by Craig Cabell
Pratchett thought he had experienced a mild stroke after having a brain scan. Two or three years earlier he had noticed that his typing skills had diminished slightly. At first he put it down to the onset of old age, but he decided to have some tests to make sure. Initially he passed a mental test, answering such basic questions as ‘what day is it?’ But after reassessment he was diagnosed in December 2007 with a rare form of Alzheimer’s known as Posterior Cortical Atrophy (PCA). Pratchett describes the symptoms as a shrinkage of the back of the brain. He was quick to tell his publisher and many fans about his illness.
According to the Alzheimer’s Society, PCA is a progressive degenerative condition where damage to brain cells is particularly focused at the back of the brain. The death of brain cells is accompanied by accumulation of toxic proteins, amyloid and tau, that form plaques and tangles in the same way as in typical Alzheimer’s disease. PCA is a rare condition, although it is noted that it has gone unnoticed in the past.
Typical Alzheimer’s disease is most commonly associated with deterioration in memory, followed by a gradual progressive decline in other cognitive functions including language, calculation, planning and perceptual skills. In contrast, individuals with PCA initially tend to have well-preserved memory but instead show a problem with vision due to the loss of the motor effects of the eye, and experience difficulties performing skilled movements and literacy skills.
The duration of the PCA condition is poorly understood. Some people live approximately the same length of time as individuals with typical Alzheimer’s (on average ten to 12 years following the onset of symptoms), while others live longer. Pratchett says that if you’re going to get Alzheimer’s, his version – PCA – is the one to get, and he has not been outwardly depressed about it to his fans or in the media.
The author broke the news of his illness to his fans in ‘Discworld News’, available through Pratchett’s websites, in the following way: ‘Folks, I would have liked to keep this one quiet for a little while, but because of upcoming conventions and of course the need to keep my publisher informed, it seems to me unfair to withhold the news.
‘I have been diagnosed with a very rare form of early onset Alzheimer’s…’
Pratchett was upfront and honest about the disease to all those around him. When asked why he had decided to tell everyone, he replied: ‘Why not? I haven’t done anything wrong.’
On 13 June 2008 he appeared on Frost Over the World, when he told David Frost about his condition and how philosophical he was about it. He explained that he would carry on writing for as long as he could. As he wrote in ‘Discworld News’: ‘All other things being equal, I expect to meet most current and, as far as possible, future commitments but will discuss things with various organisers.
‘Frankly, I would prefer it if people kept things cheerful, because I think there’s time for at least a few more books yet.’
After making a substantial donation to Alzheimer’s Research UK in March 2008, $1 million (significantly more than £500,000), he became their patron, saying: ‘I think it’s something we can beat.’
These were bold words, but it wasn’t Pratchett’s only remit for becoming patron, as he explained: ‘We should be aware that it is an illness, it’s not some visitation from heaven… It’s not something to be ashamed of. [You haven’t got it] because you’ve done something wrong.’
Making people aware of the disease, taking away the stigma, is also part of Pratchett’s quest. He explained that research was going in the right direction and that promising results were not too far away, but would a breakthrough occur in time to help him? He was – and is – optimistic. At the age of 60 Pratchett told the media that he was ‘trying to be philosophical’ about the disease and hoped that a means of combating his form of it could be found.
It was Pratchett’s father who always told him to be philosophical about things, and the author has tried to do that, but he finds it very hard to do in the wee small hours of the morning when one feels at one’s most vulnerable.
Between 2008 and 2010, the general public has seen a significant change in Pratchett’s ability to speak in public. When he delivered his speech when he donated his $1 million, he coped well with notes in front of him, but two years later at Trinity College, Dublin, he found it too difficult and somebody else read out his speech, while he passed comment from the sidelines.
For a creative man like Pratchett, his illness can be seen on the computer screen in front of him. For somebody who had always enjoyed the process of writing from a very young age and who had studied to be a professional journalist, it is a terrible and life-changing frustration, but one he has faced with courage and determination, so this chapter is not a downbeat one.
Pratchett has kept fans and fellow sufferers – and their families – informed about the way in which the disease has been affecting him. His transparency on the subject is terrifying and heart-warming at the same time, but one cannot fail to be in awe of the man and the steps he has taken to make the general public more aware of this disease and its effects. He has explained to audiences that he could spell a difficult word but then forget how to spell an easy one. He would give an assured interview, but if he took his pullover off he might not be able to put it back on again. At Trinity College he made the audience laugh about a similar predicament with his Y-fronts. ‘You need to know about my pants!’ he declared, tackling a dreadful subject with humour.
It was while writing Unseen Academicals that Pratchett finally gave up the keyboard and used voice recognition software to dictate his books. His book Nation had recently done extremely well in the book charts and at the hands of the critics, and the constant encouragement of the fans was inspiring. Indeed, when he made his donation to Alzheimer’s Research UK, an internet challenge was released called ‘Match it for Pratchett’, where an equal amount would be sought from fans as a donation to the cause. How can one be down with this type of support?
The Match it for Pratchett website encouraged people to dress as orangutans and swing from trees, and spread the word of the good cause on Facebook, Twitter and blogs – anything that could generate much-needed funds for the charity. The response was overwhelming, and still is. T-shirts were printed and books sold on eBay, among many other fundraising activities. It is not just the money raised that deserves praise; it’s the humour of millions of fans and their dedication to Pratchett’s cause and the way they embrace his interests.
‘You couldn’t say: It’s not my fault. You couldn’t say: It’s not my responsibility.
You could say: I will deal with this.’
(A Hat Full of Sky)
CHAPTER EIGHTEEN
The Dark Red Wings of Misery
‘When I was a journalist, people would die “after a long illness”, and that illness was cancer. But when people started to call cancer by its real name, research began in real earnest, so I thought, I’m not going to lie in a hole somewhere… I hope to make quite a lot of noise.’
Terry Pratchett
On 17 April 2011, The Sunday Times ran an article about the actor Sir Patrick Stewart and his support of Dignity in Dying, ‘an organisation that wants to change the law to allow people of sound mind to call it quits on life before the full horror of a terminal disease sets in’.
Although an extremely fit 70-year-old who embarks on a 40-minute run every day, Stewart became aware of the possibility of debilitating or terminal illness after being diagnosed with coronary heart disease five years previously. However, in 2009 he joined Dignity in Dying very quietly. It took them a few months to realise that this was Sir Patrick Stewart the Shakespearean actor and former captain of the USS Enterprise (Star Trek: The Next Generation), at which point they asked him to be a patron, along with Sir Terry Pratchett.
Pratchett was mentioned in the Sunday Times article and also took part in a BBC documentary, Terry Pratchett: Choosing to Die, which was screened on 13 June 2011. In it he visited a man called Peter who has chosen to die at Dignita
s, an assisted-death clinic near Zurich. The documentary was slammed by charity Care Not Killing, which believed that the documentary may have shown the ‘benefits of assisted death with very little redress’.
Even setting aside religious beliefs, there is strong opinion for and against assisted death, with people differentiating between those who have a terminal illness and those who have a debilitating illness. Pratchett put his views over very clearly during the programme, speaking directly to the camera away from the main film and the two people documented in it and their decisions to die at Dignitas. The second man, Andrew, was only 42 years old but had multiple sclerosis and had tried to commit suicide before going to Dignitas. Here was a man who had really made the decision to end his own life, despite the anxiety it would cause his mother.
I can only speak personally about the programme, because the debate about assisted death is far too big for this book. One could read the title – Terry Pratchett: Choosing to Die – as Sir Terry contemplating taking his own life because of his illness, and I think that that is partially true. He was facing his own demons and drawing his own conclusions, and the painful thing is that I believe he wasn’t happy with the final conclusion. He said he would like to die out in the sunshine. He qualified this by saying that sometimes the sun shone in Switzerland, but this wasn’t what his heart was telling him. It was telling him that he should be able to make the decision one fine English day, walk out into his beautiful garden and die with dignity.
Very early on in the documentary Pratchett asks: ‘Is it possible for someone like me – or someone like you – to choose the way we die?’To me, there is a longing to know the answer to this question. I believe that he didn’t like Dignitas. In his heart he didn’t want to choose to die there, and the reason for that – again my personal belief – is that he believes he can change the law in Britain to help assisted death.
Another important part of the documentary, and probably the most personal part, was when Pratchett went to Belgium to meet the widow of the author Hugo Claus. She explained to him that her late husband had wanted to finish a book before taking his own life, but unfortunately he lacked the skill to do so because of his Alzheimer’s. He had requested death by euthanasia, which is legal in Belgium, and chose death while drinking champagne and smoking cigarettes.
‘Not being able to dictate any more, not being able to write any more. Not being able to communicate any more…’
Terry Pratchett (Terry Pratchett: Choosing to Die)
Directly after Pratchett’s programme, Jeremy Paxman chaired a BBC Newsnight debate about the many different reasons for and against assisted death. The subject was way too vast for a 30-minute programme, and just as the debate started to heat up it had to be brought to an end. The Bishop of Exeter was concerned about the vulnerable, while the stance of the group Right to Die was a clear push towards a policy that would allow the terminally ill to die through assisted death. In a filmed interview shown throughout the programme, Pratchett told Paxman that he believed that it should be possible for somebody stricken with a fatal illness to choose, with medical help, assisted death.
Two years previously, in October 2008, Daniel James, a 23-year-old English rugby player who had been paralysed while playing his beloved sport, had travelled to Switzerland to take his own life. His parents accompanied him to Dignitas and told the press that their son had a ‘right to die’. No charges were brought against them. Two months later, in December 2008, the Sky Real Lives channel had made front-page news by broadcasting footage of the assisted death of US citizen Craig Ewart at Dignitas. On 25 January 2009, Julie Walters starred in a BBC1 drama called A Short Stay in Switzerland. Its theme was assisted death, and the script centred around a doctor (Walters) who knows she faces a traumatic death and decides to take matters into her own hands. The programme was extremely moving and showcased a very important and controversial topic.*
‘People didn’t talk much about that sort of thing in those days. Suicide was against the law. Johnny had wondered why. It meant that if you missed, or the gas ran out, or the rope broke, you could get locked up in prison to show you that life was really very jolly and thoroughly worth living.’
(Johnny and the Dead)
The drama was based on the true story of Anne Turner, a retired English doctor who took her own life in 2006 at Dignitas because she no longer could endure progressive supranuclear palsy, an incurable degenerative disease.
With television making people aware of the facts of assisted deaths (not real-life euthanasia cases) and respected celebrities – indeed knights of the realm – openly defending the need for assisted suicides, the stigma began to break down around the issue, just as it had years before when people suddenly needed to utter the word ‘cancer’ and take issue against it. ‘As every true wizard knows,’ Pratchett explains, ‘once you name something, you begin to beat it.’ And there we see the reality inside Pratchett’s fantasy world coming through.
In 2011 Sir Terry Pratchett and Sir Patrick Stewart continued to be at the forefront of breaking down and analysing the need for assisted deaths. Their actions and words continued to make the public aware of the grim possibilities in their own future and encourage them to discuss those possibilities openly. ‘The bug-bear is the term “suicide”,’ Pratchett says. ‘It’s the woman on the viaduct… [and] that is a world away from someone discussing with their family that their life should end at a certain point… with the help of a doctor.’ Bug-bear or stigma? It’s the understanding of the dilemma people find themselves in, the hopelessness. How many people have witnessed the last days of a loved one begging to die?
Pratchett believes that assisted death is a ‘sensible’ option if you are faced with a terrible illness. He discussed the deaths of his father and grandfather, 86 and 84 respectively, from cancer, and said that 90 is no longer an exceptional age to live to. He is resigned to the fact that his Alzheimer’s has surfaced early, but still wishes to live as long as his father and grandfather did. There is scientific evidence to suggest that he could, but if the Alzheimer’s becomes too much he would want to opt for the dignity of human life over the sanctity of human life, and surely that should be his decision.
‘I have no fear of death whatsoever. I suspect that few do, what they all fear is what might happen in the years or months before death.’
Terry Pratchett (‘Book World’, The Washington Post, 1 October 2008)
This chapter and its predecessor have tackled two separate subjects, Alzheimer’s disease and assisted deaths. Although separate subjects, one has led to the other as far as Pratchett is concerned. He has spoken openly on both issues, sharing his views and discussing what the government has done, or rather hasn’t done, to support sufferers. He explains that there are as many sufferers from Alzheimer’s as there are from cancer in the UK, but only a fraction of the money is put into Alzheimer’s research. ‘You hear of people surviving cancer,’ Pratchett says, ‘but I’ve never heard of anyone surviving Alzheimer’s.’
Alzheimer’s and assisted death are poignant subjects for Pratchett. He is a humanist and so, although he doesn’t believe in God, he does believe in some kind of order to the universe, as hinted at in his science fiction novels. For Pratchett, this rubbishes the idea that one cannot take one’s own life, because to him life is not a blessing from God. He explains that there is no solace for him in that idea, which one could argue violates his own human rights, or at the very least gives him no opportunity to exercise his own solution to his incurable illness when the plight becomes too much for him. This is just an extra frustration for the writer to endure.
So how does he deal with that? He explained that when he was diagnosed he felt quite alone medically. He then asked – through his website – for top brain surgeons and neurologists to approach him, and they did. These were people who had grown up reading his books and still enjoyed them, and now they were people at the top of their profession. Pratchett could use them as a sounding board for the jumbled ma
ss of ideas and perceptions he had heard about Alzheimer’s.
For example, it was said that mercury amalgam fillings in his teeth could have been a cause of the disease. He put this suggestion to the body of professionals – whom he nicknamed the Greek Chorus – but they were unconvinced. However, they did say that replacing all those fillings wouldn’t do any harm, so Pratchett spent approximately £3,000 and had them all replaced. Afterwards he asked for two that he could make into cufflinks, but was told that they had to be disposed of as hazardous waste. He marvelled at the idea that he could walk into the dentist with them in his mouth but couldn’t walk out again with them in his hand. He did offer to put them in his mouth and walk out the door, but the dentist was having none of that!
We conclude that Pratchett has a disease that is likely to kill him, that he cannot take his own life when the pain and suffering become too much, and the reason for that is the beautiful notion that God created life and we shouldn’t take that away from ourselves. But if we did conclude that, we would know nothing about the spirit of Terry Pratchett – his optimism, his humour, his strength and his fight to make a difference.
Pratchett has used his imagination to make himself very rich and very famous, but he is asking people across the world (not just in the UK), through the worthy societies he now backs, to use their own imaginations (and apply some lateral thought) and change the way we deal with terminal illnesses. He is also asking the politicians to tackle radical subjects in a brave and pragmatic way – a shame, then, that as a rule politicians are not that brave and have very little imagination. But Pratchett will continue to break down the stigmas of Alzheimer’s and assisted death, and educate people in order to change public perception. Once the public understands more, then more pressure can be applied to the powers that be to change policy and do something for terminally ill people.