Tracking CF Disease Progression with CT and Respiratory Symptoms in a Cohort of Children Aged 6-19 Years

IntroductionCystic fibrosis (CF) lung disease is characterized by bronchiectasis and trapped air on chest computed tomography (CT). ObjectiveWe aim to validate bronchiectasis and trapped air as outcome measures by evaluating associations between changes in bronchiectasis, trapped air and patient-reported respiratory symptoms. MethodsA longitudinal cohort study has been conducted. CF patients (aged 6-19 years) who had two routine CTs and completed twice a Cystic Fibrosis Questionnaire-Revised within 2 years (referred to as T-1 and T-2), in the period of July 2007 to January 2012 were included. Bronchiectasis and trapped air were scored using the CF-CT scoring system. Correlation coefficients and student's paired t tests were performed. ResultsIn total 40 patients were included with a median age at T-1 of 12.6 years (range 6-17 years), and at T-2 14.5 years (range 8-19 years). At T-1, bronchiectasis (r=-0.49, P<0.01) and trapped air (r=-0.34, P=0.04) correlated with CFQ-R Respiratory Symptoms Scores (CFQ-R RSS). At T-2 similar correlations were found with the CFQ-R RSS. Over 2 years, there was significant progression in bronchiectasis (P=0.03) and trapped air (P=0.03), but not in CFQ-R RSS. Changes in bronchiectasis and trapped air were not associated with changes in CFQ-R RSS. ConclusionOur results indicate that bronchiectasis and trapped are sensitive outcome measures in CF lung disease, showing a significant association with CFQ-R RSS at two-time points. However, progression of bronchiectasis and trapped air over 2 year does not necessarily correlate to changes in quality of life. Pediatr Pulmonol. 2014; 49:1182-1189. (c) 2014 Wiley Periodicals, Inc.