Huntington's disease care guidance consolidated for clinicians

Clinical guidelines for Huntington's disease have been brought together for the first time in a single resource to support more consistent care for patients.

Huntington's disease is a degenerative neurological condition caused by a faulty gene, which affects around one in 10,000 people in the UK. While it is often associated with movement difficulties such as involuntary jerking or trembling, the disease also affects cognition and behaviour as nerve cells in the brain gradually degenerate. The newly consolidated guidelines, available on the Huntington's Disease Association (HDA) website, address a wide range of care considerations, including mental health support, social and community care services, and end-of-life planning. Such centralised guidance is intended to help healthcare professionals better diagnose, manage and coordinate long-term care for people living with the condition.

Dr Sarah Gunn, an NHS clinical psychologist at the University of Leicester and neuropsychology specialist who contributed to the mental health guidelines, said: 'Huntington's is a rare and complex condition, and healthcare professionals may feel unsure about how best to support people living with it… These new sets of guidelines bring together available evidence to support informed and confident clinical practice, and cover the different types of difficulties and needs commonly seen in adults affected by Huntington's disease.'

Improving coordination of care is a major focus for the HDA. Alongside the clinical guidance, the organisation has launched a report, 'Support at every step: Improving care coordination for people living with Huntington's disease', based on responses from more than 250 people affected by Huntington's disease. The report highlights that 65 percent of respondents did not have a named professional responsible for coordinating care across different services, something the HDA says is essential for delivering effective, joined-up support.

At the launch event for the report, HDA representatives met with MPs to discuss the challenges facing families affected by Huntington's disease. Steve Duckett, an HDA trustee who cared for his wife, Sandy, before she died from the condition, said: 'One of the biggest challenges was organising Sandy's care. On some weeks, I could be on the phone to eight or nine different healthcare services. It felt like navigating a confusing maze and often I knew more about Huntington's disease than the professionals. Having one person to speak to who knew about the condition and could have helped manage Sandy's care would have made a world of difference.'

The availability of consolidated care guidance may help improve support for people living with Huntington's disease in the immediate future, alongside ongoing research into new treatments. Recent developments include a microRNA-based gene therapy being developed by researchers at University College London, which has shown potential to slow disease progression (see BioNews 1308); and genome editing approaches tested in mice by researchers at the University of California, Los Angeles, to repair the faulty genes responsible for Huntington's disease and Friedreich's ataxia (see BioNews 1291).

David Stephenson, head of policy and public affairs at the HDA, said: 'These guidelines, developed by leading specialists in neuropsychiatry and clinical psychology, and endorsed by the Association of Clinical Psychologists UK and the European Huntington's Disease Network, give practitioners the knowledge they need to offer effective support.'