Chapter 10
Falling Asleep Standing Up: Narcolepsy and Hypersomnia
In This Chapter
Looking at narcolepsy
Figuring out what causes narcolepsy
Spotting narcolepsy’s symptoms
Getting medical help
Living with narcolepsy
Recognizing hypersomnia
You may think of narcolepsy as some rare, exotic disease; however, it’s not that uncommon. Although narcolepsy afflicts 1 in 2,000 Americans, it affects people in different ways. And unlike in movies such as Rat Race, narcolepsy isn’t a laughing matter. Notwithstanding its unique presentation and prevalence, narcolepsy usually remains undiagnosed for many years. Why? One reason is that many primary care physicians (PCPs) don’t know to suspect narcolepsy when their patients complain of overwhelming sleepiness and unrelenting fatigue. Why? Because sleep medicine likely wasn’t in the curriculum at their medical school when they trained.
For years, even decades, people who suffer from narcolepsy have been misdiagnosed, misunderstood, and frequently mistreated. For a long time, many psychiatrists and psychologists persisted in characterizing narcolepsy as a mental illness, a result of the patient’s desire to “escape” difficult or stressful situations.
Fortunately, a handful of insightful researchers combined their findings to determine that narcolepsy is actually a medical disorder with, at least in part, a genetic basis. Even though researchers still don’t entirely understand the condition, people with narcolepsy increasingly can access accurate diagnostic procedures and effective treatments. These improvements, along with effective counseling, help them deal with narcolepsy’s numerous psychological and social consequences. But patients may have to be a bit forceful and persistent to make sure they get the help they need to effectively manage their condition.
Although both narcolepsy and hypersomnia are characterized by overwhelming daytime sleepiness, people with hypersomnia don’t suffer from the muscle weakness or paralysis that may accompany narcolepsy, nor do they have sudden, unexpected episodes of sleep. However, hypersomnia is no walk in the park. People with hypersomnia, whether it be posttraumatic or idiopathic, stay in a persistent state of confusion and disorientation. They may also suffer from poor coordination, impaired concentration, and seriously diminished quality of life.
In this chapter, we take a look at narcolepsy and hypersomnia, which are both dyssomnias that cause excessive sleepiness. We discuss symptoms, diagnostic procedures, and different treatments. We also explain what distinguishes the conditions from each other.
Understanding Narcolepsy
Narcolepsy is more than just a condition that makes people fall asleep at inopportune times. It’s a neurological sleep disorder, triggered by a physical problem or defect deep within the brain. Narcolepsy isn’t related to poor sleep habits or a psychological illness, but rather it results from the brain’s inability to regulate sleep-wake cycles normally. According to the National Institute of Neurological Disorders and Stroke, narcolepsy is the third most frequently diagnosed sleep disorder among patients complaining of sleepiness.
Patients with narcolepsy repeatedly become drowsy during the day. They may fight off the feeling until they either become alert again or fall asleep. The sleepiness can occur in inappropriate places like while at a party or at inappropriate times, such as the middle of an important business meeting. Patients may also experience loss of muscle strength (or tone), paralysis, or hallucinations.
The classic description of narcolepsy includes unplanned sleep episodes that are called sleep attacks. Even though they may last for only a few seconds, sometimes they may continue for several minutes. Regardless of their duration, the person with narcolepsy is asleep or partially asleep. Even in cases where the patient remains somewhat aware of the surrounding envi-ronment during a sleep attack, he is unresponsive during the episode. Not all patients with narcolepsy have sleep attacks; some individuals mainly suffer from episodes of severe sleepiness.
Sleep paralysis: It’s for your own good
Evolution favors traits that improve survival. Over the eons, Mother Nature selects these traits based on their ability to enhance our vitality; in this manner, humanity has been designed for survival. Sleep paralysis is just one of those protective mechanisms. The brain is so active during REM sleep that sleepers would try and act out their dreams if their bodies weren’t paralyzed. Sleep paralysis temporarily disables the pathway between the brain and the muscles, so that even if you want to get up and fight that bull or grand plie your way across the stage at The Met, you can’t do it. Your brain keeps you safely in bed. You only think you’re a great matador or prima ballerina, and the world is probably a safer place for it.
In fact, some people actually suffer from a sleep disorder that undoes the paralysis during REM sleep so they actually get up and act out their dreams. They often hurt themselves in the process. This disorder, which is a parasomnia called REM sleep behavior disorder, usually is brought to the sleep specialist’s attention after a severe injury. It’s sometimes precipitated by antidepressant medicines but can also occur spontaneously.
In narcolepsy, sleep paralysis is problematic because it occurs at the wrong time; that is, it happens while the person is still fully or partially awake. As a result, the individual is aware that he or she is paralyzed. The inability to move at all usually leads to tremendous distress and feelings of helplessness, which is especially true when a person isn’t aware that the condition is temporary. (Note: There is also a parasomnia called sleep paralysis in which sleep paralysis occurs in isolation.)
In severe cases, sleep attacks may occur several times a day, and without warning. Sometimes in response to excessive daytime sleepiness, a person will take a nap. Patients with narcolepsy often awaken from daytime naps feeling refreshed and restored, at least temporarily. The feelings of fatigue and exhaustion later slowly creep back upon them, and the cycle begins again. Finally, the nighttime sleep may be fragmented and disturbed.
People with narcolepsy suffer from a rapid eye movement (REM) sleep abnormality and extreme daytime sleepiness. They still get enough REM sleep, but they do so at different times than people who don’t have narcolepsy. People with normal sleep patterns experience about 90 minutes of non-rapid eye movement (NREM) sleep at the start of the night, when their brain waves become slower and more regular, which is followed by a period of REM sleep, when the brain waves become more active again. (REM sleep is the part of sleep that is associated with dreaming; see Chapter 1 for more details.)
By contrast, in people with narcolepsy, the order and duration of NREM and REM sleep are abnormal. When they go to bed at night, they fall almost immediately into REM sleep without cycling through NREM sleep. REM sleep onset can be so rapid that the brain may not yet be fully asleep. Therefore, the person with narcolepsy may experience his or her dreams as vivid hallucinations. She may be aware of the loss of muscle tone and sleep paralysis that normally only accompanies dream states. Worst of all, sometimes this sudden onset of REM sleep doesn’t wait until bedtime. Sometimes an emotional response can trigger it in the middle of the day. Some people with narcolepsy also experience REM sleep or fragments of REM sleep at unpredictable intervals throughout the day.
Narcolepsy affects men and women in fairly equal proportions and occurs in all racial groups, although for reasons unknown, some people in some countries like Israel have a significantly lower incidence. Symptoms first appear between the ages of 15 to 25 years, although in cases with a genetic component, onset may occur much earlier. Some people are 35- to 45-years-old when their symptoms first appear, and older women may experience an onset of symptoms at menopause. Occasionally a child as young as 2 may exhibit symptoms.
Narcolepsy can be sporadic, meaning it appears with no specific pattern in the general population, or familial, meaning it has a genetic component. Familial narcolepsy affects 8 to 12 percent of people with narcolepsy. It appears earlier in life than the sporadic form, and it may affect more than one member of the same family. For more information on the genetic form of narcolepsy, see “The genetic component” section later in this chapter.
Narcolepsy affects an estimated 125,000 to 200,000 Americans, but because researchers don’t completely understand it, fewer than 50,000 patients have actually been identified and are receiving treatment. A recent study concluded that the average time between onset of symptoms and a correct diagnosis is 14 years.
Pinpointing narcolepsy’s causes
For years, no one had any idea what caused narcolepsy. People with narcolepsy were accused of everything from being lazy to being possessed by demons. In fact, some scholars believe that Biblical characters described as “possessed” or having “fits” may actually have been suffering either from narcolepsy or epilepsy. But now, researchers are focusing their attention on multiple factors that may work in concert to produce the condition’s characteristic symptoms. What is known for certain: Narcolepsy disrupts the mechanism that coordinates the different sleep states.
Hypothala-what?
The hypothalamus is one of the brain’s main control centers. It has the very important job of maintaining the body in a state of normalcy or homeostasis, meaning everything remains in balance and everything functions just like it should. Among its many tasks, the hypothalamus regulates blood pressure, body temperature, fluid and electrolyte balance, and body weight.
In general, the hypothalamus is the seat of physiological motivation. The hypothalamus mediates the basic drives — hunger, thirst, sleepiness, and libido. In a way, the hypothalamus is the brain’s Madison Avenue. It advertises to the behavioral system a need (drive) that activates you to get whatever you require to satisfy that need. For example, if your body needs nutrients, the drive you feel is hunger. The function of eating isn’t really to relieve hunger, it’s to provide nutrients. But hunger is the immediate motivation for people to seek food.
Some researchers are looking at a group of nerve cells that contain the substance hypocretin, located in a part of the brain called the hypothalamus, while other researchers are investigating the role played by human leukocyte antigens or HLA. Both factors have been implicated in the development of narcolepsy. Still other researchers have determined that an abnormality in the part of the brain that controls REM sleep is at least partially to blame.
In addition, researchers have located and described variant forms of genes that are strongly identified with narcolepsy. But not one of these factors is sufficient to cause narcolepsy by itself.
Severe head trauma has caused some cases of narcolepsy. This type of narcolepsy is called secondary narcolepsy (or posttraumatic hypersomnia), because it starts after the injury. And finally, because other cases start at the onset of puberty or menopause, some researchers think that hormonal changes may also play a part.
We discuss all these possible causes in the following sections.
Hypocretin neurons
Special neurons throughout the brain secrete a neurotransmitter called hypocretin or orexin. (A neurotransmitter is a chemical messenger that excites or inhibits activity between two nerve cells or neurons in your brain. Neurotransmitters are amino acids — protein building blocks — or peptides, which are compounds made up of two or more amino acids.)
Although scientists don’t fully understand all the functions of hypocretin neurons, they do know that these neurons seem to play an important role in how the human body functions because of the widespread distribution of hypocretin receptors in the brain. After researchers found many hypocretin receptors in the area of the brain known to regulate sleep-wake cycles, they postulated that hypocretin plays some sort of role in regulating sleep.
Researchers also discovered that people with narcolepsy have something that people with other sleep disorders or neurological conditions don’t have — low levels of hypocretin in their cerebrospinal fluid and markedly fewer hypocretin receptors in their brains. Researchers started investigating the connection between these low hypocretin levels and missing hypocretin receptors and narcolepsy, and have concluded that a relationship does exist.
Now the challenge is to discover what causes hypocretin receptors to die in narcoleptics’ brains and why their levels of hypocretin are so much lower than normal. The answers to these questions may point the way to the development of more effective treatments for narcolepsy.
Brain abnormalities
Some recent studies have indicated that people with narcolepsy are missing hypocretin neurons in their hypothalamus, so their levels of hypocretin are abnormally low. No one yet knows why these hypocretin neurons are missing; some researchers speculate that an infection, trauma, or an immune attack generated for unknown reasons from within the patient’s own body may have destroyed them.
Sleeping and waking are complex activities controlled by groups of neurons or nerve cells in many parts of the brain and the brain stem. These neurons and nerve cells perform a delicate balancing act to keep all parts of the brain and body in synchronization. Gene mutations may upset this balance and contribute to narcolepsy by inhibiting the brain’s ability to coordinate the timing of REM sleep.
The role of human leukocyte antigens
Another line of research is looking at the role that human leukocyte antigens (HLA) may play in narcolepsy. HLAs are proteins on the surface of white blood cells that play an important part in the body’s immune defense system.
Many people with narcolepsy have variants in their HLA genes, but many others don’t. In fact, some people in the general population have these variants but don’t have narcolepsy. When researchers discovered that many people with narcolepsy have a very high HLA concentration in their blood, they speculated that an underlying infection or traumatic injury may trigger an autoimmune response that leads to the onset of narcolepsy symptoms. However, researchers need to conduct more studies before they can prove that narcolepsy is an autoimmune disease.
The genetic component
Researchers have found some patients who experience an early onset of the symptoms of narcolepsy have a genetic component to their condition, but genetic factors alone don’t account for the condition. There has to be some other risk factor present, like a problem with the immune system, an infection, trauma, stress, or hormonal changes before the symptoms of narcolepsy appear.
Although narcolepsy doesn’t necessarily “run in families,” researchers have noticed an undeniable hereditary connection in up to 10 percent of cases. At the very least, relatives of a person with narcolepsy have a higher risk of developing the disorder. Still, compared to disorders than are strictly hereditary, like Huntington’s disease, the risk is comparatively small.
Head trauma
When someone suffers a head injury severe enough to produce lingering muscle weakness or loss of sensation, the patient may also develop the symptoms of narcolepsy. The resulting acquired condition is called posttraumatic hyper-somnia. Mild head trauma usually doesn’t cause narcolepsy, although it may cause hypersomnia. (For more on hypersomnia, see the section “Coping with Hypersomnia” later in this chapter.)
Hormonal changes
Many cases of narcolepsy show up at the onset of puberty while others start somewhere between the ages of 35 to 45 or at menopause. The timing is significant because during these periods, the body is experiencing changing levels of hormones, either increasing as in the case of puberty, or decreasing as is the case with menopause. As a result, researchers speculate that changing hormone levels may play some role in the development of narcolepsy, but they realize that more research is needed before the connection is clear.
Identifying narcolepsy’s symptoms
Not everyone with narcolepsy experiences the same symptoms. The most common symptom is excessive daytime sleepiness. But about 70 percent of patients also suffer from cataplexy. A smaller group of patients has sleep paralysis and/or hypnagogic hallucinations, or some less common symptoms like blurred vision or disturbed nighttime sleep. Only 15 to 25 percent of people with narcolepsy display all four of the major symptoms (excessive daytime sleepiness, cataplexy, sleep paralysis, and hypnagogic hallucinations).
Researchers believe that cataplexy, sleep paralysis, and hypnagogic hallucinations are all caused by an intrusion of REM sleep into wakefulness. These symptoms are considered REM sleep abnormalities.
Excessive daytime sleepiness
Excessive daytime sleepiness (EDS) is usually the initial symptom of narcolepsy and also the symptom that brings patients to doctors looking for some relief. Daytime sleepiness in narcolepsy can be debilitating and interfere with activities of daily living.
Some complications of sleepiness include
Depression
Difficulty in maintaining concentration at work, home, or school
Extreme exhaustion
Inability to stay alert when sedentary
Increased irritability
Involuntary sleep episodes (sleep attacks or microsleeps)
Lack of energy
Memory lapses
Persistent mental “fog”
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Cataplexy
Cataplexy is sudden but temporary paralysis caused by the loss of skeletal muscle tone without the loss of consciousness. Severe shock or trauma can bring it on, but cataplexy is most commonly associated with narcolepsy. In narcolepsy, cataplexy is brought on by emotion, often laughter.
Although sleepiness is a normal phenomenon that may be difficult for a doctor to distinguish as something unusual or noteworthy in a patient with narcolepsy, cataplexy is never normal. Cataplexy is exclusively a symptom of narcolepsy. It’s also one of the condition’s most distressing symptoms because it’s so unpredictable, can cause serious injuries, creates so much embarrassment for the patient, and is triggered easily.
Cataplexy parallels the disruption of muscle control that occurs during REM sleep, a recently discovered fact that added to doctors’ understanding of what narcolepsy is and how it affects the brain.
Cataplexy’s severity can vary significantly from person to person. Some people only experience a mild weakness in their head, neck, or arm muscles, which they can compensate for. But in severe cases, the patient experiences a complete loss of muscular control and suffers a sudden collapse. They may not be able to speak or even keep their eyes open, but they’re still conscious. Depending upon his location at the time of the episode and the attack’s severity, a patient with cataplexy may suffer serious or even life-threatening injuries.
Cataplexy can be extremely frightening for the patient, especially if the first onset occurs at an early age. After patients understand what they’re dealing with, they can overcome their fear, but episodes of cataplexy are still very difficult and stressful, no matter how well prepared a person may be.
Sleep paralysis
Sleep paralysis is similar to cataplexy in that it inhibits a person’s ability to move or speak, but the difference is that sleep paralysis occurs only when a person is going to sleep or waking up. The brain is halfway between waking and sleeping, so the person usually has an awareness of the event. Hallucinations may occur at the same time as sleep paralysis, and some people report a sensation of not being able to breathe.
Like hypnagogic hallucinations and automatic behavior, sleep paralysis isn’t exclusively a symptom of narcolepsy; badly disturbed sleep can produce episodes of sleep paralysis, and some families have a familial form of the condition.
Like cataplexy, sleep paralysis can be very frightening, especially the first time it happens. Some people are afraid that they’re permanently paralyzed or may even think they’re on the verge of death.
Sleep paralysis occurs in approximately 60 percent of people with narcolepsy. The good news is that sleep paralysis generally has a very short duration, from a few seconds to a few minutes at most.
Hallucinations
About 50 percent of people with narcolepsy experience hypnagogic or hypnopompic hallucinations. These are dream-like visions that occur during the transition from waking to sleeping or vice versa. Hypnagogic hallucinations occur when a person is falling asleep while hypnopompic hallucinations happen when a person is waking up. Hypnagogic and hypnopompic hallucinations are essentially dream activity that appears at the wrong time, and shouldn’t be confused with hallucinations that accompany some psychiatric disorders.
These vivid hallucinations may incorporate all the senses — touch, taste, scent, hearing, and vision. They may also involve a sense of movement or include elements of the person’s environment in the imagery portrayed. Frequently, the hallucinations are frightening or bizarre, which can lead patients to fear for their sanity.
Less common symptoms
Narcolepsy can also produce a variety of less common symptoms including:
Disturbed nighttime sleep: Even though many people with narcolepsy sleep an adequate amount at night, their sleep may be so disturbed that it’s not refreshing.
In addition to the interval and order of REM and NREM sleep being mixed up, people with narcolepsy may experience multiple nighttime awakenings and severely fragmented sleep.
Automatic behavior: Up to 40 percent of people with narcolepsy engage in something called automatic behavior. They may perform routine chores and tasks automatically without any conscious awareness of what they’re doing. For example, imagine that you’re leaving work and have a letter to mail. You decide that on the way home you’ll swing by the post office, which is only one block out of your way. You get in your car, turn on the radio, and proceed home — and you don’t remember that you were going to stop at the post office until you get out of the car. That drive home was done on “autopilot,” and you have likely engaged in an automatic behavior. Another type of behavior that can occur during a sleep attack is a continuation of an ongoing activity. For example, if someone is writing when she experiences a sleep attack, she may just keep on writing, but her handwriting will drift across the page or deteriorate into illegible squiggles. Similarly, someone who is cooking may accidentally start a kitchen fire.
After an episode of automatic behavior, you may have no memory of having performed the task at all. There have been documented instances of sleep-deprived soldiers who apparently fell asleep while marching, but who kept right on marching even though part of their brain was asleep.
Diagnosing and treating narcolepsy
An accurate diagnosis is essential in order for a person with narcolepsy to receive the treatment she needs to manage her condition. Unfortunately, many members of the medical community aren’t familiar with narcolepsy, so some patients don’t get their diagnosis quickly. When people first start to feel EDS, they may just think they need to sleep more. Typically most don’t seek medical treatment until the onset of the later and more dramatic symptoms like cataplexy.
EDS is such a common complaint in the sleep-deprived world that doctors don’t automatically ascribe it to narcolepsy. It can also be a symptom of sleep apnea, insomnia, depression, and a host of other physical and emotional complaints.
A complete physical examination and medical history are the first steps in the diagnostic process. Your doctor asks you to keep a sleep diary (see Chapter 2), noting the time and duration of your sleep attacks and episodes of extreme sleepiness. Then it’s time for a visit to a sleep lab.
Sleep lab tests
To ascertain a diagnosis of narcolepsy, you need a polysomnogram (PSG) and multiple sleep latency test (MSLT). The PSG is an overnight test conducted in a sleep lab that measures abnormalities in sleep cycles by continuously monitoring a variety of bodily functions including heartbeat, respiration, and brain waves. (See Chapter 3 for more information on the polysomnogram and MSLT.)
The morning after the PSG, the patient gets a MSLT that monitors the tendency to fall asleep and how long it takes the patient to fall asleep. A sleep latency of five minutes or less indicates excessive sleepiness and suggests the possibility of narcolepsy. Patients with narcolepsy also have REM sleep on two or more naps during their MSLT.
Assessing available treatments
Patients with narcolepsy must work with their doctors to discover the best treatment or combination of treatments to manage their condition. A drug that works reasonably well for one patient may not work at all for another, or a patient may experience debilitating side effects from a particular drug therapy that make continuing the medication impossible. The next section looks at the most common currently prescribed treatments.
Drug therapy
The ideal treatment for narcolepsy would increase daytime alertness, reduce sleepiness, and eliminate sleep attacks and cataplexy. Although no cure for narcolepsy is in sight, in the vast majority of cases you can effectively manage the most troubling symptoms, EDS and cataplexy, with prescription drugs.
As helpful as drugs can be to manage narcolepsy, complete control of symptoms sometimes isn’t possible with the medications that are currently available. Therefore, new treatments are being developed and tested.
The original treatment for EDS was amphetamines, central nervous system stimulants that control sleepiness and reduce the incidence of sleep attacks. However, these stimulants had significant side effects including
Anxiety
Dizziness
Drug tolerance, which requires larger and larger doses to achieve the same therapeutic effect
Headache
High blood pressure
Irritability
Nausea
Physical and psychological drug dependence
Shakiness
Weight loss
What about HLA typing?
HLA typing is a blood test that measures levels of human leukocyte antigens or HLA in the bloodstream. Although researchers were excited when the test was first introduced, in many cases the test produces no relevant information. Many people in the general population have HLA profiles that are common among patients with narcolepsy; however, they don’t have narcolepsy. Conversely, some people with narcolepsy don’t have the HLA patterns usually associated with narcolepsy. The HLA connection is more clear-cut in patients with cataplexy, but a positive result on this test still isn’t a slam-dunk diagnosis.
In 1999, the Food and Drug Administration (FDA) approved a new non-amphetamine stimulant called Modafinil to alleviate EDS in narcolepsy. Modafinil has proved to be an effective treatment that doesn’t have the same undesirable side effects as amphetamines, yet produces similar good results. Another plus is that long-term use doesn’t appear to lead to tolerance, and it doesn’t interfere with scheduled daytime naps or the duration or quality of nighttime sleep.
However, Modafinil doesn’t work well for every patient, and it does have some side effects including
Congestion in the nose and throat
Diarrhea
Dizziness
Dry mouth
Headache (the most common side effect)
Nausea
Nervousness
Potential interference with hormone-based birth control
Swallowing a controversial drug to help people with narcolepsy
Gamma-hydroxybutyrate (GHB) has a deservedly bad reputation among law enforcement officials and college coeds, because it’s a notorious “date-rape” drug that sexual predators can synthesize on a stove top. In 2000, Congress passed a bill adding GHB to the list of controlled substances, making it illegal for anyone to manufacture or possess the substance without a prescription.
All the time this controversy was swirling, Phase II and III clinical trials of GHB for use in controlling symptoms of narcolepsy were proceeding. GHB proved to be very effective in reducing cataplexy, even in cases where nothing else worked. It also helps people with narcolepsy consolidate their nighttime sleep, which in turn reduces daytime symptoms. Finally, it happens to reduce sleep paralysis and hallucinations.
In October 2002, the FDA approved a particular formulation of GHB called sodium oxybate (Xyrem) for the purpose of only treating narcolepsy. Two doses are administered orally; one at bedtime and the second four hours later. Xyrem produces extreme drowsiness.
You can control cataplexy by using one of two types of antidepressant — tricyclics, like imipramine, or the selective serotonin reuptake inhibitors (SSRIs), like fluoxetine. Although these drugs don’t have as many side effects as amphetamines, they still have some significant drawbacks to long-term use, including possible impotence, irregular heartbeat, and high blood pressure. As a result, researchers are still hunting for one effective drug therapy that has fewer side effects.
Optimal management of symptoms is unique to each patient and doesn’t come overnight; it may take months of fiddling with drug combinations along with the size and timing of doses to achieve the best therapeutic effect with the fewest side effects. Be patient and allow your doctor the leeway he needs to help you find and maintain a good quality of life. A reasonable goal is significant improvement of the symptoms, meaning fewer episodes or a reduction in the severity of the symptoms. At this point, expecting a complete elimination of all symptoms isn’t realistic.
Other effective treatments
Although drug therapy offers the best control of narcolepsy’s symptoms, patients can take several steps to increase their daytime alertness. Scheduling two to three regular daytime naps of 10 to 15 minutes can help patients manage their daytime sleepiness and increase their alertness. Avoid large meals and alcohol, which can both promote feelings of sleepiness. Timed exposure to bright lights, training in stress management, and regular exercise are also helpful in maintaining alertness. In addition, you can maintain regular bedtimes and practice good sleep hygiene to minimize the chance of developing another sleep problem like insomnia. (See Chapter 6 for more information on sleep hygiene.)
Looking at the prognosis
You can manage narcolepsy, but even with the best treatment, symptoms may worsen over time. EDS may get worse, and the patient may require additional medication to maintain daytime alertness. But other symptoms, including cataplexy, may decrease or even disappear for a while.
Unfortunately, narcolepsy doesn’t grant immunity from other sleep disorders, and some patients may find that they develop other sleep disorders unrelated to narcolepsy as they get older, such as sleep apnea or periodic limb movement disorder. These disorders require additional diagnostic procedures and separate appropriate treatments.
Living with narcolepsy
People who live with narcolepsy suffer from more than just the condition’s physical effects. Narcolepsy can impact every aspect of their lives, including their family and social relationships, their career choices and ability to make a living, and their self-esteem and mental health. Obviously, the severity of narcolepsy and the level to which it’s controlled make a big difference. Individuals with well-controlled narcolepsy can live virtually normal lives. If the narcolepsy is severe and/or less well controlled, lifestyle adjustments are usually needed.
Hope for the future — hypocretin therapy
The most exciting research on narcolepsy is on hypocretin therapy. In recently completed and published studies, the administration of hypocretin-1 (Hcrt-1) increased activity and alertness, produced longer waking periods and an increase in REM sleep without changing NREM sleep, and consolidated sleep patterns to a more normal model. The most exciting news is that administration of a single daily dose of Hcrt-1 completely eliminated episodes of cataplexy, the most dangerous and distressing symptom of narcolepsy. With smaller doses, cataplexy was reduced but not completely eliminated.
The next step is human clinical trials of hypocretin, which are still several years away. If they’re as successful as the animal trials, hypocretin may be available as a prescription within the next ten years.
How disability laws affect people with narcolepsy
If your narcolepsy is severe and only poorly controlled by drugs, you truly may not be able to hold a job. The Americans with Disabilities Act (ADA) requires employers to make reasonable accommodations for employees with disabilities, which includes, in the case of narcoleptics, allowing naps, restructuring the job or work environment, changing duties, and rescheduling the workday to avoid night shifts and rotating shifts. If you inform your employer right after your diagnosis of your condition and he refuses to make any accommodations for you, the business may be in violation of ADA regulations, and you may have legal recourse. If you feel like you have been turned down for a job for which you were qualified simply because of your condition, you may also have legal recourse under ADA.
Managing work and social problems
An effective way to manage narcolepsy is to establish a reliable network of support among family members, friends, and coworkers. Before you enlist help, first you have to educate people about narcolepsy. Tell your family, friends, and business associates about your condition so that they understand what you’re dealing with and also understand what they can do to help. Instead of keeping your condition a deep, dark secret, open up and share. Reach out and ask for the assistance you may need to make the most of your situation.
For example, if you don’t tell your boss you have narcolepsy, she may catch you sleeping at your desk, assume you’re a lazy bum, and fire you. But if you tell her about your condition upfront and ask to be allowed to take two 15-minute naps during the workday when your coworkers are taking their coffee and cigarette breaks, you may find an ally who is committed to helping you succeed in managing your narcolepsy.
If your boss doesn’t understand the situation and refuses to allow you to take scheduled naps in the office, consider finding another place to work where your supervisor is more understanding and sympathetic.
Still afraid to lay your cards on the table with your boss because you worry you’ll be fired, or maybe never even get the job to begin with? The sad truth is that many people with narcolepsy let their condition define them. They feel rejected and condemned by society because they’re afraid to reach out and ask for assistance in managing their condition. Many people with narcolepsy give up and go on disability, living marginal existences, all the more tragic because life doesn’t have to be this way.
Even with your best efforts, narcolepsy may still cause problems for you with friends and family members. Many people just can’t resist making unfavorable comments about someone who falls asleep without warning in the middle of the day. In today’s Puritan society, some people still equate daytime sleeping with laziness and other undesirable character traits.
Do your best to ignore nonsense of this sort, and surround yourself with loving and supportive people who like and admire you for who you are and who accept that you have a lifelong condition that must be managed on a daily basis.
Considering the needs of children with narcolepsy
If you notice your child suffering from excessive daytime sleepiness or instances of cataplexy, ask your doctor to schedule a diagnostic workup to determine if narcolepsy is causing those symptoms. If your child is diagnosed with narcolepsy, work with your doctor to develop a treatment plan to give your child as normal a life as possible.
Your child follows the example you set. If you’re matter of fact and low key, emphasize the importance of following the treatment plan, and don’t make a big deal out of the diagnosis, you can empower your child to manage narcolepsy effectively. On the other hand, if you fuss and fret over the child or let him overhear you talking negatively about his condition, you’re adding unnecessary feelings of shame and sorrow to an already difficult situation.
Teachers can be especially harsh with children who have narcolepsy. If they’re not included in the information loop, teachers may assume your child is lazy, inattentive, and uncooperative, or that your child comes from a home where bedtimes are lackadaisical and the parents too undisciplined to keep their kids on a good schedule. Think how much better it can be to enlist the teacher as your partner to help your child succeed. A child with narcolepsy already has plenty to deal with. As his parents, you must do everything in your power to help him manage the condition and maintain as normal a life as possible.
Narcolepsy and driving
The laws about people with narcolepsy driving vary from state to state. Some states allow diagnosed narcoleptics under a doctor’s care to drive while others forbid it. One point that’s inarguable is that untreated narcolepsy poses a significant risk for both the patient and for the other drivers who may be on the road. By contrast, a well-treated patient doesn’t pose any more threat than the general public. The bottom line: If you have poorly controlled symptoms, don’t drive. It’s simply too dangerous. Contact your driver’s license bureau for regulations concerning drivers with narcolepsy.
Leading a near-normal life
If you’ve just been diagnosed with narcolepsy, you may be feeling overwhelmed and wondering how you’ll ever lead a normal life again. By following your doctor’s recommendations and paying good attention to your medication regimen and scheduled daytime naps, you can achieve good control of your most troubling symptoms.
Although narcolepsy isn’t a psychological condition, many people with narcolepsy develop psychological symptoms because of the many difficulties they encounter in dealing with other people as they try to manage their condition.
Depression is quite common among people with narcolepsy. Because one of the most effective treatments for cataplexy is certain antidepressants, you may be able to establish good control of both your depression and your narcolepsy with the one medication.
Coping with Hypersomnia
Some researchers refer to hypersomnia as a sort of sleep drunkenness in which patients never really come to complete wakefulness, suffer from poor motor coordination, confusion, disorientation, and slowness. Their sleep is very deep and frequently prolonged, characterized by long intervals of NREM sleep. Napping doesn’t refresh a patient with hypersomnia, and nighttime sleep periods may last 12 hours or more. Hypersomniacs are difficult to wake up after they fall asleep. They may exhibit automatic behavior during prolonged periods of drowsiness. Patients may also suffer from headaches, fainting spells, and reduced circulation in the arms and legs.
Hypersomnia can either be idiopathic, meaning it arises from an unknown cause, or traumatic, meaning it’s caused by a severe head or central nervous system injury. After a person develops hypersomnia, it’s generally a lifelong condition.
Hypersomnia versus narcolepsy
People who suffer from EDS without cataplexy, sleep attacks, sleep paralysis, or sleep-onset REM don’t have narcolepsy but may instead be suffering from hypersomnia, or excessive sleepiness. Like narcolepsy, hypersomnia is a lifelong condition with no known cure. Patients with hypersomnia require the same sort of emotional support as people with narcolepsy because family members, friends, and business associates may persist in characterizing them as lazy.
Hypersomnia usually begins in adolescence and only rarely starts up in people older than 30. A particular form of hypersomnia, sometimes called Klein-Levin Syndrome, is periodic. Periods of hypersomnia may last for days or weeks and are interspersed with periods where the patient sleeps normally and doesn’t suffer from EDS at all, and then the hypersomnia reoccurs. During the periods of hypersomnia, there may be overeating and sexual acting out. This “periodic hypersomnia” is thought to be a hypothalamic defect.
Getting a diagnosis and treatment
Unfortunately, hypersomnia isn’t well defined and therefore isn’t all that straightforward to diagnose. Some patients have a family history of hyper-somnia; others have suffered a recognizable inciting event like a viral infection. However, many patients have no discernable reason for the development of the hypersomnia. In some patients, hypersomnia is associated with overeating and an overactive sexual drive.
Doctors perform a complete physical to rule out other causes for the hypersomnia, and then send the patient to a sleep lab for a PSG and MSLT exam to confirm the diagnosis. (See Chapter 3 for more on PSG and MSLT tests.) Your doctor first rules out possible neurological, psychiatric, and pulmonary diagnoses before she can make a diagnosis of hypersomnia.
After your doctor has made a diagnosis, she treats hypersomnia with amphetamines and non-amphetamine stimulants to control daytime sleepiness.