Jean-Dominique Bauby lived his dream. He was 43 years old, a successful editor of the French edition of Elle magazine, freshly in love. On that December day in 1995, he was flying through life when he took his 8-year-old son Théophile out for dinner. Suddenly, he was sidetracked. He had no control over the car or himself. He slipped into a coma. He regained consciousness three weeks later. He was in hospital. He was aware of everything that was happening around him, but he could not react to anything. After a massive stroke, only one part of his body – his left eye – was responding to his brain’s commands.
The situation has not improved. He was fully conscious but could neither move nor speak. Doctors diagnosed him with locked-in syndrome, which in English is called locked-in syndrome, which, very loosely translated, means that a person is locked in his body.
Bauby literally overnight changed from a man with the world at his feet to a man who could do literally nothing. He could have sunk into depression, but something in him wanted to live. He was indeed helpless, but not completely. His left eye still obeyed him.
First, he learned to answer yes and no questions with him, then he invented a special way of communicating with his speech therapist. The speech therapist rearranged the order of the letters of the alphabet so that they followed each other in order of frequency of use. Then he read the letters to him, Bauby blinked his left eye when he came to the right letter and the speech therapist wrote it down. They repeated the exercise until all the letters of the word Bauby had in mind were on the paper.
Others have also learnt the time-consuming and exhausting communication. “It’s a pretty simple system. You read the alphabet … until I stop you with a wink at the letter you need to write,” Bauby explained. But the system was not foolproof. “One day, when I was about to ask for my lunettes, I was asked what I wanted to do with the moon(s).”
He couldn’t really chat with this way of communicating, but he could express his feelings and tell how he felt. For example, he could explain that although his body was not obeying him, he still felt sore, cold and hot. “My arms, which lie curled up under the yellow sheets, hurt, although I can’t tell whether they are burning or chilling me.”
Once he had mastered the new communication, Bauby set himself a goal: he would write a book. “I had to rely on myself if I wanted to prove that my intelligence quotient was still higher than that of a fool,” he explains why this was so important to him.
The nurses and doctors at the hospital near Calais in the north of France quickly grew to love him for his will to live, but dealing with such a life was not easy. “There are times when accumulated problems trigger uncontrollable nervous laughter,” he explained.
“One day… I find it funny that in my forty-fifth year I am being washed and turned, my buttocks wiped and swaddled like a newborn baby. I even find a secret pleasure in this complete slide into infantilism. But the next day, I find the same process intolerably sad. A tear slips down through the soapy water the nurse has smeared on my face.”
He sometimes felt that he was living the life of a “desert crab buried in a rock”; that his existence was not only “reduced to the level of a jellyfish”, but that it was “also terrifying to watch”; that he belonged to the plant stall, not to the human race. He reflected that he had swapped places with an old friend who had spent “years in a darkened Beirut underground dungeon” while being held hostage by Hezbollah.
He depicted his life in two metaphors, the diving bell and the butterfly. The first represents the body in which he is trapped, the second the imagination. Over time, his ‘diving bell became less oppressive and my thoughts flew like a butterfly. There is so much you can do. You can drift in time and space, go to the Isle of Fire or to King Midas’s court.”
In his imagination, he moved wherever he wanted and did all sorts of things, like cooking. In his mind’s eye, he brought back memories of the smells, tastes and textures of food: “You can sit down to a meal at any hour, without planning or rituals. If it’s a restaurant, you don’t have to book in advance … The beef burgundy is tender …”
Hope
In his book, he recalled how, just days before he suffered a stroke, he visited his ageing father and stood next to “a black and white photograph of himself on a miniature golf course. I was 11 years old, my ears stuck out and I looked a bit like a boy who was a bit restricted”. Later, his father sent the photograph to the hospital. They turned it over so he could read what was written on the back. “In his strong, angular handwriting, my father only wrote: Berck-sur-Mer, April 1963.” Life had played a trick on Bauby: he was now in hospital in Berck-sur-Mer.
Shortly before his stroke, he started controlling his calorie intake, not knowing that he would lose 33 kg over the next 20 weeks. To prevent his mind from deteriorating in the same way as his body, he kept his emotions as strong as possible. Anger, in particular, drove him forward. Sarcastic outbursts were a constant, yet there was a great deal of tenderness, humour and lyricism in him.
In the face of all that has hit him, he has managed to remain as poised as he once was. He couldn’t swallow the saliva, so it slid down his chin. He would not allow it to be wiped off with hospital napkins, he wanted his own made of cashmere.
At the time of writing, his prognosis was unclear. He hoped that his digestion and breathing would improve enough for him to be able to eat on his own, and even to “breathe in enough air to make my vocal cords vibrate”. On 9 May 1997, just two days after the French version of his book The Diving Bell and the Butterfly was published, or a year and a half after a stroke, he died.
His story was made into a film, just as he wanted it to be. The story in the film is adapted, but the two stories later told by his ex and new partners did not overlap.
Florence Ben Sadoun’s new life companion is portrayed in the film as a woman who dared not even visit him in her condition. She herself claimed in her book that she remained loyal even after the stroke. That she drove three hours away two or three times a week to read to him and help him write his book. That she was by his side even when he finally died of pneumonia.
“He was the man I loved. I never saw him any other way. We continued to love each other. We even argued. That’s what kept us both alive.” According to her, his former partner and mother of his two children, Sylvie de la Rouchefoucauld, was staying with her new love in America at the time of his death.
But Sylvie later told a different story. It was true that Bauby had cheated on her all the time, and it was true that the news that he was leaving this time hit her hard. She had only found out a few months before the stroke, when her son was eight and her daughter nine. But she remained loyal to him, too, and regularly drove the 500 kilometres north with the children. That she was at his bedside day after day and never knew that his current girlfriend also came to visit him. When she asked him what she, Sylvie, was to him, he told her that she was the mother of his children and his best friend.
His daughter Celeste later recalled her first reaction at the sight of her unmoving father. That’s not my daddy, she screamed and ran away. After that she got used to him, played and talked with him, but for years neither she nor her brother could accept his stroke or his death.
Anyone home?
Syndrome of locked-in consciousness is a rare neurological phenomenon that severely affects the patient physically and mentally. It is most often the result of trauma, stroke or brain tumours, but less commonly of drug overdose, hypoglycaemia or nerve cell damage as a consequence of multiple sclerosis. The syndrome is divided into the classic Bauby syndrome, the incomplete syndrome, in which the sufferer can move an arm or a leg for cognition, and the complete syndrome, in which the body is completely unresponsive and consciousness is intact.
Because the sufferer is fully aware of everything around him or her and can think normally, the syndrome of locked-in consciousness is different from other related conditions. In brain death, none of the brain’s functions function anymore. In a coma, there is a complete loss of consciousness, the sleep-wake cycle is lost and the eyes remain closed. A coma usually lasts between two and four weeks, after which the person wakes up, and may also be diagnosed as having a dissociated consciousness syndrome, a minimally conscious state or a vegetative state.
In the vegetative state, sleep-wake cycles occur and the eyes may open spontaneously or in response to external stimuli, but all these responses seem to be reflex. In the minimally conscious state, however, the patient appears at first sight to be in a vegetative state, but sometimes shows signs of attentiveness by reaching for an object, following a command or reacting to the environment.
Researchers looked into the case of a young teacher who had contracted something flu-like. She slipped into a coma. She never woke up. A few weeks later, doctors declared her to be in a vegetative state, although her sleep-wake cycles were still present, her eyes opened and closed and she seemed to glide across the room.
The researchers wanted to know whether this was just automatism or whether there was a hint of consciousness in it, even if it was not visible. They started testing her and other patients in a vegetative state.
Positron emission tomography (PET) was used to monitor how their brains responded when they were played sounds containing logical words and sounds full of nonsense. The images showed that the same part of their brains responded to logical speech as in healthy people, but the researchers could not confirm for sure that higher brain functions were involved and that it was not just a reflex response.
To get a definitive answer, they found volunteers, medical colleagues, who agreed to put them in a coma for a short time. They ran the same tests on them and got the same results, confirming that the part of the brain that recognises logical words responds automatically.
So what do they need to do to get a “conscious” answer? They decided to try the “follow my lead” technique. Of course, they couldn’t tell people who didn’t respond to anything to shake the doctor’s hand or say their name, but they could compose the questions differently and monitor brain function with fMRI, or functional magnetic resonance imaging.
In the first part of the experiment, healthy participants were tested to see which questions their brains respond most actively to. When they are told to sing Christmas carols in a coma, or when they have to imagine going from room to room at home or playing tennis? They found that the last two questions elicited the strongest response.
Now they have tested patients in a vegetative state. When one patient was told to imagine herself walking from room to room, one part of her brain came alive. When she was told to imagine playing tennis, another came to life. This led them to conclude that she was still conscious, even if she could not react to her surroundings.
In the following years, the experiment was refined. Now, patients had to answer questions by walking through the rooms and playing tennis. If the answer to the question was no, they had to imagine themselves walking through the rooms. If the answer was yes, they had to play tennis, because different brain centres are activated in different cases.
Then they asked patients about things their relatives had confided in them. For example, one patient was asked if his father’s name was John. He imagined a game of tennis and answered yes. He also answered other questions correctly, which meant that he understood the instructions they gave him before they started testing him and remembered them throughout the test, that he understood the question and that he remembered his life before he slipped into the state he is living in now.
After testing, this patient’s status was changed from vegetative to minimally conscious because he could communicate with his surroundings, but only with the help of fMRI or functional magnetic resonance imaging. The status was also changed for the young teacher. Several months after the test, she started to recover against all expectations. Today she is in a wheelchair and needs constant care, but her cognitive functions have recovered and she has also learned to speak again.
Husband against parents
But such cases are extremely rare. Terri Schiavo was not one of them. On 25 February 1990, 26-year-old Terri suddenly fainted. Help arrived too late. Her brain was deprived of oxygen for several minutes. A few weeks later, doctors declared her in a vegetative state.
No one knew what had happened to her. Later, they found that her body was extremely low in potassium, but even from this they could not draw a conclusion as to why she lost consciousness and why she did not wake up.
Her husband Michael, who was with her when she lost consciousness at home, wanted her to stop being artificially fed. He insisted that she would never want to live like that. Her parents argued the opposite, that she would have wanted to live. For her husband, the woman he had married was no more, and her parents saw in her all that they had kept in their memories.
Although Terri was breathing on her own, doctors assessed that she was unable to form thoughts or emotions, so there was no consciousness in her. A fight broke out between her husband and her parents. Soon, judges, politicians and all those who saw in her case an opportunity to put their own pot of interests on the line got involved. The litigation lasted fifteen years. The case moved from court to court until the final verdict was that Terri’s husband had the right to decide on her life and death.
On March 31st 2005, she was stopped being artificially fed. An autopsy showed that her brain was damaged beyond recovery. Nevertheless, the questions of whether patients in a vegetative state are really unaware of anything, what quality of life means and in what case it is acceptable to end such a life have remained unanswered, and Terri is an ideal subject for manipulation on the subject.
Much earlier, the case of 21-year-old Karen Ann Quinlan was complicated, but with parents and doctors on different sides. On 14 April 1975, Karen and her friends went to a bar. She had been drinking too much and had washed down an unknown quantity of sleeping pills with alcohol. Suddenly she became unconscious. A friend dragged her home and only then noticed that she was no longer breathing. He started to resuscitate her and called for help.
It was too late. A subsequent medical report said that her brain had been deprived of oxygen at least twice for 15 minutes each. She was now unresponsive to anything else, in a vegetative state. She was put on a ventilator and started to be fed artificially.
Her parents turned to a priest for help. Mr and Mrs Quinlan adopted Karen Ann when she was just four weeks old. Two years later they had another child and two years after that another, but there were no problems in the family until Ann finished high school. She thought about college and finally decided to work.
When the redundancies started and she was left without him, her friends told me, she lost her way. She moved into a house that was almost like a commune, started drinking heavily and occasionally took tranquillisers. In the end, this cocktail cost her her life.
But only consciously, her body was kept alive with devices. But Karen Ann visibly deteriorated over the following months. She was losing weight. She used to be about 55 kilograms, now she was only about 35. She no longer resembled the girl she had once been.
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Parents would, doctors wouldn’t
Until now, the Quinlan’s wanted the doctors to do everything in their power to keep her alive, but now they have had to accept that their daughter is gone. A local priest helped them to face the painful truth. He reassured them that the Church teaches, and so does Pope Pius XII’s 1957 declaration, that no moral obligation dictates the insistence on extraordinary measures to preserve life where there is no realistic hope of recovery.
Finally, the couple made a difficult decision: they will demand that their daughter be taken off the ventilator. They arrived at the hospital and informed her doctors of their decision. They smoothly refused. They could not, saying that there was no legal or medical precedent to follow and that they could be sued for negligence. Earlier, Mr and Mrs Quinlan had signed a declaration clearly authorising the doctors to switch off the respirator and absolving them of any liability.
Mr and Mrs Quinlan were forced to take their private problem to court. Their lawyer told the judge that keeping their daughter alive “after the dignity, beauty, hope and meaning of earthly life have evaporated” constituted cruel and unusual punishment in violation of the Eighth Amendment. He wanted the court to give the parents the right to decide on their daughter’s life.
The lawyer appointed by the court to defend Anna Karen argued that removal of the ventilator would legally constitute murder, or at least euthanasia, both of which are against the law and medical codes. The judge ended up ruling that he could not make that decision because it was a medical decision and the disconnection could violate laws dealing with homicide.
On appeal, the High Court, by a unanimous vote of 7 judges to 0, found that “in the end, we reach a point at which the rights of the individual take precedence over the interests of the State”. Three weeks later, Karen Ann was taken off the machine that helped her breathe. Against all expectations, she began to breathe on her own.
The judge also allowed the parents to request that the artificial feeding be stopped. They did not. They were convinced that the respirator was causing her pain, and they did not think she had any problems with the feeding. Karen Ann lived another 9 years like this, although she eventually weighed only 29 kilograms, lying curled up under the blankets like a baby, her eyes wandering aimlessly to and fro.
Her father visited her every day on his way to work, her mother whenever she could. The radio was always playing at her bedside, sometimes with music she liked. They knew she couldn’t hear it, but it seemed unacceptable for her to lie in complete silence. She died on 11 June 1985 of pneumonia, which her parents refused to allow to be treated, and her case became a precedent for many who followed.
A medical miracle
A year earlier, Angilee Wallis had prayed: “Please God, let my son survive!” They had no phone at home and neighbours came and told her about the accident. Her 19-year-old son Terry and his friend Chubs had driven their car off a bridge and into a river. Terry had married his girlfriend of 15 years, Sandy, not long before and they had a daughter, Amber, six weeks before the accident.
Angilee, Amber and Chubs’ loved ones rushed to the hospital, only to learn that the boy had been flown by helicopter to the trauma ward at Springfield Hospital. The three-hour drive was the longest of Angilee’s life as a mother of four.
At the hospital, she was told that Terry had suffered severe head injuries. That he would probably be paralysed. That they were trying to stop the swelling in his brain with medication, but to no avail. Before she was allowed to see him, she was warned that she would see many machines and tubes. “We are giving Terry lots of painkillers and he is not awake, but it is possible that he will hear you, so stay calm. We must not distress him further,” the nurse warned her.
Angilee entered the room and left immediately. She could not control her emotions. She was shaking, crying and gasping for air. She took a deep breath, calmed down and returned to the room. “As I watched my son lying there unconscious, I was overwhelmed with love and fear. But looking at him gave me hope.”
Even though he crashed his car into the river, he only had a tiny cut on his head with three stitches. Other than that, there were no visible injuries. His hands were twitching. She thought this was a good sign, but the nurse disappointed her by saying it was a bad one. The trembling was indicative of brain seizures. Angilee swallowed her saliva but could not hold back her tears. “Hang in there, Terry. I love you. I’m right here with you,” she told him, though she still couldn’t believe that it was really her son lying on the bed, always so full of energy.
He was still in danger of death. His friend had already been taken. Days passed, his brain swelled. Angilee and her husband Jerry heard again and again every day that it was impossible to predict the final consequences. While her husband cared for their two young children, Angilee slept in the waiting room for weeks, waiting for the final outcome.
Terry had an accident in July 1984, and in October the doctors said there was nothing more they could do for him. He is in a permanent vegetative state. He was moved to a care centre two hours from their home. Angilee and Jerry heard from all sides that it might have been better if he had died. They decided that God wanted him to live.
Angilee went back to work and spent every other weekend with her son. Christmas was approaching. She couldn’t imagine it without him. She wanted him to come home. As he was in a vegetative state, this was difficult. “I was scared, but I was determined: I wanted Terry home for Christmas.”
Now that he was being syringe-fed, she decided she could do it herself. Terry was transferred to the car, taken home and put to bed. Friends arrived, all talking to him as if he were still the old Terry. Although he was unresponsive, his mother was sure he knew the difference between a care centre and a home.
And because she believed in it with all her heart, Terry was brought home every other weekend from then on. At the end of the first year after the accident, he was moved to a care centre closer to home. Now his family could visit him more often, but they continued to take him home every other weekend.
A year has passed, two years have passed. A decade had passed and the 20th anniversary of Terry’s accident was approaching, but his parents’ perseverance had not yet worn off. His mother increasingly noticed her son’s mild reactions to the environment. One time he winked, another time he laughed. Once, as they were driving home, she asked him a question and he banged his head on the seat. Angilee didn’t notice anything behind the wheel, but her husband knew he had answered her.
Doctors changed his state from vegetative to minimally conscious. Still, no one expected anything from him.
Nineteen years after the accident, Angilee entered his room. She greeted him. Suddenly, the word came out of his mouth. She almost collapsed with shock. She burst into tears, hugged him and wanted him to repeat the word again. Again she heard, “Mama.”
Terry laughed and said “Mum” again. He said nothing more that day, but after 19 years of silence, he suddenly spoke. That weekend he was brought home again. Angilee managed to get him to say the word pepsi. The next night she turned him over at 4am, as he always had to be turned over. She noticed that he was trying to tell her something. She encouraged him to keep trying. After great agony, he said, “Mummy and the eyes.”
When she asked him the next day what he wanted to drink, she expected him to say Pepsi. It was the only word he knew. She heard the word milk. Now he has been assigned a speech therapist at the care centre. His words were still clumsy and difficult to understand, but he was slowly learning to speak.
Why did Terry wake up?
Now Terry has attracted the interest of the experts. Why did he wake up and others didn’t? He remembered everything that had happened before the accident, but he could not remember anything new. He thought he was still 19 years old and George Bush was still President of the United States.
Experts have imaged his brain. They looked at the two shadow lobes located just behind the ears. They are responsible for processing experiences and turning them into memories. The right one was damaged and destroyed. But memories should be stored in the left one. Further imaging showed that it was also damaged. For example, because of this, Terry thought he could walk, even though he could not, and could not learn anything new about himself and the world around him.
The temporal lobes are also control centres for other parts of the brain and thus provide self-monitoring. Because they were not working, Terry spoke badly, even to his daughter Amber, who he still remembered as a newborn baby of six weeks. His young wife, however, went off with their daughter soon after the accident. No one resented her.
Terry’s brain was then imaged with fMRI and again 18 months later. For three years, the images were compared with images of healthy brains and people in a vegetative state to find out why Terry woke up and why he could talk.
Finally, the riddle was explained. In Terry’s case, the nerve fibres connecting the nerve cells were severed, but the cells remained intact. And because they were like that, they could slowly form new connections. Karen Ann Quinlan’s nerve cells, on the other hand, were damaged by the lack of oxygen, so recovery was not possible.
Terry did not wake up suddenly and speak, but new connections were slowly formed in his brain over 19 years until the web was untangled and strong enough for him to wake up. Unfortunately, the experts could not have known that he would, so they did not monitor his brain comparatively at the time, but they were able to compare the images they took over a period of 18 months.
They clearly showed new connections, further supporting their hypothesis that the brain was forming new connections during the vegetative and minimally conscious states. However, they could only make them up to a certain limit. Terry was still bed-bound, had to be turned, could not remember anything and spoke haltingly, but he was awake and talking.
Although testing the brain’s responsiveness in people who are in a vegetative state has advanced over the last decade and the level of awareness can now also be monitored by electroencephalography (EEG), which is much cheaper than fMRI and can be performed at the patient’s bedside, it is still not possible to predict which patients have a chance of recovery and which do not.
But it is possible to complicate matters. In theory, this could be a way of asking people who are in a vegetative state whether they want to live or die. But how reliable is their answer? Is the consciousness of a patient who says yes or no really so clear that they can make such an important decision, experts wonder? And is it acceptable from a legal point of view, especially if it goes against the will of the relatives? There are no answers yet.
