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An ailing brain with imagination undimmed

Pratchett
Terry Pratchett at the Discworld premiere

Last year Terry Pratchett, the bestselling author of the Discworld fantasies, was diagnosed with Alzheimer's. How does the writer cope with a disease that whittles away at his brain?

When writer Terry Pratchett was told he had Alzheimer's disease, his first thought was "that's a bit of a bugger". That, and "I hope they hurry up and find a cure quick."

In December 2007, at the age of 59, the Discworld author was diagnosed with a rare early-onset form of the disease called Posterior Cortical Atrophy, or PCA.

It was last summer when he first started to suspect all was not well, and went to see his doctor. Given a brain scan and a Mini Mental State Examination (MMSE), a brief 30-point questionnaire commonly used by medics to screen for dementia, he was told that all was well. "I passed the test - it's actually quite hard to fail I think."

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But as time wore on, he remained convinced that all was not well.

"We had what I called a Clapham Junction day, when you know the phones were ringing. There were lots of things to do and I was just kind of flat-lining almost. I just couldn't deal with it and I thought 'there's more, there's more'."

He was referred to Addenbrooke's Hospital in Cambridge, where the diagnosis was finally made.

Firing on all cylinders

In the most common form of Alzheimer's, the main symptom is loss of memory, but PCA affects the back of the brain and so it is motor skills and vision which are hardest hit.

Terry Pratchett
Still thinking and speaking coherently, Pratchett's typing has gone downhill

"It's unusual because people deal with me and they refuse to believe I have Alzheimer's because at the moment I can speak very coherently, I can plot a novel," Pratchett says. "I type badly - if it wasn't for my loss of typing ability, I might doubt the fact that I have Alzheimer's.

"It's now hunt and peck, and there will be a moment sometimes when the letter A just totally vanishes and I don't quite know what happens.

"It's as if the keyboard closes up and the letter A is not there anymore. Then I'll blink a few times and then the letter A comes back."

But though his typing has slowed, the good news for his many fans is that his imagination remains undimmed, "going on at the same old rate".

It is this imagination that conjured up Discworld - a parallel universe filled with magical characters who live on a flat world sitting on the backs of four elephants, who are in turn standing on the shell of a giant turtle.

He has sold more than 55 million books worldwide and his work has been translated into 33 languages. But life as an in-demand public figure posed specific problems when it came to diagnosing his condition.

"The basic test, for example, will ask you questions like what day of the week is it, what is the date? I have a PA so there are really only two types of day - is my PA in or is my PA out? What day of the week is it? Well if he's not here then it's probably the weekend."

Rock hyrax
A rock hyrax is not the first animal many would think to list

And as a writer of fantasy fiction, and therefore a keen observer of the outlandish, this further complicated matters for those seeking to make a diagnosis.

"One of the questions was how many animals can you name.

"And I said 'let me think... There's the rock hyrax which is the closest living relative to the elephant, and then there's the thylocene which is the possibly extinct Tasmanian werewolf. How many more would you like me to name?'"

Best money can buy

But his status as the UK's biggest selling author after JK Rowling does bring huge benefits. He has the money to pay for facilities and treatment - particularly the drug Aricept, without which he says he can't function properly.

I sent out a message saying 'it's only worth asking me if you can help if you're high-end brain specialist'

"My wife said 'you would pick up the shirt and look at it as if you were looking at some kind of new thing that you had to wear. And now you just put it on and just do the buttons up and you don't think about it'."

And continuing to write keeps his ailing brain in good condition, as does walking, gardening, watching films and playing computer games.

"One piece of advice I was given by a specialist was 'surround yourself with toys'. He didn't mean buy stuff, but try to make your life as interesting as possible keep yourself active, keep yourself interested."

Once news of his illness was announced, another great source of advice were his numerous readers, whom he refers to as "the Greek Chorus".

"My fans across the world started to contact me, and in fact the website fell over and it was just getting ridiculous. So I sent out a message saying 'it's only worth asking me if you can help if you're a high-end brain specialist'.

"About a dozen or more contacted me saying 'I don't know about high-end but I'm head of the department of whatever at so-and-so university'."

While he has yet to be slipped some secret healing potion, what these medical boffins have done is offer expert opinions on the research going on behind closed doors.

"Something like a cure I suspect is in the pipeline. But I think it's a fairly long pipe, so I don't think it will be in time for me. One lives in hope."

Pratchett admits that his disease does pose immense challenges. "You get dark days. It's one step beyond can't find your mobile phone, can't find your car keys."

But although he knows his is a degenerative condition for which at present there is no cure, he refuses to be defeated.

"In addition to his bladder, his hairstyle and his sense of humour, my father willed to me his stoicism," Pratchett says. His father died of cancer aged 86, and remained active throughout.

"If there's nothing you can do about something, then don't do it. You have to learn to program your life around the fact."


Below is a selection of your comments.

What an inspiration he is to all people with long term illnesses.
Kim, London

We have all of Terry Pratchett's Discworld books, together with the Tiffany Aching series and just love them so much. He is the only author whose writing is capable of making me giggle uncontrollably, which can be slightly funny in the coffee shop! Reading this article and having an advanced techie for a husband, I wonder if Terry could learn to work with a voice activated computer and dictate his thoughts? Then he would not need to worry about typing. I love his imagination and truly pray for more witchy books - more Tiffany Aching, and more Guards books.
LibraGirl1, Malvern

Terry might try aerobic exercise in addition to walking since it increases blood flow and will help slow down its progression and improve memory. Having been close to a colleague's wife who succumbed to it over an extended period of time, it is worth the fight, and keeping abreast of new treatments.
Candace, New Jersey, US

My husband was diagnosed with early onset dementia at age 52 and, 5 years later, although his health is deteriorating, we still live a reasonably normal life. Every day we get up we discuss what we are going to do with the day and concentrate on that. We simply ignore what we can't do and plan ahead to give us both something to look forward to. That allows us to stay as positive as you can with this illness. We are also very fortunate that we have a superb consultant who literally leaves no stone unturned and took whatever steps were necessary to ensure that my husband has access to all the drugs currently available. That is no mean feat in today's NHS. The big drawback for us is that there is virtually no support by way of respite, even for a few hours once a week, to allow me some much needed "me time". That would be of immeasurable help for both of us as it would also enable my husband to have some variety of life away from me. A combination of this lack of respite and no proper drug treatment however would make an already bleak outlook completely desolate. We owe it to the increasing numbers of people who have this desperate illness to do everything we are capable of to help in every possible way.
Phredd, South Yorkshire

Pratchett is a brilliant writer who uses his humour and well-developed sense of morality to tell wonderful stories. I should hope he uses these talents to help publicise the continuing requirement for stem cell research funding. This research offers by far the best hope to find cures for degenerative brain illnesses like Alzheimer's.
Huw Evans, Brighton

It's critical for well-known people like Mr. Pratchett to publicly speak out in as many forms as possible about such afflictions. By doing so he is providing the best-palliative medicine to individuals and their caretakers with the dreaded A-disease. Simply, invaluable service that money can't buy.
Kamil Rustam, Houston, Texas


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