Moray man Brian Watt, from Hopeman, who is seeking to raise awareness of Huntington’s disease, says: ‘Huntington’s disease won’t stop me living me life!’; his comments come as new University of Aberdeen research is published into rates of the disease in Scotland
A Moray man who set up a support group for fellow Huntington’s disease patients after his own diagnosis with the degenerative illness, says it’s important to keep doing what you love and stay positive.
Brian Watt (69), from Hopeman, was speaking to raise awareness of the disease and the need for people who may have it to get tested, after new figures revealed the north of Scotland has one of the highest rates for it in the entire world.
A new study led by the University of Aberdeen has revealed that the disease rate in northern Scotland is 14.5 per 100,000 people - more than five times higher than the global rate of 2.71.
The devastating disease is caused by a neurodegenerative gene and runs in families. Every child of someone affected has a 50:50 chance of inheriting the gene, which slowly damages the brain, eventually taking away the person’s ability to walk, talk, eat and drink, make their own decisions and care for themselves.
The new analysis, which was published in Neuroepidemiology, showed that, on average, every person who has been diagnosed with Huntington’s disease will have at least another 2.2 relatives who have the gene - meaning there are hundreds of people in the north of Scotland who could be considered for effective treatments for Huntington’s disease when these become available in the future.
The north of Scotland’s high figures were calculated after researchers used NHS family-based records to find there are more than 160 adults living in Grampian, Highland, Orkney, Shetland, and the Western Isles who have the gene but have not been tested - and scientists believe the figure will be even higher as not everyone with symptoms seeks diagnosis.
The research was led by Professor Zosia Miedzybrodzka of the University of Aberdeen and NHS clinical lead for Huntington’s disease in the north of Scotland (covering Highland, Grampian, Orkney, Shetland, and the Western Isles), based in NHS Grampian, alongside Heather Cruickshank, genetic counsellor in NHS Grampian.
And they say it is “crucial” that health boards have accurate figures for the numbers of people who have the disease “so that [they] can properly plan for care and for treatments when they become available in the future”.
Brian, who is a former manager of the Chivas whisky distillery in Keith and continues to work part-time at the Moray Motor Museum in Elgin, said he was glad he got tested and received his diagnosis back in 2016.
He recalls: “I knew there was Huntington’s disease in my family – my father and my sister were both diagnosed.
“I wasn’t sure whether to go ahead with the test, but I am glad I did.”
Brian received counselling – offered by NHS Grampian's Huntington’s disease clinic in Aberdeen - before taking the test and he felt this was ‘hugely important’ and ‘absolutely necessary’ to be able to process the news regardless of the result.
Almost immediately after receiving the news that he has the gene, Brian set about organising a support group in his local area for people with Huntington’s disease and their families. The group is still going strong as a Branch of the Scottish Huntington’s Association and has helped support more than 30 families in the Moray area.
Brian keeps busy and believes a positive outlook to be vital when living with Huntington’s disease: “It could be tempting to just lie in bed all day and feel depressed about having this disease. But you have to keep going – keep busy doing things you enjoy.”
Brian walks his two terriers along Hopeman beach every day, works part-time at Moray Motor Museum in Elgin and organises quarterly meetings of his support group.
Brian says: “Zosia said to me the best way to manage Huntington’s disease is with a balance of lifestyle, attitude and medication and I think that is spot-on. I try to keep a healthy lifestyle and positive attitude and I’m doing just fine.”
Prof Miedzybrodzka believes it is vital that more accurate figures for Huntington’s disease are known. She said: “No-one has properly counted just how many people who haven’t been tested yet must have the gene.
“It is crucial that we know this, and that it is accurate, so that health boards can properly plan for care and for treatments when they become available in the future.
“A 2022 Scottish Government report underestimated Huntington’s disease rates and did not account for numbers of people at risk in a way that our data has.”
Ms Cruickshank added: “Despite high rates of testing, most people at risk of developing Huntington’s disease in Scotland have not had a test. There is a massive worldwide effort seeking treatments for Huntington’s disease.
“Services need to plan to treat these as-yet-uncounted people, as well as those currently diagnosed. Regional variations in rates will become more important, including genetic counselling and testing, management, and treatment delivery.
“Better knowledge of the numbers of people who could benefit will encourage investment into drug discovery.
“Having a test remains a free choice for people from Huntington’s disease families and our research means that care can be planned for all those at risk, without people who don’t want a test having one.”
Scottish Huntington’s Association chief executive Alistair Haw said: “This latest study further strengthens the case for immediate action to expand specialist services for families impacted by Huntington’s.
“Huntington’s disease is a hugely complex, widely misunderstood and extremely difficult to manage condition. Specialist services are not some ‘nice to have’ optional extra but an absolute necessity to prevent patients reaching crisis point and presenting to acute emergency statutory services.”
