Narcolepsy

Narcolepsy is a chronic sleep disorder characterized

by overwhelming daytime drowsiness and sudden
attacks of sleep. People with narcolepsy often find it
difficult to stay awake for long periods of time,
regardless of the circumstances. Narcolepsy can cause
serious disruptions in your daily routine

TYPES OF NARCOLEPSY

 Type 1 narcolepsy (previously called narcolepsy with cataplexy [see symptoms section

for definition]). Persons with type 1 narcolepsy have excessive daytime sleepiness plus
cataplexy and/or low levels of a chemical in the brain called hypocretin.
 Type 2 narcolepsy (previously called narcolepsy without cataplexy). Persons with type 2
narcolepsy have excessive daytime sleepiness but do not have cataplexy and have normal
levels of hypocretin.

CAUSES
 an inherited genetic fault.
 hormonal changes, including those that take place during puberty or the menopause.
 major psychological stress.
 a sudden change in sleep patterns.
 an infection, such as swine flu or a streptococcal infection.
 having the flu vaccine Pandemrix.

RISK FACTORS
There are only a few known risk factors for narcolepsy, including:
 Age. Narcolepsy typically begins in people between 10 and 30 years old.
 Family history. Your risk of narcolepsy is 20 to 40 times higher if you have a family member
who has narcolepsy.

SIGNS AND SYMPTOMS

 Excessive daytime sleepiness. People with narcolepsy fall asleep without warning, anywhere,
anytime. ...
 Sudden loss of muscle tone. ...
 Sleep paralysis. ...
 Changes in rapid eye movement (REM) sleep. ...
 Hallucinations.

PATHOPHYSIOLOGY

DIAGNOSIS/LABORATORY RESULTS

Narcolepsy is diagnosed after your healthcare provider performs a detailed medical and sleep
history, physical examination, medication history and sleep studies (which are performed in a
sleep disorders center). You may also be asked to wear a wrist motion sensor (called an
actigraph) for a few weeks or keep a sleep diary, which consists of keeping notes about how easy
it is for you to fall asleep and stay asleep, how many hours of sleep you get each night and how
awake you feel during the day.

Two essential sleep studies to confirm a diagnosis of narcolepsy are the polysomnogram (PSG)
and the multiple sleep latency test (MSLT). These tests are usually performed in a sleep
disorders center and require an overnight stay.

 The PSG is an overnight test that takes continuous multiple measurements, including
heart rate, oxygen level, breathing rate, eye and leg movements and brain waves while
you sleep. A PSG reveals how quickly you fall asleep, how often you wake up during the
night and how often REM sleep is disturbed (a common finding in people with
 narcolepsy). This study also helps determine if your symptoms are caused by another
condition, such as obstructive sleep apnea. Most people with narcolepsy show disruptions
in normal sleep patterns, with frequent awakenings.
 The MSLT is performed during the daytime, the day after the PSG test. During MSLT,
you will take five short naps, scheduled two hours apart. The MSLT measures how
quickly you fall asleep and how quickly you enter into REM sleep.

MEDICAL MANAGEMENT

There is no specific cure of the disease. One of the best ways to manage excessive daytime
sleepiness is to take frequent, brief naps evenly spaced through the day.

Lifestyle:
These include:

Stress can make the condition worse.

Eating a healthy, balanced diet can help improve your levels of alertness.
Taking regular exercise but stop at least three hours before you go to bed

MEDICATIONS

A number of medications with varying mechanisms of action in the brain are now available for
the treatment of excessive daytime sleepiness (EDS).

Wake-promoting medications

These agents help patients stay awake during the day but do not treat cataplexy or other REM
sleep-related signs of narcolepsy. Modafinil (Provigil®) or armodafinil (Nuvigil®) are often
tried first because they have fewer side effects and are less addictive than traditional stimulants.

More recently approved medications are solriamfetol (Sunosi®) and pitolisant (Wakix®).
Solriamfetol is used to improve wakefulness in adults with narcolepsy. Pitolisant (Wakix®) is
the first medication approved to treat EDS in narcolepsy that is not classified as a controlled
substance (substances with greater risk of abuse or addiction). Solriamfetol and pitolisant work
on the brain in different ways than other available agents.

Sodium oxybate
Sodium oxybate (Xyrem®) is the only FDA-approved medication used to treat daytime
sleepiness and cataplexy in patients with narcolepsy. It is taken in liquid form before bedtime
and 2.5 to 4 hours later and not during the daytime. Due to its high sodium content, patients
using sodium oxybate are advised to limit salt in the diet.

Stimulants

Once the mainstay of narcolepsy therapy, traditional stimulants such as

amphetamine/dextroamphetamine or dextroamphetamine mixed salts (Adderall®, Dexedrine®)
and methylphenidate (Ritalin®, Focalin®, Concerta®) are very effective for treating EDS but
have a higher risk of side effects than wake-promoting agents. Stimulants can produce side
effects similar to that seen with caffeine, such as agitation, nervousness and palpitations. They
are generally started at a low dose and increased gradually as needed. Careful monitoring is
required, as high blood pressure, heart arrhythmias (irregular heartbeats) and drug abuse have
been reported.

Antidepressants

Cataplexy, hallucinations, disrupted nighttime sleep and sleep paralysis are often treated with
two types of antidepressant medications: tricyclic antidepressants (TCAs) and selective serotonin
reuptake inhibitors (SSRIs). Examples of TCAs include protriptyline (Vivactil®), clomipramine
(Anafranil®) and desipramine (Norpramin®). Examples of SSRIs include fluoxetine (Prozac®),
atomoxetine (Strattera®) and sertraline (Zoloft®). SSRIs generally have fewer side effects than
TCAs. These agents are prescribed off-label, meaning that clinical trials have not been
performed and the FDA has not approved them for the treatment of narcolepsy.

It may take several weeks and/or several trials of different medications to find which one(s) work
best for you and which dosage works best. Your doctor may also recommend avoiding
antihistamine products (an ingredient in many cold products), as these products block the action
of a substance in the blood (histamine) that helps you stay awake.

NURSING INTERVENTION
 Encourage daytime physical activities but instruct the patient to avoid strenuous activities
before bedtime.
- In insomnia, stress may be reduced by therapeutic activities and may promote
sleep. However, strenuous activities may lead to fatigue and may cause insomnia.
 Encourage patient to take milk.

L-tryptophan is a component of milk which promotes sleep.

 Instruct the patient to follow a consistent daily schedule for rest and sleep.
 - Consistent schedules facilitate regulation of the circadian rhythm and decrease the
energy needed for adaptation to changes.
 Remind the patient to avoid taking a large amount of fluids before bedtime.
- This will refrain the patient from going to the bathroom in between sleep.
 Inhibit the patient from daytime naps unless needed.
- Napping can disrupt normal sleep pattern; however, older patients do better with
frequent naps during the day to counter their shorter nighttime sleep schedules.
 Introduce relaxing activities such as warm bath, calm music, reading a book, and
relaxation exercises before bedtime.
- These activities provide relaxation and distraction to prepare mind and body for
sleep.
 Tell patient to write a journal regarding problems before retiring.
- Journaling allows the patient to “set aside” problems or any mental activities just
before going to sleep.

COMPLICATIONS

The complications associated with narcolepsy include:

 People with narcolepsy also often have depression and anxiety, but it’s not clear whether
these are symptoms of narcolepsy or due to its symptoms affecting their quality of life.

 Because of excessive sleepiness and cataplexy, your social life may be affected. For
example, you may have difficulty staying awake during social gatherings, or you may
lose muscle control when laughing.

 Perhaps because of lower activity levels or a slower metabolism, many people with
narcolepsy are overweight. Adults with narcolepsy weigh about 15 to 20 percent more on
average than the general population.

 A 2017 study suggests that people with narcolepsy may have an increased risk for
suicidal behavior.

Just because you’re living with narcolepsy doesn’t necessarily mean you’ll have these
complications.

Working closely with your healthcare team to address any symptoms or treatment side effects is
one of the best ways to avoid or lessen narcolepsy complications.