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Palliative Care In Cystic Fibrosis

BMJ SUPPORTIVE & PALLIATIVE CARE(2020)

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Abstract
Cystic fibrosis (CF) is a rare congenital disease responsible for respiratory infections, pneumothorax and chronic respiratory failure. Median life expectancy is increasing and recently reached 50 years. Since it is incurable (except with a lung transplant), patients with CF may benefit from early palliative care team (PCT) involvement, which should partner with CF physicians. We report a case of CF enrolled by a PCT and discuss the benefits of collaboration between PCT and CF teams.\n\nA single 35-year-old man was followed up every 3 months in consultation by a cystic fibrosis reference centre. Respiratory failure was severe (forced expiratory volume in one second=20%). In 2017, he refused lung transplant because he found no meaning in extending life. He even expressed a wish to hasten death and had inquired about euthanasia or assisted suicide in Belgium. He explained that he never thought he could live so long. As a child, his paediatric CF physician told him his illness would be rapidly life-threatening. Consequently, he never invested in his personal or professional …
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Key words
end of life care, respiratory conditions
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