Management of CF Lung Disease
Severe Disease
As lung deterioration progressed, oxygen therapy and assisted ventilation are required. Lung transplantation became an option for patients with CF several years ago, but there is considerable morbidity and mortality associated with it; the 5-year survival rate is approximately 50%.18
Despite considerable therapeutic advances, patients with CF and their families usually have to make untimely end-of-life decisions.
Reference:
18. Yankaskas JR, Marshall BC, Sufian B, Simon RH, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest 2004 January;125(1 Suppl):1S-39S.