The optimisation of noninvasive ventilation in amyotrophic lateral sclerosis: a systematic review

Abstract

BackgroundNoninvasive ventilation (NIV) prolongs survival and quality of life in amyotrophic lateral sclerosis (ALS); however, its benefits depend upon the optimisation of both ventilation and adherence. We aimed to identify factors associated with effective initiation and ongoing use of NIV in ALS to develop evidence-based guidance and identify areas for further research.MethodsWe searched 11 electronic databases (January 1998 to May 2018) for all types of quantitative and qualitative studies. Supplementary grey literature searches were conducted. Records were screened against eligibility criteria, data were extracted from included studies and risk of bias was assessed. We present findings using a narrative synthesis.ResultsWe screened 2430 unique records and included 52 quantitative and six qualitative papers. Factors reported to be associated with NIV optimisation included coordinated multidisciplinary care, place of initiation, selection of interfaces, ventilator modes and settings appropriate for the individual patient, and adequate secretion management. The literature indicated that patients with significant bulbar dysfunction can still derive considerable benefit from NIV if their needs are met. Research emphasises that obstructive airway events, mask leak and uncontrolled secretions should be addressed by adjustments to the interface and machine settings, and the concomitant use of cough augmentation.ConclusionThis review highlights that NIV optimisation requires an individualised approach to respiratory management tailored to the differing needs of each patient. Ultimately, this should lead to improved survival and quality of life. This review expands on recommendations in current international guidelines for NIV use in ALS and identifies areas for future research.

Long-Term Mechanical Ventilation: Recommendations of the Swiss Society of Pulmonology

“…In ALS, multidisciplinary management is associated with optimized timing of NIV. Even selected patients with bulbar dysfunction can derive benefit from NIV [175].…”

Transcutaneous CO2 monitoring as indication for inpatient non‐invasive ventilation initiation in patients with amyotrophic lateral sclerosis

“…9 When comparing planned outpatient versus inpatient titration, waiting time for initiation and adverse events (death and admission for respiratory failure) were higher in the outpatient group and survival was extended for those admitted; however, this was a very small group of patients (n = 12). 10 There are several limitations to this case series. Foremost is the small sample size and retrospective nature of a case series.…”

Effects of prolonged interruption of rehabilitation routines in amyotrophic lateral sclerosis patients

“…Furthermore, the decay between T0–T1 and T1–T2 was significant in the bulbar, upper limbs, and lower limbs subdomains but not in the respiratory subdomain. This might be associated with the continuous respiratory management, which is highly supported when needed with mechanical ventilation, educational programs (on adherence and effective use), including home telemonitoring follow-up and assistance (Pinto et al, 2010; O'Brien et al, 2019). The fact that patients are confined at their homes may also generate fewer respiratory symptoms (like dyspnea) due to decreased mobility and metabolic demand (Pinto and de Carvalho, 2015).…”