscale. Statistically significant differences were identified with regards to Vitality (VT) and Mental Health (MH). The analysis of the influence of CF patients' age on their quality of life, conducted with the use of the SF-36 questionnaire, showed that there were statistically significant correlations (p < 0.05) between age and the following domains: Physical Functioning (PF), Role Physical (RP), Bodily Pain (BP), Vitality (VT), Social Functioning (SF), Mental Health (MH) and Physical Component Summary (PCS). The comparison between the QoL of patients with CF and those of a control group of individuals not suffering from CF demonstrated that there were statistically significant differences in all subscales. Patients' quality of life was significantly poorer than quality of life of individuals without CF.


CONCLUSIONS
1. Patients with CF generally perceive their quality of life as low.2. There are differences in the assessment of quality of life between women and men.3. The older the patients, the worse their quality of life assessment in most subscales.4. There are critical differences in the quality of life assessment between patients with CF and a control group encompassing individuals without CF. Patients with CF find their quality of life poorer.