When Dad developed a worrying habit at 58, doctors put it down to stress. In fact it was the first sign of an unfathomably cruel disease
George Hardy was known to be an intelligent and articulate man who, as a top pharmaceutical research scientist, had dedicated his life to finding new drugs to beat viruses.
So when he began to lose the ability to find the right word for common household objects soon after he took early retirement at the age of 58, his family were rightly concerned. His TV director son Ben, 50, recalls watching George standing in the kitchen holding a knife, unable to remember what it was called.
Conversations gradually became more stilted and frustrating as he floundered to get the right words out. But when he got a simple maths sum wrong when arranging to loan Ben some money, the family knew something was seriously wrong.
A ‘traumatic’ few months of tests, however, including a referral to a private dementia specialist, got them nowhere.
Ben Hardy, right, with his father George, who was diagnosed with primary progressive aphasia (logopenic) in 2011 at the age of 61
‘The problem was that Dad’s memory was fine,’ says Ben, 50, a director for shows such as QI, I’m A Celebrity and Naked Attraction. ‘He passed all of their tests. Scans apparently showed there was no issue with his brain. The doctor sat us down and told us he could find no evidence of Alzheimer’s, and that as far as he could tell there was no neurological problem.
‘He said Dad’s symptoms were most likely due to stress caused by his relationship with Mum. But they had no relationship problems, and it left Mum devastated.’
What George, from Robertsbridge, East Sussex, and his family experienced is sadly all too common.
Eighteen months after his first appointment, and aged just 61, George was finally diagnosed with a rare form of dementia after the family sought a second opinion. Called logopenic aphasia, it is part of a spectrum of conditions known as primary progressive aphasia, or PPA, which affect speech and language.
George’s form of PPA means people struggle to find the right words. Another type stops people from being able to physically produce sounds to make words, while a third slowly robs people of the ability to understand the meaning of words.
These conditions are 100 times less common than Alzheimer’s, affecting just 10,000 people in the UK, and often hit people ten to 20 years earlier than other types of dementia.
But there is little research on why and how they develop, no effective treatments to slow their progression, and even diagnosis is difficult. Crucially, those affected often pass GPs’ standard memory tests, which means dementia may not be considered. Patients may instead be told they have had a stroke, or are having a nervous breakdown.
Jason Warren, professor of neurology at the Dementia Research Centre at University College London, says: ‘Unquestionably, these conditions are under-diagnosed. There may be many more people out there who are only picked up once their symptoms deteriorate into something which looks much more like standard dementia. It’s not uncommon for these people to still have quite good memories. We have this picture of dementia that involves people losing their general mental capacities, but these can be very selective diseases. One of my patients with PPA is completely mute and can no longer form words physically, but still writes poetry.
Ben, pictured as a child with his father, says: ‘Dad was such a clever man and it was so out of character for him to struggle the way he did in the early stages’
George’s form of PPA means people struggle to find the right words. Another type stops people from being able to physically produce sounds to make words
‘But awareness of these conditions is poor, and people are often passed around different specialists and told they have other things wrong with them.
‘Even if you get referred to a dementia clinic, a PET brain scan or MRI scan is not enough to get a diagnosis – it really involves asking the right questions and considering PPA.’
Dr Leah Mursaleen, of Alzheimer’s Research UK, says that while there is nothing to slow the progression of PPA, speech and language therapy can help communication. But this can also be difficult to get and waiting lists are long, adds Prof Warren.
Ben, who lives in Addlestone, Surrey, with his wife Angela, 45, and their seven-month-old daughter Pollyanna, is speaking now about his father’s PPA to help raise money for Alzheimer’s Research UK’s Walk For A Cure fundraiser. The series of organised walks, taking place over the next few months, are in aid of research into finding a cure for dementia. Ben says George was offered speech and language therapy after his diagnosis and used an iPad app to find the words he needed but neither had much benefit.
His condition deteriorated until he could no longer communicate and he went into a care home after becoming a danger – falling over, wandering off in just his underwear, turning on kitchen hobs and causing fires and leaving taps running. He died in September 2021.
Prof Warren says: ‘We don’t understand yet why a particular person gets PPA but it’s rare for them to be genetic and seems to be just bad luck. There’s a lot of interest in trying to understand whether conditions like dyslexia might predispose people to develop it.’
Ben says: ‘Dad was such a clever man and it was so out of character for him to struggle the way he did in the early stages. He became very frustrated, although his memory remained OK and he still knew exactly who we were, right to the end. This disease is so poorly understood.’
Sign up for Alzheimer’s Research UK’s Walk For A Cure at walk.alzheimersresearchuk.org
