At the age of just 54, Geoff Pike was diagnosed with dementia, turning his family’s world upside down. ‘There were lots of tears, it was a nightmare for us,’ says his wife Helen, 59, a retired nurse and hospital manager.
The couple, from Gretton in Northamptonshire, were ‘at that stage of life where we had so many plans, including retiring early and travelling’, says Helen. ‘Suddenly everything changed.’
Geoff, now 61, a former national sales director for a private medical insurer, is one of around 70,800 people in the UK living with early-onset, or young-onset, dementia – defined as when symptoms start before the age of 65. Last week, Birds Of A Feather star Pauline Quirke revealed her own diagnosis in 2021, at 61.
There are concerns about a rise in cases of young-onset dementia – a study of GP practice records in England in 2022 revealed an alarming 69 per cent increase since 2014 (from 28,800 cases).
The causes of dementia in younger people are essentially the same as for older patients: Alzheimer’s, where proteins build up in the brain, forming plaques and tangles; and vascular dementia, caused by problems with the blood supply to the brain, as a result of heart disease, for instance.
Younger people may be more at risk of rare types of the disease such as frontotemporal dementia (FTD), which tend to initially cause more unusual symptoms, such as personality changes or difficulty concentrating.
These rarer types are most commonly diagnosed in those aged 45 to 65, but this may be because they’re not being spotted in older people, suggests Professor Sir John Hardy, chair of molecular biology of neurological disease at University College London.
He and other experts believe the rise in young-onset dementia may be down to something as simple as more accurate data collection: ‘I don’t think it’s due to more people having risk factors such as type 2 diabetes and high blood pressure, as research shows that cases of dementia in general are actually going down.’
Helen Pike with her husband Geoff, who is one of around 70,800 people in the UK living with early-onset, or young-onset, dementia
Geoff has FTD, which affects 16,000 people in the UK – it leads to a loss of cells in the front and side of the brain, areas that control behaviour (some people may start swearing, or neglect personal hygiene) and judgment.
Although it can progress slowly, survival time after an FTD diagnosis is between two and 12 years.
Geoff’s form of FTD is primary progressive aphasia, which particularly affects language (patients may struggle to understand words, or speak in a hesitant, or ungrammatical, way).
It’s caused by a genetic mutation that leads to low levels of a protein, called progranulin, which helps protect the brain against inflammation and ageing. One in eight people with FTD have a relative with the condition, and there’s often a genetic element.
Indeed, Geoff, who had no symptoms at that point, was only diagnosed after a chance remark in 2016 when a visiting cousin mentioned that young-onset dementia ran in their family. Helen recalls: ‘She told us that Geoff’s mother Mary – who died at 66 – and Mary’s mother (who died aged 54) both had FTD.
‘Their uncle, Mary’s brother, had taken part in a research project with Mary at the National Hospital for Neurology and Neurosurgery in London.
‘She assumed we knew all about this, but Geoff’s mum and dad had never told him or his two brothers – they thought she had died of Alzheimer’s, so it was a terrible shock to us both and very scary,’ says Helen.
‘Once Geoff knew he had a 50:50 chance of inheriting the gene for this he was distraught, as he had witnessed what had happened to his mother. He said he would rather die from anything else than FTD and even talked about going to Dignitas in Switzerland if he tested positive.’
Last week, Birds Of A Feather star Pauline Quirke revealed her own diagnosis in 2021, at 61
Geoff was referred for, and then a year later underwent, genetic tests at the same hospital where his mother had been involved in the research project. (One of his brothers died of bladder cancer at 53 and was never tested; the other has chosen not to be tested – at 78 he has no symptoms.)
‘Getting the results was probably the worst day,’ says Helen. From then on, it was an agonising wait for symptoms to start. By 2018, a year after his diagnosis, Geoff’s speech was deteriorating, and Helen says some people wrongly thought he was drunk, or was stupid as he couldn’t understand sometimes.
‘At first we tried to keep Geoff’s diagnosis a secret, apart from close friends and family, but looking back I think that was a mistake – things were much easier once everyone knew.’
The couple decided to retire and leave their home in Jersey to move close to family in the Midlands. ‘I was sad to retire, but I knew if I didn’t do it then, I wouldn’t have that time with Geoff,’ says Helen.
‘We drove round New Zealand in a camper van, took a trip across Europe, travelled the Golden Triangle in India, went to Victoria Falls in Africa and went sailing.
‘We crammed a lot in, as we knew the clock was ticking but I’m so grateful we had that time. Although Geoff had talked about assisted dying, because he was still relatively OK for a time he didn’t want to go then – and by the time he had deteriorated, he was no longer legally capable of making the decision.’
Geoff had decided soon after his diagnosis in 2017 that he wanted to take part in research trials, as his mother and uncle had done. As well as an observational study (to study the progression of symptoms) he enrolled in a trial for a new drug, latozinemab, specifically for his mutation.
‘At first, he’d say, “This one might cure me”, but deep down I think he knew it wouldn’t come in time,’ says Helen. ‘But he still wanted to make a contribution. Just taking part in a trial made us feel more positive. I also volunteered to be part of a research control group.’
Geoff decided shortly after his diagnosis that he wanted to take part in research trials, and enrolled in one for a new drug called latozinemab
The research involved, over five years, monthly trips to London for infusions, cognitive tests, MRI scans and lumbar punctures.
Dr Cath Mummery, a consultant neurologist at the National Hospital for Neurology and Neurosurgery, says: ‘Families like Geoff’s are critical in developing therapeutics for this horrible disease because it’s rare and there is a smaller pool of people to study.
‘Just taking part in an observational study can help, improving our understanding of the symptoms and pathology of the disease and it gives us a ready-made group for clinical drug trials. Otherwise it would take decades to recruit for a trial.’
Results from the trial of latozinemab – which normalises levels of progranulin and is one of the most promising drugs – are expected later this year. If successful, it would be the first drug for FTD.
By 2021, Geoff’s condition had deteriorated – he became nonverbal and could no longer understand the cognitive tests involved, so he had to drop out of the trial.
Helen says: ‘Geoff can no longer speak, read or write, but he still recognises me, friends and close family, although he can’t communicate. We are mainly confined to the house – if I need to see a doctor or dentist, I have to find someone to sit with Geoff.’
Helen describes herself as a ‘widow in waiting’ but is determined to look after Geoff at home right to the end. ‘I’m sure Geoff would be delighted to know he’s contributed to the possibility of the first-ever treatment for FTD. Being in a trial gave him hope at a terrible time. I’d encourage others to do the same.’
