The effect of discontinuing hypertonic saline or dornase alfa on mucociliary clearance in elexacaftor/tezacaftor/ivacaftor treated people with cystic fibrosis: The SIMPLIFY-MCC Study

The availability of highly effective CFTR modulators, especially elexacaftor/tezacaftor/ivacaftor (ETI), has changed the lives of people with cystic fibrosis (pwCF) via marked improvements in lung function, nutrition, exacerbation frequency, and symptoms [1 –3]. As a result, pwCF and their providers have questioned the need for other supportive therapies. Because ETI substantially improves mucociliary clearance (MCC) [4], the additive benefit of hypertonic saline (HS) and dornase alfa (DA) are particularly uncertain [5].

Source: Journal of Cystic Fibrosis - February 13, 2024 Category: Respiratory Medicine Authors: Scott H. Donaldson, Timothy E. Corcoran, Joseph M. Pilewski, Beth L. Laube, Peter Mogayzel, Agathe Ceppe, Jihong Wu, Kirby Zeman, Steven M. Rowe, David P. Nichols, Alex H. Gifford, William D. Bennett, Nicole Mayer-Hamblett, SIMPLIFY MCC Study teams Tags: Short Communication Source Type: research