The course of the terminal phase in patients with amyotrophic lateral sclerosis

Abstract The fear of “choking to death” is on the mind of most patients suffering from amyotrophic lateral sclerosis (ALS). So far, however, there have been no systematic surveys concerning the dying phase in a general ALS population. We therefore performed a structured telephone interview with the relatives of 121 patients who died from ALS and were followed by the Motor Neuron Outpatient Clinic of the Department of Neurology, University of Munich, Germany. These data are compared with those obtained by a retrospective analysis of medical records of 50 ALS patients who were followed by the Wisdom Hospice, Rochester, UK.The data show that most ALS patients (Germany 88 %, UK 98 %) died peacefully, and no patient “choked to death”. The symptoms most frequently reported for the last 24 hours were dyspnoea, coughing, anxiety and restlessness. Around half (G 55 %, UK 52 %) of the patients died at home. The main palliative measures in place during the terminal phase were: home mechanical ventilation (G 21 %, UK 0 %), percutaneous endoscopic gastrostomy (G 27 %, UK 14 %), morphine (G 27 %, UK 82 %) and benzodiazepines (G 32 %, UK 64 %). The use of these palliative measures was judged to be beneficial by almost all relatives. These data support the hypothesis of a peaceful death process in ALS and should be communicated to patients and their relatives, at the latest after the onset of dyspnoea, to relieve unwarranted fears.