Every night for the last twelve years, Anne has slept by the phone. She suffers from multiple sclerosis, a cruel illness which restricts her mobility and whose symptoms, for her, include fatigue. But that is not the reason for the phone. Anne waits for calls. A few minutes after half past two in the morning, perhaps, the phone will ring.
Sometimes the caller is a nuisance, which is why Anne prefers to keep her surname to herself. But more often than not, somebody is ringing for a few words of reassurance. They ask Anne a straightforward question.
Am I still alive?
Anne tells them that, yes, they are alive and then they hang up, released from their confusion, at least for tonight.
The callers have narcolepsy, a debilitating malady best known for its more public symptoms. People who live with narcolepsy are prone to sudden attacks (and they are attacks) of sleep, often at inconvenient times, such as when they are under a shower or at a staff meeting. To be fair, anybody can fall asleep at a staff meeting; the condition of being able to stay awake during one is so rare that medical science doesn’t even have a name for it. But sleeping under the shower is another matter, as is falling asleep with a hot saucepan in your hand or while crossing a busy street. Narcolepsy doesn’t always give warnings of the onset of sleep; there is no dusk before dark. The waking state just goes off like a light.
The other public symptoms of narcolepsy include extraordinary sleepiness during the day and a condition known as cataplexy, the real hallmark of the condition, in which some of a person’s muscles suddenly lose their strength and that person may just fall to the ground, lose grip on something they are holding or perhaps have their facial muscles fall into an appearance over which they have no control. These situations may last minutes or seconds depending on the individual. They can be triggered by emotional surges such as laughing at a joke or becoming angry.
There is a private side of narcolepsy which is even more frightening.
One of the features of narcolepsy is the ability to slip straight from wakefulness into REM sleep. This can happen in other situations, such as with tired youngsters or those who have had sleep apnoea for years and finally discover effective treatment. But it is an aberration. For most people, as we saw just before midnight, there are four distinct stages of sleep which precede REM sleep and each of these is characterised by a different pattern of brain waves. These four stages culminate in ‘deep sleep’, a period in which the brain is least active and in which sleep takes over as the boss of your life. In this state, sleep regulates a number of tasks that need to be accomplished after the wear and tear of another day. That busy beaver known as the cerebal cortex has a break while growth hormone and melatonin do you favours which you would only mess up if you had any say over the matter. You don’t dream in deep sleep. Your mind gets out of the road so your body can look after itself. Deep sleep is the healthiest part of your day, the time when you’ve left the room so your body can talk about you honestly.
About an hour and a half after falling asleep, a profound change comes over the nature of sleep, marking the arrival of REM sleep. In this state of Rapid Eye Movement, the frenetic activity of your eyes behind closed eyelids indicates the brain is tired of being stuck on the sideline and wants to start playing again; more than that, it wants to captain the team. In deep sleep, your brain is still while your body is quiet and stable with slow regular heart rate and low blood pressure. In REM sleep, however, your body is physiologically active and your brain gets restless. Most dreams, although not all, take place in REM sleep when the body is paralysed, as a natural defence mechanism to stop us acting out our dreams, one of nature’s really good ideas. REM sleep is so different from the other parts of sleep that we can think of three states in our lives: wakefulness, sleep and REM sleep. It may seem strange that REM was not clinically observed until 1953. But there is a simple reason why a species so obsessed with itself seems to have overlooked such an important part of its daily behaviour for 40,000 generations. Everybody is asleep when it happens.
There is fierce debate over the function of REM sleep, an experience which is by no means unique to humans. There are people who believe REM is the heart of sleep, the culmination of the four stages that came before it, the crucial factor in the consolidation of memory, although the relationship between REM and memory is a particular bone of contention. Francis Crick, one of the discoverers in 1953, along with Watson and Wilkins, of the double helix of DNA, proposed in the 1980s that ‘in REM sleep, there is an automatic correction mechanism which works to reduce . . . possible confusion of memories.’ In other words, he saw REM sleep as integral to the working of the mind’s elaborate filing system, also known as memory. Others believe that REM has more affinity with wakefulness than sleep and is, in fact, a preparation for wakefulness.
Narcolepsy allows the rest of us to understand what can happen when the cycle of sleep gets disrupted and for some reason REM sleep decides to come before the other stages. People with narcolepsy are often in REM within minutes of falling asleep.
One of the symptoms of narcolepsy is sleep paralysis, a situation in which a person is awake, conscious and their mind is fully active. But they can’t move. For a few terrifying moments, the body and brain simply don’t seem to connect with each other.
The other private symptom is hallucination. The immediate onset of REM can bring immediate dreams. The problem is that without the slow process that usually leads to REM, a person with narcolepsy can have genuine problems telling if their dreams are real or not. They may wake up in the morning and head off to appointments they have only dreamed about, or start looking desperately for the keys of cars they have only dreamed they own. To make matters worse, the dreams are often nasty.
That is why they might ring Anne in the small hours. They have just dreamed that they are dead. They need an outsider to tell them this is not true.
Anne, a nurse, first encountered narcolepsy when she was training in 1956; one day she found a fellow nurse propped up against a wall, able to hear but not move. She later found herself married to a school teacher who has narcolepsy, a condition which is not life threatening but which has treatment rather than a cure. It is a long-haul illness. Anne’s husband waited for ages before he found a doctor who was able to respond appropriately. In the meantime, he would come straight home from school and fall asleep. He would then need another nap later in the evening to wake himself up enough in order to get himself to bed. Once he was in bed, he also had PLMD (Periodic Limb Movement Disorder), which meant that his night’s sleep usually cost Anne a few bruises. Some of the couple’s children also have narcolepsy; a genetic factor has often been observed but the condition is not inevitable. It is possible for one identical twin to have narcolepsy and the other not. Part of dealing with all this has meant, for Anne, being available to help others:
‘A woman rang me at some ungodly hour, just after half past two. There was an angel in her room so she needed to know if she was dead yet. I told her she wasn’t and she said that was fine but she sounded slightly disappointed. She said that it had been very pleasant flying around the room.’
None of this experience has led Anne, a devout Lutheran, to doubt her faith in her own dreams.
‘Ever since I was nine or ten years old, I have been getting dreams from God in which I have been told to pass on messages. On five occasions I was told to inform my aunt that she was pregnant. Twice she miscarried but three of those children are still alive. When my mother-in-law died, she hadn’t even been sick but the moment the phone rang I knew what it was. Some people are just given this biblical gift and I am one of them. I don’t know how it happens but God uses me. I come out with my voice but it’s God talking. So when I am counselling, I will be guided in certain ways. I am guided to what God wants me to say.’
Anne does not answer the phone between dawn and noon.
Narcolepsy is rare, although the incidence of it varies from country to country. It is more common than leukemia and affects a similar percentage of people to Parkinson’s Disease, at least 1 in 2,000. It was initially thought to be far more freakish and, like many sleep disorders, narcolepsy has a long history of not being taken seriously. It was first brought to book in 1881 by a French doctor, John Baptiste Edouard Gelineau, one of those characters in the history of medicine who gets a fresh perspective because he stands a little apart from mainstream research.
Born in 1828, Gelineau became a naval doctor and had some risqué adventures in the Indian Ocean, before settling down to private practice in a rural community where he had time and space to indulge his interest in natural history. He made his money when, in 1871, he developed a heady brew of bromide, antimony and picrotoxin which he marketed as a cure for epilepsy and sold in tablet form. The success of the product probably says more about the desperation, at the time, of families living with epilepsy than it does about the efficacy of the pills. The history of sleep medicine is likewise full of wonder cures. People will pay almost anything for a decent night’s sleep.
Gelineau went on to establish a neurological clinic in Paris. One day, a 38-year-old approached him with a bewildering problem. The man was a vital member of the local community: he sold wine barrels. This active businessman was prone to sudden episodes of sleep in any situation; he also had a proclivity to fall down for no apparent reason, a condition Gelineau called astasia but which we know as cataplexy. Gelineau’s observations were astute. He noted that astasia was different from epilepsy, in that an epileptic seizure tended to make muscles tighten and contract. Astasia had the opposite effect; the muscles turned to jelly and, furthermore, the attacks seemed to switch off as suddenly as they switched on. He rightly observed that these attacks seemed to follow occasions of strong emotion which, for a French wine barrel merchant, were not infrequent. Gelineau was also correct in his deduction that the problem was somehow located in the brain. In 1880, he coined the term narcolepsy to describe the whole complex. But the medical mainstream was not especially interested in the findings of a maverick and it was not until the discovery of REM in 1953 that his work was given credit. Meanwhile, Gelineau had found there were other ways to help people sleep. In 1900, he returned to Bordeaux to make wine.
Elizabeth Hickey became aware of the symptoms of narcolepsy following a bout of glandular fever when she was sixteen years of age. But her struggles with the condition go back even further. At school, she had trouble concentrating and was often in bother with teachers who liked her to sit near the front of the class so they could keep an eye on her. This old-fashioned approach to discipline may have some valid basis: by creating mild stress for the pupil, a teacher may be helping them release enough adrenalin to stay awake. Elizabeth’s behaviour might now be described as Attention Deficit Hyperactivity Disorder (ADHD); she believes her brain was doing gymnastics to compensate for poor sleep and to keep her awake during the day.
‘I am just so thankful that nobody in my family treated me like I was a bad person, so my self-esteem never suffered. Looking back, that was a huge positive.’
Elizabeth is middle-aged now and still has narcolepsy. The drug which enables her to function is Ritalin – interestingly, the same drug that can arouse controversy for its use in treating children with ADHD. There’s a theory that ADHD affects poor sleepers who get through the day by having numerous very short ‘microsleeps’ which may last only seconds but which are long enough to rupture concentration and reduce the world to fragments. There are others who’d say that poor sleep is a symptom rather than a cause of ADHD.
Elizabeth’s story shows all the classic signs of narcolepsy. At the age of eleven, she surprised herself by being able to swim across a pool. She was delighted by her achievement and the emotion triggered a cataplectic episode as a result of which she lost the ability to move her limbs and, except for the vigilance of a friend who fished her out, she would have drowned. At the age of sixteen she had a summer holiday job in a doctor’s surgery where she saw a man whose arm had been nearly severed in a car accident. The shock sent her into a paralysis that lasted five hours.
Years later, she found herself as the mother of twins living in Toowoomba in regional Queensland, and struggling to cope with profound exhaustion. She was so sleepy all the time that she found it hard to function but was inclined to attribute this to the demands of having two babies in the house. One day, some friends from Brisbane arrived unexpectedly on her doorstep. The sheer delight of seeing them once again activated her cataplexy and she fell to the floor.
Cataplexy is just one of a suite of symptoms. As a girl, Elizabeth had an extraordinary number of imaginary friends, each of which was absolutely real to her, and she now wonders if this was a result of narcoleptic hallucination. She can well recall driving with her children in the backseat and experiencing terrifying hallucinations about oncoming trucks. She has experienced sleep paralysis and, with that, the most frightening dreams.
‘I dreamed that things were eating me up. That I was being interfered with. It was horrible. The dreams would come with a real, physical pain.’
On top of that, Elizabeth would just fall asleep. Anywhere at all. At any time.
‘I had to give up the piano. I used to just fall asleep at the keyboard.’
Elizabeth’s suffering was intensified by the inability of anyone, including herself, to recognise the problem for what it was. When she was thirty-one, a sister-in-law suggested that Elizabeth’s inability to stay awake might be due to more than just her little twins. She got a referral to a neurologist but the very visit to the surgery caused sufficient adrenalin to be released that she was more awake than she had been for ages and the doctor ended up wondering why she had come to see him. Elizabeth says she then went into denial for a further eight years. Overall, it took twenty-five years of groping in the dark, of feeling like she was living under water, until, at the age of forty-one, another doctor was able to scratch the word narcolepsy into her file.
Narcolepsy is, admittedly, not always an easy diagnosis to make and not one to reach in a hurry. One of the tools in diagnosis is the Multiple Sleep Latency Test (MSLT) which needs to be performed in a sleep lab during the day. In this test, you are wired up to an electroencephalograph (the EEG) and asked to fall asleep, a number of times, during a period in which you would otherwise be up and about. The test can see how readily you fall asleep, which is called sleep latency, a simple but effective measure of sleepiness and hence an important clue to sleep deprivation. The EEG can also see if REM sleep jumps the queue, and pushes its way into the initial stages of sleep causing no end of trouble. But the MSLT is fallible, partly because it takes place in such odd conditions for sleep. It is possible to do well on the test and still have narcolepsy. It is equally possible to have early onset REM and not have narcolepsy.
Family and community have been significant in enabling Elizabeth to live more comfortably with the condition; for her, community has meant church community as well as the community of other people forced to share their lives with narcolepsy. Both diet and medication have also played a major role. For various reasons, weight gain is a factor in a number of sleep disorders.
Current thinking is inclined to attribute narcolepsy to low levels of hormones called orexins (also called hypocretins) which are produced by a part of the brain called the hypothalamus, an amazing little gadget which is a bit like the brain’s brain. It regulates sleep, appetite and body temperature, three aspects of our lives which are closely related. Disturb one and the chances are you will disturb the other. Orexin levels are often measured by a spinal tap, an intrusive procedure which can have slight risks of its own. Certain drugs which can help with narcolepsy, such as modafinil, have been found to stir the orexins. But Elizabeth uses large amounts of Ritalin and has long argued that the maximum recommended doses of this drug are nowhere near sufficient for her needs.
‘If I don’t take enough, I may as well not take any.’
Ritalin is not a substance to be toyed with and Elizabeth is well aware that there is a list of drugs, including some antidepressants, with which it has a dysfunctional relationship. Nevertheless, although Ritalin is often used to keep people awake, for her it means she can get into the depths of sleep without having to wade through shoals of hallucination. At the end of the day, she finds it is important she gets to bed before the effects of the drug have worn off. Otherwise, she can end up spending several hours in the kitchen, pottering around with no idea what she is doing or why. This is called automatic behaviour, a form of waking sleepfulness which can beset anybody but is part of a range of sleeping disorders, especially narcolepsy. At this stage, only further medication will get Elizabeth to bed. She needs to wake up enough in order to sleep.
It’s been a long journey for Elizabeth and thousands like her. She wanted to be a doctor and did well enough at school to qualify for the course. But in the final years of high school she was already falling asleep so frequently that she doubted she could cope. She took a degree in pure mathematics and psychology. Despite such obvious achievements, her condition led to self-doubt.
‘I used to be so ashamed. I was working as a computer programmer. This was in the early days of computers when they had punch cards. I would be falling asleep at the keyboard. It was humiliating.
‘My journey has been one of self-acceptance. Before I was properly diagnosed, narcolepsy just took me over and engulfed me. There was nothing of me left. Then I met people with the same disorder who were really learning to live their lives. Now I can see myself as a person who just happens to have a disorder. I am a person. Narcolepsy is a disorder. There’s a difference.’