ReKOMendacje PostępowAnia w mukowiScydozie (cystic fibrosis; CF) dla lekarzy Podstawowej Opieki Zdrowotnej - KOMPAS CF - część 2*

With the prolonged life, patients with cystic fibrosis require multi-specialty care including not only the treatment of the underlying disease but also its complications and associated diseases. Complications of bronchopulmonary disease, both infectious (fungal infections) and non-infectious (haemoptysis, pneumothorax, respiratory failure) are often a direct threat to life and require rapid identification and proper management. Dietary treatment aims to prevent malnutrition and disturbances of water-electrolyte management with cystic fibrosis characterized by metabolic alkalosis with hyponatremia and hypochloraemia (pseudo Bartter syndrome). At every stage of the disease, there are gastrointestinal complications that require special and appropriate treatment: from meconium ileus, through distal intestinal obstruction syndrome, reflux disease, pancreatitis and cystic fibrosis related diabetes or liver diseases. Patients with chronic inflammation and polyps of paranasal sinuses required laryngological care.The end of life is a time to choose whether to continue palliative treatment or to qualify for lung transplantation.