This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.Patients with cystic fibrosis (CF) experience episodic deteriorations of their condition, termed ?exacerbations?, which commonly require hospitalization or intensive treatments at home. As CF lung disease progresses, these exacerbations may reoccur several times a year, thus heavily impacting apatient?s quality of life and increasing healthcare resource utilization. Our understanding of the biological processes that lead to exacerbations is limited, however. We hypothesize that various triggers, including viral infection, slow the clearance of mucus from airways, thus causing bacterial infection to worsen and airway blockage to progress. We propose to test this hypothesis by measuring mucus clearance (by tracking the disappearance of labeled particles after they are inhaled) in patients while they are well, andagain after the development of an exacerbation. We will also use new, sensitive methods to determine if respiratory viruses are present in the sputum of these patients while they are ill. Finally, we will measure substances in sputum that, if altered in an exacerbation, could influence mucus clearance rates. The data collected in this study may point toward the importance of maintaining/restoring mucus clearance therapeutically in order to avoid exacerbations while also identifying triggers that may be targeting with new therapies.
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