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Feature Article

Primary care for young people with cystic fibrosis

KATHERINE FRAYMAN, John Massie

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Abstract

Cystic fibrosis (CF) is a relatively common, complex chronic condition. This article provides an overview of the common aspects of care for patients with CF and highlights the potential for general practitioners to complement the care provided by specialist CF services at each life stage. 

Key Points

  • Improved survival means that cystic fibrosis (CF) may now be regarded as a chronic, multisystem disease of adolescence and young adulthood.
  • Care for patients with CF is typically provided at specialised CF centres by multidisciplinary teams.
  • Engagement of young people with CF with a general practitioner is particularly important to complement the care provided by specialist CF services at each life stage.
  • Treatment considerations in patients with CF include minimising respiratory exacerbations and deteriorating pulmonary function, treating gastrointestinal manifestations such as pancreatic insufficiency and distal intestinal obstruction syndrome, monitoring nutritional status and treating CF-related diabetes.
  • Adolescence brings an added layer of complexity to management and adolescents with CF may show poor adherence with treatments.
  • Addressing health concerns not specific to CF, including developmental concerns, mental health and sexual health and contraceptive advice is an area that has lacked attention.

    Picture credit: © SI Photography/iStockphoto.com

    Model used for illustrative purposes only

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