Abstract
Cystic fibrosis (CF) is one of the serious, progressive, as yet incurable diseases. It is clinically manifested mainly by the image of chronic sinopulmonary disease.
The basic therapeutic principles based on complex symptomatic treatment are extended by treatment with chloride channel modulators and intensification of respiratory physiotherapy using the PhysioAssist device (Simeox). Due to the age-related gradual progression of irreversible pulmonary changes, it is necessary to focus on the mucociliary airway clearance technique from an early age and to extend standard airway clearance methods with instrumental support in patients with good compliance to ensure effective expectoration and minimize the development of acute exacerbations.
Effective attenuation leads to a reduction in the number of hospitalizations and antibiotics, to a slowdown in the deterioration of lung function, and thus to an improvement in the quality of life.