‘I decided to shut down family’s crippling gene’
A woman has told how she has decided not to have children of her own after a cruel genetic illness took the lives of three generations of her family. Andrew Nuttall reports
AWOMAN has spoken out about a genetic condition that has claimed the lives of multiple family members, and caused her to make the tough decision to never have children of her own.
Katie Davies, from Ruabon, near Wrexham, said there’s too much of a risk that she might pass on the cruel illness, Huntingdon’s disease, a genetic disorder that affects the nerve endings of the brain.
It causes progressive damage that leads to physical, cognitive and emotional changes and each child of a parent with Huntingdon’s disease has a 50% chance of inheriting the gene.
For the past eight years her 59-year-old mother, Fiona Jones, has been a resident at Pendine Park’s Penybryn care home in Wrexham, which specialises in looking after people who have the condition that’s been described as Parkinson’s, motor neurone disease and Alzheimer’s rolled into one.
In a cruel twist of fate, Fiona’s twin sister, Alison, also fell victim to the disease and passed away in 2020 after spending her final years living at Penybryn alongside her.
Initially, Alison’s illness progressed at a faster, more noticeable rate than Fiona’s when it was in its early stages.
But not long after her sister’s symptoms were evident in her mid- to late thirties Fiona also became afflicted with the first emotionally devastating effects of the progressive debilitating illness.
Katie decided to speak out to raise awareness of the chronic illness that’s transformed her mum from a bright and cheerful head of the family into a struggling, stumbling and sometimes unpredictable personality suffering wild mood swings and strange fixations.
The eldest daughter had to take on the role of stand-in mum to her younger brother and sister, Ted and Kia, when their own mum could not cope.
Katie explained that her mum and aunt knew in younger years that they had a 50-50 chance of inheriting Huntington’s disease from their father, who died from the condition, as did his own father.
She said: “It was Mum who first spotted the early symptoms in Aunty Alison, though at that point she was not symptomatic herself. She noticed how my aunty would struggle to find the right words sometimes and was prone to stumbling and strange jerky movements.
“Involuntary spasms are one of the first symptoms, along with stumbling and what can be misconceived as clumsiness. Sadly,
Mum soon began to manifest similar behaviour herself, though at first it was at a less prominent rate than my aunty.”
Katie spoke about how her childhood was “different than those of other kids in our street”. She said that they had to take on board lots of the routine tasks that a parent would normally do.
Katie has lived under the shadow of the disease all her life, but she has purposely chosen not to find out if she is carrying the Huntington’s disease gene or not.
Because she does not know if she has inherited the gene, Katie has also chosen not to have children so there is no danger of her passing it on to descendants.
She said: “For years I lived in constant fear that I might be next, until I was referred for counselling to help me with my mental wellbeing and stop thoughts of the disease taking over my whole life. Now I just live my life each day as it comes and enjoy time with Sion and our family.
“In one way, Sion and I have already had the experience of bringing up a family as following my mum’s diagnosis we took on the responsibility of raising my brother, Ted, and sister, Kia, when they were both still primary school age.”
She said: “It is such a difficult thing to have to explain and I did not want it to colour our relationship. But I knew there would be a time when I’d have to tell him what was actually wrong, especially as mum’s behaviour grew more erratic.
“I knew one day the kids would have to live with me. When I did tell him he was so supportive and understanding, as he’s been ever since. It is our dearest hope that the more awareness is raised and the more we know about this genealogy, no-one else will have to suffer like mum and Aunty Alison and we will one day be able to eradicate Huntington’s forever.”
Katie’s mum Fiona is one of 10 residents living with the disorder who are now being cared for at Penybryn, which has just been recommended to become a fully accredited Huntington’s disease care centre.
The recommendation to officially acknowledge the contribution of Pendine Park came from Huntingdon’s expert Dr Alberto Salmoiraghi, a consultant psychiatrist and medical director with the Mental Health and Learning Disability Division at Betsi Cadwaladr University Health Board, who is a regular visitor to Penybryn.
Penybryn manager Tracey Cuthill said it was an honour for the home to be recommended to become officially accredited as a Huntington’s disease centre.
She said: “It is an acknowledgement of the work we all do and we are proud to be considered as a centre of excellence by families and medical professionals.”