History of Narcolepsy

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(No Transcript)
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Goals of this Presentation

1. Learn how to prepare for a successful pediatric
   sleep study
2. Learn what to look for and how to respond during
   the study
3. Learn about pediatric sleep disorders and their
   treatments

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Children
Not just short adults
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Pediatric Polysomnography Requires Patience and
Preparation

• Polysomnographic procedures may be fear provoking
  to children
• Children require more time to set up for a
  polysomnogram than do adults
• Crying and removing electrodes may extend set up
  time past the childs usual bedtime, resulting in
  an overtired child

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A Family Centered Care Approach

• Parents are the experts on their child and a
  constant in their childs life
• Procedures should be conducted to create the
  least amount of trauma for the child
• The test environment should be inviting and
  child-friendly
• Psychological preparation of the child and parent
  are fundamental to the procedure
• Coping-skill development enhances a childs sense
  of mastery and control over a potentially
  stressful experience

Zaremba et al, JCSM, 2005
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Important Mind-Set Changes by the
Polysomnography Staff
FROM TO
needs of the staff needs of the child, parent
Good Guy Bad Guy parent, child and tech on the same team
a child lying down performing the procedure with the child sitting
Zaremba et al, JCSM, 2005
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Preparing the Family for a Polysomnogram

• Provide detailed information about the test
• Schedule testing for the childs usual bedtime
• Communications Confirmation letter sent with
• Logistics of reaching the center
• What to bring (food, transitional objects)
• No caffeine, no naps, no hair oils
• Answer questions as they come up

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What the Parent Should Know

• No acute or very recent medical issues
• Parents should call to cancel if child is ill
• Recommend shampoo night before
• Avoid scalp oils
• Avoid new braids
• Avoid caffeinated beverages
• Comfortable, loose two piece pajamas
• Bring a favorite book, video
• Bring usual medications

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Creating a Calm Environment

• Take time to establish rapport
• Explore the childs past experiences and coping
  strategies
• Create a good first impression
• Have books or toys on the bed
• Cover set up supplies, equipment if possible
• Use a calm and soothing tone of voice

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Child and Family Preparation

• On the study night
• Allow the child to explore room and sensors
• Define each persons job
• Develop a plan for coping
• Maintain patience, flexibility, positive attitude
• Lavish the child with praise
• Focusing on the desired behavior

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Engaging the Parent

• Make the parent part of the team
• Encourage the parent to interact in a reassuring
  way with the child
• Respond positively to parents questions and
  concerns
• Provide parents with explanations of the
  procedures

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Optimizing the Environmentfor Sleep and Safety

• Quiet away from doors, overhead paging
• Dark shades over windows
• Can you see, hear, communicate with child?
• Call button, two-way communication for
  calibrations
• Need for infrared lighting
• Safety
• Outlet plugs, no sharp corners, bed rails up
• Hypoallergenic, latex free supplies, no sharp
  corners
• Access emergency equipment, personnel

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Ground Rules for Bedroom Electronics

• No active phones or pagers in sleep room
• Arrange local phone access for parent
• Cell phones must be muted
• No calls in the room after lights out
• Plan video or TV to end before lights out
• Avoid electronic games immediately before bed

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Explanations

• Short, objective and concrete explanations are
  appropriate for younger children
• Children may regress when upset
• May need to aim explanations at a developmental
  level less than childs age
• Be honest and careful in your word choice
• Sarcasm and teasing may be misinterpreted and
  should be avoided

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Tips for Improving Cooperation

• Younger children may want to sit in their
  parents lap during set-up
• Distractions are often useful (stickers, bubbles,
  toys, favorite video)
• Medical play may reduce anxiety (put the
  electrodes on a doll)
• Older children can help by holding electrodes or
  sensors

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Positions for Comfort
Zaremba et al, JCSM, 2005
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Pediatric Polysomnography
EEG
EOG
Nasal EtCO2
Nasal Oral Airflow
Chin EMG (2)
Microphone
SaO2
EKG
Tech Observer
Video Camera
Respiratory Effort
Leg EMG (2)
Documents arousals, parasomnias, abnormal
sleeping position, and attends to any technical
problem
Records behavior
Courtesy of Dr. Carol Rosen
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During the Night

• Children need more frequent adjustment of sensors
  during the night than adults
• Nearly all studies of children require that the
  sensors be replaced at some point during the
  night
• Technologists should warn the patient and the
  parent that they will be entering the room during
  the night

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Documentation

• Due to the prevalence of parasomnias, childrens
  studies need frequent documentation
• Children may have significant sleep disorders
  without dramatic polysomnographic findings
• Recordings may be ambiguous at times (i.e., when
  breathing sensors have been displaced)
  technologist observations become crucial to
  interpretation
• For example discovered nasal pressure
  transducer pushed to side of face restored to
  proper position

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Describe What You See

• Helpful
• Sat up abruptly--staring and mumbling
• Patient breathing quietly
• Mom moving, wakes child
• Went into room, snoring from mother, not patient

• Not Helpful
• Possible seizure
• Cant hear patient
• Patient moving in bed
• Artifact
• Sounds from room

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The Spectrum of Pediatric Sleep Disorders
Prevalent in Children and Adults Prevalent in Children Using Different Criteria Than in Adults More Prevalent in Children Than Adults Unique to Children by Definition
Delayed sleep phase syndrome Periodic limb movement disorder Obstructive sleep apnea Restless legs syndrome Narcolepsy Sleepwalking, sleep talking Sleep terrors Nightmares Behavioral insomnia of childhood
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Estimated Prevalence of Sleep Disorders in
Children

• Insufficient sleep 10 (higher in teens up
  to 33)
• Behaviorally based - 25
• Sleep related breathing disorders - 2
• Narcolepsy 0.05
• Sleep/wake timing (delayed sleep phase) - 7
  teens
• Partial arousals (parasomnias)
• Night terrors 2 - 3
• Sleep walking 5
• Rhythmic movement disorder 3 -15
• Restless legs syndrome 2

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Who Should Have a Polysomnogram?
Guidelines for Investigation of Sleep Related
Breathing Disorders in Children

• All children should be screened for snoring
• Habitual snoring with labored breathing
• Witnessed apnea
• Restless sleep
• Evidence of daytime sleepiness
• And be sent for a polysomnogram if they show
  physical signs of sleep apnea
• Growth abnormalities
• Signs of upper airway obstruction
• Evidence of pulmonary hypertension

American Academy of Pediatrics, 2002
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Prevalence of Sleep Related Breathing Disorders
in Children

• Habitual snoring 10
• Sleep disordered breathing 2
• Risk factors
• African-American heritage
• Family history of OSA
• History of prematurity
• Chronic conditions - cerebral palsy, trisomy 21,
  achondroplasia and other genetic syndromes
• Obesity (less risky than in adults)
• No gender difference in prepubertal children

Rosen et al 2003
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Many Pediatric Diagnoses Do Not Require a
Polysomnogram

• Usually requires polysomnography
• Obstructive Sleep Apnea, Pediatric
• Narcolepsy
• Usually diagnosed by tests other than
  polysomnography (i.e., ICU monitoring)
• Primary Sleep Apnea of Infancy(formerly Primary
  Sleep Apnea of Newborn)
• Congenital Central Hypoventilation Syndrome
• May require polysomnography with extended EEG
  montage
• Complicated or atypical parasomnia

• Usually does not require polysomnography
• Behavioral Insomnia of Childhood (Sleep Onset
  Type)
• Behavioral Insomnia of Childhood (Limit-Setting
  Type)
• Sleepwalking, Night Terrors
• Sleep Enuresis
• Restless Legs Syndrome
• Sleep Related Rhythmic Movement Disorder

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Evaluating Breathing during Sleep in Children

• Children experience less desaturation with apnea
• Carbon dioxide monitoring is recommended (lt 12
  years)
• Monitoring behavior, body position, snoring is
  important
• Additional measures of effort such as esophageal
  pressure monitoring may be helpful in special
  cases

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Scoring Rules

• Apnea is recurrent partial or complete airway
  obstruction despite continued effort
• Adult -- respiratory event is 10 seconds or
  longer
• Child two missed breath duration
• ETCO2 levels above 50 mm Hg for more than 10 of
  sleep time may be abnormal

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Types of Sleep Related Breathing Disorders in
Children

• Upper airway resistance syndrome is common
• Repetitive respiratory effort related arousals
  without discrete apnea or hypopnea
• No changes in oxygen saturation or ETCO2
• Obstructive hypoventilation is common
• Upper airway narrowing with gas exchange
  abnormalities, but without clear apnea or
  hypopnea
• Most prominent in REM

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The Spectrum of Obstructive Sleep Related
Breathing Disorders in Children
APNEA
HYPOPNEA
OBSTRUCTIVE HYPOVENTILATION
RESPIRATORY EFFORT RELATED AROUSAL
SNORING
HIGH
LOW
Degree of Obstruction
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Normal Breathing NREM Sleep
Note time scale
Delta activity, K complexes, spindles in EEG
Very regular breathing
No oxygen desaturation or CO2 elevation
8 y/o with daytime sleepiness
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Normal Breathing REM Sleep
Rapid eye movements, low voltage fast EEG pattern
Breathing, heart rate somewhat irregular
8 y/o with daytime sleepiness
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RERA
Arousal (alpha activity at arrow)
Recurrent episodes of flattened nasal air
pressure and minimal oxygen desaturation
10 y/o with restless sleep
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Apnea and Hypopnea
Hypopnea between 30 and 70 air flow
Apnea less than 30 air flow
9 y/o with snoring and gasping at night and poor
school performance
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ICSD-2 Diagnostic Criteria Obstructive Sleep
Apnea, Pediatric

• The caregiver reports snoring, and/or labored or
  obstructed breathing, during the childs sleep. 
• The caregiver reports observing at least one of
  the following
• Paradoxical inward rib-cage motion during
  inspiration 
• Movement arousals 
• Diaphoresis 
• Neck hyperextension during sleep 
• Excessive daytime sleepiness, hyperactivity, or
  aggressive behavior 
• A slow rate of growth 
• Morning headaches 
• Secondary enuresis 

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Obstructive Sleep Apnea, Pediatric

• ICSD-2 Diagnostic Criteria (cont.)
• Polysomnographic recording demonstrates one or
  more scoreable obstructive respiratory events per
  hour (i.e., apnea or hypopnea of at least two
  respiratory cycles in duration)
• Note Very few normative data are available for
  hypopneas, and the data that are available have
  been obtained using a variety of methodologies.
  These criteria may be modified in the future once
  more comprehensive data become available.

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Obstructive Sleep Apnea, Pediatric

• ICSD-2 Diagnostic Criteria (cont.) 
• Polysomnographic recording demonstrates either i
  or ii.  
• i. At least one of the following is observed
• a. Frequent arousals from sleep associated with
  increased respiratory effort 
• b. Arterial oxygen desaturation in association
  with the apneic episodes 
• c. Hypercapnia during sleep 
• d. Markedly negative esophageal pressure swings  
• ii. Periods of hypercapnia, desaturation, or
  hypercapnia and desaturation during sleep
  associated with snoring, paradoxical inward
  rib-cage motion during inspiration, and at least
  one of the following 
• a. Frequent arousals from sleep 
• b. Markedly negative esophageal pressure swings

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Obstructive Sleep Apnea, Pediatric

• Many children have associated cognitive problems
  and difficulty at school
• Pediatric obstructive sleep apnea is frequently
  associated with adenotonsillar hypertrophy
• Adenotonsillectomy is effective in most children
• When applied to pediatric recordings, adult
  polysomnographic measures alone (i.e., AHI) may
  underestimate the number of patients who would
  benefit from adenotonsillectomy

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CPAP Therapy for Children

• Continuous positive airway pressure is an
  effective second-line treatment in pediatric
  patients
• A desensitization program is an extremely
  important part of treatment
• Successful trials reported in 74 of patients,
  with 86 of those able to use the therapy
  long-term

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Primary Sleep Apnea of Infancy
(formerly Primary Sleep Apnea of Newborn)

• ICSD-2 Diagnostic Criteria
• Apnea of Prematurity. Prolonged central
  respiratory pauses of 20 seconds or more in
  duration (or shorter-duration events that include
  obstructive or mixed respiratory patterns and are
  associated with a significant physiologic
  compromise, including decrease in heart rate,
  hypoxemia, clinical symptoms, or need for nursing
  intervention), are recorded in an infant younger
  than 37 weeks conceptional age.
• Apnea of Infancy. Prolonged central respiratory
  pauses of 20 seconds or more in duration (or
  shorter-duration events that include obstructive
  or mixed respiratory patterns and are associated
  with bradycardia, cyanosis, pallor, or marked
  hypotonia), are recorded in an infant with a
  conceptional age of 37 weeks or older.

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Primary Sleep Apnea of Infancy

• Should be distinguished from Acute Life
  Threatening Events (ALTE), an ill-defined
  disorder based on parental complaints and Sudden
  Infant Death Syndrome (SIDS), a post-mortem
  diagnosis
• A polysomnogram is the best way to evaluate
  breathing during sleep
• Prognosis is excellent with infrequent events
• Prognosis guarded when frequent resuscitation is
  required and events persist over time

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Congenital Central Alveolar Hypoventilation
Syndrome

• ICSD-2 Diagnostic Criteria
• The patient exhibits shallow breathing, or
  cyanosis and apnea, of perinatal onset during
  sleep. 
• Note In severely affected infants, consequences
  of hypoxia, including pulmonary hypertension and
  cor pulmonale, may also be present. 
• Hypoventilation is worse during sleep than during
  wakefulness.
• The rebreathing ventilatory response to hypoxia
  and hypercapnia is absent or diminished.
• Polysomnographic monitoring during sleep
  demonstrates severe hypercapnia and hypoxia,
  predominantly without apnea.

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Congenital Central Alveolar Hypoventilation
Syndrome

• Present from birth
• Requires lifelong treatment
• Mechanical ventilation or pacing
• Most patients do not need treatment when awake
• Associated with abnormality of the PHOX2B gene
• Associated with Hirschsprung's disease

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Narcolepsy in Children

• Narcolepsy with cataplexy is rare in children
  younger than four years old
• Daytime sleepiness frequently presents as
  reappearance of napping in a child that has
  stopped napping
• Sleepiness at school may be manifest by symptoms
  similar to attention deficit disorder
• Diagnosis may be clinical or supported by
  findings from overnight polysomnography with
  multiple sleep latency testing. Alternatively,
  measurement of levels of hypocretin in
  cerebrospinal fluid may be appropriate for
  certain patients.

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Recognizing Sleepiness in Children

• Sleepy children do not always act sleepy
• Parent may endorse other terms like seems
  overtired
• Children with insufficient or disrupted sleep can
  show
• Inattention
• Hyperactivity
• Behavioral disturbances
• Poor school performance
• Persistent, overt sleepiness is uncommon in
  preadolescent children unless the disorder is
  severe

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Pediatric MSLT

• Use standard MSLT protocol from AASM Practice
  Parameter
• Review procedure with child and parent and answer
  any questions
• It is recommended that parents leave the testing
  room during naps
• Ask if child needs to go to the bathroom
• Put up side rails if necessary
• Remind the child, I will come back in to the
  room when the nap test is over.

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SOREMP in a Child
Nap 1 lights out
Alpha activity
Reduced tone
Nap 1 0030
Rapid eye movement
12 y/o referred for excessive daytime sleepiness
and cataplexy symptoms
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Nocturnal Sleep Decreases with Age
Minutes of sleep
Ohayon et al SLEEP 200427(7)1255-73.
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Napping is Normal in Very Young Children
Age (months)
Acebo et al. SLEEP 2005 28(12) 1568-1577.
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Sleep Latency during MSLT Naps Decreases in
Adolescents with Increasing Tanner Stage
NOTE Mean sleep latency is longer in children
compared with adults
Data from Carskadon MA. The second decade. In
Guilleminault C, ed, Sleeping and waking
disorders indications and techniques. Menlo
Park Addison Wesley, 1982 99-125
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Sleep Latency Increases with Age after
Adolescence
From Arand et al, SLEEP 200528(1)123-144.
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Interpreting Pediatric MSLT Results

• Two or more sleep onset REM periods are necessary
  to support a diagnosis of narcolepsy
• Age has a complicated and profound impact on MSLT
  mean sleep latency
• Limited normative data is available
• Mean sleep latencies that might be considered
  normal for adults are often abnormal for children
• The ICSD-2 states, The MSLT has not been
  validated as a diagnostic test in children
  younger than eight years of age.

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Parasomnias

• Children are often referred to the sleep center
  because of unusual behaviors during the night
• Sleepwalking
• Sleep terrors
• Nightmares
• Seizures

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Abnormal Breathing and EEG Activity in Sleep
9 y/o with known epilepsy and snoring
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Sleepwalking and Sleep Terrors Partial Arousal
Parasomnias

• Partial arousal parasomnias
• Occur during first half of night
• Arise from slow wave sleep
• Child is not awake
• Sleepwalking
• Child moves around room or house
• May be quiet or agitated
• May engage in purposeful activities, like
  unlocking door
• Sleep terrors
• Child abruptly sits up screaming
• Appears frightened and agitated

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• Night Terrors
• Deep NREM sleep
• First third of night
• Child confused or agitated
• Difficult to reassure
• Intense arousal lasting 2-10 min
• Abrupt return to sleep
• No recall in the morning

• Nightmares
• REM sleep
• Last half of night
• Child alert describes dream content
• Comforted by parent
• Difficulty going back to sleep
• Recall the following day

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Technologist Response to Unusual Behaviors

• Parasomnias can lead to injury
• Be sure patient is safe
• Parasomnias sometimes resemble seizures
• Seizures (especially frontal lobe) can resemble
  parasomnias
• During study describe what you see
• Note event on record when it is happening
• Sitting up yelling
• Patient mumbling cant understand words
• Patients left arm and leg twitching
• Mother trying to comfort, patient keeps yelling
  mommy
• Patient trying to get out of bed

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Confusional Arousal
5 y/o with witnessed apnea and restlessness
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Restless Legs Syndrome
ICSD-2 Diagnosis in Adult Patients

• The patient reports an urge to move the legs,
  usually accompanied or caused by uncomfortable
  and unpleasant sensations in the legs.
• The urge to move or the unpleasant sensations
• begin or worsen during periods of rest or
  inactivity (lying or sitting)
• are partially or totally relieved by movement,
  such as walking or stretching, at least as long
  as the activity continues
• are worse, or only occur, in the evening or night

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Restless Legs Syndrome

• ICSD-2 Diagnostic Criteria
• The child meets all four essential adult criteria
  for RLS listed above and relates a description,
  in his or her own words, that is consistent with
  leg discomfort.
• OR
• The child meets all four essential adult criteria
  for RLS listed above but does not relate a
  description in his or her own words that is
  consistent with leg discomfort.
• AND
• The child has at least two of the following three
  findings 
• i. A sleep disturbance for age 
• ii. A biological parent or sibling with definite
  RLS 
• iii. A polysomnographically documented periodic
  limb movement index of five or more movements
  per hour of sleep 
• Note Criteria for probable and possible
  childhood RLS have been developed for research
  purposes and are included in a National
  Institutes of Health diagnostic workshop report. 

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Restless Legs Syndrome (RLS) Periodic Limb
Movement Disorder (PLMD)

• Prevalence in children 0.5-2, familial link
• RLS - growing pains
• PLMD leg jerks - whats normal
• Relationship with hyperactivity?
• Can be associated with
• Iron deficiency/low ferritin
• Chronic renal disease
• Diagnostic controversies in adults
• Scant data in children
• May present as insomnia or sleepiness

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Criteria for Sleep Related Rhythmic Movement
Disorder

• ICSD-2 Diagnostic Criteria
• The patient exhibits repetitive, stereotyped, and
  rhythmic motor behaviors.
• The movements involve large muscle groups.
• The movements are predominantly sleep related,
  occurring near nap or bedtime, or when the
  individual appears drowsy or asleep.
• The behaviors result in a significant complaint
  as manifest by at least one of the following
• i. Interference with normal sleep
• ii. Significant impairment in daytime function
• iii. Self-inflicted bodily injury that requires
  medical treatment (or would result in injury if
  preventable measures were not used)

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Sleep Related Rhythmic Movements

• Repetitive movements
• Head banging or head rolling
• Body rocking
• Before sleep, light sleep, or even awake
• Prevalence of rhythmic movements decreases with
  age
• At nine months 59
• At eighteen months 33
• At five years 5
• No gender difference
• Polysomnogram or treatment rarely indicated

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Sleep Enuresis
ICSD-2 Diagnostic Criteria

• Primary
• The patient is older than five years of age
• The patient exhibits recurrent involuntary
  voiding during sleep, occurring at least twice a
  week.
• The patient has never been consistently dry
  during sleep.

• Secondary
• The patient is older than five years of age
• The patient exhibits recurrent involuntary
  voiding during sleep, occurring at least twice a
  week.
• The patient has previously been consistently dry
  during sleep for at least six months.

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Prevalence of Enuresis
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Developmental Overview of Common Non-respiratory
Sleep Problems
Newborn/ Young Infant Older Infant and Toddler Pre-schooler School Age Teenager
Usually normal Developmental Self limited Night wakings Difficulty settling Night terrors Night wakings Bedtime resistance Night terrors Sleep walking Insufficient sleep Bedtime resistance Sleep walking Insufficient sleep Delayed sleep phase Narcolepsy
Rhythmic movements Bedtime fears Rhythmic movements Bedtime fears Nightmares Enuresis Bruxism
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Behavioral or Life Style Sleep Problems

• Sleep onset association disorder
• Limit setting disorder
• Poor sleep hygiene
• Caffeine
• Irregular schedule
• TV/computer/cell phone/electronics in bedroom
• Overlap with delayed sleep phase
• Perpetuated by weekend sleep-in and late day naps
• Management change behaviors

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Behavioral Insomnia of Childhood (Sleep-onset
Type)

• ICSD-2 Diagnostic Criteria
• Falling asleep is an extended process that
  requires special conditions
• Sleep-onset associations are highly problematic
  or demanding
• In the absence of the associated conditions,
  sleep onset is significantly delayed or sleep is
  otherwise disrupted
• Awakenings require caregiver intervention for the
  child to return to sleep.

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Sleep Onset TypeTypical Presentations

• Child falls asleep during rocking or patting,
  needs to be rocked or patted after night waking
• Child falls asleep feeding, needs to be fed to
  fall asleep
• Child falls asleep with parent singing, reading
  or lying next to child, but cannot fall sleep
  alone
• Child falls asleep in car seat, needs to be
  driven around to fall asleep

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Behavioral Insomnia of Childhood (Limit-setting
Type)

• ICSD-2 Diagnostic Criteria
• The child has difficulty initiating or
  maintaining sleep
• The child stalls or refuses to go to bed at an
  appropriate time or refuses to return to bed
  following a nighttime awakening
• The caregiver demonstrates insufficient or
  inappropriate limit setting to establish
  appropriate sleeping behavior in the child

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Limit-setting TypeTypical Presentations

• Child is two years or older
• Stalling behaviors at bedtime
• Needs a drink or food
• Multiple stories
• Crying, clinging
• Gets out of bed (curtain calls)
• Parents behavior contributes to problem
• Irregular or inappropriate schedules
• Inconsistent application of rules
• Secondary gain for child

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Contributing Factors

• Circadian rhythms develop over the first few
  months of life infants have frequent awakenings
  and irregular schedules at birth
• Homeostatic drive to sleep is blunted by frequent
  napping
• Environmental factors such as warmth, soothing
  sounds and vestibular stimulation promote
  sleepiness
• Learned associations serve as triggers for sleep
  onset

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Behavioral Insomnia of ChildhoodTreatment
Options

• Extinction
• Graduated extinction (Ferberizing)
• Positive routines
• Faded bedtime with response cost
• Scheduled awakenings
• Parent education
• Medications (efficacy unproven in children)
• Prescription
• Over-the-counter