title: 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 However, a proportion of patients receiving long-term ventilation decide to have it withdrawn.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.
authors.affiliation: a Charité - Universitätsmedizin Berlin , Department of Neurology, Outpatient Clinic for ALS and other Motor Neuron Disorders , Berlin and.
authors.forename: Dagmar
authors.initials: D
authors.lastname: Kettemann
authors.affiliation: a Charité - Universitätsmedizin Berlin , Department of Neurology, Outpatient Clinic for ALS and other Motor Neuron Disorders , Berlin and.
authors.forename: Andreas
authors.initials: A
authors.lastname: Funke
authors.affiliation: a Charité - Universitätsmedizin Berlin , Department of Neurology, Outpatient Clinic for ALS and other Motor Neuron Disorders , Berlin and.
authors.forename: André
authors.initials: A
authors.lastname: Maier
authors.affiliation: b Charité - Universitätsmedizin Berlin , Department of Infectious Diseases and Pulmonary Medicine , Berlin , Germany.
authors.forename: Simone
authors.initials: S
authors.lastname: Rosseau
authors.affiliation: a Charité - Universitätsmedizin Berlin , Department of Neurology, Outpatient Clinic for ALS and other Motor Neuron Disorders , Berlin and.
authors.forename: Robert
authors.initials: R
authors.lastname: Meyer
authors.affiliation: a Charité - Universitätsmedizin Berlin , Department of Neurology, Outpatient Clinic for ALS and other Motor Neuron Disorders , Berlin and.
authors.forename: Susanne
authors.initials: S
authors.lastname: Spittel
authors.affiliation: a Charité - Universitätsmedizin Berlin , Department of Neurology, Outpatient Clinic for ALS and other Motor Neuron Disorders , Berlin and.
authors.forename: Christoph
authors.initials: C
authors.lastname: Münch
authors.affiliation: a Charité - Universitätsmedizin Berlin , Department of Neurology, Outpatient Clinic for ALS and other Motor Neuron Disorders , Berlin and.
authors.forename: Thomas
authors.initials: T
authors.lastname: Meyer
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D005858:Germany; D006801:Humans; D008297:Male; D008875:Middle Aged; D063087:Noninvasive Ventilation / Q000379:methods*; D010166:Palliative Care / Q000379:methods; D018709:Statistics, Nonparametric; D013727:Terminal Care / Q000379:methods*; D014140:Tracheotomy / Q000379:methods
keywords: Amyotrophic lateral sclerosis; augmented symptom control; course of dying; end-of-life care; motor neuron disease; palliative care; sedation; withdrawing from ventilation