The secret happiness of locked-in syndrome
Shirley Parsons has been trapped in her body for 14 years. But like many other sufferers, her mind is thriving, she tells
Until her life changed radically and forever at the age of 41, Shirley Parsons was a successful solicitor in Exeter, southwest England. Her husband ran a nearby farm. Beef and sheep. They are still married, though these days she doesn’t see him all that often.
On a Sunday morning in 2003, Shirley woke up with a really bad headache and a sensation of vertigo. She decided to stay in bed, but dragged herself up in the afternoon to help with feeding on the farm. “I suddenly felt dizzy so I sat down on the hay,” she recalls, “and the next thing I knew was when I woke up in intensive care two weeks later.”
Doctors discovered that Shirley had a mutation in the gene for factor V, the stuff that helps blood clot. The single-letter misprint in the code meant that one of her factor V proteins has a glutamine amino acid instead of an arginine. This gives her thrombophilia, the tendency for her blood to clot more than it ought to.
Without realising, Shirley had developed deep vein thrombosis, and a clot had dislodged and travelled to her brain. It caused brainstem bleeding, and a massive haemorrhagic stroke. She was told that she wasn’t expected to live.
Shirley has now been paralysed from the neck down for 14 years and five months. She has locked-in syndrome: her mind is intact, and indeed thriving, but her body has long since closed down.
By the time I met Shirley at her house on the edge of Dartmoor, we’d already chatted via email for a number of months. She was helping me with research I was doing for a book about the extreme potential of human beings, specifically in terms of our ability to feel happiness. Over email, I’d been struck by her thoughtfulness, and by her extraordinary reserves of strength and resilience (when I put that to her, she said, “Most people would use the words stubborn and awkward!”).
But the thing that interested me the most was her approach to life. “I’ve come to the conclusion that my brain’s default setting is happiness,” she said on one occasion.
Face-to-face, Shirley can answer yes – no questions with her eyes: she looks up for “yes” and side to side for “no”. For more complicated answers, she uses her computer, which she operates using a cheek switch, and specialist software called EZ Keys.
It’s a roasting day on the edge of Dartmoor, and Shirley’s face is flushed and sheened. Her mouth hangs open, which is the default setting now she’s paralysed. It gives her a hospitalised look. You see people who look like this and you assume that they are paralysed in their minds, too. Labels such as “vegetative state” don’t help. I know that’s not the case with Shirley, but it’s still disconcerting, to see her face-to-face, unable to move on the outside but knowing that she’s buzzing, on the inside.
I asked her, apologising if it was a strange or even offensive question, whether there was a sense that she was happier now than before she became locked-in. She found the question easy to answer.
“Although on the face of it, it seems a ridiculous question, rather bizarrely I think that I am happier,” she said. “Before the stroke my life was noisy and hectic but now most of the time it’s quiet, peaceful and calm. Over the years I’ve grown accustomed and become content with my life.”
Shirley’s evaluation, that she is happier now than before her illness burst, is probably the opposite of what most people who aren’t paralysed would expect her to say. To us, being locked-in sounds horrendous.
It’s an assumption that drove Marie-christine Nizzi, an instructor at the department of psychology at Harvard University, to survey patients with locked-in syndrome. “What I found was that they have a much more positive quality of life than what we project from the outside,” she told me. “The patients report being happy, or being satisfied with their lives. The majority are happy.”
Nizzi’s work follows that of Steven Laureys, who runs the Coma
‘Before my life was noisy and hectic, but now it’s quiet, peaceful and calm’
Science Group at the University of Liège in Belgium. In 2008, Laureys decided to conduct a quality-of-life survey of people with locked-in syndrome.
Sixty-five patients were included in the survey; 47 of them professed themselves happy, and the rest responded that they were unhappy. Laureys concluded that perhaps the happy locked-in people had succeeded in recalibrating their lives.
I accept that some locked-in patients are content, even happy. But surely no one locked-in feels better about their lives now? “I asked that exact question,” said Nizzi. “The answer was yes. They knew themselves better, and they found their life’s meaning more strongly now than they had before.” Being locked-in forces you to halt. It forces you to look for a meaning that you may not have sought before.
Tim Harrower is Shirley’s consultant neurologist at the Royal Devon and Exeter Hospital, and saw her when she was in rehab there after her stroke. “The big breakthrough with locked-in patients happens when you work out a way to communicate,” he says. “It changes everything, because they can express their needs.”
You might have an itch, or you’re in pain, or you just want the damn TV turned off. Then it’s a case of adapting to what you can and cannot do, and finding something cerebral to enjoy. “Being able to accept your situation and adjust to your limitations is the biggest problem really,” Harrower says. “That doesn’t happen straight away, it can be years down the line.”
Shirley had been locked-in for about five years when she decided to begin a degree course. She was missing her job; she was bored, basically. “It was the loss of my job which prompted me to study,” she told me. “I was too young to retire and needed something intellectual to fill the void.” She took a BA, and later a postgraduate certificate, in social sciences with politics, graduating in 2012. It must have been challenging, I offered. “The main challenge,” she said, drily, “was that it took me ages to type assignments.”
It seems that if you suffer from locked-in syndrome, it helps to have a positive outlook on life. As Shirley put it: “I don’t doubt that my happy disposition and inability to maintain upset or anger has helped.”
Shirley’s story is incredible, but as those academic studies show, it’s not unique. I also spoke with Kati van der Hoeven, a former international model who became locked-in after a stroke at the age of 20. Kati now runs a Youtube channel, where she helps other sufferers find happiness. Many locked-in people get in touch with her. Over email, she told me she no longer considers what happened to her a tragedy. She is happier now than when she was a model: “For me happiness is love, not just receiving it but also being able to give it, to share it. I would say it’s having a purpose in life and making a difference in other people’s lives, as little that it may be.”
There are two things I take away from this. One is that the majority of people who contact Kati haven’t made peace with their condition. For all I’ve been citing the research that shows most locked-in people are happy, it would be crazy to pretend it’s not a crushing blow to come to terms with.
The other is that everyone needs a purpose, whatever their situation in life. Shirley and Kati show us that happiness doesn’t require beauty, athleticism, fame or riches – the things we often chase in life. A positive outlook, however, plays a huge role in the level of contentment we report.
If you look back on your life and you have spent 20 or 30 years of it locked-in, you might think, and you’d surely be justified, that you’d been dealt a rough hand. Or, if you were Shirley, you might say, well, s--happened, but I did some good things, I took those degrees, I improved my mind; I understood myself and the meaning of life. Shipwrecked in my own body, I explored myself, and I found hidden treasure.
This article is an edited extract from Superhuman, by Rowan Hooper. Published by Little, Brown Book Group (RRP £20) on Thursday. To order your copy for £16.99 plus p&p call 0844 871 1514 or visit books.telegraph.co.uk