Cystic Fibrosis Pulmonary Exacerbation

Cystic fibrosis (CF) is an autosomal recessive condition caused by mutations in the CFTR gene, which leads to thickened secretions resulting in damage to multiple organ systems. It is a condition that is often encountered by providers who care for pediatric patients, including in the emergency department. CF and its complications are typically managed at CF care centers across the United States. However, massive hemoptysis and pneumothorax are complications that may present to a local emergency department and require emergent intervention with specific considerations. Although it is important to keep these complications in mind, the frequency with which CF patients are presenting to emergency and inpatient settings has been drastically affected by the advent of cystic fibrosis transmembrane conductance receptor (CFTR) modulator therapies. These therapies remain limited to patients with certain mutations, and therefore knowledge of the care of CF complications remains extremely important for medical providers.