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Evaluation of the Sleepy Child: What to Do When the Sleep
Study is Normal
Stacey L. Ishman, MD, MPHProfessor, Otolaryngology – Head & Neck Surgery
And Pulmonary (Sleep) MedicineCincinnati Children’s Hospital Medical Center,
Cincinnati, OH
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Disclosures
Medtronic – Consultant – There will be no discussion of Medtronic devices in this talk
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Objectives
1. Describe the appropriate clinical assessment of the sleepy child • screening questions/ questionnaires• exam findings • testing to screen for non-SDB causes
of daytime sleepiness in children
3
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Objectives2. Be familiar with the incidence, identification
and impact of medical conditions that contribute to daytime tiredness including:• narcolepsy• nocturnal seizures • restless leg syndrome • circadian rhythm disorders
3. Recognize the importance of adequate sleep hygiene and review the components of good sleep habits 4
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Practice Change
Recognize the need to extend the number of hours of sleep for most children
5
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Evaluation of the Sleepy Child
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Evaluation of the Sleepy Child
EpidemiologyWhat does a sleepy child look like?HistoryPhysical ExamDifferential DiagnosisRole of PSG
Goal=Broaden our view of the causes of sleepiness
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Iglowstein I, et al. Pediatrics 2003;111:302–307
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Pediatric Sleep Disorders
InsomniaSleep-related Breathing DisordersHypersomnia (not related to breathing)Circadian Rhythm DisorderParasomniaSleep-Related Movement Disorders
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Sleep DisordersInsomnia: – 307.42 Psychophysiological (Conditioned)
Insomnia– 307.41 Adjustment Sleep Disorder– 307.41 Inadequate Sleep Hygiene– 307.42 Behavioral Insomnia of childhood– 995.2 Insomnia due to drug effect
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Pediatric Sleep Disorders
Hypersomnia (not related to breathing):– 347.01 Narcolepsy with cataplexy– 347.00 Narcolepsy without cataplexy– 780.54 Recurrent Hypersomnia (Klein-Levin
Syndrome)– 780.54 Idiopathic Hypersomnia– 995.2 Hypersomnia due to drug effect– 307.44 Hypersomnia due psychiatric
condition
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Pediatric Sleep Disorders
Circadian Rhythm Disorder:– 780.55 Delayed Sleep Phase Syndrome– 780.55 Advanced Sleep Phase Syndrome– 780.55 Irregular sleep wake type– 995.2 Circadian Rhythm disorder due to drug
effect– 780.55 Circadian rhythm disorder due to a
known physiologic condition
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Pediatric Sleep Disorders
Sleep-Related Movement Disorders:– 333.99 Restless Leg Syndrome– 780.58 Periodic Limb Movement Disorder– 780.58 Sleep-Related Leg Cramps– 780.58 Sleep-Related Bruxism– 780.58 Sleep-Related Rhythmic Movement
Disorder
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Pediatric Sleep Disorders: Prevalence
Approximately 25% of all children experience some type of sleep problemYet, less than 25% of patients/parents with sleep problems seek physician helpPrevalence of various problems varies largely with age
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Pediatric Sleep Disorders: InfantsColic (1-4 months)– Melatonin metabolism?
Conditioned InsomniaAdjustment Insomnia
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Pediatric Sleep Disorders: School-Age
Sleep HygieneAdjustment issuesAnxiety related sleep issuesObstructive Sleep ApneaParasomnia
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Pediatric Sleep Disorders: Adolescents
Sleep Hygiene!!!– Diet
CaffeineChocolate
AnxietyObstructive Sleep apneaNarcolepsyKlein-Levin Syndrome
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Evaluation of the Sleepy Child: HistoryChief Complaint = sleepy?Questionnaires / Surveys The Sleep Diary – Bedtime routine (Child AND parents)– Actual sleep duration– Sleep Environment
MedicationsDiet Family HistoryOther factors
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The Sleepy Child ≠ The Sleepy Adult
Adults ChildrenFatigue HyperactiveSomnolence Behavioral issuesOccupational Hazards School
performanceVigilance Tasks Cognitive
dysfunction
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Evaluation of the Sleepy Child: HistoryBEARS history– Bedtime routine
Time, environment, distractions– Excessive Sleepiness
How much, actual sudden sleep, naps?– Awakenings
Time, quantity, why?– Regularity
weekends/vacation better?– Snoring
Mindell JA, Owens JA. A clinical Guide to Pediatric Sleep, 2003
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Evaluation of the Sleepy Child: HistoryMedications– Allergy meds (Diphenhydramine)– Antidepressants– ADHD medication timing
Diet– Caffeine – Chocolate / sugar
Family History– Parasomnia– Insomnia/RLS
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Evaluation of the Sleepy Child: Physical Exam
Height / WeightBMIObservation of interaction with parent(s)Sleep Apnea / Obstruction risk– Craniofacial structure– Occlusion– Neck Size– Tonsil size / Mallampati Score
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Howard NS, Brietzke SE. Otolaryngol Head Neck Surg. 2009 May;140(5):675-81
010
2030
40P
reop
_RD
I
1 1.5 2 2.5 3Mallampati1
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Role of Sleep StudyUsually not required for majority of sleep disorders– Insomnia– Straight-forward Parasomnia– Circadian Rhythm Disorders
Can be helpful in certain situations– Concerned about seizure– Movement disorders
May consider empiric treatment – Narcolepsy / Severe Hypersomnia
Multiple Sleep Latency Test (MSLT)– Still concerned about OSA
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Narcolepsy, Seizures and Restless Leg
Syndrome
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Symptoms of Narcolepsy
TetradPentad
• Excessive daytime sleepiness (EDS)
• Cataplexy• Hypnagogic hallucinations• Sleep paralysis
• Fragmented nocturnal sleep
• Other associated featuresAdapted from AASM 2005 Slide Set: Narcolepsy
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Excessive Daytime Sleepiness
• Sleep attacks on a background of chronic sleepiness or fatigue
• Frequent napping, usually refreshing• Memory lapses and automatic
behaviors• Impaired attention / concentration
• Decreased work performance• Increased drowsy driving crashes
• Visual disturbancesAdapted from AASM 2005 Slide Set: Narcolepsy
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Excessive Daytime Sleepiness
29
50
60
70
80
90
100
10:00 AM 12 noon 2:00 PM 4:00 PM 6:00 PM300
400
500
600
700
800
10:00 AM 12 noon 2:00 PM 4:00 PM 6:00 PM
Wilkinson Addition Test Digit Symbol Substitution Test
Adapted from Mitler et al 1982
ControlNarcoleptic
Adapted from AASM 2005 Slide Set: Narcolepsy
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Cataplexy
30
• Muscle weakness triggered by emotions• Joking, laughter, excitement, anger• Brief duration, mostly bilateral• Sudden onset
• May affect any voluntary muscle• Knee / leg buckling, jaw sagging,
head drooping, postural collapse• Consciousness maintained at the start
Adapted from AASM 2005 Slide Set: Narcolepsy
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Cataplexy
31
0
10
20
30
40
50
60
70
80
90
100
Laughing Joking Anger Stress Sex
Perc
enta
ge o
f Pat
ient
s
Adapted from AASM 2005 Slide Set: Narcolepsy
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Cataplexy
32
0102030405060708090
100
Legs / knees Jaw Slurred speech Generalized(falling to ground)
Perc
enta
ge o
f Pat
ient
s
Adapted from AASM 2005 Slide Set: Narcolepsy
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Hallucinations
33
• Vivid hallucinations at sleep onset (hypnagogic) or awakening (hypnopompic)
• Auditory: sounds, music, someone talking to them
• Visual: colored circles, parts of objects
• Can be vividly realistic and anxiety provoking
Adapted from AASM 2005 Slide Set: Narcolepsy
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Sleep Paralysis
34
• Sudden inability to move on falling asleep or on awakening
• Episodes are generally brief and benign, end spontaneously
• Can cause significant anxiety• Associated with
• Sleep deprivation • Narcolepsy• Obstructive sleep apnea
Adapted from AASM 2005 Slide Set: Narcolepsy
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Narcolepsy Age of Onset
35
0
5
10
15
20
25
5 10 15 20 25 30 35 40 45 50 55 60 65 70Perc
enta
ge o
f Pat
ient
s
Age
Adapted from AASM 2005 Slide Set: Narcolepsy
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Narcolepsy Age of Onset
36
• Under-recognized / misdiagnosed• Sleepiness may present as:
• Learning disability• Attention deficit hyperactivity
disorder• Cataplexy may be mislabeled as
psychogenic behavior• May be secondary to other disorders
Adapted from AASM 2005 Slide Set: Narcolepsy
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Narcolepsy Evaluation
37
• History• Sleepiness, cataplexy, other disassociated
REM sleep features• Polysomnography (PSG)
• Exclude other causes for EDS (insufficient sleep, apnea)
• Identify and treat associated conditions• Multiple Sleep Latency Test (MSLT)
• Objective sleepiness• Sleep onset REM periods (SOREMPs)
• CSF Hypocretin levelsAdapted from AASM 2005 Slide Set: Narcolepsy
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Narcolepsy Symptom Prevalence
38
Adapted from AASM 2005 Slide Set: Narcolepsy
0
10
20
30
40
50
60
70
80
90
100
Perc
enta
ge o
f Pat
ients
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Narcolepsy Sleep Study Findings
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• Short sleep latency• Sleep onset REM period in 50% of
narcoleptics• Sleep fragmentation (REM and NREM)
• Increased number of arousals• Increased stage 1 sleep• Low sleep efficiency
• Frequently associated with periodic limb movements
Adapted from AASM 2005 Slide Set: Narcolepsy
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• The MSLT is: • A validated objective measure of the
tendency to fall asleep• Indicated as part of the evaluation of
patients with suspected narcolepsy to confirm the diagnosis
• For 2 or more SOREMPs during MSLT:• Sensitivity was 0.79• Specificity was 0.98
From Arand et al 2005Adapted from AASM 2005 Slide Set: Narcolepsy
Narcolepsy Sleep Study Findings
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• Excessive sleepiness• Scheduled naps
• Modafinil• Sodium oxybate• Amphetamine (meth- and dextro-)• Methylphenidate• Selegiline
From Arand et al 2005 Adapted from AASM 2005 Slide Set: Narcolepsy
Narcolepsy Treatment
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• Cataplexy, sleep paralysis, halluc• TCAs
• Amitriptyline, clomipramine• Fluoxetine
From Arand et al 2005 Adapted from AASM 2005 Slide Set: Narcolepsy
Narcolepsy Treatment
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Nocturnal Seizures
43
• Rarely witnessed, often poorly described• Can be confused with parasomnias • Profoundly disrupt sleep structure
• Therefore disrupt daytime functioning
Basil. Nocturnal Seizure. Seminar Neurology 2004;24(3):293.
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Seizure Differential
44
Adapted from Basil. Nocturnal Seizure. Seminar Neurology 2004;24(3):293.
Table 1 Characteristics of Specific Sleep Disorders and Seizures
Sleep Walk/TalkSeizure Sleep Terrors RLS Narcolepsy
Incontinence + - - -Tongue biting + - - -Confusion + + - -Tonic-clonic movmnts + - - -Drooling + - - -Amnesia + + - -Occur while awake + - + +
PLMS, periodic limb movements of sleep; RLS, restless legs syndrome
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Seizure Onset by Sleep Stage• Seizure onset primarily in Stage 1/2• Rare during REM
Basil. Nocturnal Seizure. Seminar Neurology 2004;24(3):293.
450
10
20
30
40
50
60
70
Stage 1 Stage 2 SWS REM
% seizures% sleep
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Seizures - History• Important to differentiate from parasomnias• Speech often stereotypical in seizures
• Vs. sleep talking=speech is random• Occur predominantly in Stages 1/2
• Vs. sleep walking=usually in SWS• Atypical characteristics suggestive of seizure
warrant full EEG evaluation• Nocturnal injury• Tongue or lip biting• Morning muscle soreness
Basil. Nocturnal Seizure. Seminar Neurology 2004;24(3):293.
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Seizure & OSA• In some individuals, OSA can
• Provoke epileptic seizures or • Be the primary reason for intractability
of epilepsy• Seizures are likely triggered by
hypoxia during an apneic event• Severe hypersomnolence can result
in sleep attacks with apparent sudden loss of consciousness
Basil. Nocturnal Seizure. Seminar Neurology 2004;24(3):293.
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Seizures - Sleep Study• If suspected, consider an extended
montage EEG (more EEG leads)• Look for spike and wave activity
• Especially at transitions periods such as sleep onset
• REM usually spared
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emedicine.medscape.com/article/1138154-media
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RLS Diagnosis in Children• What are the 4 Essential criteria
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• A compelling urge to move the limbs • Symptoms worse/only at rest • Variable/temporary relief by activity • Symptoms worse in the evening /night
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RLS Diagnosis in Children• Supportive criteria
• Sleep disturbance for age• Biological parent or sibling with RLS• Sleep study documenting periodic limb
movement index ≥5/h
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RLS Diagnosis• History
• Primarily a clinical diagnosis• 7 to 10% of population in US/ N Europe• Increases with age• Strong FH correlates with earlier age of
onset (<45yo)• Incidence
• 2x more common in women• More common in whites than blacks• More common in iron deficient, pregnant
and end-stage renal disease 51
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RLS Aggravators
• Nicotine• Caffeine• SSRIs• Metoclopramide• Prochlorperazine maleate• Dopamine antagonists• Diphenhydramine• Alcohol
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RLS Diagnosis• Supportive clinical features
• In Children• Behavioral and mood difficulties
• Including ADHD• Cognitive issues• Sleep difficulties
• Adults and children• Sleep disturbance• Periodic leg movements• Response to dopaminergic therapy• Family history• Normal medical/physical evaluation
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RLS Diagnosis
• Supplemental workup • Polysomnography• Iron profile• And/or neuropathy screen
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RLS – Sleep Study
• Rhythmic or semi-rhythmic movements of the legs
• Found in 90% of RLS patients during sleep• > 5 periodic limb movements/hour
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RLS – Iron Studies
• Iron deficiency defined as:• Ferritin level ≤ 40 ng/ml
• acute-phase reactant• may misrepresent the true
iron stores, so the transferrin saturation determined along with it
• Transferrin saturation percentage ≤ 16% 56
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RLS Treatment• Behavioral techniques - *1st line in peds• Sleep Hygiene - * 1st line in peds• Iron – very low or iron deficiency
• Ferritin below 40• Dopaminergic agents
• Pramipexole - Rotigotine• Ropinirole • Levodopa - augmentation
• Antiepileptics – gabapentin, pregabalin, carbemazepine
• Opiates – oxycodone, tramadol57
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Circadian Rhythm Disorders
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Incidence of Circadian Rhythm Disorders
NSF- 1/15 children in age range of 5-13 years have CRD200,000 children in USAdolescents reported prevalence up to 16% with Delayed Sleep Phase Syndrome
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Internal Clock in Children
Birth- Infant sleep pattern irregular4-6 Months- Sleeping more nightBy 18 months, most children sleep all nightRegular adult sleep wake cycle usually established by age 5
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Physiology of Circadian Rhythm
The suprachiasmatic nucleus (SCN)= pacemakerLight is the strongest influence on the sleep/wake cycleMelatonin secretion signals sleep period
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Normal Adolescent Sleep Changes
– Adolescents have a normal shift of sleep time by 2 hours
– Delayed melatonin secretion with puberty
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Types of Pediatric Circadian Rhythm Disorders
Free Running or non-24 hours typeIrregular Sleep Wake PatternAdvanced Sleep Phase SyndromeDelayed Sleep Phase Syndrome
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Advanced Sleep Phase Syndrome
–Sleep and wake very early 6pm- 3am–May be genetic–1% Elderly–Young Children- the early awakening is a
problem for parents
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General Treatment of CRDs
Firm sleep/wake schedule- fixed AM arising time most importantAvoid weekend variationSleep Hygiene- caffeine, computer, cell phones, lights, TV
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Delayed Sleep Phase- Night Owls
– Delay in sleep time by 2 or more hours– Difficulty arising in the morning– Sleeping in very late on weekends– Results in sleep deprivation during the week– Occurs in 7-16% of Teens
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Manifestations of DSPS
SleepinessInsomnia- difficulty going to sleepSchool dysfunctionAuto accidents due to sleepinessFamily conflict
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Factors contributing to DSPS
Evening light exposure -suppresses melatonin secretion– Computer screens– Video Games– Bright overhead lighting
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Treatment of DSPS
Sleep HygieneFirm Sleep ScheduleMelatonin Chronotherapy
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Sleep Hygiene
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Sleep Hygiene
Environmental factors contributing to poor sleep in children – Infant – teenage
Impact of poor sleep hygieneWays to improve sleep hygiene
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Sleep Hygiene: General
Comfortable Sleep environment– Correct temperature– Quiet– Dark
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Cheerios & Pampers Advice
Age 1-2 years–Put child to sleep when drowsy, but not
already asleepPre-school age–Establish a routine
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Limit-Setting Sleep Disorder
Behavioral insomnia of childhood– Bedtime stalling and repeated demands
Treatment– Consistency in routine and limit setting
Day AND night time– Time warning(s) (bedtime in 30, 10, 5 minutes)– Positive reinforcement
ChartsRewards
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How many hours of sleep does the average teenager need?
Older school-aged children and teens need on average about 9.25 hours of sleep a day
Carskadon MA et al. Sleep 1980
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How many hours of sleep does the average teenager get?
Sixth-graders sleep an average of 8.4 hrs on school nights12th-graders sleep on average 6.9 hrs/ night– 2 hours less sleep than recommended
Only 20 percent of teens get 9 hours of sleep on school nights
Sleep in America Poll 2006
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Teens and Electronics
97% of children and teens have at least one electronic item in their bedroom– television, computer, phone or music player
Adolescents with ≥4 such items in their bedrooms are much less likely than their peers to get a sufficient amount of sleep
– Sleep in America Poll 2006
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Cell phones4/5 teens carry a cell phone40% increase from percent of teens in 2004
– wireless trade association CTIA and Harris Interactive 2008
On average kids get their first cell phone between 10-11 yoNearly half of kids 8-12 years old own cell phones in the U.S
– Nielsen report 2008
42%of teens surveyed say they can text blindfolded
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Cell phone use and sleep
Prospective study of 1,600 13-15 yo60% talk or text message after lights out A year later, teens who used their cell phone more than once a week after lights-out were 5x more likely to say they felt tiredThe later teens stayed awake with their phones, the more tired they were
– Van Den Bulck Sleep 2007
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Computers72% of teenagers have own desktop computer25% of teenagers have own laptop computerTeens spend an average of 2.9 hours/day on-line
– CBS news survey 2006
55 % of households have rules about how much time children/teens are allowed to spend on-line
– Pew Internet and American Life Project Parent and Teen Survey 2006
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Computers
22% of school age children admit they IM while their parents think they are asleep
i-safe America poll 2007
51% of kids check their social network daily– 23 % of parents think their kids check their networks
daily
22 % of teenagers check their social networks more than 10 times/day– 4 % of parents think their kids check their social
networks more than 10 times/day– Common Sense Media poll 2009
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Television43% of school aged children, 33% of preschool children and 20% of infants and toddlers have TVs in their bedroomsChildren with TVs in their bedrooms – Go to sleep 20 minutes later– Sleep 40 minutes less per night – Loss of 2 hours sleep every week
– Sleep in America Survey 2006
58 % of households have limits on number of hours of TV viewing allowed
– Pew Internet and American Life Project Parent and Teen Survey 2006
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Caffeine
76% of children are getting caffeine from some source on a daily basis
Journal of the American Dietetics Association 2004
31% of teens consume at least 2 caffeinated beverages/ day
Sleep in America Poll 2006
31% of US teens drink energy drinks on a regular basisSimmons research poll 2006
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Effects of Sleep Deprivation
School performanceMoodObesityAutomobile accidents
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Recommendations: Teens
Regular bedtime and sleep scheduleRelaxing bedtime routine– Reading, music
Remove distractions from bedroom– TV, Phone, computer– Use parental controls, check phone logs
Avoid caffeine– Especially after lunch
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RecommendationsParental Awareness
Most teens know they're not getting enough sleep– Half of teenagers admit they get less sleep than they need to
be at their best– 51 percent say they feel too tired or sleepy during the day
90 percent of parents believe their adolescents are getting enough sleep on school nights
– Sleep in America Poll 2006
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Parental Awareness:Sleep Needs
www.sleepfoundation.org
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SummarySleep hygiene issues are by far the most common pediatric sleep problems
Sleepiness is not the most common way children with sleep disorders present
The sleep history and sleep diary are the most useful tools to make a diagnosis
The physical exam is of limited usefulness
PSG is only used in a minority of cases
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Summary
• Narcolepsy can occur in children and is characterized by: • Excessive daytime sleepiness (EDS)• Cataplexy• Hypnagogic hallucinations• Sleep paralysis
• PSG with MSLT is the primary tool for diagnosis
90
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Summary• Seizures must be differentiated from
parasomnias• Can be increased with concomitant
OSA• Extended montage EEG with the
PSG may pick up nocturnal seizures• Tend to occur in NREM, rare in SWS
and REM• Full Neurologic evaluation with
Extended EEG may be required91
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Summary• RLS is characterized by 4 essential
features:• An urge to move, usually associated
with paresthesias• Onset or exacerbation of symptoms
at rest• Relief of symptoms with movement• Symptoms manifesting in a circadian
pattern92
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Summary• RLS in children – alternate criteria:
• Four essential adult criteria plus two supportive criteria
• Supportive criteria• Sleep disturbance for age (including
daytime sleepiness)• Biological parent or sibling with RLS• Sleep study documenting periodic limb
movement index ≥5/h
93
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Summary
Circadian Rhythm Disorders are common in children and adolescentsEven if SDB present, look for other sleep disordersIn Teens- be especially aware of DSPS and the possibility of sleep deprivation
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Summary
Awareness of problem/ sleep needs of childrenSet a good example– Most adults are sleep deprived as well!
Consistency important Sleep conducive environmentControl use of electronics and caffeineLegislation
Driving restrictions for teensSchool start times
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Thank You
Stacey Ishman, MD, MPH
Professor, Otolaryngology – Head & Neck Surgery Surgical Director, Upper Airway Center
Cincinnati Children’s Hospital Medical Center
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Practice Change
Recognize the need to extend the number of hours of sleep for most children
97