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Huntington’s disease: From witch hunts to global waypoints in integrated care

The Salem witch trials of the late seventeenth century are notorious, but their connection with a devastating neurological condition is less well known. There is good evidence that some of the unfortunate victims of the trials may have had Huntington’s disease (HD). The condition has always been blighted by secrecy, misunderstanding and stigma; it was a full two centuries later that it was properly described and understood by Long Island GP, Dr George Huntington.

The condition causes severe motor changes (including loss of mobility, speech and ability to swallow), mental illness and impaired cognition caused by a type of early onset dementia. This is devastating enough, but the impact is deepened by the hereditary nature of the disease. Each child of an HD parent has a 50/50 chance of inheriting the gene and, therefore, the disease and symptoms which inevitably follow. In Scotland today between 4000 and 6000 people are at risk of inheriting the disease from the 1100 diagnosed cases.

This legacy of Huntington’s disease has created a vacuum of silence in which families have struggled with isolation, lack of access to support and, frequently, huge personal tragedy. It is difficult to imagine a group of people more in need of well-coordinated support. Sadly – like so many rare diseases – all too little is known about HD, resulting in health and social care services being confounded by its complex and often challenging presentation. Patient care and support can suffer badly as a result.

If only it was possible to have a national care framework that had at its core the principles of person-centeredness, an integrated health and social care approach and a well thought out set of multi-agency arrangements? What if it was spearheaded by a lead professional who focussed on providing specialist support and assisting people to navigate through the health and social care maze? What if it spanned the spectrum of issues that really matter to the families who live with the condition, from genetic testing, telling children about the condition, getting good symptom management and support at the end of life?

For families living with HD, and indeed the health and social care professionals they depend upon, that really would be a godsend. What’s more, if it proved possible to construct such a framework for a condition as notoriously complex as HD, might it not act as a trailblazer for other conditions and provide a model for their support networks to follow?

With funding and partnership support from the Scottish Government, that is what the Scottish Huntington’s Association set out to do in 2016. Developed by a geographically diverse multi-disciplinary expert group, and drawing extensively upon the lived experience of families, the Framework is now in its final draft, and out for public consultation.

The completed framework will be a waypoint in the journey towards improving care and support for people living with Huntington’s disease. It has been designed to avoid being a prescriptive document that rigidly sets out unrealistic expectations from the centre. In 2017 the work begins to develop local versions of the framework for each of Scotland’s 14 NHS Boards. This will mean HD families and professionals will have a readily available, flexible, interactive web-based resource to empower and guide them to the specific support they need, where they need it.
The framework will also set a standard of care throughout Scotland and will be the first for HD condition anywhere in the world. It will enable families and professionals to ask for more appropriate levels of support and, where required, to challenge deficits in support. By 2018 we hope to be in the position of having mapped significant change; perhaps we will also be able to celebrate a greater degree of awareness and understanding of the disease, something that people caught up in its wake desperately require and deserve.

If successful, it is our hope that this approach can significantly benefit people living with HD and, in due course, other conditions – not just in Scotland but throughout the world. So we’d better get it right. With the help of your readers, we know we can.

The consultation on the draft National Care Framework for Huntington’s disease remains open until 9 December. To get involved visit: hdscotland.org/careframework

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