The Narcolepsy Network finds the answer through patients’ first-hand experience with narcolepsy.
I’ve been invited to write this editorial to discuss the “inside” experience of narcolepsy and the mutual support that sleep centers and patient groups can offer each other. While I have included personal incidents, we must remember that no one patient is typical and that each individual’s medical and support needs differ. I am 58 years old and was fortunate to be diagnosed promptly upon onset and description of my symptoms to a young family doctor about 22 years ago. Presently, I have two part-time jobs including a solo family law practice and serving as executive director of the Narcolepsy Network.
The network is a national nonprofit corporation both founded and directed by patients. Our mission is to meet the needs of families with narcolepsy by providing educational, referral, and supportive resources. We direct patients to sleep centers and, in turn, depend on centers to inform their patients about our assisting network and the comprehensive educational and social support we can provide. The symptoms of narcolepsy are so disruptive and so permeate the daily lives of those afflicted with it that any diagnosis and treatment provided by a sleep center remain woefully incomplete if not accompanied by an ongoing concern for assisting patients with necessary psychosocial adjustments.
The need that people with narcolepsy have for prompt diagnosis and comprehensive social support is made more difficult by the fact that the vast majority of those affected with the disorder remain undiagnosed. Prevalent studies estimate that the number of persons with narcolepsy in this country ranges from 125,000 to 200,000; but about 65% of these people have not yet sought the services of a sleep center and remain unaware that their symptoms represent a neurological disorder. The rest struggle an average of almost 15 years from initial onset of symptoms to eventual diagnosis during which time they remain untreated and/or misdiagnosed. Tragically, many narcoleptics are mislabeled and start to believe that they are lazy, unmotivated, irresponsible, or, even worse, suffering from depression, personality disorders, mononucleosis, fatigue, and other medical problems. The general public, including patients, primary care physicians, and health care professionals, remain alarmingly unaware of narcolepsy as a treatable neurological condition. Dispelling this ignorance will probably take a generation and will come from the combined efforts of sleep professionals, medical school faculties, scientific journals, and public media attention. (The publication of this guest editorial by Sleep Review is a welcome example.)
It is no surprise that a person with suspected narcolepsy who is finally referred to a sleep center shows up with an invisible burden of confusion and self-doubt. Often, a patient’s initial reaction to being diagnosed with narcolepsy is clear relief: “I really do have something with a medical name and it can be treated. I can’t wait to tell my family, my work associates, my peers, and friends!” What remains unclear, and often not discussed, is the effort that will be required over successive years to struggle with medication changes, lifestyle adjustments, and new ways of interacting with loved ones and peers. Narcolepsy’s impact on a person’s family, work, and social interactions is substantial and pervasive. Accordingly, the care provided by sleep center professionals must go well beyond mere diagnosis. This can be accomplished easily by informing a patient about local support groups.
Main Goals
The Narcolepsy Network’s specific goals include: serving as a national resource center; educating families, the public, and health care professionals about narcolepsy; developing and maintaining support groups; facilitating early diagnosis and effective treatment; participating in scientific research; and advocating to protect the rights of people with narcolepsy.
To date, we have about 3,000 members, which includes individual patients, their family members and friends, and professionals involved in both research and treatment of narcolepsy. We have dozens of patient support groups in cities across the country. The founders envisioned an organization to serve the needs of patients, and provided in our bylaws that the direction of the organization remains in the hands of patients. We are governed by a Board of Trustees—the majority of whom must have narcolepsy and are elected by our members. All programs and services were conducted by voluntary efforts until 1998 when we first employed an office staff. In 1999, our national office relocated to Cincinnati where it is presently administered by myself and Colleen Rettig, who serves as a part-time office manager. The operating budget has grown to $200,000, which is funded mostly by private, individual, and corporate donations along with patient and professional membership dues. Because many of our members are unemployed and/or disabled because of narcolepsy, membership is open to everyone, regardless of the ability to pay nominal annual dues of $35.
In addition to our national resource center, we distribute a quarterly newsletter, conduct national and regional patient conferences, maintain dozens of patient support groups (often in association with sleep centers), host an informative Web site (narcolepsynetwork.org), participate in clinical trials, and advise members regarding disability laws and other civil matters. Because narcolepsy is a neurological sleep disorder, we cooperate extensively with other organizations concerned with sleep deprivation and disorders including the National Center on Sleep Disorders Research, the American Academy of Sleep Medicine, the National Sleep Foundation, and the National Organization for Rare Disorders.
Sleep Center Support
Most important, we share a common concern with sleep centers of improving the disrupted lives of persons with narcolepsy. The referral of people with suspected narcolepsy and educational materials for competent and caring sleep professionals are the first and best way we serve members. Similarly, sleep centers’ assistance in maintaining support groups and informing patients of our services is the most effective way to help patients avoid isolation and assure ongoing comprehensive support for treatment. Some individual sleep centers develop and host their own support groups for their patients. More often, however, a sleep center will encourage and assist several of their patients to start a support network and will assist by providing a meeting space, educational materials, and mailings. For example, in Cincinnati, Columbus, and St Louis—each a major city with several excellent sleep centers—our patient support groups function independently from any one center, but cooperate with and ask assistance from all. Narcolepsy Network has excellent publications describing the start-up and activities of support groups.
Characteristic Symptoms
A closer look at the nature of narcolepsy’s characteristic symptoms helps to explain the need for extensive patient support. The four classic symptoms are excessive daytime sleepiness, cataplexy, hypnagogic hallucinations, and sleep paralysis. These are often accompanied by secondary or auxiliary symptoms, such as disrupted nighttime sleep, automatic behavior, interruptions to memory and concentration, intense fatigue, and clinical depression.
Excessive daytime sleepiness (EDS) is commonly experienced as a recurring and overwhelming need to sleep or nap in both appropriate and inappropriate circumstances, and usually without feeling rested or refreshed afterwards. The experience of such sleep has been described in various ways. Personally, I feel it is unlike any other normal sensation of sleepiness; I describe it as a dizzying experience of being unwillingly swept away, like when standing in the surf and being lifted off your feet and washed away by a crashing wave. EDS also takes the form of sudden and unexpected sleep attacks. Using another personal example, imagine talking with your family at dinner and suddenly falling asleep in mid-sentence during which the first words are understandable, but the last come out as if from a dream; or reviewing your written notes, and finding the start of a line clearly legible, only to end in a toddler’s scribble.
If EDS is the first and universal symptom of narcolepsy, cataplexy is, according to many, the most unusual, dangerous, and threatening to self-esteem. It is estimated to affect only about two thirds of persons with narcolepsy. But when present, it is regarded by many sleep physicians as diagnostic of narcolepsy. Cataplexy is a sudden loss or paralysis of muscle tone, and may be partial or total. When partial, it may appear as a sagging jaw, twitching face, slumping head, thick and unintelligible speech, limp arms, and buckling knees—all depending on which muscles are affected. And when one is in total cataplexy, it can cause an abrupt collapse to the ground with no control over the direction of fall. One’s head can strike whatever object may be in the way. This can result in bruises, sprains, lacerations, and concussion. (I have experienced all—on concrete, in water, off docks, and climbing stairs.) Severe injuries and even death may result from such a sudden collapse. An attack of cataplexy may last from less than a minute up to a half-hour before normal muscle tone is regained. When such attacks occur in public, well-intentioned paramedics, police, or lifeguards quickly respond. Fortunately, most attacks are brief; and even when the attack is total, a person can often stand up after a few moments. (What a profound moment when a person tries to explain narcolepsy and cataplexy to concerned and confused onlookers!)
Like EDS, cataplexy is a normal sleep behavior occurring at an abnormal time. It is characteristic of rapid eye movement (REM) sleep—that stage of nighttime sleep during which we dream. The loss of muscle tone is nature’s way to prevent us from acting out our dreams. But for people with narcolepsy whose sleep is fragmented, such muscle paralysis suddenly appears often without warning during daytime wakefulness. It is usually triggered by an emotional response such as laughter, anger, surprise, competitive exertion, or elation.
The two other primary symptoms of narcolepsy are hypnagogic hallucinations and sleep paralysis, which can also be quite frightening. But because they usually occur immediately before and after sleep, they are less visible to the public. The hallucinations occur at sleep onset and are frequently experienced as vivid, realistic, and personally threatening intrusions into one’s home or bedroom by strangers, animals, or even spirits (personally, I find them so real that I would never keep a weapon near our bed).
Sleep paralysis may accompany the hallucinations, but often occurs independently. It is a frightening experience upon falling asleep or awakening, to be unable to move your limbs, talk, or even breathe deeply. Like the other main symptoms of narcolepsy, hallucinations and sleep paralysis are normal sleep behaviors characteristic of REM or dream sleep. Normally, dreams occur as a late stage of sleep. But for persons with narcolepsy who, because of fragmented sleep, pass into dreams immediately or even before falling completely asleep, the dream and/or paralysis is experienced when still somewhat awake.
Many persons with narcolepsy also experience secondary or auxiliary symptoms. Among these is automatic behavior, which is the performance of a routine task without conscious awareness of performing it and without later memory of it. An example might be when mowing the lawn, or even the neighbor’s lawn, without knowing you are doing it until you see it done. It is another form of sleepiness without knowing one is asleep.
Another auxiliary symptom is the experience of diminished memory and concentration skills. This is particularly disturbing to younger persons with narcolepsy and can be a substantial barrier to choosing or continuing a course of academic study or maintaining competency in the workplace.
Sleep researchers tell us that a healthy sleep regime including 8 hours of sleep with normal occurrence of the various sleep stages is essential both to consolidating the day’s learning into memory and to peak performance the following day. It is predictable, then, that the disrupted and fragmented sleep that is characteristic of narcolepsy would negatively impact memory and concentration.
Conclusion
This inside review of narcolepsy may explain why patients need and benefit from sleep centers, which provide a comprehensive approach to treatment. In addition to tests and prescriptions, this would include exploring patients’ successes and failures in making necessary psychosocial adjustments.
During recent years, I have had the opportunity to meet many people with narcolepsy. Their stories and determination to continually seek the right balance of medications, sleep habits, and personal lifestyle have been revealing and inspiring. We know that narcolepsy is only a part of who we are as people. Past discoveries as to the cause of narcolepsy with cataplexy and the development of new drugs to reduce symptoms owe a large part of their success to collaborative efforts. Communication between researchers and practitioners, cooperation between sleep centers and pharmaceutical companies that conduct trials, and the willing participation of patients and support groups provide necessary input into this major scientific breakthrough.
Experience has taught us that the individual sleep center is pivotal to positive patient response. We at Narcolepsy Network need and welcome such centers as partners and professional members, assuring prompt availability of our educational resources and support for patient groups.
Robert L. Cloud is the executive director of the Narcolepsy Network Inc, Cincinnati.
