Mason is a 15 year-old who is sleeping too much.
The most common cause of hypersomnolence is insufficient sleep syndrome.
Narcolepsy has varying prevalence depending on the populations.
Narcolepsy occurs in childhood, with a median age of symptom onset of
14.7 years
By the end of this module, you will be able to:
- Describe components of a comprehensive sleep history that are specific to pediatric and adolescent patients.
- Explain the role of the PSG and MSLT in the diagnosis of Narcolepsy.
- Differentiate when a child or adolescent should be referred to a Sleep Disorders Center with experience in treating children and adolescents.
- Explain the roles of team member (physician, specialty physician, nutritionist, respiratory therapist, social worker, nurse) for patients diagnosed with EDS, Narcolepsy, and KLS.
What are Hypersomnolence Disorders?
Hypersomnolence is the complaint of having excessive daytime sleepiness. Parents commonly complain that their child falls asleep during the day, particularly during school. Daytime hypersomnolence presents frequently in the pediatric population with an incidence of 4% in preadolescents and increasing to at least 20% in high school seniors (Neveus T et al. Acta Paedatr, 2001 & Ohayon M et al. J Am Acad Child Adolesc Psychiatry 2000). Gender differences in daytime sleepiness have not been determined. Often in pediatric populations, increased sleepiness presents in conjunction with increased behavioral difficulties, increased depressed mood, and lower quality of life scores (Stores G et al. Pediatrics 2006).
Hypersomnolence may be expressed in the childhood population due to a confluence of several factors. As children age, the optimum number of hours of sleep declines with a corresponding delay in bedtime (delay in sleep onset). This delay couples with early schools start times between 7:15-9:00 AM and limits the amount of time children are able to sleep. The prevalence of technologies such as cell phones, tablets, computers, and televisions in children’s bedrooms also affords another avenue of restriction of children's time asleep. Finally, some children have dysfunctions in the wake promoting areas of the brain, leading to increased daytime sleepiness.
Pediatric hypersomnolence may be caused by several different etiologies. These include insufficient sleep syndrome, sleep-disordered breathing, narcolepsy, and Kleine-Levin syndrome (KLS). (Kotagal S. Sleep Med Clin 2012)
Causes of Hypersomnolence Disorders
1 Insufficient Sleep Syndrome
The most common cause of pediatric hypersomnolence - Insufficient sleep syndrome - typically presents in adolescents as sleep-onset times are delayed due to both physiologic and social factors (jobs, sports, extra-curricular activities) while wake times remain relatively stable due to the fixed start times of schools. Adolescents may further exacerbate this problem with excessive use of caffeine or nicotine, illicit drug use, physical activity late at night, and use of screens (TV, computer, video games) in the bedroom and in bed. Additionally, the rise of texting and social media with cell phones and tablets in bed has further decreased the amount of sleep that children obtain on a regular basis. Late night use of social media even has a name: “Vamping,” in reference to both vampires and to “virtual camping”.
2 Narcolepsy
This is a primary hypersomnia that is characterized by excessive daytime sleepiness, cataplexy, sleep paralysis, and hallucinations when going to or when waking from sleep. Patients with narcolepsy have rapid sleep onset, yet their sleep is very fragmented. The daytime sleepiness in narcolepsy is described as irresistible. Cataplexy does not occur in all patients with narcolepsy, but is pathognomonic for the disease. Cataplexy is a sudden loss of muscle tone or control in the legs, face, trunk, or neck usually in response to emotional stimuli.
- Type 1 Narcolepsy: Caused by a loss of orexin-producing neurons in the hypothalamus. This results in the clinical symptoms of narcolepsy, including cataplexy. Low cerebral spinal fluid (CSF) orexin levels are seen, if tested.
- Type 2 Narcolepsy: Consists of clinical features of irrepressible need to sleep without evidence of cataplexy. Symptoms of hallucinations and sleep paralysis may still occur. CSF orexin levels are in the normal range.
3 Kleine-Levin Syndrome
Kleine-Levin syndrome is an extremely rare condition that is characterized by recurrent episodes of hypersomnolence with sleep periods lasting up to 18 consecutive hours and behavioral issues such as irritability, aggression, changes in appetite, and hypersexual behavior. Episodes of sleepiness and behavioral changes may last from several days to weeks. Asymptomatic periods may last for months. KLS typically occurs during adolescence and has a slight male predominance. KLS is thought to be caused by hypothalamic, thalamic, and fronto-temporal dysfunction and hypoperfusion that may be autoimmune in nature (Portilla P et al. Rev Neurol (Paris) 2002).
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Medicine  |
Medical providers (MD, OD, NP, PA) serve the role of obtaining the history and physical, assessing the patient and developing the differential diagnosis for the patient’s stated complaint. Medical Providers then order lab studies, sleep testing, and develop treatment strategies for patients with hypersomnolence. This may include altering the patient’s sleep schedule or sleep hygiene, ordering blood work, referring to a sleep lab for an overnight polysomnogram (sleep study), or starting medications. |
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Nursing  |
A registered nurse (RN) may obtain the history to assess the individual’s sleep habits and sleep quality. The advanced practice nurse (APN) or RN may use the “BEARS” instrument to screen for major sleep disorders affecting children. Education regarding sleep hygiene will reinforce the messages that the physician or medical providers have delivered. |
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Nutrition  |
A registered dietitian (RDN) is a vital part of the multidisciplinary team to address obesity, as it is a highly prevalent issue related to multiple sleep disorders. Dietitians assess weight, height, BMI percentiles, diet history, and physical activity level. Nutrition intervention may include: counseling on a healthy diet, increasing daily physical activity, and referral to a pediatric weight management clinic. |
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Pharmacy  |
A Pharmacist (R.Ph., or PharmD) specializes in the use of medications and pharmaceuticals to improve health and manage chronic medical conditions. Pharmacists are critically important to assess the treatment plan to ensure that there are no interactions or side effects which will negatively impact the patient. Additionally, pharmacists can be useful in suggesting alternative therapies for patients with known allergies or complications to medications. |
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Respiratory Therapy  |
A respiratory therapist (RT) plays a role in performing spirometry studies, sleep studies, and other tests that can rule out and/or confirm diagnosis (for instance, Obstructive Sleep Apnea). RTs can refer and educate on the importance of exercise as related to sleep issues. RTs assist in setting up and educating patients and families on respiratory equipment that may be needed. |
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Social Work and Mental Health  |
A social worker is often a good person to start with when a patient or family has questions, but does not know where to turn. Social workers can guide families to helpful resources, such as counseling and financial assistance. They can also provide information on navigating issues such as Individualized Education Plans (IEP) or 504 Plans (see resource section) for school age children, and Vocational Rehabilitation for adolescents. |
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Family Perspective and Leadership  |
As a Family Representative, it would be helpful to educate the whole family unit on issues that can emerge with hypersomnolence that could disrupt the daily harmony and routine. Areas to address would be the development of anxiety, depression and obesity, as well as the importance of maintaining a good schedule and healthy sleep habits. |
Mason
Apple
Apple is an 11 year-old white female with a history of falling asleep during the day.
Supplemental Websites
Narcolepsy Network.org
This site has information on various services and supports for people with Narcolepsy, including how to talk to school personnel, asking for accommodations with standardized testing, and other helpful tips.
Wake up narcolepsy
This
is a non-profit dedicated to increasing awareness of Narcolepsy and to funding research. Here is one very helpful page has information on Narcolepsy and SSI.
Wrightslaw
This site has many helpful resources regarding special education, for example, the difference between an Individualized Education Plan (IEP) and a 504 Plan.
The National Sleep Foundation
The National Sleep Foundation maintains a list of state laws related to sleepy driving. These are changing rapidly as awareness is increasing, so be sure to check with your state for the most up to date information.
Family Voices
This national organization connects families of children with special needs (like narcolepsy) to resources and to each other. Each state also has a Family-to-Family Health Information Center, where parents can receive information from other parents.