29 may 2011
By Sharon Feinstein
Sorry Mr Pratchett, but with Alzheimer’s there’s no point in being angry
Best-selling author Terry Pratchett, 63, calls it ‘an embuggerance’, and describes himself as ‘slipping away a bit at a time’.
But Nadia Razis, who suffers from the same rare kind of Alzheimer’s disease, is not angry.
After a six-year battle to discover the cause of her terrifying symptoms, she says knowing her fate has led to a sense of peace and acceptance.
The 58-year-old teacher from Norfolk explains: ‘Terry Pratchett is furious and bitter that this is happening to him. But I’m not at all.
‘When I was diagnosed, my doctor told me I could have as few as six years left. And I know that before I die, in the not too distant future, I might not recognise my husband Vic or my two children.
I might not be able to feed or dress or wash myself, and I might not even know.
‘No one wants to be a burden but I have no choice. Yes, I have plenty of obstacles, but there’s still quite a lot I can do.
‘Of course, I was upset when I was diagnosed. But I came to terms with it. I’m happy. I don’t feel, “Why me?”‘
Pratchett has recently spoken out in favour of assisted suicide – something that Nadia has also considered.
She says: ‘I can understand where he is coming from but I don’t think it’s a road I would ever go down.
‘I know my family will look after me. Perhaps life will become intolerable, but I hope not. At the moment I am making the most of what I have.’
Posterior cortical atrophy (PCA) is a progressive degenerative condition involving the loss of brain cells, particularly at the back (posterior) of the brain – a variant of Alzheimer’s disease, the most common cause of dementia.
Best-selling author Terry Pratchett has said he is ‘slipping away a bit at a time’ with his condition
There are about 570,000 dementia sufferers in Britain, and numbers are expected to swell to one million by 2021, in line with the ageing population.
There are a number of different kinds of dementia, but the conditions are all characterised by a decline in mental abilities.
Believed to affect one in 100 of those aged 65, and one in 25 of those aged 70, by the age of 80, one in six will suffer from dementia.
However, PCA strikes earlier – typically around the age of 50, the age Nadia was when she first started putting on her clothes back to front.
She recalls: ‘I started getting very upset and weepy not knowing what was going on. For months I simply thought they were symptoms of the menopause, and didn’t even visit a doctor.
‘Then, I remember my daughter-and I were sharing some ice cream and she said, “Mum, you’re using your spoon the wrong way round.”
‘One day I suddenly couldn’t use a can opener – I just forgot how to do it. Or I would put down my briefcase and then become convinced it had disappeared, even accusing people of stealing it.
‘Then it was as if the room spun around and I suddenly saw it was where I had left it.
‘The defining moment was when I looked at a clock and couldn’t translate what the time was. I knew there was definitely something wrong, but nobody could tell me what and that made me feel desperate.’
Nadia, who has two children, Yalena, 31, and Stephan, 28, underwent a raft of tests. She visited opticians, and her GP countless times.
A brain scan and a Mini Mental State Examination, a brief 30-point questionnaire commonly used by doctors to screen for dementia, both came back normal.
Two years ago she was finally referred to neurologist Dr Sebastian Crutch at the Institute of Neurology, University College London.
There is no diagnostic test for PCA but visual examinations, tests of cognitive skills (memory, perception and literacy, for example), blood tests, and scans help to exclude potentially treatable causes such as infection, inflammation or brain tumour. Shrinkage of the back part of the brain as a result of brain-cell loss may be visible on a scan.
Sir Terry Pratchett was knighted by the Queen in February 2009 – just over a year after he announced he had been diagnosed with early onset Alzheimer’s disease
When Pratchett was diagnosed with PCA, he said: ‘I felt totally alone, with the world receding from me in every direction, and you could have used my anger to weld steel.’
But when Nadia’s diagnosis finally came, she felt her world had slotted back into place.
‘If these weird things are happening and you don’t know why, you think you’re going crazy,’ she says. ‘But the minute I got that diagnosis, my despair and sense of chaos were over.’
Nadia’s NHS consultant prescribed Aricept. There is no cure for Alzheimer’s but this drug has been found to slow and even reverse the disease.
‘Since I’ve been taking it, the symptoms have become far less noticeable. I feel more organised, and less forgetful,’ says Nadia. ‘But my vision is still getting worse.
‘I can write neatly but I can’t read back what I have written. So I have had to give up work. It’s rather sad, but what can I say? Vic does read to me, which is nice.
‘My family are very anxious to help and make things easier for me but at times it must irritate them. It’s definitely brought us closer. We feel that we’re all in this together. I try to live with what I do have, rather than with what I’ve lost.’
Dr Crutch says: ‘Dementia in those under 60 is rare, but not unheard of. About 60per cent of dementia is due to Alzheimer’s. Of those, five per cent – about 17,000 individuals – have the PCA type. At the moment the prognosis of someone with the condition is approximately ten to 12 years to live following the diagnosis.’
Alzheimer’s disease itself does not kill. But loss of motor control leads to trouble swallowing, which can cause those afflicted to inhale their food, causing pneumonia – which is the most common reason for death.
‘Those with PCA often have considerable delays in diagnosis, partly because the condition is quite rare and many health professionals do not recognise it, though this situation is improving.
‘The onset isn’t like typical Alzheimer’s disease, being characterised more by visual than memory problems. People notice difficulties with handwriting, spelling or numbers, some describe seeing washes of colour or having greater difficulty reading large rather than small print.
‘These symptoms can be mistaken for problems with the eyes. As a result, many patients with PCA are directed to opticians and ophthalmologists rather than neurologists.
‘We are working hard to try to increase awareness of the impact of PCA and other rare or early-onset forms of dementia, and have set up some support groups to provide people with information and advice.
‘Aricept offers symptomatic treatment, but it doesn’t cure the disease and is not effective for everybody.’
Nadia, though, is optimistic
‘On holiday with Vic in Botswana recently, every night I watched the sun set, and listened to the symphony of animal sounds echoing though the bush. It was truly wonderful.
‘I know I don’t have that many days left. But I’m enjoying life.’