TY - JOUR T1 - The optimisation of non-invasive ventilation in amyotrophic lateral sclerosis: A systematic review JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00261-2019 SP - 1900261 AU - David O'Brien AU - Theocharis Stavroulakis AU - Susan Baxter AU - Paul Norman AU - Stephen Bianchi AU - Mark Elliott AU - Maxine Johnson AU - Mark Clowes AU - Aldara Garcia-Sánchez AU - Esther Hobson AU - Christopher McDermott Y1 - 2019/01/01 UR - http://erj.ersjournals.com/content/early/2019/06/26/13993003.00261-2019.abstract N2 - Background Non-invasive 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.Methods We searched eleven electronic databases (Jan 1998 – 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.Results We screened 2430 unique records and included 52 quantitative and 6 qualitative papers. Factors reported to be associated with NIV optimisation included: co-ordinated 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.Conclusion This 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.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: David O'Brien reports a bursary from The Wolfson Foundation during the conduct of the study.Conflict of interest: Dr. Stavroulakis reports grants from National Institute for Health Research (NIHR) under the Research for Patient Benefit RfPB) programme during the conduct of the study.Conflict of interest: Dr. Baxter has nothing to disclose.Conflict of interest: Dr. Norman has nothing to disclose.Conflict of interest: Dr. Bianchi has nothing to disclose.Conflict of interest: Dr. Elliott reports personal fees from Philips-Respironics, personal fees from ResMed, and personal fees from Fisher and Paykel, outside the submitted work.Conflict of interest: Dr. Johnson has nothing to disclose.Conflict of interest: Dr. Clowes has nothing to disclose.Conflict of interest: Dr. Garcia-Sánchez has nothing to disclose.Conflict of interest: Dr. Hobson reports receiving a grant from the National Institute for Health Research (NIHR) under the Research for Patient Benefit (RfPB) programme. Further support was provided by the NIHR Sheffield Biomedical Research Centre (BRC). She also received equipment from Philips Respironics as part of a previous research study.Conflict of interest: Professor McDermott reports grants from the National Institute for Health Research (NIHR) under the Research for Patient Benefit (RfPB) programme during the conduct of the study. Further support was provided by the NIHR Sheffield Biomedical Research Centre (BRC). He is also a member of the Data Safety Monitoring Board for Orphazyme and Orion Pharma outside of the submitted work, and received equipment from Philips Respironics as part of a prior research study. ER -