An insight into Huntington's Disease

It is Huntington’s Disease (HD) Awareness Week, and we’re proud to help share more about this often misunderstood condition.

At St Andrew’s Healthcare Northampton, we care for around 24 people with the disease, ensuring they – and their families – receive the care, advice and support they need.

Here Andy Broughton, a Social Worker who works with our HD patients, shares his thoughts…

It is the cruellest and most heart breaking illness I have ever come across; some describe it as the worst bits of Dementia, Parkinson’s and Motor Neurone Disease all rolled into one. But it doesn’t have to mean the end of the world; people can ‘live well’ with Huntington’s Disease and I love working in a team who help people do just that.

Huntington’s Disease is an extremely rare, genetic neurodegenerative condition which affects the central nervous system, impacting on the individual’s muscle control, such as ‘chorea’ (random, jerky movements), their ability to speak and swallow, as well as their cognition and mental health.

The physical symptoms typically begin to show around the age of 30, and life expectancy after this is around 15 to 25 years; symptoms can begin to show from early adolescence and this is known as juvenile Huntington’s, which happens in around 10% of cases. The genetic link is strong; if an individual’s parents have it, they have a 50/50 chance of inheriting themselves, and while some symptoms can be managed, there is no known cure.

> Download our Huntington's Disease quick reference guide

There are only an estimated 8,000 people living with Huntington’s Disease in the UK, which is around 0.01% of the population. In Northamptonshire, although the actual number is likely to be higher, there are at least 100 known individuals ‘at risk’ of having Huntington’s, but as only the minority choose to be tested, it is difficult to properly quantify.

For those living with it, it can have the most terrible, life-changing affect. As time goes on, the individual can begin to lose their independence and ability to self-care, eventually becoming totally dependent on others for their most basic of needs. Their cognitive abilities will begin to decline; they will lose the ability to communicate with others and to swallow, causing a significant risk of choking and chest infections.

For many of those affected, they may have also seen their grandparents, parents, siblings and even (in cases of juvenile Huntington’s) their children go through the same process.

HD and mental illness

While Huntington’s Disease does not automatically lead to mental illness, the rates are higher than that of the general population and can cause irritability, mood disorders and, in around 11% of cases, serious mental illness such as Psychosis. When faced with such a future, it is completely understandable why rates of suicide are seven to 12 times higher in those diagnosed with Huntington’s Disease than in the general population.

For these reasons I can completely understand why so many people have said to me they’d struggle to work in a Huntington’s Disease service. But I don’t, I love it! Because for me it’s simple: there is currently nothing we can do to cure them of this terrible disease, but there is an awful lot we can do to improve their quality of life; to minimise the distress and suffering they face and give them back some independence and dignity, some joy and happiness, and something worth living for.

I work in an amazing, dedicated team who work tirelessly to improve the quality of life for those living with this Huntington’s. Over the years we have been able to do some amazing things for our patients; from supporting one young lady to go to Disneyland Paris, and reuniting families, to more simple things that made such a massive difference - like getting a tattoo or helping them to keep eating their favourite foods. Perhaps most importantly, we have been able to support people to have a good, peaceful and dignified death, with their loved ones around them.

These are the things we can’t always measure or put an outcome on, but we know they are by far the most important matters to the individual, which makes all the difference to them. This is why I love working with those living with Huntington’s.

We have, in association with the Huntington's Disease Association, created a mini guide about HD.