Narcolepsy is a neurological disorder that affects the natural cycles of sleeping and waking. It is characterized by excessive sleepiness during the day. This rare disease affects 2–5 out of every 10,000 people (1). No cure of the disease is currently possible. Therefore the treatment concentrates on relieving the symptoms as far as possible.
Orexin is a natural hormone produced in our brain that helps us to stay awake (2-4). In most people with narcolepsy, the small region of their brain where orexin is produced is not working properly. Certain cells in this area are irreversibly damaged by an autoimmune process – that is, the patient's own immune system has attacked the cells in this brain region. The probability of this type of autoimmune reaction is partly inherited. Among people who have a genetic disposition towards this autoimmune condition, the risk of developing the disease may be increased by infections or by the vaccination against swine flu (5).
Healthy people sleep in cycles of about 90 minutes each. A normal sleep cycle includes phases with stable and deep sleep, followed by a phase with rapid eye movement (REM). In the REM phase, the person’s eyes move around quickly while their eyelids are closed, and this is the phase in which we have most of our dreams (6).
In people with narcolepsy, the sleep cycles are disturbed. Instead of spending time in the “non-REM" phases of stable and deep sleep, patients go into REM phase shortly after falling asleep (7).
The classic symptoms of narcolepsy include abnormal sleepiness during the day, disturbed sleep at night, hallucinations, sleep paralysis and cataplexy. The intensity of the symptoms can be very different from one patient to another, and some symptoms (e.g. cataplexy) may be completely absent (8).
The sleepiness during daytime is the central symptom of narcolepsy. Patients often describe it as irresistible and uncontrollable. They feel tired and lethargic during the day and may fall asleep suddenly. These naps are often very short, and the patients feel refreshed and more alert afterwards (7,9).
One consequence of daytime sleepiness may be ‘automatic behaviours’ in which patients continue activities being half-asleep - these actions may be executed with errors or purposeless (7).
A less well-known but common symptom of narcolepsy is disturbed sleep at night, in which patients wake up frequently (9).
Narcolepsy patients often suffer from intense hallucinations while falling asleep or waking up. These can include seeing, hearing or touching things that don’t exist in reality but are often experienced as highly realistic. The hallucinations are mainly perceived as threatening or unpleasant (7).
In healthy people sleep paralysis, which is the total inability to move, is normal during sleep and prevents them moving around while dreaming. Patients with narcolepsy, in contrast, often suffer from sleep paralysis at the beginning or end of sleep and they may consciously experience being paralysed. This could last from a few seconds to some minutes and can be a very frightening experience (7).
Cataplexy is the term for a brief temporary loss of muscles tonus. It ranges from mild cases, in which for example the facial muscles go limp, to severe forms, where a patient loses the strength to stay upright and drops. Episodes of cataplexy can be triggered by emotions such as laughing, anger or surprise and usually last only a few seconds (10).
Narcolepsy can have very negative effects on quality of life. Patients regularly experience social exclusion – due to the symptoms and their own efforts to manage them, and also to a lack of understanding of their condition by the people around them.
Narcolepsy can also cause problems with partners and family. If sufferers try to avoid emotional situations, they may appear reserved and distant. Talking openly about the disease can improve people’s understanding of the situation and restore patients’ self-confidence. This is a key to maintaining as active a life as possible.
Since daytime sleepiness also causes a higher risk of accidents, narcolepsy also influences the kind of work patients can do. Some jobs are incompatible with the disease, for example driving motor vehicles, operating hazardous machinery, or working at height (for example as a roofer). Monotonous activities can increase the risk of falling asleep, and irregular working hours can make the disease worse overall.
People with narcolepsy can take several precautions that make travelling easier. Travelling with a trusted companion is a great help. If this is not possible, many airports and railway stations offer help in the form of support staff or wheelchairs. These can help to avoid agitation and physical exertion that could trigger cataplexy. To enable quick and correct treatment in emergencies, it is recommended to carry an emergency medical information card with you. It is also important to note that many medications prescribed for narcolepsy fall under controlled substances laws, and formalities have to be observed when travelling with them. For trips of up to 30 days in duration within the Schengen area, a note from the prescribing doctor, certified by the district medical officer, must be carried (1). For travel outside the Schengen area, the patient must check the legal situation in the country or countries they are travelling to and must take appropriate measures to comply with the rules.