Only when Lanarkshire woman Rachael Steele began to consider being tested for the incurable disease that she’d a 50/50 chance of inheriting from her mum did she realise the significance of “in sickness and in health.”
The 33-year-old data analyst desperately wanted to plan for the future with her partner, Graham Cameron – but the couple’s fears were realised when she received a positive result for the faulty gene, passed down through the generations, that causes Huntington’s disease.
That was back in 2018 – and, mercifully, Rachael is still healthy and has no symptoms of the condition.
“There was counselling beforehand, which helped prepare me for whatever the result was going to be,” she told Lanarkshire Live.
“One of hardest things was telling my mum, June, who has Huntington’s disease. It was devastating – much worse than when she found out about herself. But now, some time has passed and we’re getting on with things.
"It does take a toll on Mum, but I’m thankful she’s keeping okay right now.”
As Rachael explained, being tested is a very personal decision, and many people at risk of Huntington’s choose to live their lives without finding out. Because there is no cure, they consider that knowing would not change their destiny.
“For us, we were moving towards getting married and maybe thinking about starting a family. Having a clear picture means we can look at our options and make decisions,” she said.
Each child of someone who has the faulty gene is at 50/50 risk of inheriting it, too. Severe symptoms of Huntington’s can include
a loss of body control, leading to involuntary, repetitive jerky movements and the inability to walk, talk, eat, drink or swallow. Thinking processes may change and early on-set dementia can develop, as can mental illness.
And such a complex combination of symptoms can result in the need for 24-hour care.
Said IT specialist Graham, 37: “When Rachael decided it was time to get herself tested, I was very anxious as I was fully
aware that she had a 50/50 chance. I was ready to support her with anything that may happen, while obviously hoping for the best result.
“My heart sank when we found out the test had come back positive. I felt helpless, knowing there was nothing I could do to change it. All I knew was I had to be there for Rachael for whatever she needed.
“Since then, we try not let this interfere with our life together. I know that further down the line, the effects will become more apparent. But I remain positive that this will not be for a long time yet and I will always be there for Rachael, for whatever happens.”
Right from the start, the couple were open with friends, family and work colleagues.
And when they tied the knot almost a year ago, the bride and groom ditched traditional favours and instead made a donation to Scottish Huntington’s Association and gave every guest a pin badge.
That came as a surprise for mother-of-the-bride, June, who had no idea until she reached her table that every place setting included the special gift.
“Mum was very touched and our guests were delighted,” said Rachael, of Hamilton. “It was great to see everyone up dancing, wearing the bright blue badge. It’s never been a secret, but there were probably a few people on Graham’s side who didn’t realise how Huntington’s disease affects my mum.”
Now, as they approach their first wedding anniversary, Rachael has become more involved with the work of Scottish Huntington’s Association as voluntary chairperson of the charity’s new Lanarkshire fundraising group.
The funds it generates will go towards lifeline services provided by the charity, including: HD specialists who provide emotional support and ensure the right package of care is in place; a financial wellbeing service that helps with access to benefits and other financial products; and a youth service for children and young people growing up in Huntington’s families.
The Lanarkshire group has been holding regular online meetings to plan its first fundraising event: a big Christmas fayre on Saturday, December 3, at West and Wardlawhill Church in Rutherglen.
“We’re determined to raise as much money and awareness as possible,” said Rachael. “Mum and I both have Scottish Huntington’s Association HD specialists, and every group member has a connection with Huntington’s disease, so we all know first-hand how much families depend on the charity’s services.”
Another of the charity’s fervent supporters is Lanarkshire mum-of-three, Gillian McNab, who inherited the faulty gene from her late mum, Myra.
Strathcarron Hospice palliative care nurse Gillian watched her own mother’s gradual decline from Huntington’s disease – and, as a woman whose vocation is to care for people who are nearing the end of their lives, she knows that her own daughters will one day do the same.
At the age of 52, Gillian, of Lanark, is asymptomatic. And she feels blessed to have time to treasure life and ensure that not a moment of it is wasted.
That has included raising tens of thousands for SHA in recent years through dare-devil skydives, wing walks and danceathons.
During SHA’s 30th anniversary in 2020, not only was she named Volunteer of the Year, Gillian also became a ‘You, Me and HD’ speaker, taking her message across Scotland in a bid to overcome the stigma that surrounds the disease.
Another inspirational supporter of Scottish Huntington’s Association is East Kilbride teenager, William Hillhouse.
Although he knows there’s a 50 per cent chance that he has inherited the devastating neurological condition from his dad, 16-year-old William is singing and dancing his way through life, while taking every opportunity as his career in musical theatre progresses to raise awareness of the charity that supports his family.
Super talented William – a student at the Dance School of Scotland in Glasgow – has appeared in pantos with David ‘The Hoff’ Hasselhoff and his heroes, The Krankies.
“I’ve travelled all over the country and I take every chance to speak to people about Huntington’s disease and its symptoms,” he said.
“I’m a glass-half-full person who hopes for the best – because my mum’s positivity inspires me to be the same. I’ve known about the risk to me since I was young.
“Mum got in touch with Scottish Huntington’s Association to make sure I have the right support. I’ve been meeting up regularly with one of the charity’s youth advisors ever since and it’s made a huge difference to how I live my life.
“It means I understand much more about the disease and there’s always someone to answer my questions and help me to understand the changes in my dad’s health.”
Increasing awareness and fundraising is a vital part of Scottish Huntington’s Association’s work to ensure that all Huntington’s families have access to the care and specialist support they need, regardless of where in Scotland they live. It also enables young people like William to have one-to-one sessions with a youth advisor and respite breaks such as summer camps and activity days.
Volunteer fundraisers take part in a variety of challenges including marathons, skydives, the Three Peaks Challenge, the Kilt Walk and earlier this year, Dance 100 in George Square. They also organise their own events such as dinner dances, quiz nights and fetes.
* Huntington’s disease is a complex neurological condition with symptoms that typically begin to develop between the ages of 30 and 50. It is hereditary, meaning that it impacts upon entire families over generations rather than on individuals alone.
Each child of a person who has Huntington’s disease is at 50 per cent risk of having inherited the condition.
As Huntington’s disease progresses it can impact a person’s:
* Movement (or motor skills). People with Huntington’s can experience repetitive involuntary movements resulting in mobility, balance and coordination problems, as well as difficulties with speech and swallowing.
* Thinking processes (or cognition). Huntington’s causes a type of early onset dementia, which affects ability to process information, make decisions, solve problems, plan and organise.
* Mental health. People may also experience a decline in mental health. Depression, anxiety, irritability, obsessive pre-occupations and apathy are among the most common mental health problems experienced. Psychosis may also occur.
Around one in 5000 people in Scotland have Huntington’s disease. Here, 1100 people have been diagnosed with the disease and a further 4000 to 6000 are at risk of having inherited it from their parents.
Symptoms generally progress slowly over a long period time. Those who have the disease may eventually lose the ability to walk, talk, eat, drink, make decisions or care for themselves. It typically takes between 10 and 25 years from a person developing
Huntington’s symptoms until the end of life.
Although worldwide research is continuing, there is no cure for Huntington’s disease. Many of its symptoms, however, can be managed with a combination of medication, alternative therapies and appropriate support from specialist services delivered
by Scottish Huntington’s Association (SHA) and medical, health, social care providers.
SHA is the only charity in the country exclusively dedicated to supporting families impacted by HD.
To find out more about how to get involved, visit hdscotland.org
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