Pediatric Edition // SLEEP & WELLNESS MAGAZINE
SLEEP & WELLNESS MAGAZINE // Pediatric Edition
FINDING A WAY
Narcolepsy victims ride a grinding, endless roller coaster – in
and out of deep sleep at night due to the loss of brain cells
that regulate sleep and wakefulness. For someone without the
disorder, it would be like going without sleep for 48-72 hours,
day after exhausting day.
TO GIVE
SMILES
BACK TO KIDS
Narcolepsy with cataplexy causes sudden, brief episodes of
muscle weakness or paralysis. These episodes are similar to
the paralysis that occurs naturally during REM sleep, except
the victim remains fully conscious.
Narcolepsy can rob children of
simple childhood pleasures
AM I DYING?
Generally, the tendency to lose muscle control is brought on
by an emotion, such as laughter, happiness or fear. Cataplexy,
present in up to 70% of narcoleptics, can be a terrifying event,
especially when first experienced and particularly in children.
By Monica Gow
Stunned by suddenly being
unable to move, many fear that
they may be forever paralyzed or
even dying. Yet, even when the
episode is severe, cataplexy
does not result in permanent
dysfunction. After episodes end,
people rapidly recover their full
capacity to move and speak.
FOR JASON, AGE 15, HAPPINESS IS NOTHING
TO GIGGLE AT. “If he’s laughing hard and he
hasn’t taken his medication, he collapses to the
floor,” says Kelly, his mom. “So he avoids laughter,
even smiling, at all cost. It’s very hard on him.”
Jason is one of some 200,000 Americans and 3 million worldwide living with cataplexy, a debilitating
symptom of narcolepsy, the sleep disorder characterized by excessive daytime sleepiness (EDS). In
most cases, symptoms first appear between the ages
of 7 and 25; however, the disease may appear at a
younger age or in older adults.
Among cataplexy sufferers,
episodes can differ greatly in
intensity. They can include arm
weakness, sagging jaw, drooping head, slumping shoulders,
slurred speech, generalized weakness, or knees buckling.
Attacks typically last from a few seconds to several minutes.
Fortunately, Jason’s narcolepsy was diagnosed when he was
in first grade, and medication has made a world of difference
in his quality of life. Sadly for many sufferers, however, sometimes it takes up to 10 years to have the disease diagnosed.
Imagine living for years with such devastating symptoms.
If left undiagnosed and untreated, childhood-onset
narcolepsy can interfere with psychological, social,
and cognitive function and can challenge academic
and social activities. For some young people, low
self-esteem that results from poor academic performance may persist into adulthood.
“My son Casey, a freshman in high school, has to
nap at school, after school and sometimes once
more before bedtime,” says his mom. “Somehow, he
has to squeeze in sports and homework.” She adds,
“Casey just wants to blend in with his classmates,
even though he deals with constant sleepiness at
school. A boring class is beyond torture for kids
with narcolepsy.”
When Casey was diagnosed, at age 10, his folks
could still control bedtime, “Now, at 15,” says his
mom, “he thinks he can do whatever he wants and
still function. A kid with narcolepsy has to to adhere
to a strict bedtime, and sleepovers with friends are
rare to non-existent due to his sleep patterns and his
night-time medication schedule.”
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GETTING AT LEAST
20 MINUTES OF
PHYSICAL EXERCISE
PER DAY FOUR TO
FIVE HOURS BEFORE
BEDTIME CAN ALSO
IMPROVE SLEEP
QUALITY.
“
CATAPLEXY, PRESENT
IN UP TO 70% OF NARCOLEPTICS, CAN BE A
TERRIFYING EVENT,
ESPECIALLY WHEN
FIRST EXPERIENCED
AND PARTICULARLY
IN CHILDREN.
“
Even when treated with the best medicines, narcolepsy is extremely difficult to manage in children.
Kids with narcolepsy – and their families – often
find this crucial time of growing up, with its associated peer pressure, especially challenging.
Narcolepsy affects both men and women at any age without
regard for ethnicity, although narcolepsy symptoms are usually first noticed in teens or young adults. Narcolepsy can
also develop early in life, probably more frequently than is
generally recognized. Children as young as three years old
have been diagnosed with narcolepsy.
MISDIAGNOSIS IS COMMON
Frequently, when a child falls asleep repeatedly in class,
teachers – if they are unfamiliar with the disease – misdiagnose the behavior as laziness, lack of motivation, or a discipline or drug problem.
Fortunately, school staffs are becoming better informed. For
Casey, says his mom, “The school bent over backward to help
us, and we caught it quickly. The teacher called us to say
Casey fell asleep in class and fell down at recess, and kids
told him he looked ‘drunk’ because of his cataplexy.”
“Cataplexy can be really tough for a happy kid that likes to
laugh,” she adds. “Many kids with undiagnosed and untreated cataplexy teach themselves to hide their emotions. Your
heart goes out to them.”
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Pediatric Edition // SLEEP & WELLNESS MAGAZINE
Typically, people with EDS describe life as a stubborn sense
of mental murkiness, an absence of energy, a depressed
mood or severe exhaustion. “Imagine trying to pay attention
in class, take notes, or work on a computer while fighting
crushing sleepiness,” Jason’s mother observes.
Generally, a 30-minute nap brings refreshment – but often for
just a few hours. This creates a punishing agenda for the
entire family. Imagine the logistical and social challenges
confronting a child or teen who has to nap every three to four
hours. How does he make and keep friends, a critically important building block in a child’s development? Which friends
should learn of the child’s narcolepsy, and when? Not surprisingly, many children are embarrassed by their condition and
guard their friendships and privacy judiciously.
For the family, life often revolves around managing where
the child is when sleepiness comes on. Outings often have to
cut short.
“When your child has a medical problem,” says Casey’s mom,
“they naturally receive the most attention, so it can be tricky
and challenging to make sure all the children in the family
feel like they’re getting the equal notice.”
RESEARCH TO FIND A CAUSE AND A CURE
Over the past 15 years, scientists have made considerable
progress in understanding the causes of narcolepsy. This
work has shown that narcolepsy and cataplexy often develop
because of a loss of brain cells that produce hypocretin, a
neurotransmitter that regulates wakefulness.
Neurotransmitters are chemicals that neurons produce to
communicate with each other and to regulate biological
processes. Loss of hypocretin results in an inability to regulate sleep.
Cell loss generally begins in the teens or young adulthood
and results in lifelong narcolepsy. The cause remains
unknown but appears to be autoimmune in nature. That is,
the body’s immune system selectively attacks hypocretincontaining brain cells.
People living with narcolepsy are genetically predisposed
and the gene is triggered normally in their youth. One in four
people in the U.S. carry the genetic marker for narcolepsy
WHAT TREATMENTS ARE AVAILABLE?
Research to find a cure for narcolepsy continues. Medicines,
lifestyle changes and other therapies can help relieve many
of the symptoms, and treatment is based on the type and
severity of the indicators.
Medication
Though the loss of hypocretin is believed to be irreversible
and permanent, excessive daytime sleepiness and cataplexy
can be controlled in most people through drug treatment. As
with all such matters, you should talk with your doctor about
what medication is right for your child.
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SLEEP & WELLNESS MAGAZINE // Pediatric Edition
Medications to relieve Excessive Daytime Sleepiness (EDS)
• Sodium Oxybate (XYREM®), also FDA-approved
for reducing cataplexy
• Wakefulness Promoting Medications including
Modafinil (Provigil®) and Armodafinil (Nuvigil®)
• Central Nervous System Stimulants such as
Methylphenidate (Ritalin®, Ritalin SR®, Methylin®,
Methylin ER®), Mixed Amphetamine Salts (Adderall
IR®, Adderall XR®), Dextroamphetamine (Dexedrine®,
Dexedrine SR®), and Lisdexamfetamine (Vyvanse®)
Medications to reduce cataplexy (partial or full muscle
weakness) and other REM sleep disturbances
• Sodium Oxybate (XYREM®), also FDA-approved
for reducing EDS
• Antidepressants that repress REM sleep, such as:
– Serotonin Norepinephrine Reuptake Inhibitors,
like Venlafaxine (EffexorSR®)
– Norepinephrine Reuptake Inhibitors, like
Atomoxetine (Strattera®)
– Selective Serotonin Reuptake Inhibitors, like
Fluoxetine (Prozac®) and Sertraline (Zoloft®)
– Older Tricyclic Antidepressants, like Protriptyline
(Triptil® and Vivactil®), Imipramine (Janimine®
and Tofranil®), Desipramine (Norpramine® and
Pertofran®), and Clomipramine (Anafranil®)
Advocating at School
Your voice as a parent advocate can make all the difference
with your child’s school and physician. The doctor can help
by authoring letters describing specific accommodations
needed at school. At school, seek out and stay connected with
a teacher, guidance counselor or nurse who will be your inschool advocate.
Schools should be encouraged to modify class schedules of
children and adolescents with narcolepsy. And parents
should inform school personnel of their child’s special needs,
including medication requirements during the school day.
Use your child’s situation as an opportunity to educate the
school about narcolepsy. You may be pleasantly surprised by
the warm reception you’ll receive.
Behavioral Strategies to Help Children
Medication alone won’t enable kids and adolescents with
narcolepsy to consistently maintain a fully normal state of
alertness, so behavioral strategies play a crucial role in treating the disease.
Many kids take short, regularly scheduled naps at times
when they tend to feel sleepiest. Improving the quality of
night-time sleep can reduce, but not cure, EDS and help
relieve persistent weariness. Common-sense measures to
enhance sleep quality can be especially important for young
people with narcolepsy, including:
• Maintain a regular sleep schedule
• Avoid caffeine-containing beverages for several
hours before bedtime
• Maintain a comfortable, adequately warmed
bedroom environment
• Engage in relaxing activities such as a warm
bath before bedtime.
Getting at least 20 minutes of physical exercise per day four
to five hours before bedtime can also improve sleep quality.
Exercise has also been shown to help people with narcolepsy
avoid excess weight gain, a common side effect of the disease.
Sports, whether organized or individual, can help kids with
narcolepsy both physically and socially. “Otherwise,” says
Casey’s mom, “it can seem as if they have little more than
school and sleep. It’s really hard to throw a sport into the mix,
but in the long run it’s worth it for Casey’s overall well-being.”
Although the disorder itself is not fatal, safety precautions,
particularly when driving, are vitally important for anyone
living with narcolepsy. People with untreated symptoms are
involved in automobile accidents roughly 10 times more frequently than the general population. Accident rates are nor-
mal, however, among individuals who have received appropriate medication.
Finally, because people with narcolepsy may become socially isolated due to embarrassment about their symptoms,
support groups can prove beneficial. The empathy and
understanding that these relationships offer narcolepsy sufferers can be crucial to their overall sense of well being, providing them with a network of social contacts who can offer
practical help and emotional support.
REASON FOR OPTIMISM
For its sufferers, living with narcolepsy can mean lifelong,
debilitating weariness, cataplexy and a compromised lifestyle.
Slowly, the public is becoming more aware of the disease and
its consequences, leading to advances in research toward a
cure. For the thousands of young people and adults living with
narcolepsy, these developments are providing more than optimism. They are leading to proven treatment.
Jason’s mom sums it up. “Treatment has given our son his
smile back. But only through more research can he hope for
a normal life someday.” S&W
About the Author
Monica Gow is Executive Director of Wake Up Narcolepsy, a
non-profit organization dedicated to raising awareness of narcolepsy and finding a cure for this debilitating neurologic disorder. www.wakeupnarcolepsy.org. Monica is also a parent of a
child with narcolepsy.
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