20.8 : 囊性纤维化：管理

Cystic fibrosis, or CF, is an autosomal genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator or CFTR gene.

CF primarily affects the respiratory system. The treatment aims to optimize lung function, control respiratory infections, and facilitate the clearance of mucus.

These complications are managed with medications and therapies that improve oxygenation and airway secretion clearance.

Airway clearance techniques like specialized breathing, coughing exercises, and therapy vests help loosen lung mucus.

Medications, including antibiotics, anti-inflammatory drugs, bronchodilators, and mucus thinners, improve lung function, fight infections, and clear mucus.

CFTR modulator therapies, such as ivacaftor and lumacaftor, correct the faulty CFTR protein.

Dornase alfa, also known as pulmozyme, is a recombinant human deoxyribonuclease I medication that reduces the viscosity and elasticity of purulent sputum by breaking down extracellular DNA.

In advanced CF cases, surgical interventions like lung transplants may be considered.