Live and let die: existential decision processes in a fatal disease


Decisions and determinants of decisions to prolong or shorten life in the course of fatal diseases like ALS are poorly understood. Decisions and desire for hastened death of N = 93 ALS patients were investigated in a prospective longitudinal approach three times in the course of 1 year. Determinants of decisions were evaluated: quality of life (QoL), depression, feeling of being a burden, physical function, social support and cognitive status. More than half of patients had a positive attitude towards life-sustaining treatments and they had a low desire for hastened death. Of those with undecided or negative attitude, 10 % changed attitudes towards life-sustaining treatments in the course of 1 year. Patients’ desire to hasten death was low and decreased significantly within 1 year despite physical function decline. Those with a high desire for hastened death decided against invasive therapeutic treatments. QoL, depression and social support were not predictors for vital decisions and remained stable. Feeling of being a burden was a predictor for decisions against life-supporting treatments. Throughout physical function loss, decisions to prolong life are flexibly adapted while desire to shorten life declines. QoL was stable and not a predictor for vital decisions, even though anticipated low QoL has been reported to be the reason to request euthanasia. In contrast, feeling of being a burden in decision making needs more attention in clinical counselling. Considering a patient’s possible adaptation processes in the course of a fatal disease is necessary.

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This is an EU Joint Programme—Neurodegenerative Disease Research (JPND) project. The project is supported through the following organisations under the aegis of JPND— e.g. Germany, Bundesministerium für Bildung und Forschung (BMBF, FKZ), Sweden, Vetenskaprådet Sverige, and Poland, Narodowe Centrum Badán i Rozwoju (NCBR). This work was supported by the Deutsche Forschungsgemeinschaft (DFG 336/13-2 und BI 195/54-2) and the (BMBF #01GQ0831 and BMBF #01GM1103A).

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The authors declare that they have no conflict of interest.

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Correspondence to Dorothée Lulé.

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Lulé, D., Nonnenmacher, S., Sorg, S. et al. Live and let die: existential decision processes in a fatal disease. J Neurol 261, 518–525 (2014).

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  • Palliative care
  • Quality of life
  • Depression
  • Decision
  • Amyotrophic lateral sclerosis