K2 International guidelines project: an update of non-pharmacological recommendations for huntington’s disease care

Background An evidence-based framework for a basic minimum standard of care is required, not only to maximise health and quality of life for patients and their families, but also to set standards to underpin future clinical trial design. A first set of guidelines outlining some aspects of non-pharmacological management was published in 2012. In an effort to combine and formulate guidelines including pharmacological and non-pharmacological approaches a further review of the available evidence was conducted. Aim The aim is to update recommendations for non-pharmacological management in HD as part of the International Guidelines Project. Methods The guidelines will be developed using a standard methodology provided by the Haute Autorite de Sante (HAS) in France. This formal method ensures the quality and reliability of the guidelines. A systematic and comprehensive review of all available literature aims to identify and critically appraise evidence addressing the effectiveness of various symptomatic treatments. Results From the systematic literature review, 120 papers were identified and extracted of which 77 were considered in the development of the recommendations. There continues to be insufficient evidence to make strong recommendations. Nevertheless, there is a growing body of evidence supporting multi-disciplinary cooperation for the management of global functioning in HD. Furthermore, there is increasing evidence in support of the use of physiotherapy for impairments in strength, stability, and functional independence. Whilst there remains a low level of evidence for the use of speech and language therapy, in the management of dysphagia and weight loss, and occupational therapy, for the adaption of a patient’s environment. Conclusion Although there is progress in areas of Physiotherapy, a paucity of high quality evidence is still lacking in many other areas. Further research is urgently needed on how to manage swallow, weight loss and communication.