Abstract
Although the prognosis of patients with cystic fibrosis continues to improve, many still die in early adulthood of respiratory failure. 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 not all patients are suitable for transplantation and the shortage of donor organs means that up to 40 % of patients on a transplant waiting list die before donor organs become available. Disease-modifying therapies, supportive care, emergency care, and palliative care all run in parallel for these patients. Palliative care is a key component of a comprehensive adult cystic fibrosis service, and this is often organized in an integrated model of care with specialist palliative care clinicians forming part of the multidisciplinary CF team and working collaboratively with the CF team.
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Bourke, S.J., Quibell, R. (2013). Cystic Fibrosis. In: Bourke, S., Peel, E. (eds) Integrated Palliative Care of Respiratory Disease. Springer, London. https://doi.org/10.1007/978-1-4471-2230-2_10
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DOI: https://doi.org/10.1007/978-1-4471-2230-2_10
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