Cystic Fibrosis

With advances in medical care and with the introduction of highly effective modulator therapies, the predicted median survival of individuals with cystic fibrosis has increased dramatically over the last several decades. As a result, over half of the current cystic fibrosis population is 18 years of age or older, making it likely that many primary care providers will see patients with cystic fibrosis in their adult medical practices. With expanded knowledge of cystic fibrosis genetics and genotype:phenotype relationships, it is also possible that primary care providers will be able to recognize and diagnose previously missed adults with cystic fibrosis. Though the comprehensive care of patients with cystic fibrosis is best directed by one of the nationwide Cystic Fibrosis Foundation-accredited care centers, primary care providers will need to play an increasingly critical role in the long-term co-management of adult patients with cystic fibrosis. The purpose of this chapter is to educate the primary care provider about common medical problems experienced by patients with cystic fibrosis, to provide some fundamental knowledge of the cornerstones of chronic cystic fibrosis disease management, and to highlight key issues when co-management with subspecialists with expertise in the field of cystic fibrosis is indicated. Key concepts in care management for cystic fibrosis patients transitioning from pediatric- to adult-oriented healthcare systems will also be reviewed.