Treatment-cystic fibrosis

Lancet 2021;397:2195)

• Acute exacerbations: may be assoc w/ persistent drop in FEV1 (AJRCCM 2010;182:627); continue aggressive airway clearance, target abx based on sputum cx (incl double coverage for PsA); common pathogens include PsA, S. aureus, non-typeable H. flu, Stenotrophomonas, Burkholderia, NTM

• Chronic mgmt: airway clearance with chest PT, inhaled hypertonic saline, inhaled DNAse (dornase alfa), SABA; oral azithromycin if chronic respiratory symptoms, inhaled tobramycin or aztreonam if persistent PsA infection

• CFTR potentiator (ivacaftor) or corrector (lumacaftor, tezacaftor) depending on mutation; combo (elexacaftor+tezacaftor+ivacaftor) if homozygous for ΔF508 (Lancet 2019;394:1940)

• Lung transplantation; refer to lung transplant center when FEV1 <30% predicted, rapidly declining FEV1, 6MWT <400 m, evidence of PHT, significant clinical decline