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.

Assessment and Management of Respiratory Dysfunction in Patients with Amyotrophic Lateral Sclerosis

“…Interestingly, the amyotrophic lateral sclerosis functional rating scale (ALSFRS), a disease-specific rating scale that assesses functional impairment, has been shown to predict both length of hospital stay as a result of acute respiratory failure and survival after initiation of invasive ventilation in these patients (Lo Coco et al, 2007). Notwithstanding its effect on survival, only a minority of patients with ALS receive invasive mechanical ventilation, at least in the western Countries (Moss et al, 1993;Miller et al, 2000;Neudert et al, 2001). On the contrary, in Japan the frequency of invasive ventilation is considerably higher.…”

Palliative Care for Motor Neurone Disease

“…There is now evidence that, with good palliative care, death is not distressing and most often it is peaceful (Neudert et al 2001).…”

“…The deterioration of a patient with MND is variable. Patients usually deteriorate slowly and it gradually becomes apparent that the disease has reached the terminal stage, but for many patients the changes are rapid, over a few days (Neudert et al 2001). One study showed that for 65% of patients the period between the acute deterioration and death was under 24 h (Neudert et al 2001).…”

“…The patient develops a respiratory tract infection, which initially may seem trivial, but the respiratory function deteriorates and over a few days, sometimes over a few hours, there is a rapid deterioration leading to death (Neudert et al 2001). One study showed that 86% died from respiratory failure, 6% from heart failure, 4% from pneumonia, 1% from suicide and 3% from other causes (Neudert et al 2001).…”

“…However, distress is rare and with good palliative care, including the management of dyspnoea, pain and swallowing problems, and support of patient and family, the vast majority of deaths from MND can be peaceful (Neudert et al 2001;O'Brien et al 1992). An international study showed that only 5% of a group of 171 patients in the UK and Germany died in distress, and 91% died peacefully (Neudert et al 2001).…”