This is a good article in the Daily Mail (London) about a man struggling with progressive supranuclear palsy.
The PSP Association’s chief executive is quoted as follows:
“PSP is a devastating neurological terminal illness, which first steals a person’s balance, then progressively robs them of their ability to walk, talk, eat, drink and see, yet they remain mentally alert. It can best be described as a living hell. Most people have never heard of this illness, and don’t recognise its symptoms – even among the medical profession. In its early stages, a lot of people feel lethargic and down, and may wrongly be given antidepressants. These may help some patients, but the side-effects may make others feel worse. In one in three cases of misdiagnosis, people are told they have Parkinson’s Disease and given drugs which do not help and have unpleasant side-effects such as hallucinations.”
‘When I kept falling over, my wife said I was drunk’: One man’s battle with the disease that killed Dudley Moore
By Lesley Gibson for MailOnline
UPDATED: 21:55 EST, 18 August 2008
On a good day, Ray Nind falls five times. In the past year, he’s been rushed to hospital six times to be treated for the resulting injuries – usually cuts to the back of his head that need stitches.
‘When I first began falling, people thought I was drunk,’ says Ray, 56. ‘Even my wife thought I had been drinking. She kept asking if I’d “had a few” on the quiet, but I honestly hadn’t. I didn’t know why I kept falling. Now, fortunately, everyone who knows me is aware I fall because I’m ill.’
Until recently, Ray, who lives in Ilford, Essex, ran a building business, employed more than 20 staff and drove a three-litre Jaguar. ‘Now I just watch TV all day,’ he says. ‘It’s all I can do.’
His wife Jenny, 54, worked alongside him looking after the secretarial side of the business, but is now his full-time carer.
Ray has a brain disease called Progressive Supranuclear Palsy (PSP) for which there is no treatment or cure. One in three people is predisposed to it because of a genetic fault.
While 4,000 Britons are known to be living with PSP, experts believe the real number is nearer 10,000.
The condition affects those over 40, and equal numbers of men and women.
Daily Mail diarist Nigel Dempster died from it in July 2007, aged 65. Comedian Dudley Moore also died from the condition, in March 2002, aged 66.
The cause is unknown – blows to the head and environmental toxins are possible triggers – but the mechanism is understood.
Basically, cells in the brain produce too much of a certain protein, called tau; this causes neurons (nerve cells) to die. Messages can no longer be sent from the brain to certain muscle groups, especially those affecting the legs, speech, swallowing and vision.
The part of the brain that controls mood and personality may also be affected, leading to patients becoming unusually depressed, withdrawn and apathetic, or aggressive and demanding. The condition does not affect intelligence or memory.
A cruel and unknown disease
PSP is a cruel disease – life expectancy is five to eight years from onset. Although there is no cure or treatment for the condition, drugs can be given to alleviate certain symptoms in some patients.
The PSP Association is battling to make both the public and the medical profession more aware of this devastating illness because without early and accurate diagnosis, patients may be given inappropriate drugs and cannot get the information and support they need.
Chief executive Jane Hardy says: ‘PSP is a devastating neurological terminal illness, which first steals a person’s balance, then progressively robs them of their ability to walk, talk, eat, drink and see, yet they remain mentally alert. It can best be described as a living hell.
‘Most people have never heard of this illness, and don’t recognise its symptoms – even among the medical profession. In its early stages, a lot of people feel lethargic and down, and may wrongly be given antidepressants. These may help some patients, but the side-effects may make others feel worse.
‘In one in three cases of misdiagnosis, people are told they have Parkinson’s Disease and given drugs which do not help and have unpleasant side-effects such as hallucinations.’
It took ‘only’ 18 months for Ray to receive his diagnosis – shorter than the average diagnosis time of four years. But, initially, his private neurologist put his symptoms down to a stroke.
When Ray’s symptoms worsened, Jenny kept saying it couldn’t be a stroke, but her husband insisted that ‘consultants are supposed to know best’.
In fact, it had been Jenny who first realised something was wrong, in 2001 when Ray started tripping over kerbs, and steps, for no apparent reason.
‘His elderly father had died just before that and I thought he’d been drinking to numb his grief – yet he insisted he hadn’t,’ she recalls.
But it was the change in his personality that alarmed her most. Normally very sociable, Ray wanted to spend more and more time on his own and lost interest in going out.
‘All he wanted to do was watch TV all day,’ says Jenny. ‘Even his choice of programmes had changed. He started watching rubbish, such as low-life chat shows. He also became unusually irritable.’
Three years ago, she woke up at 5.30am to find Ray had disappeared, taking the car. ‘I was so worried – this just wasn’t the kind of thing he did. When he hadn’t returned at 6am I called him at work.
‘He answered and explained he’d got up at 2.30am and, not realising what time it was, had set off for work. The police had stopped him and accused him of driving erratically. Convinced he’d been drinking, they’d taken him back to the cells, where he’d spent two hours before they released him and he went in to work.’
It was only when Ray’s colleagues voiced their concerns that he consulted a neurologist in April 2005. The doctor agreed something was wrong but was perplexed because brain scans showed no abnormalities.
This is not unusual – PSP cannot be detected on a brain scan. Even when, a few months later, Ray developed slight paralysis and began slurring his speech, it was blamed on a stroke.
Ray was still working part-time, but his business was failing, partly because he was ill and could not give it the attention it needed. His falls were now occurring on a daily basis, too.
‘He’d fall getting out of the car, or opening the boot – always backwards or sideways,’ remembers Jenny. ‘His eyes were also different, as if he were looking up at the ceiling instead of straight at me.’
Ray’s illness was causing his body increasingly serious damage. He fell down some stairs and fractured his vertebra. He scalded himself trying to fill a hot water bottle to ease the pain and needed specialist burns treatment.
In September 2006, after he had fallen on the living room coffee table so badly he needed several stitches, the couple refused to believe the stroke theory any longer.
At their nearest hospital in Romford, Essex, an NHS neurologist examined the numerous MRI scans and notes from the previous few months. He asked Ray to look up and down, count his fingers, walk in a straight line, and questioned him on his physical capabilities.
As PSP does not show up on scans, specialists use symptoms such as regular backward falls and an upward gaze to check for the illness. Finally, five years after the initial symptoms and 18 months after first seeing a doctor, Ray was correctly diagnosed.
Ray recalls: ‘We’d never heard of PSP so Jenny looked it up on the internet when we got home. As we read the information, all my symptoms suddenly made sense. The falling backwards or sideways, slurred speech, changes in personality and difficulty gripping and swallowing were all listed.’
But Ray also read that the illness would worsen, until he became bed-bound and unable to talk. Because of difficulty swallowing, aspirational pneumonia, when food literally goes down the wrong way, is a real problem; it can cause infection in the lungs, which is often fatal.
Ray says: ‘My first thought was: “I’m going to die much sooner than I want to.” Aside from that, I was too shocked and numb to feel anything else. For weeks, we dressed, ate and went about our daily business like zombies. Gradually, we accepted the news.’
With inspiring courage and stoicism, Ray says he has come to terms with the fact he is dying. His mother died in 1994 from motor neurone disease – a neurological disorder with similarities to PSP. But there is not believed to be a genetic link.
‘Jenny and I both realise there’s no point in being in denial,’ he says. ‘There’s no point in saying I’ll get better, because I won’t. So we’ve faced facts. We know I will die, but we try not to think about it.’
However, it isn’t easy. Ray aches all over because the muscles he cannot use properly cramp. His eyes are dry and sore because he cannot blink, and he has double vision because his eye muscles do not get messages from the brain.
With his slurred speech, saying more than a few words is tiring. He struggles to grip his cutlery and needs a wheelchair for outings. ‘It’s very annoying being unable to do anything for myself,’ he says.
There are times when Ray forgets how incapable he is, and gets up to do something, only to fall and hurt himself. Six weeks ago, he cut his head again after going out unsupervised in his electric wheelchair and falling out.
For Jenny, of course, caring for Ray is a heartrending job. The outgoing, independent man she married 28 years ago must be dressed, washed and fed.
Yet she is incredibly chatty, cheerful and doggedly determined to see the bright side in everything, even if she is despairing inside. ‘We haven’t been to casualty yet this year, and that’s great,’ she jokes.
Although there is no treatment for PSP, Ray takes anti-depressants and sleeping tablets. The couple also have the support of a multidisciplinary team arranged by their GP, including an occupational therapist who has provided them with disability aids, a speech and language therapist to help with communication, and care assistants to help Jenny.
The couple have planned for the future as much as they can. ‘We’ve set up wills and cleared our debts, apart from the mortgage,’ says Jenny. ‘And thanks to the GP, we have the support we need. I just hope that our story is a lesson to other people who might be affected by PSP.
‘We want to make people aware of this illness so they don’t go through what we did. We spent months struggling without any help or support because Ray was misdiagnosed.
‘Although this disease is incurable, knowing what was wrong with Ray was such a relief. It meant we could make sense of his symptoms and get some help.’
For Ray, it’s simply a case of trying to stay strong for as long as he can. ‘It’s very frustrating. I used to be proud to be the one that looked after the family, and now there’s been a complete role reversal. I’m an old-fashioned man and it’s been hard to accept that, but I’ve had to.’