Background: Little is known about non-terminally ill cystic fibrosis (CF) patients who die by voluntary cessation of therapy. The current study was undertaken to provide an international snapshot of this problem. Methods: An online survey was distributed to the medical directors of the CF Centres affiliated with the US Cystic Fibrosis Foundation and Cystic Fibrosis Australia (with the inclusion of New Zealand); the same letter was sent to every clinician member of the European Cystic Fibrosis Society.
Results: More than 200 patients were reported as being in the situation described above. Data analysis was eligible in 102 patients (4 children, 25 adolescents and 73 adults). 92% were capable of judgement. Time-consuming and low immediate-impact therapies, such as respiratory physiotherapy, were most frequently discontinued first. Resignation was the main reported reason, followed by reactive depression and lack of familial support. 69% received palliative care and 72% died in the six months following refusal.
Conclusions: Death of non-terminally ill CF patients due to voluntary cessation of therapy exists. This challenging situation should be discussed in multi-disciplinary teams so that the most appropriate attitude, suited to the individual situation, is defined.