Cystic fibrosis in pregnancy

Advances in the diagnosis and treatment of cystic fibrosis have resulted in increased survival for those afflicted with this disease. As a result, more women are reaching their reproductive years and will subsequently conceive. Multiple organ systems are affected during the course of cystic fibrosis and may impact on pregnancy; however, pulmonary complications play a major role in morbidity and mortality. Therefore, this article primarily focuses on the pulmonary aspects of cystic fibrosis as they relate to pregnancy. The current literature suggests a favorable outcome for the pregnancy when there is limited pulmonary dysfunction. Chronic severely impaired pulmonary function is associated with poorer pregnancy outcome; however, it is the acute deterioration of pulmonary function that may in fact be associated with the highest morbidity and mortality for both mother and infant. Aggressive management of pulmonary infection and function is essential, and pulmonary status should be evaluated on a monthly basis. The forced expiratory volume in 1 second (FEV₁) and forced vital capacity (FVC) can be used to monitor pulmonary status. Close surveillance, aggressive management, and a team approach can and has resulted in successful pregnancy outcome in those women with cystic fibrosis.