Pulmonary Complications in Cystic Fibrosis: Past, Present, and Future Adult Cystic Fibrosis Series

(1)Cystic fibrosis (CF) is an autosomal recessive genetic condition with multisystemic disease manifestations, the most prominent of which occur in the respiratory system. Despite signifi-cant developments in disease understanding and therapeutics, each contributing to improved lung function and survival in patients with CF, several pulmonary complications, including pneumothorax, massive hemoptysis, and respiratory failure, continue to occur. In this review, we briefly describe each of these complications and their management and discuss how they impact the care and disease trajectory of individuals in whom they occur. Finally, we discuss the evolving role that palliative care and CF transmembrane conductance regular modulator ther-apies play in the natural disease course and care of patients with CF.

Automated summary

Cystic fibrosis is an autosomal recessive genetic condition with significant manifestations in the respiratory system. Despite advancements in treatment, pulmonary complications like pneumothorax and respiratory failure still occur. Palliative care and CF transmembrane conductance regulator therapies play evolving roles in managing the care of CF patients.