Table 1.

Treatment of the respiratory condition in CF patients (pulmonary symptomatic treatment).

Treatment of Obstruction

Manual and instrumental physiotherapy

Physical exercise

Bronchodilators

Mucolytics: human dornase alfa

Hypertonic substances: 6 or 7% saline/Mannitol

Treatment of Inflammation

Oral/inhaled corticosteroids (ICS)

Ibuprofen

Azithromycin

Treatment of Infection

Treatment of initial colonization by Pseudomonas aeruginosa

Treatment of other pathogens

Chronic maintenance treatment

Treatment of exacerbations

Treatment of Chronic Respiratory Failure

Oxygen therapy

Noninvasive mechanical ventilation (transplant bridge)

Pulmonary transplant