ANALYSIS OF CLINICAL DATA OF PATIENTS WITH CYSTIC FIBROSIS IN 2014–2017: ADULT CYSTIC FIBROSIS CENTER EXPERIENCE

Abstract

Aim. To analyze the clinical data of cystic fibrosis (CF) patients treated at the Adult cystic fibrosis centre of the Hospital of LithuanianUniversity ofHealth Sciences Kauno klinikos in 2014–2017.Methods.A retrospective analysis of patients treated in the Adult CF Center from January 2014 to December 2017 was performed according to the criteria and requirements of the European CF Society Patient Registry. Results. An analysis was made of 16 CF patients (6 women and 10 men) treated at the Adult CF Center in 2014–2017: 12 patients in 2014, and 14 patients for each rest of the year. During this period, 2 patients
died, and 2 new persons began treatment at the centre. 81.2% of patients (n = 13) were diagnosed with CF before the age of 18, 18.8%. (n = 3) – at adult age. All patients were genetically confirmed, and 9 had a high chloride level in sweat test. At least one ∆F508mutation was detected in all patients, and both ∆F508mutations were detected in 5 subjects (31.3%).During the analyzed period, themean age of CF patients increased from 24.6 years (2014) to 27.8 years (2017). Lung function,measured by themedian forced expiratory volume in the first second (FEV1), remained similar – 59.7% of predicted in 2014 and 60.5% of predicted in 2017.Mean body mass index (BMI) also remained unchanged – 19.6 kg/m2 – over the entire analyzed period. According to the results of the sputum microbiological examination, the most common chronic infection was Staphyloccocus aureus infection – 75.0% of CF patients in 2014; 57.1% in 2015, 58.3% in 2016 and 64.3% in 2017 Meanwhile, chronic Pseudomonas aeruginosa 
infection was found less frequent – 18.3% in 2014, 14.3% in 2015, 16.7% in 2016 and 14.3% in 2017. Intermittent treatment with the inhaled antipseudomonal antibiotic tobramycin was administered to 2 patients, one of whom was switched to inhaled colistin due to developed resistance. Symptomatic treatment was based on inhaled mucolytic dornase alfa, pancreatic enzymes and bronchodilators. One patient was diagnosed with CF–related diabetes mellitus, 2 subjects were diagnosed with chronic respiratory failure and received continuous oxygen therapy. Three patients were on the lung transplant waiting list, 2 of whom died. Conclusions. During the period 2014–2017, the mean age of adult CF centre patients increased, lung function and BMI remained stable, and chronic P. aeruginosa infection was relatively rare; all this shows the effectiveness of CF centremedical care.