This week marks a poignant anniversary for Maria McNeilly and her husband Alan. On November 30 last year, bright, chatty, Spanish-born Maria, who loves Strictly Come Dancing and chatting on the phone, suffered the first of a series of strokes that would leave her almost completely paralysed.
Alan had taken Maria — Marini to those who know her — to their local hospital after she began to feel light-headed and dizzy. She even suffered mini-strokes while on the ward but doctors there misdiagnosed her symptoms as labyrinthitis, an ear infection, and sent her home with anti-sickness tablets. Five days after she was discharged, Marini had a massive stroke at home in Guildford, Surrey.
At the hospital, Alan was given the news that his wife of 33 years had lost all feeling in her body. The only movements she could make were to shift her head slightly and move her eyes left, right, up and down. She remained in hospital while her condition stabilised and Alan began to contemplate what lay ahead.
“I would ask her a very simple question and she wouldn’t respond in any way. I thought, ‘That’s bad news’,” says Alan, 58. “I’d never heard of locked-in syndrome.”
It wasn’t until Marini, 56, was transferred to the Royal Hospital for Neurodisability (RHN) in Putney, South London, that her condition was properly diagnosed. The stroke had caused a lesion in her brain stem, meaning that the top half of the brain, where conscious thought happens, was intact but the “bridge” from her brain to her body was damaged. Marini was fully aware of herself and what was around her but had no way of moving or communicating. She was “locked” in a body that wouldn’t do as she wanted.
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“The morning after we arrived here, I came in and they had dressed her in her own clothes,” Alan says, closing his eyes and momentarily tightening his lips. “It was such a beautiful sight.”
Today, Alan stands over his wife holding a board of colour-coded letters, and she uses the direction of her gaze to indicate what she wants to say.
“I was very frustrated because I had always relied on my voice,” she spells out when I ask her what it was like in those early days. Together, the couple can communicate remarkably quickly but it has taken time to work out the system. Alan has thousands of sheets of paper at home with the beginnings of sentences that they couldn’t finish because Marini became too tired or frustrated. Over time, though, they began to master their system and, thanks to sophisticated therapy at the hospital, Marini has regained movement in her fingers and face. She says that her mother in Spain, to whom she is very close, helped to keep her going — before her stroke they rang up huge telephone bills talking to each other. Then Marini opens her mouth wide and sobs, big tears running down her cheeks.
“In all those years of marriage I didn’t see my wife cry once,” says Alan, going to her and stroking her shoulder. “Now she cries every day.”
If the tears are hard for him to witness, for Marini they are something of a relief: before she came to the RHN, she could not cry. “She had only two facial expressions,” says Alan. “One was plain nothing, one was pain. Now at least she can express her emotions. She can cry and she can laugh — make a joke and she laughs like a drain.”
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Some “locked-in” patients can be paralysed so severely that no one recognises that they are conscious inside — as in the case of Rom Houben, whose story made headlines last week. Houben had no control over his eyes and lived with locked-in syndrome for 23 years while doctors insisted that he was in a permanent vegetative state and had little or no brain function.
A survey at the RHN in 1996 found that 43 per cent of those patients who arrived with a diagnosis of vegetative state had been misdiagnosed and in fact had varying levels of consciousness. One patient had been “locked in” for eight years, his family unaware that he was conscious, before he was diagnosed at the RHN. Therapists introduced him to technology that allowed him to communicate. His first words in almost a decade were to tell his wife that he loved her.
Dr Ashraff Ali, consultant in neuro-rehabilitation at the hospital, says that misdiagnosis is now happening less. His team have devised a thorough 12-week assessment programme for patients diagnosed as being in a vegetative state. “It tests all five senses and looks at how the patient responds to external stimuli,” says Dr Ali. “It starts with something like shining a light in their eyes but we also use objects that mean something to them. If the patient is a guitar player, we would show them a guitar or play music that they like.” The input of relatives and friends, who recognise their loved one’s expressions, is vital. In 1972, a family member noticed Julia Tavalaro trying to smile after she heard a joke. For six years doctors had believed her to be in a vegetative state, but the smile prompted them to diagnose locked-in syndrome. Tavalaro went on to become a poet and author.
Immediately after Marini arrived at the RHN and was diagnosed as being in a locked-in state, staff began working on a personal plan with special equipment that allowed her to communicate using her eyes. Alan realised that his wife was in there: alive, awake her personality unaltered.
Locked-in syndrome has been described as the closest thing to being buried alive, and when Rom Houben’s story broke last week there were some who said that he should have been allowed to die. But although recovery is very unlikely, many patients with this rare condition feel optimistic. The stories of people such as Tavalaro and Jean-Dominique Bauby, the French writer who dictated his memoir, The Diving Bell and the Butterfly, one letter at a time by blinking one eye, are testaments to what is possible.
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“The quality of life is not as bad as the rest of the world thinks,” says Dr Ali. “If you ask people with locked-in syndrome about their quality of life, with 0 as medium, minus 5 as the most unhappy situation you could be in and 5 as the most happy, most answer 3 or 4 — much higher than what those working with them had thought.”
Marini agrees: “I feel very motivated thanks to the enthusiasm here at Putney,” she spells out. “What I want is to get better. I have faith.”
It has been a year since that black week when Alan watched as Marini lost her ability to walk, to talk, to swallow, to dance. Now, the couple are hoping that she will soon be able to come home, but Alan is struggling to work out how he will pay for the expensive equipment and modifications they will need to their house, after he was made redundant from his job as an IT manager. Although their health authority is paying for Marini to stay at the RHN, it has said that it cannot pay for the technology they need to live a meaningful life — such as a computer with a screen of letters that reads the user’s gaze and interprets the words that he or she wants to say.
But Alan, too, shows a patience and strength that are humbling — so much so that Marini begins to weep again when he says: “We had a life before, which is gone. But we have a new life now and, given what recovery Marini has made, we can have some kind of enjoyment.”