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It is a multi-system disease affecting the exocrine glands, principally the lung and digestive systems. It is estimated that a population of 250,000 is likely to have 35 residents with CF, 15 of which will be adults. For those born since 1990 it is expected that 50% will live beyond 40 years. As it is a progressive condition with an improving prognosis, there is a rapidly increasing number of adult patients with inevitably more severe problems, whilst the paediatric population is relatively static.

As a multi-system disease, a specialist centre requires a multi-disciplinary team approach to care. This team comprises experienced clinician, specialist nurse, respiratory physiotherapist, dietician, clinical psychologist and ideally, social worker, all of whom spend a substantial part of their working time dealing with CF.

Patient care is delivered by a hub and spoke arrangement with the specialist centre at the hub supervising but sharing care with local hospitals and primary care teams. Whilst details of the model may vary between patients and areas, the key is that specialist supervision oversees local provision with the patient seen centrally at diagnosis for initial assessment and then at minimum, annually for review. Currently, most provision for adults is only at specialist centres, whereas the hub and spoke arrangement is common for children.

The definition of a specialist centre is not rigid and is based on a combination of patient treatment services, numbers attending there, the presence of the multi-disciplinary team outlined above, the ability to manage the more unusual manifestations of the condition and the additional functions it carries out such as research, education and standard setting.

Specialised services for adults and children are not the same and should be commissioned separately but with regard to each other and in particular, services should be arranged to ensure a smooth transition from paediatric to adult care. Paediatric services should be provided in a paediatric setting where there are appropriate paediatric support services.

It is the nature of the service that is specialised not its physical location. All care for CF patients whether within the specialist hospital or the shared care facility should be regarded as specialised. In fact, any care provided by the CF team, which may include support given in a patient's home will come within the remit of this definition.