CYSTIC FIBROSIS MORTALITY. RETROSPECTIVE ANALYSIS OF FIVE YEARS IN A HIGHLY COMPLEX INSTITUTION. MEDELLÍN-COLOMBIA

Abstract

Introduction: cystic fibrosis (CF) is a chronic, progressive disease with multisystem involvement and high mortality. An early diagnosis and a multidisciplinary approach lead to longer survival and better quality of life. Objective: to characterize the patients who died with CF diagnosis in the period between 2011 and 2017 in the Hospital Universitario San Vicente in Colombia. Methodology: descriptive study of case series, with retrospective data collection. Results: 168 patients with CF were found in follow-up at the institution during the study period, of which 17 died (10,1%). Eleven deaths corresponded to children under 15 years old (64.7%), with equal distribution of gender (women 52.9% and men 47.1%). The median age at diagnosis was 3 years and median death 12 years. The most frequent clinical compromise was sinopulmonary and gastrointestinal. Pulmonary hypertension occurred in 29.4%. All the patients had a severe functional and nutritional compromise and 82.4% were colonized by Pseudomona aeruginosa. None had multidisciplinary management and most had a low socioeconomic level. BiPAP was used as a palliative measure in 6 patients and all died from ventilatory failure. Conclusion: CF mortality in our population continues to present at an early age. The diagnosis of the disease is still made late, compared to developed countries. There was a high proportion of bacterial colonization of the airway and the patients presented a severe clinical and functional status before dying.