'Locked-in syndrome': is there any hope for sufferers?

Just over 14 months ago, Christine Simpson's horizons were wide. Her twin sons had grown up and were in good jobs, her husband Colin had taken early redundancy to spend more time at home, and they were planning to take a cruise to celebrate her 54th birthday.

Then, in the space of a few short minutes on 3 February last year, Christine's world fell apart. She suffered a massive stroke that has caused total paralysis and a condition called locked-in syndrome. Now, Christine cannot eat, breathe, speak or swallow, and is ventilated through a tube. Once an independent, healthy woman, she can communicate only by rolling her eyes.

Currently, locked-in syndrome (LIS) is incurable. Although there is little research about the life expectancy of sufferers, one study of 139 patients found that 83 had died within two years of being hit by the condition, and, of those, 91 per cent had died within the first six months, mainly because of respiratory problems. Jean-Dominique Bauby, a former editor-in-chief of French Elle magazine, developed LIS in 1995 and dictated a moving book called The Diving Bell and the Butterfly about his condition. He died a year later.

Colin describes the day that everything changed. "Chris called me and said she had a headache," he says. "When I got home she said she was feeling nauseous. She hadn't been to a doctor for years, but I asked our GP to come round. We were waiting for him but she was getting worse, so I called an ambulance. She had the stroke before it got here."

Christine was taken from her home in Old Harlow, Hertfordshire, to the nearby Princess Alexandra Hospital, where she was given an emergency tracheostomy. Because she was on a ventilator, it was a week before she could have an MRI scan to find out what had caused the strokes. They found that they had been triggered by a bleed to her brain stem, but what had caused the bleed was a mystery. Paralysed and unable to breathe, some doctors believed Christine was in a persistent vegetative state. But Colin became convinced that she was not.

Talking in front of his wheelchair-bound wife in a specialist neurological centre near Harlow, Colin cannot bring himself to give full details of the discussions he had with medical staff. "Until this happened I hadn't had much to do with the NHS and the medical world, he says. "But what angers me is the way they make judgements about quality of life even if they don't know the full facts. I kept being told she had no quality of life and that it wasn't going to get any better. But the doctors only came round during the day and what often happens with people in Christine's condition is that their body clocks get all messed up so they wake up at weird times.

"My sons and I stayed with Christine round the clock, and I saw that she seemed more alert at 3am. I was sure that she could hear and see me, so I started to say, 'Chris, if you can understand me, look down,' and she would. But when the doctors came round in the morning, they wouldn't believe me. They just said I was seeing what I wanted to see, that it couldn't be happening."

Eventually, doctors accepted that Christine had LIS, a rare condition that is believed to have affected only 250 people in the UK. It occurs when a certain area of the brain stem is damaged, and its rarity means that experts still do not know whether it can be cured.

Sitting in a wheelchair next to her husband as he talks, Christine becomes visibly distressed when he mentions the impact of her condition on their sons. Her legs judder and her breathing through the tube becomes laboured. Colin asks her to look down if she wants him to carry on. She does.

"It is incredibly frustrating for Chris, who is such a strong-willed person," he says. "We have been married 36 years and we have always communicated very well, but it is different now. I can only ask her 'yes and no' questions, but I know she doesn't want to give up."

Christine is now in one of Brtain's only specialist centres caring for people with such severe neurological disorders, the Capio Jacob Centre in Hertfordshire. She is receiving physiotherapy to stop her limbs from weakening and has her husband by her side almost constantly. The wall of her room is covered in pictures - of her wedding; her sons; mementos of holidays. Colin is insistent that this is a life she may return to. "She has improved a lot just by being here. I don't think she has classic locked-in syndrome because she can smile and cry."

The future is uncertain but there is hope. Last week, Christine took possession of a new virtual reality device that is based on computer games. A headband will allow her to use her eye movements to control a computer and point to letters, making up words that will then be fed through a voice synthesiser. Research is also being carried out into brain-computer interfacing, whereby people can use their thoughts to communicate. In the future it is hoped that people in Christine's condition can literally think "BBC1", for example, and a computer will change the television channel for them. And a British-based company, ReNeuron, is about to start clinical trials in the US of stem cell treatment that could repair the damage done to the brain stem. If all goes well, a treatment for LIS may be available soon.

Christine has already defied the doctors' original prognosis and outlived many others who have suffered LIS. Colin says: "I'm reading a book at the moment by a woman who had a similar thing to Christine and has been helped by traditional Chinese medicine. There are some really exciting things happening in this field."

As he wheels his wife to the television room, he strokes her long hair and says: "She's still my Chris."