Imprisoned in his body after suffering a stroke, Manuel Longo, Malta’s only known case of locked-in syndrome, tells Ariadne Massa that although there is little left to motivate him his will to live has never been snuffed out.

Manuel Longo eyes the housefly buzzing around his room and feels its tickle as it crawls up his arm.

The thought of death scares me, so I don’t even think of suicide

He wants to scratch the itch and shoo the fly away, but instead he closes his eyes and wills it to leave.

“Even a small fly can make me miserable,” the 50-year-old says, communicating by deftly beaming his laser on the alphabet to construct a sentence, letter by letter to form each word.

Mr Longo is conscious of all that is happening around him – his mental faculties are all there, he can feel, taste, hear, see and smell – but he is completely paralysed and is unable to talk.

“I’m like a thinking corpse,” he says, his shoulders moving as he silently chuckles in self-deprecation, revealing a sense of humour that has also remained intact.

Mr Longo, Malta’s only known case of locked-in syndrome, was a busy man with not enough hours in the day to do all he wanted when he suffered a stroke at the age of 38.

This is a “cruel”, rare condition where the brainstem is damaged by the stroke, leading to paralysis. Few survive such a catastrophic event and when they do, victims remain imprisoned in their own body.

Locked-in syndrome made international headlines last month after Briton Tony Nicklinson lost his High Court battle for doctors to legally end his life, seven years after a stroke left him paralysed.

Mr Nicklinson, who said he had been condemned to a life worse than death, started refusing food after the verdict and seven days later he died from pneumonia.

His howl of despair when he learnt his case for assisted suicide was turned down was beamed across the world, forcing a debate on the ethical minefield of the right to die.

One person who keenly watched the news unfold, from the permanent confines of his room in St Vincent de Paul home for the elderly, was Mr Longo.

“Everybody is different and maybe those seeking assisted suicide really need it...

“It should be a choice as long as the person is still all there and can make a conscious decision,” he says, his eyes speaking volumes but unable to communicate quickly enough as he spelled out his thoughts one letter at a time.

“Tony must have had his reasons. Maybe he never came to terms with locked-in syndrome.

“I can understand what he was going through,” he adds.

Did Mr Longo ever entertain the idea of ending his own life?

“No thanks! Even though I have nothing to motivate me,” he says, his wife patiently dictating each sentence.

His 47-year-old wife – who for the past 12 years has had her own life symbolically locked in as she raised their two boys, went to work and visited her husband six evenings every week – expressed the wish not to be named for fear the routine that keeps her sane will be upset once more by the unwanted attention and pitying stares.

She is an active participant throughout the interview, quickly pointing out: “Manuel was always petrified of dying, even before the stroke”.

Mr Longo slowly nods his head: “The thought of death scares me, so I don’t even think of suicide.”

Feeding him thickened coffee – all his food has to be pureed as his tongue and mouth muscles are also paralysed – with a spoon, the couple share a joke.

As he chuckles, his wife smiles and warns: “Don’t laugh or you’ll choke and I’ll be charged with aiding and abetting you.”

She adds: “We’ve spoken about this several times and if it [locked-in syndrome] had to happen to me, I wouldn’t want to be saved.”

However, Mr Longo is happy he was saved, even though he admits his future is bleak. Why is he so terrified of dying?

“The great unknown scares me... what is there, if anything, after this life? Probably nothing and I just become bug food,” he says, explaining he had never been religious.

Asked to explain the plastic statues of Our Lady of Lourdes containing holy water on his shelf and the rosary beads dangling close to his head, Mr Longo chuckles and says he leaves them there not to offend the people who brought them; plus he was not in a position to move them either.

“I guess if I was religious I’d have something to look forward to seeing that most religions promise an afterlife, which is much better than what we have here on earth.”

Reflecting on the day the life he once knew was changed, Mr Longo manages to smile at the irony that his loss of freedom coincided with Independence Day 12 years ago – September 21, 2000.

He was in Cremona, Italy, on a work-related trip overseeing the testing of a machine when at about 5pm he felt dizzy. He sat down and kept his head lowered, thinking it was low blood pressure.

After it passed, he headed to the bathroom, but instinct told him not to close the door.

“Suddenly everything was spinning and I began shouting for help.

“Shortly after I collapsed,” he says, recalling how his colleague sensed something had happened and went to his rescue.

In the ambulance medics cut through his polo shirt to help him breathe and – failing to realise he was still conscious – said: “This one’s a goner”.

“I desperately tried to open my eyes but nothing happened. I remember thinking that if this is dying, at least it doesn’t hurt,” he says.

After four days in a coma in Italy, Mr Longo came round but was hallucinating.

Completely paralysed and unable to talk ,he was terrified that he was somehow being kept alive for his organs.

He retained this fear until he was transferred to Malta. During the months he spent in the hospital’s ITU, Mr Longo lost all notion of time.

He was forced to watch whatever the nurses were following on TV – usually serials such as Ipokriti and Simpatiċi, which he disliked.

Around this time he learnt to communicate by blinking his eyes.

His wife learnt to use an alphabet that started with the most commonly used letters – ETAOINS as opposed to ABCDEFG.

She would start reciting it slowly and he would blink when she reached the letter he wanted, then start all over again for the next letter.

His wife explains that in the first year her husband was extremely ill; he was in pain, constantly drooling and unable to move, except blink his eyes.

His story is nearly identical to that of Jean-Dominique Bauby, the editor-in-chief of French Elle magazine, who created awareness about locked-in syndrome by writing The Diving Bell and The Butterfly, a memoir he dictated using his one good eye.

The only difference between Mr Longo and Mr Bauby is that Mr Longo has two good eyes and has, over the years, succeeded in regaining some movement in his neck.

Mr Longo has watched the film based on Mr Bauby’s book four times and each time was equally emotional.

“It helps people understand a bit my world. Ideally, anybody taking care for me should watch it but in practice it’s impossible...”

These days Mr Longo considers himself to be one of the lucky survivors of a brainstem stroke because he can move his head, blink, open and close his mouth and breathe without needing a machine.

Technology and the internet have drastically improved his life – using the laser that is attached to his spectacles he can change TV channels, surf any website he wants, send text messages, download a film and even write e-mails through a laser-operated keyboard.

The laser has also helped do away with reciting the alphabet and instead he simply points to the letter he needs to communicate.

However, this still requires patience and his message can be lost in translation.

“Very often, during a conversation, I want to add my part but by the time somebody notices I want to say something, the relevant moment will have passed and my comment is no longer valid,” he explains.

Those who are unaware of his condition assume he is a vegetable, tending to shout and flap their hands at him in the hope of being understood.

“Sometimes, I’m treated as though I’m retarded, which really aggravates me.”

Consequently, visitors are rare. Apart from his wife (her daily visits are “the highlight”), and his two sons he is proud of – who, however, are living and studying abroad – he has one other friend who visits religiously every Sunday. Others visit occasionally.

Sometimes, I’m treated as though I’m retarded, which really aggravates me

Before the stroke, Mr Longo was obsessed with computer gizmos, fixing things, off-roading and model aircraft.

A self-taught engineer who did everything himself as he thought he could do it best, having to be completely dependent on others was one of the hardest things to adjust to. Patience is something he had to discover in hospital.

Who is Manuel in there?

“I don’t know anymore. The hardest thing to accept is not being me,” is his reply.

His day is a predictable routine. “I would say I am normally sad and bored.

“Remember, I live here and my physical world is my room. Picture this: when I wake up I see the usual ceiling and can’t look elsewhere because I can’t move. Enough said?”

He does not dread any part of the day and does his utmost not to let his mind wander.

“When I do [let it wander] it is about all the mistakes I did, the future (bleak), ways to improve my existence...”

So if death does not scare him, what does?

“That my wife dies before I do... I am like a vegetable; the only difference is I can think.

“My father died when I was seven years old and I often wondered if I would see my children grow.

“The answer is ‘yes’, but in this state?”

amassa@timesofmalta.com

Conscious, but paralysed and voiceless

Locked-in syndrome (LIS) is a rare neurological disorder characterised by complete paralysis of voluntary muscles in all parts of the body, except those that control eye movement.

It is usually the result of a stroke in the base of the brainstem. The patient is alert and fully conscious but cannot move.

There is no cure for LIS, nor is there a standard course of treatment. The prognosis is poor and the majority of patients do not regain function.

Once a patient becomes medically stable, and is given appropriate medical care, life expectancy increases to several decades. In fact, Mr Longo describes himself as “boringly healthy”.

Even if the chances of good motor recovery are very limited, existing eye-controlled, computer-based communication technology allows the patient to control their environment and access the internet.

Healthy individuals and medical professionals sometimes assume that the quality of life of an LIS patient is so poor it is not worth living.

On the contrary, chronic LIS patients, such as Mr Longo, typically report a meaningful quality of life and their demand for euthanasia is surprisingly infrequent, according to the American National Centre for Biotechnology Information.