MEDICATIONS IMPROVING AIRWAY CLEARANCE IN CYSTIC FIBROSIS

Abstract

Cystic fibrosis (CF) is a rare autosomal recessive disorder usually affected the lungs and digestive systems. Despite the fact that gastrointestinal lesions can cause serious health problems, patients more difficult tolerate mucus secretion, severe coughing, short ness of breath, infectious disease exacerbations that often lead to disease progression or lethal outcomes. Effective evacuation of thick, sticky airway mucus can decrease CF pulmonary exacerbation rate, improve the patient's quality of life, slow the progression of the disease. The choice of drugs enhancing respiratory mucociliary clearance is not narrow, but their effectiveness depends on the thick bronchial secretions pathophysiological features. According this there is only one approved mucolytics in clinical practice that lowering viscosity and elasticity of CF patients mucus. It is inhaled dornase alpha (Pulmozyme®) - phosphorylated, glycosylated recombinant human protein deoxyribonuclease 1, that degrades neutrophil DNA. Drug efficacy and safety is based on clinical trials. Other mucoactive medications such as hypertonic saline and mannitol working as expectorants may also be effective mucociliary clearance enhancing agents. They have approved indications of thinning the thick mucus in patients with cystic fibrosis but their action principle is different and these drug are less efficient than inhaled dornase alpha.