This guideline covers the diagnosis and management of cystic fibrosis. It specifies how to monitor the condition and manage the symptoms to improve quality of life. There are also detailed recommendations on treating the most common infections in people with cystic fibrosis
To improve diagnosis and achieve standardized definitions worldwide, the CF Foundation convened a committee of 32 experts in CF diagnosis from 9 countries to develop clear and actionable consensus guidelines on the diagnosis of CF and to clarify diagnostic criteria and terminology for other disorders associated with CFTR mutations.
Optimising growth and nutrition in people with cystic fibrosis (CF) has been shown to positively influence lung function and survival. These updated guidelines from the Thoracic Society of Australia and New Zealand incorporate new topic areas including the nutritional implications of new genetic modulatory therapies, the emergence of overweight and obesity in CF, and complementary nutritional therapies.
Non-tuberculous mycobacteria (NTM) are ubiquitous environmental organisms that can cause chronic pulmonary infection, particularly in individuals with pre-existing inflammatory lung disease such as cystic fibrosis (CF). Pulmonary disease caused by NTM has emerged as a major threat to the health of individuals with CF but remains difficult to diagnose and problematic to treat. The US Cystic Fibrosis Foundation (CFF) and the European Cystic Fibrosis Society (ECFS) developed these consensus recommendations for the screening, investigation, diagnosis and management of NTM pulmonary disease in individuals with CF.
Cystic Fibrosis Foundation clinical care guidelines for the prevention of Pseudomonas aeruginosa infection, the treatment of initial P. aeruginosa infection, and the use of bronchoscopy to obtain routine airway cultures in individuals with CF.
European Cystic Fibrosis Society evidence based best practice guidelines
Cystic Fibrosis Trust (CFT) Standards for the Clinical Care of Children and Adults with Cystic Fibrosis in the UK (CFT)
Cystic Fibrosis Trust (CFT) Standards of care and good clinical practice for the Physiotherapy management of Cystic Fibrosis in the UK.
European Cystic Fibrosis society guidelines on DIOS – complete or incomplete intestinal obstruction by viscid faecal material in the terminal ileum and proximal colon – distal intestinal obstruction syndrome (DIOS), a common complication in cystic fibrosis.
Cystic Fibrosis Foundation Pulmonary Guidelines – Pulmonary Complications: Haemoptysis and Pneumothorax
European Cystic Fibrosis Society evidence based guidelines onTravelling with cystic fibrosis: Recommendations for patients and care team members developed by the European Centres of Reference Network for Cystic Fibrosis (ECORN-CF) Study Group. Provides patient-oriented advice that complements medical aspects by offering practical suggestions for all aspects involved in planning and taking a trip.
Report of the UK Cystic Fibrosis Trust Antibiotic Working Group
The Cystic Fibrosis Foundation Pulmonary Guidelines – Treatment of Pulmonary Exacerbations
The Cystic Fibrosis Foundation Pulmonary Guidelines: Airway Clearance Therapies
European Cystic Fibrosis Society evidence based guidelines on neonatal screening for cystic fibrosis