This guideline covers diagnosing and managing 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.
NICE has produced a COVID-19 rapid guideline on cystic fibrosis. It recommends changes to usual practice to maximise the safety of patients and protect staff from infection during the COVID-19 pandemic.
This guideline includes recommendations on:
- service delivery, including how to organise services and multidisciplinary teams
- annual and routine reviews
- monitoring, assessment and management, including for lung disease, pulmonary infection, distal intestinal obstruction syndrome, liver disease and cystic-fibrosis-related diabetes
- preventing cross-infection
Who is it for?
- Healthcare professionals
- Social care practitioners working with people with cystic fibrosis
- People with cystic fibrosis and their families and carers
Guideline development process
The recommendations in this guideline represent the view of NICE, arrived at after careful consideration of the evidence available. When exercising their judgement, professionals and practitioners are expected to take this guideline fully into account, alongside the individual needs, preferences and values of their patients or the people using their service. It is not mandatory to apply the recommendations, and the guideline does not override the responsibility to make decisions appropriate to the circumstances of the individual, in consultation with them and their families and carers or guardian.
All problems (adverse events) related to a medicine or medical device used for treatment or in a procedure should be reported to the Medicines and Healthcare products Regulatory Agency using the Yellow Card Scheme.
Local commissioners and providers of healthcare have a responsibility to enable the guideline to be applied when individual professionals and people using services wish to use it. They should do so in the context of local and national priorities for funding and developing services, and in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health inequalities. Nothing in this guideline should be interpreted in a way that would be inconsistent with complying with those duties.
Commissioners and providers have a responsibility to promote an environmentally sustainable health and care system and should assess and reduce the environmental impact of implementing NICE recommendations wherever possible.