A mapping review of international guidance on the management and care of amyotrophic lateral sclerosis (ALS)

Abstract

Management of ALS is suboptimal. Consequently, quality improvement interventions are needed to improve ALS care. An evidence-based insight into how patients should be managed is essential when developing quality improvement interventions. Therefore, this study aimed to map, categorize and summarize international guidance on the management and care of ALS and to identify gaps in this guidance by means of a mapping review. Literature was searched for clinical practice guidelines, quality indicators and evidence-based clinical summaries. A content analysis and meta-synthesis of the included literature was performed. Interventions and outcomes used in the management and care of ALS were identified and categorized. Furthermore, the amount of guidance underpinning these interventions and outcomes was analysed. Six clinical practice guidelines, one set of quality indicators and three evidence-based clinical summaries were identified. The results demonstrated that certain domains in ALS care, mainly disease-specific domains such as breathing and swallowing, are extensively addressed in the literature whereas other subjects, such as care coordination, receive little attention. In conclusion, this mapping review provides a scientific basis for targeting and developing the clinical content of a quality improvement intervention for the management of ALS.

Key words:

• Amyotrophic lateral sclerosis
• disease management
• review
• guidelines
• quality improvement

Acknowledgements

ALS-CarE is an EU Joint Programme – Neurodegenerative Disease Research (JPND) project. The project is supported through the following funding organisations under the aegis of JPND – www.jpnd.eu: Belgium, Research Foundation Flanders (FWO); Germany, Bundesministerium für Bildung und Forschung (BMBF); Ireland, Health Research Board; Italy, the Ministry of Health and the Ministry of Education, University and Research (MIUR); The Netherlands, The Netherlands Organisation for Health Research and Development (ZonMw) and the ALS Foundation Netherlands; UK, Economic and Social Research Council (ESRC).

Declaration of interest

AAC receives salary support from the National Institute for Health Research (NIHR) Dementia Biomedical Research Unit at South London, Maudsley NHS Foundation Trust, King s College Hospital, London and reports grants from the EU Seventh Framework Programme (FP7) EuroMotor and through the following funding organisations under the aegis of JPND - www.jpnd.eu (United Kingdom, Medical Research Council). PVD holds a senior clinical investigatorship of Fonds Wetenschappelijk Onderzoek (FWO)-Vlaanderen. LHvdB serves on scientific advisory boards for the Prinses Beatrix Spierfonds, Thierry Latran Foundation, Biogen Idec and Cytokinetics; received an educational grant from Baxter International Inc.; serves on the editorial board of Amyotrophic Lateral Sclerosis and the Journal of Neurology, Neurosurgery and Psychiatry; and receives research support from the Prinses Beatrix Spierfonds, Netherlands ALS Foundation, The European Community's Health Seventh Framework Programme (grant agreement n° 259867), The Netherlands Organization for Health Research and Development (Vici Scheme, JPND (SOPHIA, STRENGTH, ALS-CarE)).

All other authors declare no competing interests.

Supplementary material available online