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Guidelines and Consensus Statements

The purpose of this document is to set out guidelines to ensure standardised care for children with cystic fibrosis looked after at the Royal Brompton & Harefield NHS Foundation Trust and District General Hospitals on a network care basis. They should be used as a guide only. The Royal Brompton Hospital is a Specialist CF Centre as defined by the Specialist Commissioners, NHS England.

With the development of the APP for our guidelines in 2017, we know that aside from the UK, the guidelines have been downloaded 53 countries - Afghanistan, Argentina, Australia, Austria, Bangladesh, Belgium, Brazil, Bulgaria, Ecuador, Egypt, France, Germany, Gibraltar, Greece, Hong Kong, Hungary, India, Iran, Ireland, Italy, Kazakhstan, Kenya, Latvia, Lebanon, Luxembourg, Macedonia, Malaysia, Malta, Mexico, Montenegro, Netherlands, New Zealand, Oman, Pakistan, Portugal, Philippines, Qatar, Romania, Russia, Saudi Arabia, Serbia, South Africa, Spain, Sweden, Switzerland, Thailand, Turkey, Seychelles, UAE, Ukraine, Uruguay, USA, and Yemen. This year we are going paperless, so the guideline will be available online and via APP only. We will only print out the ‘What’s new’ section, contacts and drug formulary for use in our clinics.

Our philosophy of care for patients with cystic fibrosis is based on current guidelines laid down by the Royal College of Physicians, Royal College of Paediatrics & Child Health (formerly British Paediatric Association), CF Trust, British Thoracic Society, and NHSE Service Specifications. These have identified significant advantages in terms of survival and morbidity for patients receiving care from specialist centres. Specialist centres offer access to comprehensive care from a multidisciplinary team consisting of consultants with a special interest in CF, trainee doctors, nurse specialists, dietitians, physiotherapists, clinical psychologists, pharmacists and social workers. The team is also responsible for producing and distributing educational material and carrying out research to improve knowledge about this disease. Special procedures and investigations are provided that may not be available at District General Hospital level (such as formal lung function and bronchoscopy). We are happy to continue with a shared care policy, as long as the NHSE National Service Specification and our signed Service Level Agreement are adhered to. We also run several out-reach clinics whereby our MDT see CF patients in their local hospitals.

Details of the Service Specification can be found –


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Athanazio RA, Vergara AA, Ribeiro AF, Riedi CA, Procianoy EFA, Adde FA, Reis FJC, Ribeiro JD, Torres LA, Fuccio MB, Epifanio M,Firmida MC, Damaceno N, Ludwig-Neto N, Maróstica PJC, Rached SZ, Melo SFO; Grupo de Trabalho das Diretrizes Brasileiras de Diagnóstico e Tratamento da Fibrose Cística.
J Bras Pneumol. 2017;43(3):219-45.

Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease. Previously, CF was limited to the pediatric age group. However, an increase in the number of adult CF patients has been observed, because of the greater number of individuals being diagnosed with atypical forms (with milder phenotypic expression) and because of the increase in life expectancy provided by the new treatments. However, there is still great heterogeneity among the different regions of Brazil in terms of the access of CF patients to diagnostic and therapeutic methods. The objective of these guidelines was to aggregate the main scientific evidence to guide the management of these patients. A group of 18 CF specialists devised 82 relevant clinical questions, divided into five categories: characteristics of a referral center; diagnosis; treatment of respiratory disease; gastrointestinal and nutritional treatment; and other aspects. Various professionals working in the area of CF in Brazil were invited to answer the questions devised by the coordinators. We used the PubMed database to search the available literature based on keywords, in order to find the best answers to these questions.
KEYWORDS: Cystic fibrosis/diagnosis; Cystic fibrosis/therapy; Cystic fibrosis/complications; Practice guideline.

© 2017 Sociedade Brasileira de Pneumologia e Tisiologia.

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