Recent outline of Treatment lines of Cystic Fibrosis in Children

Abstract

Background:  In cystic fibrosis (CF), respiratory disease is the main factor that influences the outcome and the prognosis of patients. Bacterial infections being responsible for severe exacerbations. The etiology is often multi-microbial with resistant strains. Recently, CFTR modulator therapy showed promising results in slowing the disease or even stopping the damaging effects. However, until the discovery of a specific targeted therapy for every pathogenic mutation, the lung must be treated, and its function carefully preserved. Therefore, besides clearance techniques and mucolytic therapy, the antibiotic therapy plays a crucial role in CF lung disease management and life expectancy improvement. The main goals of antibiotic therapy in CF are the prevention, eradication, and control of CF-associated respiratory infections. General principles of antibiotic therapy are to introduce aggressive treatment from the beginning, in higher doses than usual, with prolonged duration of therapy (2–4 weeks), and association of nebulized antibiotics.