European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre

Abstract

A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those who are not. Key to the effectiveness of the specialist CF Centre is the multidisciplinary team (MDT), which should include consultants, clinical nurse specialist, microbiologist, physiotherapist, dietitian, pharmacist, clinical psychologist, social worker, clinical geneticist and allied healthcare professionals, all of whom should be experienced in CF care. Members of the MDT are also expected to keep up to date with developments in CF through continued professional development, attendance at conferences, auditing and involvement in research. Specialists CF Centres should also network with other Centres both nationally and internationally, and feed Centre data to registries in order to further the understanding of the disease. This paper provides a framework for the specialist CF Centre, including the organisation of the Centre and the individual roles of MDT members, as well as highlighting the value of CF organisations and disease registries.

Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis

“…The optimal framework for adult CF centres is described in detail in the ECFS standard of care framework for the CF centre [8] which provides detailed information regarding centre staffing (table 2) for the CF multidisciplinary team and the skills required for delivery of multidisciplinary care in this context. This framework describes the differences between paediatric and adult centres for numbers of staff required, reflecting the significantly higher morbidity in patients attending adult centres.…”

“…The changing role of parents, siblings and friends during transition are also important to consider. Approaches for optimal transition are detailed in the ECFS standards of care and are well described in important disease-specific and generic guidelines [8,21,22,[25][26][27].…”

“…Experts in reproduction, genetic counselling and obstetrics and gynaecology are also needed to deal with infertility and the management of pregnancy in CF [8]. Therefore, it is most efficient for adult CF centres to be located at large teaching/academic hospitals to support clinical care, education and training and research where appropriate support is available for all aspects of care delivery.…”

“…These and other supportive therapies for complications of CF, such as allergic bronchopulmonary aspergillosis (ABPA), CF-related diabetes mellitus, liver disease, bone disease and sinus disease, have been delivered by multidisciplinary teams in CF centres where expertise has been developed, retained and shared to ensure optimal management for people with CF. Centralised care with dedicated CF teams has become and remains the cornerstone of care delivery in CF [3,8].…”

“…The task force has reviewed the current provision of care for adults with CF across Europe, estimated the likely number of adults by country (where sufficient registry data are available) and, where possible, made manpower projections for the numbers and skills of CF physicians and other healthcare professionals that will be required to provide optimal care as outlined by the recently published European Cystic Fibrosis Society (ECFS) standards of care [8]. Furthermore, we have reviewed the requirements for a curriculum for training physicians in CF care.…”