Tag Archives: Terry Pratchett

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Meeting other people living with posterior cortical atrophy

Even The Man Who Mistook His Wife for a Hat, from Oliver Sacks’ famous book, has since been speculated to have suffered from posterior cortical atrophy (PCA).

A dementia is not a natural part of ageing.

Dementia is caused by diseases of the brain.

It’s not just about losing your memory.

It’s possible to live well with dementia.

There’s more to a person than the dementia.

Sue Stephen, one of the founding members of the Dementia Alliance International (DAI), spoke about how she came to be diagnosed with PCA.

This is one of the latest additions on their blog, which I strongly recommend.  The DAI is an unique group of individuals with a strong sense of solidarity, who live with one of the various types of dementia.

“I had been noticing myself for the last few years that I was having visual issues… very bad glaring.. when I tried to read..”

She put it down to stress, but she still went ‘to get [my] eyes tested.’

And this went on for a few years. Interestingly, she reports this delay in diagnosis as rather typical for many people finding a diagnosis of PCA.

Tests for dementia of the Alzheimer type, typically initially shown up by problems in short term learning and memory, were negative.

When she was eventually given a diagnosis, she was told, “That’s it. There’s nothing I can do. Here’s a prescription for Aricept. See you in six months.”

But she described being shocked, and she had no idea what PCA is/was.

“I lost all certainty in my life. How do I make decisions in my life?”

She then sat on her month doing ‘research’, but reported ‘three wonderful things’ which came out of her research.

She found a list of symptoms which matched what she had been experiencing. “That was really good”. This enabled her to see her GP who then went referred her onto more suitable specific tests, confirming the diagnosis.

That’s one of the slight paradoxes about the way we approach a fellow citizen of ours living with a dementia. Nobody wants to be defined by any medical conditions they live with. They do not wish (rightly) to consider themselves as a collection of diagnoses.

But it is often reported by people living with conditions how it can be both a blessing to know what might happen but also a burden to know something might happen, with this information resulting from a clinical diagnosis.

She then reported discovering the work of Sir Terry Pratchett, who is living well with PCA, still writing books.

A third thing which she reports is finding a group of people on Facebook of people with posterior cortical atrophy. It’s a closed group where people can share in private “scenarios”.

“I could see in the future what symptoms might be. I look at symptoms, and I think if this happened to me, what could I do now to help?”

Stephen reports sharing these stories really helped.

Terry Pratchett went to see other people living with posterior cortical atrophy in 2010, a form of dementia.

Pratchett is an example of an individual who has shown that it is possible to live well with dementia.

Pratchett admitted he was apprehensive about the meeting, as he said it was like ‘seeing into the future’.

One person in the meeting, “Anything coming through the postbox, I can’t read. Susan has to do everything from cutting my nails to putting my shoes on. This disease.. or whatever you call it, changes your perspective.”

“It didn’t happen – it evolved.”

Pratchett though admitted despite his fears he enjoyed the meeting.

“I can see why people enjoy meeting up”

He had dinner with Graham Doggett, who is an 70 year-old author, and academic who has written books about quantum chemistry, and his wife.

“Susan is the gardener. If I help her, I can never find the wretched branches in the air to prune. They disappear.”

“I was doing computing since 1959. That’s gone.”

“I used to play piano up to grade 7. Gone.”

“Reading, writing, gone gone”.

When the term ‘living well with dementia’, one of the main disclaimers on that is that the person living with one of the dementias will have their life before to compare their experience to.

And this is bound to impact upon one’s own personal perception of living with dementia. Unless you bother to find out about the past of a person, in relation to his environment, you will not be able to understand his or her world.

The symptoms of PCA can vary from one person to the next and can change as the condition progresses.

The most common symptoms are consistent with damage to the part of the brain at the back of the head, an area responsible for processing visual information.

Consistent with this process are slowly developing difficulties illustrating its slow and progressive nature due to a disease of the brain.

Now you’re even able to follow it using sophisticated ‘imaging’ of the brain.

PCA imaging

These difficulties include visual tasks such as reading a line of text, judging distances, and  distinguishing between moving objects and stationary objects.

Other symptoms include an inability to perceive more than one object at a time, disorientation, and difficulty maoneuvering, identifying, and using tools or common objects.

Some people can experience hallucinations.

Yet further symptoms can include difficulty performing mathematical calculations or spelling, and many people with PCA experience anxiety, possibly because they know something is wrong.

In the early stages of PCA, most people do not have markedly reduced memory, but memory can be affected in later stages.

This illustrates how this particular dementia is not just about memory.

Age of onset is typically 50-65, demonstrating why dementia is not simply about ageing.

Both Stephen and Pratchett demonstrate by a country mile while there are more to people than their dementias.

If you’d like to find more about dementia, you can do a short 45 minute session close to where you live in England (and now in other parts of the UK). The initiative is run by the Alzheimer’s Society, but is a Public Health England initiative. To find out more about this initiative called “Dementia Friends”, please go to their website.

There’s more to this initiative than the badge you’ll receive in the process, but this initiative is all about improving understanding in the general population about the dementias. I hope you will wish to learn more.

People with PCA are are at risk of being let down by ‘traditional dementia support groups’.

That is partly because they are younger and face different challenges, and partly because the activities offered in day centres  puzzles, large-font books—are visual and thus actively unhelpful for people with PCA.

PCA is not new to the research community.

Already in 1902, the Czech psychiatrist Arnold Pick mentioned a woman’s inability to see and grab a lit candle held in front of her face in his essay “Ueber eine eigenthuemliche sehstoerung senile dementer,” Jahrbuecher f. Psychiatrie u. Neurol., see excerpt.

Several attempts at defining criteria for diagnosis exist.

The neurologist Frank Benson at the University of California, Los Angeles, coined the term while describing the pattern of symptoms in five patients (Benson et al., 1988).

Later, Mario Mendez, also of University of California and San Francisco proposed clinical diagnostic criteria arguing that PCA is its own syndrome, not simply Alzheimer’s disease with visual symptoms (Mendez et al., 2002).

The English dementia strategy is mindful of the needs to develop communities supportive of the needs of people living with PCA, of the need for high quality research research into PCA, and to think how people living with PCA can be best supported professionally and personally.

It’s not only people with posterior cortical atrophy who experience problems with vision, however. This is very important to be aware of.

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Meet Norman and Terry: two people living with a dementia in different ways

“Dementia is not just about sitting in a bathroom all day, staring at the walls.”

So speaks Norman McNamara in his recent BBC Devon interview this week.

Norman McNamara

This may seem like a silly thing to say, but the perception of some of “people living  with dementia” can be engulfed with huge assumptions and immense negativity.

The concept of ‘living well with dementia’ has therefore threatened some people’s framing of a person who happens to have one of the hundred or so diagnoses with dementia.

It’s possible memory might not be massively involved for someone who has been diagnosed with a dementia.

Or as “Dementia Friends” put it, “Dementia is not just about memory loss.”

Norman McNamara and Sir Terry Pratchett are people who are testament to this.

“If you made a mistake, would you laugh it off to yourself and say ‘Ha, ha, maybe it’s because I have dementia.””

If somebody else made a mistake, would you laugh at that person and say ‘Ha, ha, maybe it’s because you have dementia.” Definitely not.

There are about a hundred different underlying causes of dementia.

“Dementia” is as helpful a word as “cancer”, embracing a number of different conditions tending to affect different people of different ages, with some similarities in each condition which part of the brain tend to be affected.

These parts of the brain, tending to be affected, means it can be predicted what a person with a medical type of dementia might experience at some stage.

This can be helpful in that the emergence of such symptoms don’t come as much of a shock to the people living with them.

Elaine, his wife, noticed Norman was doing “weird and wonderful things”.

Norman says “my spatial awareness was awful”, and “I was stumbling and falling”.

Norman, furthermore, was putting “red hot tea in the fridge”, and “shower gel, instead of toothpaste, in [my] mouth”.

Dementia with Lewy bodies (DLB) is a type of dementia that shares symptoms with both Alzheimer’s disease and Parkinson’s disease. It may account for around 10 per cent of all cases of dementia. It is not a rare condition.

It is thought to affect an estimated 1.3 million individuals and their families in the United States.

Problems in recognising 3-D objects, “agnosia”, can happen.

Lewy bodies, named after the doctor who first identified them, are tiny deposits of protein in nerve cells.

See for example this report in this literature.

“Night terrors” have long been recognised in diffuse lewy Body disease.

“The hallucinations are terrific”

The core features tend to be fluctuating levels of ability to think successfully, with pronounced variations in attention and alertness and recurrent complex visual hallucinations, typically well formed and detailed.  

See for example this account.

For Norman, it was ‘prevalent in his family’.

Other than age, there are few risk factors (medical, lifestyle or environmental) which are known to increase a person’s chances of developing DLB.

Most people who develop DLB have no clear family history of the disease. A few families do seem to have genetic mutations which are linked to inherited Lewy body disease, but these mutations are very rare.

Monogenetic forms of Lewy body disorders, where a patient inherited the disease from one parent, are rare and comprise about 10% of cases. These familial variants are more common in persons with an earlier age of disease onset.

The patterns of blood flow can help to confirm an underlying diagnosis (see this helpful review).

Also, in this particular ‘type of dementia’, it can be helpful for medical physicians to avoid certain medications (which people with this condition can do very badly with). So therefore while personhood is important here an understanding of medicine is also helpful in avoiding doing harm to a person living with dementia.

However, Norman has been tirelessly campaigning: he, for example, describes how hundreds of businesses in the Torbay-area of Devon have signed up for ‘dementia awareness.”

And, as Norman says, “When you’ve met one person with dementia, you’ve met one person with dementia.”

Sir Terry Pratchett is another person living with dementia.

Sir Terry Pratchett described on Tuesday 13th May 2014 the following phenomenon bhe had noticed:

“That nagging voice in their head willing them to understand the difference between a 5p piece and £1 and yet their brain refusing to help them. Or they might lose patience with friends or family, struggling to follow conversations.”

coins

“Astereognosis” is a feature of ‘posterior cortical atrophy’ (“PCA”).

A good review on the condition of PCA is here.

Sir Terry Pratchett has written a personal reflection on society’s response to dementia and his own experience of Alzheimer’s to launch a new blog for Alzheimer’s Research UK: http://www.dementiablog.org

Sir Terry became a patron of Alzheimer’s Research UK in 2008, shortly after announcing his diagnosis with posterior cortical atrophy, a rare variant of Alzheimer’s disease affecting vision.

He went on to make a personal donation of $1 million to the charity, and has subsequently campaigned for greater research funding, including delivering a major petition to No.10 and countless media appearances.

Terry Pratchett Dementia Friends campaign

In his inaugural post for the blog, Sir Terry Pratchett writes: “There isn’t one kind of dementia. There aren’t a dozen kinds. There are hundreds of thousands. Each person who lives with one of these diseases will be affected in uniquely destructive ways. I, for one, am the only person suffering from Terry Pratchett’s posterior cortical atrophy which, for some unknown reason, still leaves me able to write – with the help of my computer and friend – bestselling novels.”

Posterior cortical atrophy (PCA) refers to gradual and progressive degeneration of the outer layer of the brain (the cortex) in the part of the brain located in the back of the head (posterior).

The symptoms of PCA can vary from one person to the next and can change as the condition progresses. The most common symptoms are consistent with damage to the posterior cortex of the brain, an area responsible for processing visual information.

Consistent with this neurological damage are slowly developing difficulties with visual tasks such as reading a line of text, judging distances, and distinguishing between moving objects and stationary objects.

Other issues might be an inability to perceive more than one object at a time, disorientation, and difficulty maneuvering, identifying, and using tools or common objects.

Some persons experience difficulty performing mathematical calculations or spelling, and many people with PCA experience anxiety, possibly because they know something is wrong. In the early stages of PCA, most people do not have markedly reduced memory, but memory can be affected in later stages.

Astereognosis (or tactile agnosia if only one hand is affected) is the inability to identify an object by active touch of the hands without other sensory input.

An individual with astereognosis is unable to identify objects by handling them, despite intact sensation. With the absence of vision (i.e. eyes closed), an individual with astereognosis is unable to identify what is placed in their hand. As opposed to agnosia, when the object is observed visually, one should be able to successfully identify the object.

Living well with dementia means different things to different people.

Pratchett further writes:

“For me, living with posterior cortical atrophy began when I noticed the precision of my touch-typing getting progressively worse and my spelling starting to slip. For an author, what could be worse? And so I sought help, and will always be the loud and proud type to speak my mind and admit I’m having trouble. But there are many people with dementia too worried about failing with simple tasks in public to even step out of the house. I believe this is because simple displays of kindness often elude the best of us in these manic modern days of ours.”

As we better understand what dementia is, our response as a society can be more sophisticated. I’ve found one of the most potent factors for encouraging stigma and discrimination is in fact total ignorance.

Both Norman and Terry demonstrate wonderfully: it’s not what a person cannot do, it’s what they CAN DO, that counts.

This is ‘degree level’ “Dementia Friends” stuff, but I hope you found it interesting.

 

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