Patterns of Non-Invasive Ventilation in Amyotrophic Lateral Sclerosis.

BACKGROUND: Non-invasive ventilation (NIV) improves quality of life and survival in patients with amyotrophic lateral sclerosis (ALS) and respiratory symptoms. Little is known about the patterns of NIV use over time and the impact of NIV on end-of-life decision-making in ALS. OBJECTIVE: This study assessed the pattern of NIV use over the course of the disease and the timing of end-of-life discussions in people living with ALS. METHOD: A retrospective single-center cohort study was performed at London Health Sciences Centre. Daily NIV duration of use was evaluated at 3-month intervals. The timing of diagnosis, NIV initiation, discussions relating to do-not-attempt-resuscitation (DNAR) and death were examined. RESULTS: In total, 48 patients were included in the analysis. Duration of NIV use increased over time, and tolerance to NIV was observed to be better than expected in patients with bulbar-onset ALS. There was a high degree of variability in the timing of end-of-life discussions in patients with ALS (356±451 days from diagnosis). In this cohort, there was a strong association between the timing of discussions regarding code status and establishment of a DNAR order (r2=0.93). CONCLUSION: This retrospective cohort study suggests that the use of NIV in ALS increases over time and that there remains a great deal of variability in the timing of end-of-life discussions in people living with ALS. Future prospective studies exploring the use NIV over the disease trajectory and how NIV affects end-of-life decision-making in people with ALS are needed.

[Weaning from mechanical ventilation. Weaning categories and weaning concepts]. / Entwöhnung von der mechanischen Beatmung. Weaningkategorien und Weaningkonzepte.

The international classification of three weaning categories (simple weaning, difficult weaning, prolonged weaning) has been modified in the German weaning guidelines: the group of prolonged weaning has been subclassified into weaning without noninvasive ventilation (NIV), weaning with NIV, if necessary with continuing NIV in the form of home mechanical ventilation, and weaning failure.Strategies to prevent prolonged weaning comprise daily interruption of sedation, daily screening of capability of spontaneous breathing by a spontaneous breathing trial (SBT) and early implementation of NIV instead of continuing invasive mechanical ventilation especially in hypercapnic patients. The comorbidity left heart failure plays a major role in weaning failure and need for re-intubation-in this case early diagnosis and if necessary modification of heart therapy are important.Specialised weaning-centres offer the option for successful weaning for about 50-60 % of patients declared as unweanable by usual intensive care units. A multimodal therapy concept with respiratory therapists, physiotherapists and speech therapy is necessary to reach this goal. In case of weaning failure a professional discharge management to invasive home mechanical ventilation is important. Competent care by physicians in the out-of-hospital area is restricted by the sectoral division of responsibility by the German health care system. Improvement in this area is urgently needed.