• Marisa Pisaturo, Alain Deppen, Isabelle Rochat, Walter M Robinson, and Gaudenz M Hafen.
• 1 Faculty of Biology and Medicine, University of Lausanne, Lausanne, Switzerland.
• Palliat Med. 2017 Jan 1; 31 (1): 82-88.

BackgroundLittle is known about cystic fibrosis patients, who are not considered to be terminally ill, and who die after voluntary cessation of treatment.AimThis study was undertaken to provide an international snapshot of this issue.DesignAn online survey was distributed across three continents.SettingDistribution to the medical directors of the cystic fibrosis centres affiliated with the US Cystic Fibrosis Foundation, Cystic Fibrosis Australia (inclusion of New Zealand) and to every clinician member of the European Cystic Fibrosis Society.ResultsMore than 200 cystic fibrosis patients not considered to be terminally ill and, who voluntarily ceased treatment, were reported by the clinicians surveyed. Detailed data were reported in 102 patients (4 children, 25 adolescents and 73 adults). Only one child, six adolescents and one adult were judged by clinicians not to be competent to make the decision to stop treatment. Time-consuming and low immediate-impact therapies, such as respiratory physiotherapy, were most frequently discontinued. Resignation was the main reported reason for discontinuing treatment, followed by reactive depression and lack of familial support. A total of 69% of the patients received palliative care and 72% died in the 6 months following cessation of treatment.ConclusionDeath of cystic fibrosis patients, not considered to be terminally ill, is reported in Europe, the United States and Australia due to voluntary cessation of treatment.

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