Cystic Fibrosis: Treatment

Abstract

Cystic fibrosis treatment is based on a symptomatic approach aimed to interrupt the cycle of mucus retention, infection, and inflammation. The treatment must start early in life to avoid permanent damage to the lung. Treatment for cystic fibrosis requires a multidisciplinary team and a specialized center where patients can carry out their follow-up. The main treatment must be based on nutrition, antibiotic treatment, and chest physiotherapy. Nutritional support is essential for both quality of life and survival. Chest physiotherapy is recommended to improve airway secretions management, essential for symptomatic relief, with inhaled treatment. Therapy must also target respiratory infections, and the use of antibiotics will depend on the clinician’s objective, such as treatment for first bacterial isolation, acute exacerbations, chronic suppressive therapy, or prophylactic treatment. New therapies may be considered, such as genetic pharmacotherapy for certain mutations and antiinflammatory treatment, but they are not considered as standard of care. Oxygen therapy and ventilation support are recommended when chronic lung damage is present and lung transplant becomes an option to improve survival.