Cystic fibrosis (CF) is the most common autosomal recessive disease of the Caucasian population characterised by chronic multi-organ impairment. Although cystic fibrosis is still incurable condition, it has been shown that holistic approach to treatment that is based on multi-professional centre care provided by trained and experienced health professionals is necessary for optimal management and improved quality of life and survival of patients.
Complex treatment focused on intensive airway clearance, good nutritional status, aggressive anti-infective and anti-inflammatory therapy and continuous search for complications is essential. New therapeutic strategies for CF focus on protein targeted treatment of impaired cystic fibrosis transmembrane conductance regulator (CFTR) that is responsible for exocrine secretion pathology.