1. Age at Onset
2. Aging and Narcolepsy
3. Cause: Head Trauma
4. Diagnosis: HLA Typing
5. Diagnosis: Mental Disorder
6. Disability Claims
7. Disability and ADA Attorneys
8. Driver's License: Reporting to the Department of Motor Vehicles (DMV)
9. Driver's License: Suspension Due to Narcolepsy – How to Contest
10. Eating Disorders, Sleep Related
11. Employment and the Americans with Disabilities Act (ADA)
12. Employment: Informing Employer
13. Employment: Shift Work
14. History of Narcolepsy
15. Medications for Excessive Daytime Sleepiness
16. Pregnancy: Use of Stimulants
17. Pregnancy: Family Planning
18. Prevalence Among Ethnic Groups
19. Surgery and Anesthesia
20. Symptoms: Automatic Behavior
21. Symptoms: Sleep Paralysis
22. Newest Medications for Narcolepsy Treatment
1. Age at onset
QUESTION: At what age or ages do narcolepsy symptoms first appear?
ANSWER: “The first symptoms often develop near the age of puberty; the peak age at which reported symptoms occur is 15 to 25 years, but narcolepsy and other symptoms have been noted as early as two years. A second smaller peak of onset has been noted between 35 and 45 years and near menopause in women.”
SOURCE: Christian Guilleminault and Angela Anagnos. “Narcolepsy” in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2001; Chapter 60.
2. Aging and Narcolepsy
QUESTION: Do the symptoms of narcolepsy increase or decrease with age?
ANSWER: “Although it has often been assumed that incident cases of narcolepsy decrease with age, data published in 1998 suggest that this may not be the case, and, in fact, exacerbation of cataplexy in the aged patient with long established narcolepsy may not be rare. Such cataplexy may mimic cerebrovascular or cardiac events and may lead to costly and unnecessary diagnostic workups.”
SOURCE: Donald Bliwise. “Normal Aging” in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2000; Page 37.
3. Cause: Head Trauma
QUESTION: Is it true that physical injury or trauma, particularly to the head can cause narcolepsy? Can this be proven in some cases?
ANSWER: There are forms of narcolepsy that can occur from trauma. We called this type of narcolepsy, Secondary Narcolepsy. It is much less common than typical narcolepsy, which is thought to be due to genetic and environmental causes. Mild head trauma, when it produces sleepiness, usually produces sleepiness that differs from narcolepsy in that it is not associated with REM sleep phenomena such as cataplexy, hypnagogic hallucinations or sleep paralysis. It may be called Idiopathic Hypersomnia, although if the association with head trauma is clear it would be called Post-traumatic Hypersomnia. When narcolepsy with cataplexy occurs following head injury it usually is due to severe injury that produces observable neurological features such as weakness or loss of sensation. For most patients with narcolepsy it is highly unlikely that mild head trauma was associated with the development of narcolepsy. However, we do not have the means to be able to say for sure that some past episodes of head trauma was not the cause of the narcolepsy. It is not possible to prove a relationship by investigative means at this time, and the association with time of onset is all that we can go on.
SOURCE: Michael J. Thorpy, M.D., Neurologist, Director, Sleep/Wake Disorders Center, Montefiore Medical Center, Bronx, NY, 4/2000.
4. Diagnosis: HLA Typing
QUESTION: All my symptoms and tests point to narcolepsy, except the blood test. Are they accurate for diagnosis?
ANSWER: “The usefulness of HLA typing in clinical practice is limited by several factors. First, the HLA association is very high (more than 90%) only in narcoleptic patients with clear-cut cataplexy; clear-cut cataplexy is defined as episodes of muscle weakness triggered by laughter, joking, or anger. Muscle weakness episodes triggered by anger, stress, other negative emotions, or physical or sexual activity may not be cataplexy if joking or laughing is not mentioned as a triggering factor. In patients without cataplexy or with doubtful cataplexy, HLA DQB1*0602 frequency is also increased (40% to 60%), but many patients are DQB1*0602 negative. Second, a large number of control individuals have the HLA DQB1*0602 marker without having narcolepsy. Finally, a few rare patients with clear-cut cataplexy do not have the HLA DQB1*0602 marker.
Despite these limitations, HLA typing probably is most useful in atypical cases or in patients with narcolepsy without definite cataplexy. A negative result should lead the clinician to be more cautious in excluding other possible causes of daytime sleepiness such as abnormal breathing during sleep.”
SOURCE: Emmanuel Mignot, M.D., Ph.D. “Pathophysiology of Narcolepsy” in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2001; Chapter 59: 671-672.
5. Diagnosis: Mental Disorder
QUESTION: Should narcolepsy be considered a mental disorder? And if not, why is it listed as such in the Diagnostic and Statistical Manual of Mental Disorders, or DSM-IV?
ANSWER: Narcolepsy is not a mental disorder. However the distinction between mental and neurological disorders has been narrowing in recent years. Many mental disorders are associated with neurotransmitter abnormalities that may have their basis in organic changes in the microstructure of neuronal activity. Unfortunately, there is a large difference between mental disorders and neurological disorders with respect to how they are viewed by the public, health care workers, and insurance companies. For this reason, how the disorder is classified can have major implications with regard to reimbursement from insurance companies and can affect employment or disability status.
The DSM-IV includes narcolepsy in its descriptions, not because it is regarded as having a mental basis as compared to an organic basis, but because its symptoms overlap with those of many mental disorders and therefore the recognition of the features of narcolepsy by psychiatrists is important to prevent misdiagnosis. Other physical or organic disorders, such as breathing disorders, which can have similar symptoms to many psychiatric disorders are also detailed in DSM-IV. The need to ensure that narcolepsy is not misdiagnosed has taken preference over considerations of reimbursement and public perception.
6. Disability Claims
QUESTION: I have filed, or am wishing to file, a private or social security disability claim. What information can Narcolepsy Network provide to assist me?
ANSWER: This is an excellent question, and Narcolepsy Network is happy to provide you with written and other information to help you educate your claim examiner, hearing officer, lawyer, or others. These written materials include brochures, pamphlets, and papers prepared by Narcolepsy Network, National Sleep Foundation, National Institute of Health, American Academy of Sleep Medicine, Merck Diagnostic Manual, and Encyclopedia of Sleep Disorders.
In addition, we can provide a list of Social Security and other disability case decisions that have been decided by the Courts, and a paper describing Social Security Disability claim procedures.
While we cannot provide individual legal advice or representation, it is our hope that these materials will assist you in educating those representing you or deciding your claim. Finally, keep in mind that many claims are not successful until the second or third request for reconsideration or appeal.
Please contact us directly for such materials.
A list of attorneys in your geographical area who practice Social Security and disability law can be obtained by calling: 201-444-1415 or 800-431-2804.
7. Disability and ADA Attorneys
QUESTION: How can I find a disability attorney in my area for my possible Social Security Disability and/or American Disabilities Act matters?
ANSWER: Some attorneys who practice Social Security disability law also handle other employment discrimination matters, including American Disabilities Act (ADA) cases. Or it may be, in a larger law office, that different attorneys will practice in each area. The best source for a referral is an attorney that you or a family member or friend may be familiar with. (Even though this may not have been one who deals with employee matters, that attorney will often be able to refer you to such a specialist whom the attorney respects and recommends.)
Another alternative is to contact the local city or state bar association, as they often maintain a referral list to match attorneys with your case matter and geographical area.
A source for Social Security Disability attorneys is the National Organization of Social Security Claimant’s Representatives (NOSSCR) at 1-800-431-2804.
The last resort is to look through the yellow pages, using these headings: Lawyers, Employment Law, and Disability.
8. Driver's License: Reporting to the Department of Motor Vehicles (DMV)
QUESTION: I am a young man living in California with possible narcolepsy. My doctor and others recommend that I visit a sleep disorder center for diagnosis and possible treatment. But I have also heard that any doctor who diagnoses me as having narcolepsy is required, by law, to report me and my diagnosis to the Department of Motor Vehicles (DMV), and that my driver’s license will then be suspended or revoked. Is this true? I need my license for many purposes, including work, and will not be able to keep my job or support myself and family without it. And what can I do about it?
ANSWER: Unfortunately, your information is largely correct, and your fears well founded. However, not all diagnoses of narcolepsy are reportable. And California, like most states, provides for hearings in which you can contest the action and show why your right to drive should not be suspended or revoked.
The new California law, effective October 2, 2000, requires every physician who diagnoses a patient, who is at least age 14, as having a disorder characterized by “lapses of consciousness” to immediately report the patient’s name, birth date, and address to the local health officer, who will then send the information to the DMV. The patient can then expect a notice of suspension from the DMV.
“Disorders characterized by lapses of consciousness” includes conditions involving:
24. “A loss of consciousness” OR “Marked reduction of alertness or responsiveness to external stimuli” AND
25. “Inability to perform one or more activities of daily living” AND
26. “Impairment of the sensory motor functions used to operate a motor vehicle”.
Narcolepsy and Sleep Apnea are individually named and included as medical conditions that may progress to the level of functional severity described.
“Sensory motor functions means the ability to integrate seeing, hearing, smelling, feeling, and reacting with physical movement, such as depressing the brake pedal of a car to stop the car from entering an intersection with a green traffic light to avoid hitting a pedestrian crossing the street.”
“Activities of daily living” are also described as including the operation of a motor vehicle.
Thus, it seems clear that a diagnosis of narcolepsy, with symptoms of serious excessive daytime sleepiness, sleep attacks, and/or cataplexy, will, more often than not, trigger mandatory reporting. But it may be your physician will not find your narcolepsy to be sufficiently severe to meet these conditions.
Other exceptions, which do not require mandatory reporting, are:
1. A patient's sensory motor functions are so severely impaired that he/she is unable to ever operate a motor vehicle. OR
2. The patient states he/she doesn’t drive and never intends to, and the doctor believes this. OR
3. The doctor previously reported the diagnosis and believes that the patient has not operated a motor vehicle since that time. OR
4. Another doctor previously reported the condition, and the present doctor believes the patient has not since operated a motor vehicle.
The California Vehicle Code also contains the following provisions regarding notices of suspension, hearings, and appeals:
- 10 days certified mail notice, setting forth the proposed action and the grounds therefore, including advice of one’s right to a hearing;
- no suspension/revocation to be effective until thirty (30) days after written notice (unless one’s mental or physical condition requires immediate action for the safety of the driver or other persons);
- procedures to demand a hearing within 10 days (but failure to respond to the notice is considered a waiver of the right to a hearing, which may or may not be reopened upon a later request;
- the right to review the DMV’s records (including medical reports);
- the right to present evidence “concerning any facts relating to the ability of the applicant or licensee to safely operate a motor vehicle”;
- right to receive the hearing officer’s findings and decision together with notice regarding the person’s right to a review hearing.
So, in California as well as in other states, one must be prepared to prove both the ability to drive safely and the right to retain one’s license. This will mean educating the DMV, the hearing officer, and perhaps your own lawyer regarding narcolepsy and the extent of your particular symptoms. (Narcolepsy Network can provide you with helpful materials.) In preparing for hearings, you should request a letter or statement from your physician, if he/she can honestly state that you are under treatment, responding positively, know your limits, and are not a risk to drive, or at least that you believe you can drive safely, and the doctor knows no reason to the contrary. However, your physician will not be able to say this unless your symptoms and driving experience are summarized in the medical record. And they will not get there, unless you regularly keep your physician informed. This can be done by phone calls, letters, and even personal driving logs that can all be included in your medical file. It is also advisable, at such hearings, to bring a witness or two, and/or a letter or two from family members, friends, or even support group members familiar with your driving habits. It might well be worth the time and cost to obtain a local attorney familiar with California law and motor vehicle issues. This is more important than his/her knowledge of narcolepsy, about which you and your doctor can provide information.
It has been the consistent position of Narcolepsy Network to recommend persons with suspected narcolepsy and/or other sleep disorders to seek prompt diagnosis and necessary treatment. Moreover, we maintain that persons with narcolepsy, who need to drive, should be allowed to drive provided they have been professionally diagnosed and are receiving proper treatment, including any prescribed medications, and are responding to and maintaining such treatment with sufficient success that they can drive safely and alertly.
At the same time, we acknowledge that some persons diagnosed with narcolepsy may require a period of time to achieve sufficient control as to be able to safely drive. Others, because of the severity of symptoms or other health conditions, may never achieve this control. We recommend that do not attempt to drive, to avoid unnecessary risk of harm to themselves and others.
Please remember that most medications for narcolepsy are “controlled substances” or “dangerous drugs”, sometimes with a sedative effect. Driving under the influence (DUI) is not limited to alcohol, and the penalties are severe.
The present California regulations raise serious concerns regarding doctor and patient confidentiality. Moreover, we fear that California’s mandatory reporting requirement may defeat the very purpose it is designed to achieve, if it causes persons with narcolepsy or other sleep disorders to avoid seeking diagnosis and treatment. However, it is not the purpose of this article to discuss those issues. Rather, our purpose is to encourage prompt and professional diagnosis, and to assist those who do so and respond positively to their treatment, and who need to drive, to retain their driver’s license privileges.
WRITTEN by Robert L. Cloud, Atty., Past Executive Director, Narcolepsy Network, Cincinnati, OH.
9. Driver's License: Suspension Due to Narcolepsy – How to Contest
Persons with narcolepsy will occasionally receive a notice that their driver’s license is suspended, due to this sleep disorder. This may happen because of an accident in which one fell asleep, or from disclosing that one takes medication for narcolepsy, or even from mandatory reporting requirements in some states that order a treating physician to report the names of patients being treated for narcolepsy or other sleep disorders. Or it can result from disclosure, when applying for a license, that one has a physical condition that might cause “impaired muscle control”, “loss of consciousness”, “episodic confusion”, and other responses that affect ones ability to drive.
Usually the notice of suspension will specify a time period within which one can appeal the suspension, and the notice may indicate specific requirements, such as a letter from a treating physician.
Narcolepsy Network acknowledges that narcolepsy, if untreated, results in a significant risk for driving. The same is true for one under treatment for narcolepsy, but not yet responding positively to medications and other treatment. Such individuals should not drive.
However, it’s also true that a person who has been diagnosed with narcolepsy and is obtaining treatment by a physician may be, for those reasons, a safer driver than most. This, of course, depends on taking prescribed medications and complying with other treatment therapies. In this case, one knows the limitations of narcolepsy, when the risk of falling asleep or experiencing other symptoms might and might not occur, and is able to drive with the assurance of remaining awake and alert.
If your license has been suspended, we suggest trying to obtain a letter from your treating physician that you are responding positively to your treatment and appear to be able to drive safely. If your physician will not recommend your driving, perhaps the physician will at least indicate your treatment and your response to the same. If this isn’t possible, then you yourself must inform the suspending agency of your condition, your treatment, and your positive response. It may be necessary to educate your lawyer and the motor vehicle agency regarding narcolepsy, including how it is treated. Narcolepsy Network can provide you additional written materials for this purpose.
CHECKLIST OF ITEMS TO PROVIDE TO THE DEPARTMENT OF MOTOR VECHILES (DMV):
[ ] Letter from your physician acknowledging your diagnosis of narcolepsy, that you are receiving treatment under his/her care, are responding well, know your limits, and are not a risk to drive or that you believe you can drive safely and he/she knows no reason to the contrary.
[ ] Letter or statement from yourself stating why you believe you are safe to drive and the precautions you take to be a safe driver who complies with your doctor’s recommended treatment of your narcolepsy symptoms.
[ ] A NARCOLEPSY: QUESTIONS & ANSWERS brochure available from Narcolepsy Network that summaries what narcolepsy is including symptoms and treatment.
[ ] Any other paperwork or documents they require.
MATERIALS AVAILABLE FROM NARCOLEPSY NETWORK:
[ ] NARCOLEPSY QUESTIONS & ANSWERS – brochure that summaries what narcolepsy is including symptoms and treatment.
[ ] HAVING NARCOLEPSY & KEEPING YOUR LICENSE by Robert L. Cloud, Atty. – presentation handout from Narcolepsy Network National Conference, New York, NY 10/24/1999; 18 pages. Includes some information about California, New York, New Jersey, Ohio, Pennsylvania, and Texas.
[ ] DRIVING WITH NARCOLEPSY by Robert L. Cloud, Atty. – presentation handout from Narcolepsy Network, Ohio Regional Conference, Columbus, OH 07/20/2002; 2 pages. Includes some information about Ohio, Kentucky, Indiana, and Michigan.
[ ] FREQUENTLY ASKED QUESTION (FAQ) TOPICS – [1] NN FAQ TOPIC: DRIVER’S LICENSE Reporting to the Department of Motor Vehicles (DMV); [2] NN FAQ TOPIC: DRIVER’S LICENSE Suspension Due to Narcolepsy – How to Contest
10. Eating Disorders, Sleep-related
Patients have sometimes described patterns of uncontrolled eating in connection with some sleep disorders, including narcolepsy. This behavior is sometimes called a sleep related eating disorder, and is characterized by repeated instances of getting up to eat after going to sleep. The disorder usually begins in adulthood. Preferred foods are described as high calorie foods, and often include items one would not normally eat while awake. Often the individual will have no memory the next day of the night’s eating episodes. This sleep disorder is said to be different from “nocturnal eating syndrome”, in which patients remember what they ate at night, and are often unable to get back to sleep. The disorder is often accompanied by sleepwalking, periodic sleep movements, and eating disorders.
The literature reports successful treatment of this and related disorders with Clonazepam, Dopaminergic agents, and anti-depressants
SOURCE: R. Benca and R. Casper. “Eating and Disorders” in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2001; Chapter 98.
11. Employment and the Americans with Disabilities Act (ADA)
Narcolepsy is a life-long neurological disorder affecting sleep. Medical research has identified the cause to be the absence of neurotransmitters, normally present in the hypothalamus region of the brain, which produce hypocretin peptides essential to regulating the human sleep-wake cycle. It is not a mental disorder, although psychological problems can develop from misunderstandings and difficulties coping with the symptoms.
Many persons with narcolepsy, after being properly diagnosed and following the treatment recommendations of an accredited sleep disorder physician, are able to continue their employment. (However, this often requires a period of adjustment to medications, and may require some adjustments in work schedules or other minor accommodations from one’s employer.) For many others, however, their narcolepsy symptoms can be so severe as to prevent them from engaging in any employment.
The Americans with Disabilities Act (ADA) requires employers (with 15 or more employees) to make reasonable accommodations to the known physical limitations of an otherwise qualified applicant or employee with a disability. A qualified individual with a disability is one who satisfies the qualifications for the job, and who, with reasonable accommodations, can perform the essential functions of the job one holds or is applying for. Disability includes a physical impairment that substantially limits one or more major life activities. Major life activities are those that the average person in the general population can perform with little of no difficulty, but which may affect essential functions of a job. The excessive daytime sleepiness and cataplexy (loss of muscle control) symptoms of narcolepsy can impact one’s ability to perform these activities.
It has also been shown, in many cases, that reasonable accommodations may be authorized by an employer to allow an employee with narcolepsy to continue in productive employment. Examples are allowing and facilitating a few brief naps during the working day; avoiding late or rotating shifts; sometimes adjusting starting and ending times, and sometimes restructuring job duties or environments.
Enforcement of one’s rights under the ADA is by filing a complaint with the local Equal Employment Opportunity Commission or State Civil Rights Commission, within 180 days of alleged discrimination.
The Family and Medical Leave Act (FMLA) may also be of benefit to an employer and employee when the later is adjusting to the treatment and medication recommendations of a physician. The provisions of this act require an employer (with 50 or more employees at a worksite) to provide an employee (having at least 12 months of consecutive service) up to 12 weeks of unpaid leave per year for treatment or recovery due to the employee’s serious health condition. The employer is entitled to 30 days notice, and must maintain the employee’s group health insurance, and allow return to the same or an equivalent position. The provisions of this act may allow for the time period sometimes required to obtain professional sleep disorder testing and to adjust to recommended treatment and medications. Enforcement is through the Department of Labor and/or a civil lawsuit.
Finally, the symptoms of narcolepsy can be so disruptive as to prevent one’s ability to perform consistent employment. In this event, the only recourse is to apply for disability benefits, under a private or employer provided disability plan, if available, and under the regulations of the Social Security Act. Disability, under the Social Security Act, means the “inability to do any substantial gainful activity by reason of any medically determinable physical or mental impairment that can be expected to result in death or that has lasted or can be expected to last for a continuous period of at least 12 months.” (Again, narcolepsy is a chronic life-long neurological condition.)
Application for Social Security Disability is made through one’s local Social Security office. Application for benefits under a private policy should be in accord with the policy provisions, and benefits under an employer provider disability plan should be requested from the employer’s human resources department.
Attorneys in your geographical area who practice disability law can be obtained by seeking referrals from family, friends, or associates; using the yellow pages; using the Internet (e.g.: lawyers.com); or by calling the National Organization of Social Security Claimant’s Representatives (NOSSCR) at 1-800-431-2804.
12. Employment: Informing the Employer
QUESTION: If my boss fires me for sleeping on the job, but doesn’t know about my narcolepsy, do I have an Americans with Disabilities Act (ADA) case?
ANSWER: Usually not. It is not necessary to disclose your narcolepsy before or after employment, particularly if you are able to manage your symptoms with medications and/or healthy sleep and living routines so that it doesn’t interfere with your work. However, if your sleepiness or cataplexy interferes enough with your job duties that you are disciplined or terminated, it may be too late to request accommodation. An employer can’t be said to discriminate against an employee’s condition he does not know about. So if your symptoms require you to take prescriptive medications during work or if you need accommodations to work effectively (naptime, schedule change, etc.) you must inform your employer before or at least when these symptoms interfere with your job activities. This request should be made in writing, with support from your sleep physician if possible, and with you retaining a copy.
13. Employment: Shift Work
QUESTION: Can variations in work shift schedules, including rotating shifts and split shifts, cause or aggravate the symptoms of narcolepsy?
ANSWER: While variations and late or nighttime shifts are not reported to cause narcolepsy, it is well accepted that they will aggravate the symptoms and make living or working with this condition more difficult. It is also suspected that such shift changes may cause narcolepsy symptoms to become manifest when otherwise latent.
“Career counseling is also important because patients and their employers must be educated regarding jobs that patients with narcolepsy should avoid, including shift work, on-call schedules, driving and the transportation industry, or any job necessitating continuous attention for long hours without breaks, particularly under monotonous conditions. Some of these difficulties can be overcome if the employer recognizes the importance of short 15 to 20 minute naps every four hours during the daytime. In addition to scheduled naps, other behavioral approaches include a regular sleep-wake schedule, the avoidance of frequent time zone changes, and overall good sleep hygiene.” This same discussion recommends avoiding shifts in sleep schedule as part of narcolepsy treatment.
SOURCE: Christian Guilleminault and Angela Anagnos. “Narcolepsy” in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2001; Chapter 60: 680-681.
Accommodating the needs of a person with narcolepsy by assuring a regular work schedule and avoiding rotating or split shifts have also been required or recognized as reasonable accommodations under the Americans with Disabilities Act in recent court cases. (Citations available upon request.)
14. History of Narcolepsy
QUESTION: When was the term narcolepsy first used, and its symptoms described?
ANSWER: “The word narcolepsy was first coined by Gelineau in 1880 to designate a pathological condition characterized by irresistible episodes of sleep of short duration recurring at close intervals. He also wrote the attacks were sometimes accompanied by falls, or “astasias,” a condition later referred to as cataplexy. In the 1930s, Daniels emphasized the association of daytime sleepiness, cataplexy, sleep paralysis, and hypnagogic hallucinations. Calling these symptoms “the clinical tetrad,” Yoss and Daly and Vogel reported a nocturnal sleep-onset rapid eye movement (REM) period in narcoleptic patients, a finding confirmed in the following years. In 1975, participants in the First International Symposium on Narcolepsy, which was held in France, defined the syndrome as follows:
The word ‘narcolepsy’ refers to a syndrome of unknown origin that is characterized by abnormal sleep tendencies, including excessive daytime sleepiness and other disturbed nocturnal sleep and pathological manifestations of REM sleep. The REM sleep abnormalities include sleep onset REM periods and the dissociated REM sleep inhibitory processes, cataplexy and sleep paralysis. Excessive daytime sleepiness, cataplexy, and less often sleep paralysis and hypnagogic hallucinations are the major symptoms of the disease.”
SOURCE: Christian Guilleminault and Angela Anagnos. “Narcolepsy” in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2001; Chapter 60.
15. Medications for Excessive Daytime Sleepiness
QUESTION: How normal should I expect to feel with medication?
ANSWER: An often heard and common sense answer is that the goal of medication is to improve daytime alertness to as close to normal as possible. A more professional and perhaps realistic answer follows.
“Treatment options should be individualized depending on the severity of the symptoms, life conditions of the patients, and the specific goals of therapy. Optimal management usually takes weeks to months to achieve and requires continued communications among the physician, narcoleptic patient, family members, employer, teachers and others. Good treatment management typically produces significant improvement of the symptoms, rather than resolution of all symptoms.”
“Pharmacological treatment of narcolepsy is determined by the number and severity of the symptoms. Severe daytime sleepiness may require treatment with high doses of stimulant medication, and sometimes a combination of stimulants may be needed. Rare or infrequent cataplexy and other auxiliary symptoms may not require drug treatment, or a prn regimen may be adequate. Insomnia and depression may also require treatment. The treatment should be catered to the individual needs of the patient. For example, improved alertness throughout the day may be critical for students and working adults, but only at certain times of the day for others (e.g., driving times).”
SOURCE: Roza Hayduk, M.D. “An Overview of the Diagnosis and Treatment of Narcolepsy for Primary Care Physicians” in Sleep Medicine Alert, Volume 5, No. 1, Winter 2001, a publication of The National Sleep Foundation.
16. Pregnancy: Use of Stimulants
“The risk of teratogenicity with commonly used stimulants is uncertain because well controlled studies of stimulant use by pregnant women are unavailable. . . . Given the uncertainties, the benefits for any given patient must be weighed carefully against the potential risks. For many patients, it may be advisable to reduce or discontinue stimulants during attempts at conception and for the duration of pregnancy. The efficacy of commonly used stimulants for treatment of narcolepsy during pregnancy is probably similar to efficacy at other times.”
SOURCE: George F. Koob. “Stimulants: Basic Mechanisms and Pharmacology” in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2000.
17. Pregnancy: Family Planning
QUESTION: If my husband or I have narcolepsy, what are the chances of our children having it?
ANSWER: “ . . . The risk of a first-degree relative to develop narcolepsy – cataplexy has been shown to be only 1 to 2%. A larger portion of relatives (4 to 5%) may have isolated daytime sleepiness, when other causes of daytime sleepiness have been excluded. These percentages are important to keep in mind because they are helpful in reassuring patients regarding the risk to their children and relatives. A 1 to 2% risk is 10 to 40 fold higher than that to the general population but remains manageable. A 4 to 5% risk for daytime sleepiness is not negligible, but similar values have been reported for excessive daytime sleepiness in the general population independent of narcolepsy.”
SOURCE: Emmanuel Mignot, M.D., Ph.D. “Pathophysiology of Narcolepsy” in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2001; Chapter 59.
18. Prevalence Among Ethnic Groups
QUESTION: Does narcolepsy affect certain ethnic groups more than others?
ANSWER: We are not aware of definitive studies showing narcolepsy to affect one ethnic group more than another. However, certain prevalence studies have suggested that: “narcolepsy/cataplexy may be less prevalent in Israel and more prevalent in Japan.”
SOURCE: Emmanuel Mignot, M.D., Ph.D. “Pathophysiology of Narcolepsy” in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2001; Chapter 59.
19. Surgery and Anesthesia
QUESTION: What precautions should I take if I go under general anesthesia for my surgery?
ANSWER: Physicians, including anesthesiologists, have inquired regarding the management of sleepiness and cataplexy while under anesthesia, or after the patient comes out of anesthesia. Many of our patients too express concern about general anesthesia during surgery.
One potential problem is the interaction of medications, specifically stimulant medications, and tricyclic antidepressants. The effects of atropine and ephedrine are enhanced by tricyclic antidepressants. The effects of phenylephrine may be either increased or decreased. Sodium thiopental will prolong the sedation during anesthesia whereas ketamine may produce acute hypertension.
Inform your doctor about all the medications and supplements you are taking. Some professionals advise withdrawal of medication for narcolepsy to prevent possible cardiovascular effects. Since this may increase the symptoms of narcolepsy during withdrawal, other professionals prefer to adjust the dosage for anesthetics instead of discontinuing medications. Recovery room staff should be aware that you may have cataplexy, hypnopompic hallucinations or sleep paralysis when you come out of the anesthesia. They should be trained to manage the symptoms of narcolepsy and avoid resuscitating patients whose breathing is impaired during cataplexy. Select a doctor who is knowledgeable about narcolepsy and the interaction of medications and whose staff is supportive and understanding.
SOURCE: Meeta Goswami, M.P.H., Ph.D., Director, The Narcolepsy Institute, Montefiore Medical Center, Bronx, NY.
Reprinted with permission from The Network, Volume 13, Number 3, Summer/Fall 2000, quarterly newsletter of Narcolepsy Network, Cincinnati, OH.
20. Symptoms: Automatic Behavior
QUESTION: What is automatic behavior?
ANSWER: "Automatic behavior refers to episodes of purposeful but inappropriate behavior occurring in sleepy persons. It is associated with impaired attention and vigilance and with partial or complete amnesia for the events. The episodes my last minutes to hours, during which the subject engages in repetitive, meaningless activities or makes errors resulting from impaired vigilance, such as missing freeway exits, driving through stop signs, or writing nonsense. Sleep-deprived soldiers who fall asleep while marching provide an example of automatic behavior. Narcoleptic patients with automatic behavior may have hallucinations during the episodes.
Polygraph recordings of sleepy subjects engaged in boring tasks show repeated "microsleeps," which are usually NREM sleep in non-narcoleptic subjects but may include features of REM sleep in narcoleptic patients; these microsleeps are probably partly responsible for automatic behavior.
Automatic behavior may be difficult to distinguish from automatisms associated with partial complex seizures, absence status, postictal confusion, transient global amnesia, metabolic or drug-induced confusional states, fugue states, or simply daydreaming. The association with repetitive or boring tasks, the relief with stimulation, and the observation by others of preexisting signs of drowsiness may help differentiate automatic behavior from confusional states. Automatisms associated with partial seizures are often preceded by auras, show greater stereotypy than does automatic behavior, and are usually followed by postictal confusion. Transient global amnesia is usually an isolated event in elderly patient without an altered level of alertness; the bewilderment that often accompanies transient global amnesia is not part of automatic behavior."
SOURCE: Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2000.
21. Symptoms: Sleep Paralysis
This is a terrifying experience that occurs in the narcoleptic upon falling asleep or upon awakening. Patients find themselves suddenly unable to move the limbs, to speak, or even to breath deeply. This state is frequently accompanied by hallucinations.
During an episode of sleep paralysis, the patient is unable to move the extremities, to speak, or to open the eyes, although he or she is fully aware of the condition and able to recall it completely later. In many episodes of sleep paralysis, but especially the first occurrence, the patient may be prey to extreme anxiety associated with the fear of dying. This anxiety is often greatly intensified by the terrifying hallucinations that may accompany the sleep paralysis. With more experience with the phenomenon, however, the patient usually learns that episodes are brief and benign, rarely lasting longer than 10 minutes and always ending spontaneously.
SOURCE: C. Guilleminult and A. Anagenos. "Narcolepsy" in Principles and Practice of Sleep Medicine. Philadelphia: W.B. Saunders Company, 2000; Page 677.
22. Newest Medications for Narcolepsy Treatment
QUESTION: What are the newest medications for the treatment of narcolepsy?
ANSWER: The two newest medications on the market are modafinil (Provigil) and sodium oxybate (Xyrem).
Modafinil (brand name Provigil, manuf. by Cephalon, Inc.) received FDA approved 12/1998 and is used to treat excessive daytime sleepiness (EDS). The dosage recommendation is 200 - 400 mg/day. Provigil is called a wake promoting medication and is not considered a central nervous system stimulant because it may affect specific areas of the brain involved in normal wakefulness. Its wake promoting effect is similar to stimulants, but its overall effect on the body is not identical (eg. minimal or no effect on blood pressure and heart rate). The precise mechanism by which modafinil promotes wakefulness is not known. For more information, contact Cephalon, Inc., 145 Brandywine Parkway, Building 300, West Chester, PA 19308-4245; Professional Services Tel: 1-800-896-5855; Web site: http://www.provigil.com.
Sodium oxybate (brand name Xyrem, manuf. by Orphan Medical, Inc.) received FDA approved 7/2002 and is used to treat the REM sleep related symptoms of narcolepsy: cataplexy, hypnagogic / hypnapomic hallucinations, and sleep paralysis. The dosage recommendation is 4.5 - 9 g, taken in 2 nightly doses. Xyrem is also known as gamma-hydroxybutyrate (GHB). Although it is primarily used to treat cataplexy, hypnagogic / hypnapomic hallucinations, and sleep paralysis, it has also been shown to improve excessive daytime sleepiness and disrupted nighttime sleep by consolidating nighttime sleep. Xyrem consolidates sleep by increasing slow-wave sleep. Call the XYREM SUCCESS PROGRAM toll-free at 1-888-867-7426 or 1-952-513-6900 for answers to any questions you or your doctor may have. Orphan Medical, 13911 Ridgedale Drive, Suite 250, Minnetonka, Minnesota 55205; Tel: 952-541-9209; Web site: http://www.orphan.com
For more information on Xyrem:
Orphan Medical press release: http://www.orphan.com/articledetail.cfm?aid=4&id=342
FDA Talk Paper on the approval of Xyrem http://www.fda.gov/bbs/topics/ANSWERS/2002/ANS01157.html
CASE REPORT: NEW TREATMENT FOR CATAPLEXY by Daniel Loube, MD; Ralph Pascualy, MD; and Sally Soest, MS of Swedish Medical Center, Seattle, Washington in SLEEP REVIEW January/February 2003, The Journal For Sleep Specialists. "Sodium oxybate is a promising new alternative treatment to the stimulants, hypnotics, and tricyclic antidepressants that were previously prescribed for narcolepsy." http://www.sleepreviewmag.com/Articles.ASP?articleid=S0301D06
For Additional Information About Medications:
http://www-med.stanford.edu/school/psychiatry/narcolepsy/medications.htmlInformation about medications for the treatment of narcolepsy from STANFORD UNIVERSITY CENTER FOR NARCOLEPSY directed by Emmanuel Mignot, M.D., Ph.D., Administrative Offices, 701 Welch Road, Room 2226, Stanford, CA 94305
PHARMACOLOGY OPTIONS FOR NARCOLEPSY, article in Sleep Review magazine March/April 2003 by Robert A. Whitman, PhD, ABSM, RRT, RPFT. While the search continues for a cure for narcolepsy, researchers are studying hypocretin neurotransmission and treating patients with the latest drug therapies.
http://www.sleepreviewmag.com/Articles.ASP?articleid=S0303F02
http://www.medlineplus.govMEDLINEplus HEALTH INFORMATION. A service of the United States National Library of Medicine and the National Institutes of Health. Includes health topics, drug information, and a medical encyclopedia.
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