Goals of this Presentation

2. Goals of this Presentation • Learn how to prepare for a successful pediatric sleep study • Learn what to look for and how to respond during the study • Learn about pediatric sleep disorders and their treatments

3. Children: Not just short adults

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 andremoving electrodes may extend set up time past the child’s usual bedtime, resulting in an overtired child

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. Important “Mind-Set” Changes by the Polysomnography Staff Zaremba et al, JCSM, 2005

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. 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. 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. 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. Engagingthe 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. 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

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

17. 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

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. 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. 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 Describe What You See

21. The Spectrum of Pediatric Sleep Disorders

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. 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

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. 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 Many Pediatric Diagnoses Do Not Require a Polysomnogram

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. 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

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 ETCO2 • Obstructive hypoventilation is common • Upper airway narrowing with gas exchange abnormalities, but without clear apnea or hypopnea • Most prominent in REM

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

30. 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

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

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. 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: • 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 

35. 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.

36. 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

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. 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. 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.

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

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

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

49. 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

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