NARCOLEPSY: WIDE AWAKE AND DREAMING

By Julie Flygare

Because I'm narcoleptic I carry the water weight of the river border

between sleeping and waking. I'm heavy with traveling that line,

like women carrying their babies across the Rio Grande:

I can see both lands but live in neither.

                                                                   ––Melinda Phelps

 

 

 

On a Saturday in February 2011, ten-year-old Haven Cavnar took a nap in the middle of the day.

 

“This was unusual,” says her mother, Diantha Cavnar. “She’s an energetic girl. I thought she might be coming down with something.” Haven’s sleepiness did not pass. Instead, it continued and worsened. Soon, she was sleeping on the way to school, in class, and after school. Haven was ranked high in her class; her sudden energy dip was obvious to everyone.

 

Around the same time, Haven started to wake repeatedly from vivid dreams during the night. During the day, her knees would buckle under her at seemingly random times. A primary-care doctor ran tests for everything under the sun, suspecting such ailments as lead poisoning and mononucleosis, but found nothing.

 

Haven’s knee weakness continued and progressed, until one day she collapsed to the floor of her classroom. Shaken by the incident, Haven’s fourth-grade teacher cried as she brought the child to her mother, who teaches at the same school.

 

Haven went in for a MRI (magnetic resonance imaging). While waiting for the results, the doctor told Haven’s mom they should not leave the hospital premises and mentioned the cancer ward across the street. A staff member pulled her aside to tell her a room was already reserved for Haven in the cancer ward. “We were terrified,” Diantha says.

 

When the MRIs showed no cancerous abnormalities, the family was greatly relieved, but they still had no insight into what was happening with Haven, whose legs were now collapsing out from under her often. Haven saw more doctors and had more tests––including one for epilepsy—but they yielded no answers.

 

It was Haven’s teacher who first found the words narcolepsy and cataplexy and called Diantha. “I’d heard of narcolepsy,” Diantha recalls, “but didn’t know it was such a serious illness.” She read more about the autoimmune disorder online and it resonated with Haven’s symptoms.

 

To test for narcolepsy, Haven underwent a twenty-four-hour sleep study in late March at a nearby sleep clinic. “I was scared because they put sticky things all over my head,” Haven says, but “I was also excited about the possibility of finding out what was wrong.” A few weeks later, in early April, she was diagnosed with “narcolepsy with cataplexy.”

 

Narcolepsy is a chronic neurological disorder of the sleep/wake cycle that affects about one in every 2,000 people, including more than 200,000 Americans, 18,000 Canadians, and 400,000 Europeans.

 

Narcolepsy is popularly portrayed as causing individuals to fall asleep without warning, yet the full spectrum of symptoms is largely unknown to the public. For example, many people with narcolepsy frequently experience disrupted nighttime sleep (insomnia) and so are often awake during the night. Narcolepsy’s predominant symptoms are excessive daytime sleepiness, a sudden loss of muscle control (called cataplexy), sleep paralysis, and vivid hallucinations while falling asleep and waking up, which are called hypnagogic and hypnopompic hallucinations, respectively.

 

During these hallucinations, individuals with narcolepsy experience extremely realistic dreams. This can feel a lot like being in two places at once—both asleep and awake. Generally, people with narcolepsy have very active dream lives.

 

            The dream is a little hidden door in the innermost and most secret recesses of the soul,

opening into that cosmic night of the psyche.

 ––Carl Jung

 

Dr. Emmanuel Mignot is the director of the Center for Sleep Sciences and the Center for Narcolepsy at Stanford University’s School of Medicine, as well as a professor of psychiatry and behavioral sciences. His research has significantly advanced the medical community’s understanding of narcolepsy in recent years. Dr. Mignot describes hypnagogic and hypnopompic episodes as “dream-like auditory or visual hallucinations, while dozing or falling asleep.”

 

For Haven, these vivid dreams are the scariest part of having narcolepsy. While dreaming, she has seen strange figures in her room that she believed to be real.

 

One morning she asked her mother, “Was it today that you told me about someone breaking into people’s houses and shooting them?” Shocked, Diantha responded, “No, Haven, I never told you that. It must have been a dream.”

 

Researchers believe that people are not born with narcolepsy, though they may be genetically predisposed to it, and that it is an autoimmune disorder. Onset of symptoms might be triggered by environmental factors, such as an upper airway infection, and/or by the immune system mistakenly attacking specific brain cells. Typically, symptoms become apparent during the teen years or the early twenties. However, children as young as three years old have been diagnosed with narcolepsy.

 

The excessive daytime sleepiness is comparable to how someone without narcolepsy might feel after staying awake for 48 to 72 hours straight. A person with narcolepsy (before receiving treatment and sometimes even with treatment) often has difficulty staying awake for long periods of time, which can compromise alertness in school, at work, or while driving.

 

Haven’s knee-buckling and collapsing were episodes of cataplexy, a sudden loss of muscle tone often triggered by emotions such as humor, happiness, annoyance, and anger. Cataplexy, a symptom believed to be unique to narcolepsy, varies in location in the body and in intensity––from a slight buckling of the knee or a sudden slackening of the jaw, to the head snapping back or falling forward, to a full-body collapse.

 

To an onlooker, a person with narcolepsy experiencing severe cataplexy may appear to be fainting, passing out, having a seizure, or sleeping. Though unable to move, speak, or open one’s eyes during a cataplectic episode, individuals

remain fully conscious and aware of their surroundings.

 

Not everyone with narcolepsy has cataplexy, and the diagnosis is designated accordingly. Meg Smith (to protect her privacy and because of her high-profile job, we are using a pseudonym rather than her real name) is a thirty-two-year-old woman who was diagnosed with “narcolepsy without cataplexy” at age nineteen. Her symptoms began in high school, but worsened substantially during her sophomore year of college. “It hit me like a ton of bricks,” Meg says. “I went from being a really great student to not––seemingly overnight.” After a few challenging months, her doctor referred her to a sleep specialist.

 

Although narcolepsy affects about three million people worldwide, it frequently goes undiagnosed or misdiagnosed as epilepsy, depression, or other psychiatric disorders. In many acute-onset cases, extensive and costly hospitalizations result in no diagnosis at all. On average, three to five years pass between onset of symptoms and diagnosis.

 

Once Meg’s case was diagnosed, she was able to start a treatment regime to manage her symptoms with multiple medications, which can lessen symptoms but do not erase them, and diligent attention to her health. Meg works in the demanding field of financial trading, so balancing her work, medication, and symptoms can be challenging. She finds that the fast-paced and intellectually stimulating environment of her job helps to keep her energized. “I’m passionate about what I do, which is essential for my success,” Meg explains.

 

Healthy lifestyle habits can make a significant impact as well. Meg exercises regularly and eats six small meals a day. “Changing my eating habits has made a huge difference, I don’t crash after meals anymore.” Planning ahead also helps. “If I have an important social obligation at night, I come home straight after work to take a three-hour nap in preparation.”

 

Wake Butterfly, it’s late and we’ve got miles to go together.

                                                                                                                                ––Basho

 

Although there is no cure, through continuous research, Dr. Mignot and other scientists are learning more about this fascinating sleep disorder. In 1999, two independent groups of researchers narrowed in on a brain molecule that causes narcolepsy-like conditions in animals. In 2000, it was confirmed that the same molecule––or more precisely, the lack of it––in humans is associated with narcolepsy. It turns out that people with narcolepsy have very low counts or none at all of a neurotransmitter called hypocretin, which is also commonly called orexin.

 

People with narcolepsy are born with normal levels of hypocretin, but subsequently lose some or all of the cells. Researchers are coming closer to understanding why this takes place. It is believed that certain genetic and environmental co-factors contribute to the autoimmune malfunction in some people’s brains, ultimately leading to a loss of hypocretin and the onset of symptoms.

 

As ongoing research explores hypocretin’s role in the sleep process, one theory is that the neurotransmitter may regulate the boundaries between various stages of consciousness, which could explain why people with narcolepsy shift so frequently between stages of wakefulness, REM sleep, and non-REM sleep. There is also some indication of a possible link between the H1N1 flu and acute onset of narcolepsy, as outlined in the accompanying sidebar.

 

If a little dreaming is dangerous, the cure for it is not to dream less 

but to dream more, to dream all the time. 

––Marcel Proust

 

There are many unanswered questions about “normal” sleep and even about why humans sleep and dream. Narcolepsy research is helping to broaden our understanding of the sleep/wake cycle. As our scientific understanding evolves, people living with this disorder continue to adapt and move forward as well.

 

Haven, who is now in 5th grade, looks forward to social studies, art classes, and field trips. She has raised awareness in her school and in her community in Roanoke Valley, North Carolina, where few had heard of narcolepsy. Now many are strong supporters, and various community fundraisers are helping Haven’s mother to pay for medical expenses and ongoing care. Meg volunteers with the Narcolepsy Network, bringing her financial expertise to the national nonprofit organization.

 

Meanwhile, as efforts and research continue, people with narcolepsy (this writer included) remain wide awake and dreaming.