Survival in ALS with home mechanical ventilation non-invasively and invasively: a 15-year cohort study in west Denmark.

Our objective was to describe patient characteristics, survival and long-term outcome in amyotrophic lateral sclerosis (ALS) patients treated with non-invasive and invasive home mechanical ventilation (HMV). A single-centre cohort study over a 15-years period (1998-2012) was performed. All ALS patients admitted to the centre were enrolled in the study. All patients were offered treatment with non-invasive and/or invasive HMV. The patients were divided into four groups: 1) no treatment; 2) treatment with non-invasive HMV; 3) treatment with non-invasive HMV followed by invasive HMV by tracheostomy; and 4) treatment with invasive HMV by tracheostomy. Patient characteristics and effects on survival were studied. Four hundred and thirty-one patients with ALS were admitted to a referral respiratory care unit (RCU) in the period January 1998 to June 2012. The average treatment time in the groups was: 1) 22.9 months (range 1-164); 2) 25.8 months (range 1-145); 3) 56.8 months (range 14-207); and 4) 33.8 months (range 6-88). Non-invasive HMV followed by invasive HMV is a possible treatment of respiratory symptoms in ALS and has a significant effect on survival.

Withdrawal of invasive home mechanical ventilation in patients with advanced amyotrophic lateral sclerosis: ten years of Danish experience.

BACKGROUND: Due to the growing use of home mechanical ventilation in amyotrophic lateral sclerosis (ALS), physicians are increasingly confronted with patients seeking discontinuation of therapy. Yet there are few systematic investigations of the withdrawal of invasive home mechanical ventilation (IHMV). This article aims to describe the medical and patient-related aspects of terminating IHMV in patients with advanced stage ALS. METHODS: A retrospective, descriptive study was made of all ALS patients cared for at our center from 2002 to 2009 who decided to withdraw their consent for IHMV. RESULTS: All 12 ALS patients (4 females) received continuous IHMV. They had a median age of 61 years (range, 39-69 years). In all cases advance directives for end of life care, particularly concerning withdrawal of treatment, were discussed before the initiation of IHMV. The median time from initiation of IHMV to the decision to terminate treatment was 22 months (range, 1-35 months). The reasons for requests were for all patients a general loss of "meaning in life." Deep sedation was achieved with high dose morphine and diazepam before disconnecting the ventilator. CONCLUSION: Patients' requests for the withdrawal of IHMV in advanced stage ALS was related to a perceived "loss of meaning in life." Termination of treatment was performed under deep sedation as a medically, legally, and ethically justified procedure.