Clinical characteristics and course of dying in patients with amyotrophic lateral sclerosis withdrawing from long-term ventilation

Abstract

Non-invasive ventilation (NIV) or tracheotomy with invasive ventilation (TIV) are treatment options in ALS. However, a proportion of patients receiving long-term ventilation decide to have it withdrawn. The objective of this study was to analyse the clinical characteristics and palliative approaches in ALS patients withdrawing from long-term ventilation (WLTV).

In a cohort study, two different palliative concepts in WLTV were studied: (1) augmented symptom control (ASC; sedation not intended) in patients with ventilator-free tolerance; (2) continuous deep sedation (CDS; sedation intended) in patients without ventilator-free tolerance. Results showed that WLTV was realised in 49 ALS patients (NIV = 13; TIV = 36). Mean daily ventilation was 23.4 h. The ALS Functional Rating Scale (ALSFRS-R) was low (5.6 of 48). Forty-one per cent of patients (n = 20) presented with ophthalmoplegia. ASC was performed in 20 patients, CDS in 29 patients. The mean time to death following disconnection was 32 (0.3–164) h during ASC and 0.3 (0.2–0.6) h in CDS. In conclusion, a low ALSFRS-R, high incidence of ophthalmoplegia and extended ventilator dependency were found before WLTV. The presence or absence of ventilator-free tolerance determined the approach to the management of symptoms, the setting for immediate end-of-life care and the course of dying in WLTV.

Key words:

• Amyotrophic lateral sclerosis
• motor neuron disease
• palliative care
• withdrawing from ventilation
• course of dying
• augmented symptom control
• sedation
• end-of-life care

Acknowledgements

DK, AF, CM and TM were supported by the initiative ‘Hilfe für ALS-kranke Menschen’. DK was a fellow of the ‘Angelika-von-Hagen-Fonds für Palliativmedizin bei der ALS’. The authors’ work was also supported by the ‘Knut Marthiens Fonds für ALS-Therapieforschung’. TM and SS were supported by the Joint Programme - Neurodegenerative Disease Research (BMBF; 01ED1402A). The project is supported through the following funding organizations under the aegis of JPND (http://www.jpnd.eu): Agency for Innovation by Science and Technology and Research Foundation, Flanders region; Bundesministerium für Bildung und Forschung (BMBF), Germany; Economic and Social Research Council, United Kingdom; Health Research Board, Ireland; Ministry of Health and Ministry of Education, Universities and Research, Italy; The Netherlands Organization for Health Research and Development, The Netherlands (national regional organizations who are funding project, by country, in alphabetical order).

Declaration of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.