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Cystic Fibrosis

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Integrated Palliative Care of Respiratory Disease

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Abstract

Although the prognosis of people with cystic fibrosis continues to improve many still die of respiratory failure in early adulthood, at a median age of 31 years. They are treated in specialist centers by multidisciplinary teams who strive to provide holistic care and support throughout the course of the disease. As the disease progresses patients have a high level of complex symptoms and a high burden of treatment. Palliative care is intertwined with potential lung transplantation, but up to 40% of patients on a transplant waiting list die before donor organs become available. Palliative care is a key component of comprehensive cystic fibrosis care, and this is now often organized in an integrated model of care with specialist palliative care clinicians forming part of the multidisciplinary team such that palliative care runs in parallel with standard care.

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Author information

Authors and Affiliations

  1. Department of Respiratory Medicine, Royal Victoria Infirmary, Newcastle Upon Tyne, UK

    Stephen J. Bourke

  2. Department of Palliative Medicine, Royal Victoria Infirmary, Newcastle Upon Tyne, UK

    Rachel Quibell

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  1. Stephen J. Bourke
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  2. Rachel Quibell
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Correspondence to Stephen J. Bourke .

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Editors and Affiliations

  1. Department of Respiratory Medicine, Royal Victoria Infirmary, Newcastle Upon Tyne, UK

    Stephen J. Bourke

  2. Policy, Ethics and Life Sciences, Newcastle University, Newcastle Upon Tyne, UK

    Tim Peel

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Bourke, S.J., Quibell, R. (2019). Cystic Fibrosis. In: Bourke, S., Peel, T. (eds) Integrated Palliative Care of Respiratory Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-18944-0_10

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  • DOI: https://doi.org/10.1007/978-3-030-18944-0_10

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-18943-3

  • Online ISBN: 978-3-030-18944-0

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