1. 1

2. 2 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

3. 3 Children: Not just short adults

4. 4 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 child’s usual bedtime, resulting in an overtired child

5. 5 A Family Centered Care Approach Parents are the experts on their child and a constant in their child’s 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 child’s sense of mastery and control over a potentially stressful experience Zaremba et al, JCSM, 2005

6. 6 Important “Mind-Set” Changes by the Polysomnography Staff FROMTO needs of the staffneeds 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

7. 7 Preparing the Family for a Polysomnogram Provide detailed information about the test Schedule testing for the child’s 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

8. 8 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

9. 9 Creating a Calm Environment Take time to establish rapport Explore the child’s 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

10. 10 Child and Family Preparation On the study night… –Allow the child to explore room and sensors –Define each person’s job –Develop a plan for coping –Maintain patience, flexibility, positive attitude –Lavish the child with praise Focusing on the desired behavior

11. 11 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

12. 12 Optimizing the Environment for 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

13. 13 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

14. 14 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 child’s age Be honest and careful in your word choice Sarcasm and teasing may be misinterpreted and should be avoided

15. 15 Tips for Improving Cooperation Younger children may want to sit in their parent’s 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

16. 16 Positions for Comfort Zaremba et al, JCSM, 2005

17. 17 Courtesy of Dr. Carol Rosen Pediatric Polysomnography Tech Observer Video Camera SaO 2 Leg EMG (2) Microphone EKG Chin EMG (2) EEG EOG Nasal EtCO 2 Records behavior Documents arousals, parasomnias, abnormal sleeping position, and attends to any technical problem Respiratory Effort Nasal Oral Airflow

18. 18 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

19. 19 Documentation Due to the prevalence of parasomnias, children’s 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”

20. 20 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 –Can’t hear patient –Patient moving in bed –Artifact –Sounds from room

21. 21 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

22. 22 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%

23. 23 Who Should Have a Polysomnogram? 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 Guidelines for Investigation of Sleep Related Breathing Disorders in Children

24. 24 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

25. 25 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

26. 26 Evaluating Breathing during Sleep in Children Children experience less desaturation with apnea Carbon dioxide monitoring is recommended (< 12 years) Monitoring behavior, body position, snoring is important Additional measures of effort such as esophageal pressure monitoring may be helpful in special cases

27. 27 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 ETCO 2 levels above 50 mm Hg for more than 10% of sleep time may be abnormal

28. 28 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 ETCO 2 Obstructive hypoventilation is common –Upper airway narrowing with gas exchange abnormalities, but without clear apnea or hypopnea Most prominent in REM

29. 29 The Spectrum of Obstructive Sleep Related Breathing Disorders in Children APNEA HYPOPNEA RESPIRATORY EFFORT RELATED AROUSAL OBSTRUCTIVE HYPOVENTILATION SNORING Degree of Obstruction HIGHLOW

30. 30 Normal Breathing – NREM Sleep Delta activity, K complexes, spindles in EEG Very regular breathing No oxygen desaturation or CO 2 elevation 8 y/o with daytime sleepiness Note time scale

31. 31 Normal Breathing –REM Sleep Rapid eye movements, low voltage fast EEG pattern Breathing, heart rate somewhat irregular 8 y/o with daytime sleepiness

32. 32 RERA Arousal (alpha activity at arrow) Recurrent episodes of flattened nasal air pressure and minimal oxygen desaturation 10 y/o with restless sleep

33. 33 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

34. 34 ICSD-2 Diagnostic Criteria: Obstructive Sleep Apnea, Pediatric The caregiver reports snoring, and/or labored or obstructed breathing, during the child’s sleep. The caregiver reports observing at least one of the following: i.Paradoxical inward rib-cage motion during inspiration ii.Movement arousals iii.Diaphoresis iv.Neck hyperextension during sleep v.Excessive daytime sleepiness, hyperactivity, or aggressive behavior vi.A slow rate of growth vii.Morning headaches viii.Secondary enuresis

35. 35 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. Obstructive Sleep Apnea, Pediatric

36. 36 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 Obstructive Sleep Apnea, Pediatric

37. 37 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

38. 38 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

39. 39 Primary Sleep Apnea of Infancy 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. (formerly Primary Sleep Apnea of Newborn)

40. 40 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

41. 41 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.

42. 42 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

43. 43 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.

44. 44 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

45. 45 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.”

46. 46 SOREMP in a Child Rapid eye movement Alpha activity Nap #1 00:30 Nap #1 lights out 12 y/o referred for excessive daytime sleepiness and cataplexy symptoms Reduced tone

47. 47 Nocturnal Sleep Decreases with Age Ohayon et al SLEEP 2004;27(7):1255-73. Minutes of sleep

48. 48 Acebo et al. SLEEP 2005; 28(12): 1568-1577. Napping is Normal in Very Young Children Age (months)

49. 49 Sleep Latency during MSLT Naps Decreases in Adolescents with Increasing Tanner Stage 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 NOTE: Mean sleep latency is longer in children compared with adults

50. 50 Sleep Latency Increases with Age after Adolescence From Arand et al, SLEEP 2005;28(1):123-144.

51. 51 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.”

52. 52 Parasomnias Children are often referred to the sleep center because of unusual behaviors during the night –Sleepwalking –Sleep terrors –Nightmares –Seizures

53. 53 Abnormal Breathing and EEG Activity in Sleep 9 y/o with known epilepsy and snoring

54. 54 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

55. 55 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

56. 56 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 – can’t understand words –Patient’s left arm and leg twitching –Mother trying to comfort, patient keeps yelling “mommy” –Patient trying to get out of bed

57. 57 Confusional Arousal 5 y/o with witnessed apnea and restlessness

58. 58 Restless Legs Syndrome 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 ICSD-2 Diagnosis in Adult Patients

59. 59 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.

60. 60 Restless Legs Syndrome (RLS) & Periodic Limb Movement Disorder (PLMD) Prevalence in children 0.5-2%, familial link –RLS - “growing pains” –PLMD – leg jerks - what’s 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

61. 61 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)

62. 62 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

63. 63 Sleep Enuresis 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. ICSD-2 Diagnostic Criteria

64. 64 Prevalence of Enuresis

65. 65 Developmental Overview of Common Non-respiratory Sleep Problems Newborn/ Young Infant Older Infant and Toddler Pre- schoolerSchool AgeTeenager 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

66. 66 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

67. 67 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.

68. 68 Sleep Onset Type Typical 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

69. 69 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

70. 70 Limit-setting Type Typical 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”) Parent’s behavior contributes to problem –Irregular or inappropriate schedules –Inconsistent application of rules –Secondary gain for child

71. 71 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

72. 72 Behavioral Insomnia of Childhood: Treatment 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