Abstract
Introduction: Cystic fibrosis (CF) is a serious genetic life-shortening disease. Quality of life (QoL) measurement
related to CF children is a relatively new field of research, which includes the patient’s perspective in research
and clinical practice.
Objectives: This study aimed to evaluate the QoL in children with CF and its association with FEV1 (forced
expiratory volume in 1 second).
Patients and Methods: This cross-sectional study was carried out on 7-14 years old children with CF attending
children’s medical center from March 2017 to March 2018. Throat swab cultures and spirometry evaluation was
performed for all patients. FEV1 was determined and the 6-min walk test (6MWT) was conducted. The patient’s
psychological status was assessed using the Persian version of pediatric QoL inventory. ANOVA, t test, and chisquare tests were used for data analysis.
Results: Seventy-six subjects with the mean age of 10.49±3.18 years were studied since 59% of them (n=44)
were boys. The mean total QoL was 65.34±17.73. Patients with lower pulmonary function had a lower QoL.
There was a significant association between FEV1 and school and emotional functioning (P=0.005 and P=0.002,
respectively). A significant association was found between SPO2 (peripheral capillary oxygen saturation) reduction
after 6MWT and FEV1 decline (P=0.001). Additionally, a significant association was detected between FEV1 and
the distance walked during 6MWT (P=0.030).
Conclusion: Regarding the association between pulmonary function and QoL in CF patients and lower QoL score
in our study, the importance of assessing pulmonary function in these patients should not be neglected.