Answering the call to address cystic fibrosis treatment burden in the era of highly effective CFTR modulator therapy

Drugs that effectively increase functioning of the cystic fibrosis transmembrane conductance regulator (CFTR) protein have revolutionized the treatment and anticipated health outcomes of many people with cystic fibrosis (PwCF). Collectively known as CFTR modulators, ivacaftor (IVA), lumacaftor/ivacaftor (LUM/IVA), and tezacaftor/ivacaftor (TEZ/IVA) improve lung function, reduce pulmonary exacerbation (PEx) risk, and alleviate respiratory symptoms of CF lung disease [1 –3]. As evidence of a systemic biological effect, these drugs can reduce sweat chloride levels towards the normal range in some individuals.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Original Article Source Type: research