17th February 2025

Management of CF

The mainstay of management is respiratory physiotherapy, such as postural drainage techniques to help clear viscous sputum and avoid chest infections. Other aspect of management may include:

• Pancreatic enzyme supplementation – to treat pancreatic insufficiency
• Specialised diet – high in calories, fat and salt to counteract malabsorption 
• Mucolytics – to help break down thickened sputum 
• Antibiotics for recurrent infections – patients with CF generally need longer courses and higher doses. Pseudomonas aeruginosa cover should always be considered, especially if the patient has been previously infected. This is a common coloniser in patients with cystic fibrosis and is identified by Gram-negative rods on blood cultures.
• Lung transplantation – bilateral lung transplant is usually the procedure of choice and should be considered once a patient is on optimal medical therapy and their disease is impacting their quality of life
• Routine immunisations and yearly flu vaccine