Pediatric sleep

C H A P T E R

16 Pediatric sleep Alexa J. Watach, Melissa S. Xanthopoulos, Olufunke Afolabi-Brown, Bruno Saconi, Amy M. Sawyer University of Pennsylvania, Philadelphia, PA, United States

Overview of sleep The average human spends one-third of his or her life asleep (Aminoff, Boller, & Swaab, 2011). Sleep is critical for overall health and wellness, impacting how we feel, function, and perform. It is not a passive or dormant state but part of an active physiological process. Sleep affects almost every bodily system, including the brain, heart, lungs, metabolism, and immune system, and plays a vital role in helping the body recover from daily stress (i.e., homeostasis) (Medic, Wille, & Hemels, 2017).

Sleep anatomy, physiology, and architecture Numerous brain structures (e.g., suprachiasmatic nucleus, hypothalamus, brain stem, thalamus, amygdala) are involved in facilitating sleep, each playing an important role in sleepewake signaling and staging while two biological mechanisms impact sleep: circadian rhythm and homeostasis (National Institute of Neurological Disorders and Stroke, 2018). Circadian rhythms, often referred to as an “internal clock,” influence sleep onset and offset by working to synchronize with environmental cues (e.g., light), hormonal cues (e.g., melatonin), and personal routines (e.g., wake time). Sleepewake homeostasis influences sleep drive, or “pressure to sleep,” by signaling the body to sleep after a sustained period of wakefulness. Sleep is divided into two phases: rapid eye movement (REM) and nonREM sleep (N). Non-REM consists of three stages: (1) N1, short stage of light sleep where the body and brain waves begin to slow from patterns of

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16. Pediatric sleep

wakefulness to sleep; (2) N2, further slowing of heartbeat, breathing, and muscle relaxation; decreased body temperature; cessation of eye movements; and slowed brain wave activity marked with brief bursts of activity; and (3) N3, deep sleep accompanied by the lowest heart and respiratory rates, and slowest brain wave activity (National Institute of Neurological Disorders and Stroke, 2018). REM sleep, stage R, is marked by rapid eye movement, increased heart rate, respiratory rate, blood pressure, and brain wave activities that appear similar to wakefulness. Individuals cycle through all stages of REM and non-REM throughout the night, with young children and infants spending more time in REM-like sleep prior to the age of 5 years, as compared with adults (Alsubie & BaHammam, 2017).

Recommended sleep duration guidelines Sleep requirements vary as individuals age. Adequate sleep duration is important for growth and development, including behavior, learning, memory, attention, emotion regulation, quality of life, and mental and physical health. Expert-informed sleep duration guidelines exist for the National Sleep Foundation (Hirshkowitz et al., 2015) and American Academy of Sleep Medicine (Paruthi et al., 2016). Endorsed by the American Academy of Pediatrics (2018), the American Academy of Sleep Medicine guidelines set forth specific sleep duration recommendations for all ages of children (see Table 16.1).

Consequences of insufficient sleep or sleep disruption Although sleep is an important and necessary pillar of health, difficulties obtaining recommended amounts of sleep are common in children, including insufficient sleep due to sleep disorders. Accumulation of

TABLE 16.1 Recommended Pediatric Sleep Durations (Paruthi et al., 2016). Age group

Recommended sleep duration, per 24 hours

Infants • 4e12 months

12e16 hours (including naps)

Children • 1e2 years • 3e5 years • 6e12 years

11e14 hours (including naps) 10e13 hours (including naps) 9e12 hours

Teenagers • 13e18 years

8e10 hours

Sleep evaluation

381

insufficient sleep increases risk of injuries and accidents, hypertension, obesity, depression, learning difficulties, and other complications (Medic et al., 2017; Owens, 2014). Sleep is also not often prioritized. Lifestyle factors (e.g., overpacked schedules, work, social events, 24/7 technology) can impact sleep opportunity, quality, and duration. Regardless of the presence of a sleep disorder, establishing a bedtime routine is important to ensuring children, and teens have the opportunity to receive an adequate amount of sleep. Although steps to establishing a healthy bedtime routine are beyond the scope of this chapter, resources can be found at www. healthychildren.org (American Academy of Pediatrics, 2018).

Sleep disorders Sleep disorders in children are prevalent with upward of 25% children affected, with higher rates for children with neurodevelopmental disorders (Maski & Owens, 2016). The International Classification of Sleep Disorders, third edition (ICSD-3; American Academy of Sleep Medicine, 2014), categorizes sleep disorders into six subtypes (Table 16.2). Children should be screened at routine well child visits for symptoms suggestive of sleep disorders. Clinical evaluation with detailed sleep and medical history and physical examination is important for differentiating between various sleep disorders. The use of objective testing complements the history and physical and should be used when needed.

Sleep evaluation A thorough sleep and medical history and focused physical examination are essential to the diagnosis and management of sleep disorders in children. Various screening questionnaires are available, which can augment information obtained through history or indicate need for further evaluation. Used in both primary care and sleep centers, the validated BEARS screening tool includes assessment of five areas of sleep: Bedtime, Excessive daytime sleepiness, Awakenings at night, Regularity/ duration, and Snoring (Owens & Dalzell, 2005). A comprehensive sleep history should start with the chief sleep complaint and include questions about onset, duration, and severity of symptoms. Details from the history can then be focused toward the primary sleep complaints. Other components of the history should include assessment of sleep/wake schedule, difficulty with initiating or maintaining sleep, sleep environment, snoring or breathing difficulties during sleep, abnormal sleep movements, and daytime symptoms (excessive

TABLE 16.2 Classification of Common Sleep Disorders Seen in Pediatric Sleep Centers (American Academy of Sleep Medicine, 2014). Disorder a

Insomnia

Definition/Types/Key features

Evaluation

Difficulty initiating, maintaining sleep or earlymorning awakening associated with daytime consequences. Common forms in children include the following: 1. Behavioral insomnia of childhood • Seen as early as 6 months of age and can persist into adolescence a) Sleep-onset association: Only falling asleep under certain conditions; requiring those to fall back asleep (e.g., rocking, bottle feeding prior to sleep) b) Inadequate limit setting: Parental difficulty setting rules/limits for bedtime; child protests/resistance (e.g., calls for water, extra hugs) 2. Psychophysiological insomnia • Heightened arousal to sleep or sleeprelated environment • Excessive worry and focus about sleep • Primarily in older children and adolescents 3. Inadequate sleep hygiene • Activities of daily living that do not promote good quality sleep • Excessive daytime napping, use of products that further disrupt sleep such as caffeine, alcohol, engaging in mentally stimulating activities around bedtime such as electronics

1. 2. 3. 4.

Clinical history Sleep log or diary Actigraphy PSG not indicated unless concerns for concomitant sleep disorder

Treatment Nonpharmacological/behavioral: 1. Adequate sleep hygiene 2. Consistent bedtime routine 3. Graduated extinction: weaning child from dependence upon parent’s presence, bottle, rocking, etc. 4. Positive reinforcement 5. Psychophysiological insomnia • CBTI; relaxation techniques; screening and management of underlying anxiety Pharmacologic: • No FDA-approved medications

Sleep-related breathing disorders

Spectrum of breathing disorders that disrupt nocturnal respiration and sleep architecture 1. OSAS • Recurrent episodes of complete or partial airway obstruction • Most common cause in children is adenotonsillar hypertrophy; other risk factors; obesity, craniofacial anomalies, Down’s syndrome • Night symptoms of snoring, labored/ paradoxical breathing; daytime symptoms of disrupted sleep, cognitive problems 2. CSA • Pathologic repeated or prolonged cessation of airflow due to decreased respiratory drive • Causes include brain stem compression (intracranial tumors, Chiari malformations), neuromuscular and genetic conditions 3. Sleep-related hypoventilation • Downregulation of ventilation during sleep a) Neuromuscular conditions (e.g., muscular dystrophy) b) Central hypoventilation syndrome, including the following: • CCHS • Rapid-onset obesity, hypoventilation, hypothalamic and autonomic dysfunction

1. Clinical history 2. Physical exam (e.g., enlarged tonsils) 3. PSG demonstrates AHI >1.5/hours of sleep (OSAS) 4. Nocturnal hypoventilation >25% of total sleep time with CO2 measurements >50 Torr 5. PFT may show restrictive lung disease in neuromuscular conditions 6. Brain MRI: Brain stem abnormalities such as Chiari malformations, tumors causing CSA 7. Genetic testing in CCHS

Nonpharmacological/surgical: OSAS: 1. Adenotonsillectomyd first-line treatment if indicated 2. Mandibular advancement in children with micrognathia/ retrognathia Other treatments if surgery is not curative/not indicated: 1. Positive airway pressure (PAP) • CPAP, BPAP, AutoPAP 2. Weight loss 3. Watchful waiting in some cases if mild OSAS (Marcus et al., 2013) CSA and hypoventilation: 1. Noninvasive ventilation 2. Invasive ventilation 3. Diaphragm pacer (CCHS) 4. Brain stem decompression (Chiari malformation) Pharmacological: In mild-to-moderate OSAS • Nasal corticosteroid • Montelukast

Continued

TABLE 16.2

Classification of Common Sleep Disorders Seen in Pediatric Sleep Centers (American Academy of Sleep Medicine, 2014).dcont’d

Disorder

Definition/Types/Key features

Evaluation

Treatment

Central disorders of hypersomnolence

Group of sleep disorders with chief complaint of excessive daytime sleepiness, not caused by disrupted night sleep, circadian rhythm misalignment, or other sleep disorder: 1. Narcolepsy • Daily episodes of uncontrollable urge to sleep • Hypnagogic/hypnopompic hallucinations • Sleep paralysis • Cataplexy in type 1; absence of cataplexy in type 2 • Sleep fragmentation 2. Kleine Levin syndrome • Discrete episodes of recurrent hypersomnia and prolonged sleep; lasts few days to several weeks • Cognitive dysfunction, altered perception • Sexual disinhibition • Hyperphagia/anorexia during episodes • Normal alertness and function between episodes 3. Insufficient sleep syndrome • persistent excessive daytime sleepiness due to not attaining recommended amount of sleep • Improves with sleep extension

1. 2. 3. 4.

Clinical history Sleep log or diary Actigraphy PSG and MSLT for narcolepsy • Mean sleep latency of
8 minutes and 2 REM periods 5. CSF hypocretin-1 concentration <110 pg/mL (narcolepsy type 1)

Nonpharmacological: 1. Regular sleepewake schedule 2. Maintain sleep hygiene 3. Address safety: Driving, school, etc. 4. Scheduled daytime napping for narcolepsy Pharmacologic: Narcolepsy: • Stimulants (e.g., amphetamines, methylphenidate, modafinil) • Cataplexy reducing agents (e.g., oxybate [xyrem], venlafaxine) KLS • Most treatments ineffective; llithium may decrease frequency and severity of episodes

Circadian rhythm sleepewake disorders

Mismatch between patient’s attempt to sleep and their endogenous circadian rhythm. Most common forms in children: 1. Delayed sleepewake phase disorder • Delay in sleep in relation to desired/ required sleep and wake time • Patients commonly described as evening types (night owls); increased energy and alertness as day progresses • EExcessive sleep inertia in morning; daytime sleepiness, irritability, learning problems • Sleep efficiency normal when allowed to choose own schedule • peak prevalence in adolescents/young adulthood 2. Irregular sleepewake rhythm disorder • Disorganized sleepewake pattern throughout the 24 hours cycle • Can be seen in children with neurodevelopmental disabilities 3. Non-24-hour sleepewake rhythm disorder • Intrinsic circadian rhythm not aligned to the 24-h light dark cycle • Progressively delayed sleepewake patterns • Can be seen in children with blindness as well as severe intellectual disabilities

1. Clinical history 2. Chronotype assessment with morningnesse eveningness questionnaires 3. Sleep log 4. Actigraphy

Nonpharmacological: 1. Adequate sleep hygiene 2. Consistent bed and wake time 7 days/week 3. Bright light exposure in morning 4. Avoid light/stimulating activities in evening Pharmacological: • Melatonin at nighttime

Continued

TABLE 16.2

Classification of Common Sleep Disorders Seen in Pediatric Sleep Centers (American Academy of Sleep Medicine, 2014).dcont’d

Disorder

Definition/Types/Key features

Evaluation

Treatment

Parasomnias

Abnormal behaviors/experiences occurring during transition into sleep, within sleep, and during arousal from sleep 1. Disorders of arousal from non-REM sleep • Typically occur during the first third of night; initiated out of slow wave sleep • Brief episodes; some up to 30 minutes • Complex behaviors but high level of cognition often absent • Inappropriate/absent responsiveness to efforts to intervene • Partial or complete amnesia following episodes a. Confusional arousals: confused behavior following awakening; no associated flushing, sweating or stereotyped behavior seen; no displacement from bed b. Sleep walking: arousal associated with ambulation and behaviors out of bed; child often clumsy with increased risk of injury c. Sleep terrors: child often frightened, agitated, confused, screaming, crying, and exaggerated autonomic response such as sweating, flushing, tachycardia; not responsive/ worsened by soothing efforts

1. Clinical history 2. PSG if episodes are frequent or severe to assess for disorders, resulting in arousal/ triggers

Nonpharmacological: 1. Reassurance as episodes decrease with age 2. Adequate sleep time 3. Appropriate sleep hygiene 4. For sleep terrors, minimize interventions to decrease duration/intensity 5. Safety measures for home (high place locks, gates near steps, alarms, etc.) 6. Scheduled awakenings 15 minutes before usual time of occurrence 7. Imagery rehearsal for nightmare disorders (not well studied in children) Pharmacological/surgical intervention: • Treatment of any underlying triggers (OSAS, RLS/PLMD, reflux)

2. Disorders of arousal from REM sleep • Occur during the final third of night/ early hours of the morning. Most common form: Nightmare disorder: disturbing dreams that awaken (associated with negative emotions); memory intact with full alertness upon awakening; often occurs in children with exposure to severe psychosocial stressors 3. Other parasomnias: Sleep-related enuresis: recurrent voiding occurring during sleep; could occur as a result of sleep disorders that disrupt sleep (e.g., OSAS) Sleep-related movement disorders

Simple stereotyped movements that disrupt sleep initiation/maintenance resulting in impaired daytime function; unexplained by other movement or neurological disorder 1. RLS • Unpleasant sensation mostly in the lower extremities • Urge to move/stretch to relieve discomfort • Worsened by rest or inactivity • Mainly in the evening prior to sleep • Associated with low ferritin stores, genetic predilection • Worsened by caffeine/alcohol/stress

1. Clinical history (in child’s own words for RLS) 2. Actigraphy 3. PSG required for diagnosis of PLMD but not RLS; rhythmic masticatory activity may be seen with bruxism 4. Ferritin level for suspected RLS or PLMD

Nonpharmacological: 1. Avoiding caffeine/alcohol 2. Gentle massage/stretches 3. Adequate sleep hygiene 4. Safety precautions (e.g., padding head of the bed for movement disorders) 5. Dental splints if bruxism occurs frequently or is severe Pharmacological: • For RLS and PLMD, iron supplementation if serum ferritin is < 50 ng/mL

Continued

TABLE 16.2 Classification of Common Sleep Disorders Seen in Pediatric Sleep Centers (American Academy of Sleep Medicine, 2014).dcont’d Disorder

Definition/Types/Key features

Evaluation

Treatment

2. PLMD • Repetitive stereotyped limb movements during sleep • Parental report of restless sleep/leg kicking • PSG demonstrates periodic limb movements (>5/h) 3. Rhythmic movement disorders • Repetitive stereotypic movements of the head, neck, or trunk, which occurs during transition to sleep • Involves large muscle groups (e.g., head banging, body rocking); self-inflicted bodily injury can occur 4. Sleep-related bruxism • Repetitive teeth grinding/clenching • Jaw muscle pain, morning headaches and fatigue; abnormal tooth wear Notes. Polysomnography (PSG), cognitive behavioral therapy for insomnia (CBTI), congenital central hypoventilation syndrome (CCHS); Food and Drug Administration (FDA), obstructive sleep apnea syndrome (OSAS), central sleep apnea (CSA), pulmonary function tests (PFT), apnea hypopnea index (AHI), positive airway pressure (PAP), continuous positive airway pressure (CPAP), bilevel positive airway pressure (BPAP), autoadjusting positive airway pressure (AutoPAP), KleineeLevin syndrome (KLS), multiple sleep latency test (MSLT), rapid eye movement (REM), restless legs syndrome (RLS), periodic limb movement disorder (PLMD). a All the prior subtypes of insomnia described in ICSD-2 have been classified under a single diagnosis (chronic insomnia disorder) in the ICSD-3 as there was limited evidence to support these distinct subtypes. Treatment approaches are still targeted toward the forms listed above.

Obstructive sleep apnea syndrome treatments

389

sleepiness, hyperactivity, inattentiveness, or irritability as well as poor school performance). Since children in younger age groups may not present with excessive sleepiness but rather other daytime symptoms (Beebe, 2011), it is important to screen for sleep disorders when children present with any behavioral or mood concerns. The physical examination should focus on identification of the sleep disorder and consequences associated with sleep pathology. Essential components of the physical examination include general appearance, evaluation of growth parameters (for failure to thrive or obesity), assessment of craniofacial or dysmorphic anomalies (e.g., Down syndrome, Pierre Robin syndrome), oropharynx/oral examination, and neurologic/mental state evaluation. Objective testing may be needed to confirm specific sleep disorders and should complement the history and physical examination. Testing commonly performed in children include polysomnogram, multiple sleep latency test, and actigraphy. As displayed in Table 16.2, treatments for sleep disorders include behavioral, medical (e.g., positive airway pressure [PAP], orthodontia), and pharmacological approaches. Promotion of adherence to any of these interventions is essential for successfully managing a sleep disorder.

Obstructive sleep apnea syndrome The remainder of this chapter is devoted to discussing obstructive sleep apnea syndrome (OSAS) treatment adherence, as adherence to other sleep disorder treatments are sparsely addressed in the scientific literature. OSAS is defined by repeated episodes of complete or partial airway obstruction (American Thoracic Society, 1996) and is estimated to affect approximately 1%e4% of children (Lumeng & Chervin, 2008). Nighttime symptoms can include snoring and labored/paradoxical breathing. Daytime symptoms can include hyperactivity in younger children; daytime sleepiness in older children and problems with attention (including while driving), mood, school performance; and other cognitive complications (American Academy of Sleep Medicine, 2014). OSAS risk is assessed via clinical history and physical examination and diagnosed with polysomnography (i.e., sleep study).

Obstructive sleep apnea syndrome treatments Adenotonsillectomy Enlargement of the tonsils and adenoids (adenotonsillar hypertrophy) is the most common cause of pediatric OSAS; therefore, surgical removal

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16. Pediatric sleep

of the tonsils and adenoids (adenotonsillectomy) is the primary treatment (Marcus, Brooks, et al., 2012). Adenotonsillectomy can be successful in resolving OSAS, although OSAS may persist postsurgery, with residual OSAS ranging from 13% to 29% in low-risk populations (i.e., nonobese, less severe OSAS, younger children, no snoring) and up to 73% in obese youth (Marcus, Brooks, et al., 2012).

Positive airway pressure Second-line treatment for pediatric OSAS is PAP therapy. PAP is a noninvasive method of treating OSAS, involving a portable machine that delivers air pressure above the closing pressure of the airway to prevent obstruction. Several types of devices exist: continuous positive airway pressure (CPAP), bilevel positive airway pressure (BPAP), and automatic positive airway pressure (AutoPAP). Pressurized air is supplied through tubing that is connected to a mask. A variety of mask interfaces exist (e.g., nasal masks, oronasal [full-face] masks); mask type is selected based on anatomical features, developmental level, and individual preference. As children grow and develop or if other changes occur (e.g., weight loss, orthodontics), repeat sleep studies should be conducted to ensure adequate treatment pressure and/or that treatment is still indicated. PAP is an efficacious treatment for postsurgical residual OSAS, nonsurgical candidates, or based on patient/family preferences. However, adherence is a major barrier to effective PAP treatment.

Other treatments Other less common treatment options/recommendations are available, such as weight loss (Marcus, Brooks, et al., 2012), watchful waiting (Marcus et al., 2013), intranasal steroids such as corticosteroids or oral leukotriene antagonists such as montelukast (Marcus, Brooks, et al., 2012), and craniofacial and orthodontic procedures or oral appliances (Cielo & Gungor, 2016; Tapia & Marcus, 2013).

Pediatric positive airway pressure adherence Pediatric PAP users are faced with challenges, such as acceptance of a treatment that may last many months or even years, establishing a new daily routine, and specific barriers (e.g., mask discomfort, children pulling masks off in the middle of the night, negative social reactions, lack of caregiver engagement) that come with wearing PAP. Although PAP is an efficacious treatment, it is only effective if the device is regularly used.

Theoretical approaches to adherence in pediatric sleep disorders

391

Untreated OSAS, as a result of PAP nonadherence, can lead to cardiovascular, neurobehavioral, developmental and growth abnormalities; with treatment, these consequences can be avoided or improved (Marcus, Brooks, et al., 2012).

Measuring positive airway pressure adherence Early measures of pediatric PAP use relied on caregiver report. Today, measuring adherence has become simpler, faster, and more accurate due to advances in technology. PAP devices contain an internal microprocessor that records the amount of time powered-on at effective pressure (i.e., amount of time the device is actually being worn). Usage data (hours, minutes, and percentage of nights used) can be downloaded directly from the machine during follow-up visits or by a data card easily ejected from the machine. Alternatively PAP can be equipped with a modem, and nightly use data are directed to a HIPAA compliant online server. Brief lapses in use, such as mask leaks, can be detected and observed, which prompt providers to discuss mask fit and mask adjustment or recommend a mask change. In adults, adherence to PAP is commonly defined as use 4 hours per night on 70% of nights; for insurance payment of PAP treatment, this threshold of use is required for a consecutive 30-day period at treatment outset (Centers for Medicare & Medicaid Services, 2016; Sawyer et al., 2011). In children, optimal adherence has not been standardized because sleep providers and researchers are skeptical that these same parameters are appropriate for children, as they have longer sleep durations and different sleep patterns compared with adults (King, Xanthopoulos, & Marcus, 2014). For clinical purposes, the adult definition of adherence is used by insurance providers to qualify the costs of PAP (Centers for Medicare & Medicaid Services, 2016). Costs for the PAP device are only covered by insurance if adherence can be proven (e.g., data card downloads or internal modems); otherwise, nonadherence will require the individual to make an out-of-pocket purchase or return of the device.

Theoretical approaches to adherence in pediatric sleep disorders Little is known about adherence predictors, including moderators and the influence of mediators, on adherence to sleep disorder treatments among children. Existing studies are isolated to examining PAP adherence in pediatric OSA. Such studies have examined an inconsistent set of influential factors, thus leading to inconclusive results on what factors are

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16. Pediatric sleep

interventional targets to improve pediatric PAP adherence (Alebraheem, Toulany, Baker, Christian, & Narang, 2018; DiFeo et al., 2012; Hawkins, Jensen, Simon, & Friedman, 2016; Lynch, Elliott, Avis, Schwebel, & Goodin, 2017; Prashad et al., 2013; Ramirez et al., 2013). Confidence in these study results is further limited by low quality and low level of evidence. To date, the published pediatric PAP adherence studies, whether descriptive, predictive, or interventional, have been nontheoretical. Intervention work has failed to identify a conceptual model or theoretical framework explicating the selection of predictors and consequent intervention(s). In the few instances when theory is mentioned, it occurs in a rather cursory manner (Lynch et al., 2017). Considering the state of the science, the discipline would be well served to move the science forward by systematically discerning what factors influence PAP adherence in children. Two approaches may be considered: (1) to use constructs from existing theories and systematically test as predictors and potential intervention targets for PAP adherence (deductive) or (2) build the science by developing population- and behavior-specific theory based on available evidence, or less rigorously, based on practice experience and intuition (inductive; Fig. 16.1). With a postmodernist lens, we advocate that the phenomenon of pediatric PAP adherence be inclusively examined as a health behavior that occurs and is measurable at the individual level (e.g., genetic, biological, sleep habits) but that is heavily laden with extraindividual influences, including persons/influences in the immediate environment (e.g., family, peers, sleeping environment, neighborhood) and also influences from the broader environment (e.g., social networks, health systems, payers, and policy). In the absence of any explicitly applied theoretical models to pediatric PAP adherence but with this lens of the phenomenon, a biosocioecological approach will help the field extrapolate predictors of adherence, beyond nonmodifiable characteristics, to define intervention targets. Given the presence of multifactorial influences, a modified

FIGURE 16.1 Two approaches for advancing the science on pediatric positive airway pressure adherence.

Positive airway pressure use

393

version of the Socio-ecological Model (Bronfenbrenner, 1977) or the Pediatric Self-management Model (Modi et al., 2012) may be a good fit for application to advance science in improving adherence to PAP therapy. Additionally, models commonly used in the adult PAP adherence literature such as the Health Belief Model (Rosenstock, 1974), Social Cognitive Theory (Bandura, 1977), or Transtheoretical Model (Prochaska & Velicer, 1997) may be advantageous to apply in pediatrics. To fully exploit this approach, a priori-defined theoretical premises must be explicit with each study; high quality studies, which are longitudinal, adequately powered, and replicated, will then provide robust evidence to identify potential intervention opportunities for improving pediatric PAP adherence. Contextual consideration, as is consistent with a biosocioecological premise, should be prioritized in studies so as to also support subsequent implementation and dissemination models for pediatric OSAS care.

Positive airway pressure use As previously described, PAP use is objectively measurable by the PAP device’s internal processor. Clinical evaluation of individuals with pediatric PAP will routinely include providerechild/adolescenteparent review of usage data, air leaks, patterns of use, and response to treatment. Currently, PAP adherence is categorized into three ways: 1. Payer strategy: use 4 hours per night on 70% of nights (adult criteria applied to pediatric population (Centers for Medicare & Medicaid Services, 2016). 2. Provider strategy: frequency of use and duration of use when used; defining patterns of use to identify consistent opportunities to intervene on nonuse periods; must also consider payer strategy for PAP payment. 3. Research strategy: investigator-defined adherent versus nonadherent use threshold (e.g., >1 hour of use, >6 hours of use); mean hours per night of use; mean hours per night of use at a defined frequency threshold (e.g., 50%e100% of nights). Published evidence addressing PAP use is predominately retrospective, observational, and limited by modest sample sizes (Table 16.3). The literature also includes samples with a wide range of ages, varying patient characteristics (e.g., developmental delays, OSAS severity, prior exposure to PAP vs. new PAP users), various measures of PAP use (e.g., chart notes, objective measure), and various PAP types (e.g., CPAP, BPAP, AutoPAP; Table 16.3). The literature also contains varying definitions of adherence (Table 16.4); the range of adherence across the included studies is

TABLE 16.3

Description of studies examining PAP use as a primary outcome. Age range years

Delay Y/N

AHI criteria events/h

PAP type

PAP naı¨ve Y/N

Metric

Author (year)

Study design

DiFeo et al. (2012)

PO

56

2e16

Y

NR

CB

Y

O

Harford et al. (2013)

RO

19

0e21

Y

NR

U

Y/N

O

Hawkins et al. (2016)

RO

140

NR

Y

1/h

CB

NR

O

Jambhekar et al. (2013)

RE

46

NR

Y

NR

U

N

O

Koontz et al. (2003)

RE

20

1e17

Y

NR

B

NR

O

Machaalani et al. (2016)

RO

99

0e18

Y

NR

CB

N

O

Marcus et al. (2006)

PE

29

2e16

N

5/h

CB

Y

O

Marcus, Beck, et al. (2012)

PE

56

2e16

Y

NR

CB

Y

O

Nathan et al. (2013)

RO

51

NR

Y

1/h

CB

N

CN

Nixon et al. (2011)

RO

32

NR

Y

NR

C

NR

O

O’Donnell et al. (2006)

RO

79

0e18

Y

>1/h

C

NR

O

Puri et al. (2016)

RO

56

<18

Y

1/h

U

Y

O

Ramirez et al. (2013)

RO

62

2e18

NR

NR

CB

NR

O

Simon et al. (2012)

PO

51

8e17

Y

NR

CBA

N

O

Xanthopoulos et al. (2017)

RO

161

10

Y

NR

C

NR

O

N

Notes. N ¼ total sample; Delay ¼ developmental delay included in sample. Autopositive airway pressure (A), Apnea hypopnea index (AHI), bilevel positive airway pressure (B), chart note (CN), continuous positive airway pressure (C), experimental (E), no (N), not reported (NR), observational (O), positive airway pressure (PAP), prospective (P), retrospective (R), unspecified (U), yes (Y).

TABLE 16.4

Pediatric PAP use and adherence.

Author (year)

Definition of adherence

Met adherence n (%)

Average time/night (h) at £1 mo M (SD) or median (IQR)

DiFeo et al. (2012)

NR

NR

3.0 (3.0) (1 mo)

2.8 (2.7) (3 mos)

Harford et al. (2013)

>4 h/n for >70%

8 (42%)

NR

NR

Hawkins et al. (2016)

4 h/n for >70%

69 (49%)

NR

NR

Jambhekar et al. (2013)

>4 h/n for >50%

22 (48%)

NR

NR

Koontz et al. (2003)

NR

NR

NR

5.9 (E1) (2 y) 8.5 (E2) (2 y) 0.7 (C) (2 y)

Machaalani et al. (2016)

4 h/n for 70%

4 wk: CPAP: 41 (75%) BPAP: 40 (91%) 1 y: CPAP: 13 (76%) BPAP: 15 (80%)

NR

6.8 (2.8) (CPAP) (4 wk) 9.3 (3.6) (BPAP) (4 wk) 8.0 (4.1) (CPAP) (1 y) 9.3 (3.8) (BPAP) (1 y)

Marcus et al. (2006)

Poor adherence <3 h/n

NR

NR

5.3 (2.5) (6 mos)

Marcus, Beck, et al. (2012)

NR

NR

3.4 (2.3) (CPAP) (1 mo) 3.1 (2.8) (BPAP) (1 mo)

2.1 (2.5) (CPAP) (3 mos) 3.1 (2.8) (BPAP) (3 mos)

Nathan et al. (2013)

4 d/wk

21 (41%)

NR

NR

Nixon et al. (2011)

>1 h/n >6 n/wk

10 (33%)

NR

4.7 (2.7) (3 mos)

Average time/night (h) at >1 mo M (SD) or median (IQR)

Continued

TABLE 16.4

Pediatric PAP use and adherence.dcont’d

Author (year)

Definition of adherence

Met adherence n (%)

Average time/night (h) at £1 mo M (SD) or median (IQR)

O’Donnell et al. (2006)

NR

NR

NR

4.7 (1.4, 7.0) (7 mos)

Puri et al. (2016)

NR

NR

3.5 (2.7) (1 wk) 2.9 (2.4) (1 mos)

2.8 (2.4) (3 mos)

Ramirez et al. (2013)

>8 h/n

45 (72%)

8.3 (2.5) (1 mos)

NR

Simon et al. (2012)

NR

NR

NR

3.4 (2.8) (3 mos)

Xanthopoulos et al. (2017)

NR

NR

2.9 (0.6, 5.8) (1 mos)

NR

Average time/night (h) at >1 mo M (SD) or median (IQR)

Notes. bilevel positive airway pressure (BPAP), continuous positive airway pressure (CPAP), exposure condition 1 (E1), exposure condition 2 (E2); control (C), hours (h), month (mos), night (n), not reported (NR), week (wk), year (y).

Positive airway pressure use

397

33%e91% based on definitions provided (Harford et al., 2013; Hawkins et al., 2016; Jambhekar et al., 2013; Nathan, Tang, Goh, Teoh, & Chay, 2013; Nixon, Mihai, Verginis, & Davey, 2011; Ramirez et al., 2013). Many studies are absent an adherence criterion (DiFeo et al., 2012; Koontz, Slifer, Cataldo, & Marcus, 2003; Marcus, Beck, et al., 2012; O’Donnell, Bjornson, Bohn, & Kirk, 2006; Puri et al., 2016; Simon, Duncan, Janicke, & Wagner, 2012; Xanthopoulos et al., 2017); in such cases, mean hours of use per night are commonly reported. Average short-term PAP use (one month) is 4.5 (SD, 3.5) hours per night, and longer-term PAP use (3mos) is 4.9 (SD, 3.8) hours/night (DiFeo et al., 2012; Machaalani, Evans, & Waters, 2016; Marcus, Beck, et al., 2012; Marcus et al., 2006; Nixon et al., 2011; Puri et al., 2016; Ramirez et al., 2013; Simon et al., 2012). A few studies included in Table 16.4 were not part of the above summary statistic. One reported two years of PAP data but could not be included in the summary statistic, as no standard deviations were provided (Koontz et al., 2003). Another study reported mean hours per night (adherent group: 7.4 hours; nonadherent group: 1.7 hours) but did not state the period of time for which adherence data were collected (Hawkins et al., 2016). Two studies reported average use with medians; 2.9 hours per night (IQR 0.6, 5.8) at one month (Xanthopoulos et al., 2017) and 4.7 hours per night (IQR 1.4, 7.0) at 7 months (O’Donnell et al., 2006). Whether hours of PAP use are reported and/or frequency of adherence (despite varying definitions of adherence), pediatric PAP use appears to be suboptimal. In addition to poor adherence rates, studies report a large initial refusal rate or high attrition due to PAP discontinuation (Machaalani et al., 2016; Marcus et al., 2006; Puri et al., 2016). This suggests that PAP may be undesirable or that PAP use is unachievable from the outset of treatment for a majority of children and adolescents; those that do attempt to use PAP, do so at levels that may not be therapeutic.

Key points Objective measures of PAP give providers, individuals, and caregivers an accurate depiction of treatment use. Although these measures are precise and provide valuable information, there is no universally accepted definition of PAP adherence for pediatrics, therefore leaving adherence to be variably defined based on payer, provider, or research strategy. Studies that assess PAP use are predominately retrospective and observational in design and are limited by modest sample sizes and have variable sample characteristics and inclusion criteria. Based on evidence to date, PAP use in children is suboptimal when compared with their recommended sleep duration.

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Factors influencing pressure airway pressure use and/or adherence Clinical observations of suboptimal PAP adherence have led researchers to explore barriers to adherence. Factors can be categorized by patient characteristics (e.g., age, race, BMI, comorbidities), technological device factors (e.g., machine, tubing, masks), disease factors (e.g., side effects of PAP or OSAS symptoms, OSAS severity), and family/socioeconomic status factors (e.g., parenting style, parental education). Studies exploring factors potentially influential on PAP adherence examine PAP use as either a continuous variable (i.e., hours of CPAP use/night) or as a categorical/binary variable (i.e., adherent vs. nonadherent). Age is a significant influential factor on PAP use, with older children using PAP for fewer hours per night than younger children (DiFeo et al., 2012; Lynch et al., 2017; O’Donnell et al., 2006; Puri et al., 2016). Other significant, although not consistently studied, factors of potential influence include sex, race, BMI, developmental delays, mask type, severity of OSAS, duration of OSAS diagnosis, having a parent on PAP, and maternal education. Females are more adherent than males (Hawkins et al., 2016; Nathan et al., 2013), African Americans are less adherent than Caucasians (DiFeo et al., 2012), children with a higher BMI are more likely to be nonadherent (Lynch et al., 2017), those with developmental disabilities are more likely to be adherent than typically developing peers (Hawkins et al., 2016; Kang et al., 2018), and higher maternal education is a significant predictor of number of nights used and hours per night used (DiFeo et al., 2012). In relation to OSAS- and PAP-specific factors, children with nasal masks are more likely to be adherent than those with full-face masks (O’Donnell et al., 2006), those with a higher baseline apnea hyponea index and greater improvements in apnea hypopnea index on PAP are more adherent than those with less severe OSAS, and those who experience less improvement in apnea hyponea index on PAP (Uong, Epperson, Bathon, & Jeffe, 2007), having a parent on PAP increases hours of use (Ramirez et al., 2013), and adherence increases with the length of time on PAP (Kang et al., 2018; Simon et al., 2012). Other potentially influential factors include caregiver self-efficacy (Xanthopoulos et al., 2017), caregiver concern (Lynch et al., 2017), child and parent-reported barriers to treatment such as not using PAP when away from home, not feeling well, or forgetting (Simon et al., 2012), and changes in PAP pressure (Nixon et al., 2011). Two qualitative studies have explored factors that influence adherence by interviewing adolescents using PAP and their caregivers. Using individual, open-ended, semistructured interviews, Prashad et al. (2013)

Factors influencing pressure airway pressure use and/or adherence

399

identified four overarching factors related to CPAP adherence from the perspective of both adolescents (12e18 years) and their caregivers: (1) structure in the home and in the family; (2) style of communication between adolescents and caregivers; (3) social reactions and attitudes; and (4) adolescent perception of CPAP benefits (Prashad et al., 2013). A recent study supported similar findings in adolescents (aged 11e17 years; Alebraheem et al., 2018). Using semistructured interviews, four themes emerged: (1) physical design of the machine (e.g., mask and tubing) is an impediment to use; (2) importance of an adjustment period to adapt and address issues unique to adolescents; (3) perceived barriers and challenges are more apparent than experiencing symptom relief; and (4) the amount of family support desired is individual and based on the unique needs of adolescents (Alebraheem et al., 2018). Given the nature of the evidence (i.e., quality, level and number of studies) addressing influential factors on PAP adherence in children and adolescents, key points are in the form of clinical and research recommendations.

Clinical recommendations Based on literature to date, providers should be aware of clinical populations that are at higher risk for nonadherence, such as older children/adolescents, males, those who are overweight or obese, and African Americans. However, many of these factors have not been supported by numerous studies so should be considered with great caution. Furthermore, providers should have an awareness of the importance of caregiver involvement to facilitate PAP use at home. Education regarding OSAS should also be a clinical priority, as with increased disease severity, treatment exposure may resolve bothersome symptoms, which positively influence PAP use (perceived treatment benefit); alternatively, knowledge of disease severity may convey heightened awareness for need of treatment and thereby positively influence PAP use (perceived treatment need).

Research recommendations Large, prospective, doseeresponse studies are needed to determine and establish the optimal thresholds of PAP adherence for pediatric populations. Additionally, large prospective observational studies are needed, with carefully selected measures relative to hypothesized factors of PAP adherence. Alternatively, use of preexisting large data sets would also be beneficial, including opportunities to use electronic health record data warehouses, to provide opportunities to more rigorously evaluate

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variables of influence on PAP adherence; this approach may be limited to characteristic variables based on available data, and age group strata should be a priori defined and employed in analyses.

Interventions to promote positive airway pressure adherence Seven published studies report PAP adherence interventions. The majority of this work has occurred within the past decade and is derived from clinical intuition and experience. It is notable that these interventions are reflective of the field’s limited knowledge about intervening on PAP adherence in pediatrics. Educational, behavioral, and/or supportive interventions have been programmatically evaluated, with few rigorous tests for efficacy. One randomized controlled trial has been conducted to date (Koontz et al., 2003), in which educational or educational plus behavioral sessions were offered to families, resulting in increased PAP use. Three studies are not generalizable as they are small case studies (Rains, 1995) with intensive inpatient intervention protocols (Harford et al., 2012; Slifer et al., 2007). Three studies are retrospective clinical program evaluations, in which “interventions” consist of increased number of follow-up appointments, additional staff, education, and/or behavioral training (Harford et al., 2013; Jambhekar et al., 2013; Riley et al., 2017). Interventions reported in the extant literature employ a combination of education, behavior modification, desensitization, and caregiver engagement. Despite varying sample sizes, study designs, and intervention types, all published studies are atheoretical and do not explicitly extend from evidence but rather clinical experience. Although all of these interventions reportedly had a positive effect on PAP adherence, there is an absence of comparators and rigorous scientific methods; therefore, caution must be exercised when evaluating interventions that report improvement of PAP adherence. In the absence of systematic evaluations of current practices and rigorous testing methods, the field remains relatively uninformed about evidence-based pediatric PAP adherence interventions.

Key points Interventions are mostly reported in the form of case studies, quality improvement, or clinical program evaluations, and caution must be exercised with reported successes of these interventions. Furthermore, the published studies and interventions are atheoretical, and future studies may benefit from interventions based on the adherence theories

Case study

401

previously mentioned. Many interventions appear to be geared toward caregivers, leaving children and adolescents as underused agents of change. Because one of the most commonly identified influential factors on PAP use is age, interventions aimed at adolescent PAP users may be prioritized and highly impactful, as increasing age appears to be consistently associated with decreased PAP use. Future interventions should take family-based approaches, encouraging both children and caregivers to become actively involved in the effort to acclimate to and use PAP. Furthermore, as PAP use can be measured objectively, this provides a unique opportunity to monitor adherence and develop interventions designed to increase use while integrating technology as an intervention platform.

Case study History: “Alex,” a 14-year-old female with Trisomy 21 presented to an interdisciplinary Sleep Clinic. Alex’s mother reported a 6-month history of excessive daytime sleepiness, both at home and at school. Alex had a relevant medical history of cardiac concerns, with complete atrioventricular canal defect (surgically repaired at ages 3 and 11 years), as well as obesity, and recurrent pneumonias. Alex’s mother reported that she had a polysomnogram at another institution as a young child (4e5 years old), which showed OSAS (severity unknown) and underwent an adenotonsillectomy. A repeat polysomnogram status postadenotonsillectomy reportedly showed abnormal CO2 but no further apnea. Her mother reported that she more recently observed apnea when Alex is asleep in the car but has not noted any snoring/apnea at night. At the time of her initial consultation, Alex slept in her own bed in her own room, with a bedtime of 8:30 p.m. and wake time of 6:30 a.m. on school nights and 9 p.m. and 9 a.m. on weekends. Alex was described as difficult to wake and excessively sleepy during the day. She also fell asleep at school several times per day, throughout most of her classes, for about 50 minutes each time, and napped on the weekends. She complained of headache and neck pain upon waking. There was no reported enuresis (e.g., bedwetting), diaphoresis (e.g., excessive sweating), history of parasomnia (e.g., sleep terrors, sleep walking), history consistent with restless legs syndrome and no history that could be elicited regarding cataplexy (e.g., temporary muscle weakness or paralysis), hypnagogic hallucinations (e.g., hallucinations near sleep onset), or sleep paralysis due to developmental delays.

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Alex weighed 70.8 kg (93rd percentile based on CDC 2e20 years weight-for-age) was 141 cm (<1 percentile based on CDC 2e20 years stature-for-age), and had a body mass index of 35.61 kg/m2 (99 percentile based on CDC 2e20 years BMI-for-age). Physical exam revealed a wellappearing child, cooperative and in no distress. There was no adenoidal facies. Vitals were within normal range. Tonsils were surgically absent. Cardiovascular and neurologic exam was normal. Differential diagnosis: The differential diagnosis of excessive daytime sleepiness included OSAS, narcolepsy, hypercarbia, hypothyroidism, and idiopathic hypersomnia. Actigraphy: Actigraphy was ordered to monitor sleep objectively; however, it was not approved by insurance. Therefore, Alex wore a Fitbit and caregivers completed a sleep diary for 9 days. Fitbit and diary information indicated that Alex was obtaining the recommended amount of sleep for her age on most nights (10e12 hours). PSG test and multiple sleep latency test: Alex underwent an overnight polysomnography, which showed an apnea hyponea index of 15.8 events/hour with oxygen desaturation nadir of 88% and several events of hypercapnia (maximum was 57 Torr). The multiple sleep latency test objectively confirmed sleepiness with a mean sleep latency of 7.8 minutes and no sleep-onset REM periods. Additional tests: Alex was evaluated by an ear, nose, and throat (ENT) physician and had no adenotonsillar regrowth based on endoscopy. Her thyroid was functioning normally. MRI was performed due to concern for Chiari malformation following results of polysomnography, and results were negative. Treatment plan and follow-up: Alex and her mother returned to clinic to discuss initiation of CPAP therapy with the physician. The family then met with the respiratory therapist and clinical psychologist. The respiratory therapist fit her for a nasal interface, and Alex was able to tolerate the mask, headgear, and CPAP pressure while in the clinic. Alex was placed on a CPAP pressure of 4 cm H2O to habituate to the CPAP prior to a titration PSG. Following is the desensitization CPAP implementation plan that was discussed with the family: • Attach both sides of the mask to the headgear. Have Alex wear the mask with the air turned on for as long as possible up to 30 minutes while engaged in an enjoyable and distracting activity such as playing on her iPad or watching her favorite television show. • Have Alex lie down on the bed or couch. Repeat above steps. • In addition to previous steps, have Alex attempt to fall asleep with the PAP (air on). When she is regularly able to fall asleep with the PAP and leave it on all night, every night, discontinue the daytime practice.

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The standard of care in the CPAP adherence program where Alex was seen includes a number of follow-up calls to check in on initiation and adherence (King et al., 2014). During initial follow-up calls, Alex’s mother identified a number of barriers to successful initiation, including being overwhelmed with other medical appointments, and concern about Alex’s fear response to the CPAP mask. Alex’s mother was concerned that the CPAP mask triggered a posttraumatic stress reaction due to her history of surgical procedures and undergoing anesthesia. The concerns and barriers were addressed by providing empathy and support, psychoeducation about common responses to stress and medical procedures and evidence-based treatment of trauma responses (i.e., gradual desensitization), and the need for managing Alex’s OSAS given her significant daytime sleepiness and the family’s concern about this symptom. Alex’s mother was instructed to repeat desensitization plan but have Alex hold the mask/place it on her face only if comfortable while engaged in distracting activities. Alex’s mother’s concerns on the next call included (1) Alex disliked the nasal mask and is interested in a full-face mask and (2) Alex was afraid of having the machine on with water in the humidity chamber due to the noise it made. We encouraged the mother to institute a behavioral reinforcement plan with incentives for having Alex sit next to the machine while it is on to help expose her and sensitize her to the noise. The mother would institute a chart or jar to provide Alex with a sticker or token for each day that she was able to sit next to the machine, with the number of minutes she was expected to sit next to the machine gradually increasing each day. Her mother then provided a reward to Alex after three days of sitting next to the machine consistently, with gradual increases in the number of days in a row that Alex was required to sit next to the machine before earning a larger reward. At Alex’s one-month follow-up, her adherence was 27% of days turning on the machine for an average of 16 minutes on days used. Primary concerns and barriers reported were mask and machine discomfort and continued fear response to medical interventions. Excessive daytime sleepiness was still present. The following plan was developed with the family: (1) Alex did not have to use the water (humidifier) for her CPAP as it is for comfort; (2) if she wanted to earn iPad time at bedtime, she needed to wear her CPAP at bedtime (longer she wore CPAP, the longer time she could have on iPad); (3) rewards closer in time (the idea of the CPAP Fairy was introduced to Alex, wherein the CPAP “Fairy” would leave surprises; and underneath her pillow while she was sleeping if she did each CPAP task); (4) encouraged Alex’s mother to stay with her at the beginning of the night so Alex did not try to take the CPAP off prior to her initially falling asleep. Alex responded positively to the CPAP Fairy and change in

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her mask and was able to fall asleep with the CPAP on by the time of her titration polysomnogram. Alex had a titration PSG 9.5 weeks following the initiation of CPAP. The CPAP modem was downloaded at the sleep lab at Alex’s titration PSG, revealing use of equipment 57.9% for an average of 2 hours, 39 minutes on nights used. At Alex’s subsequent clinic visits at 3, 6, and 9 months following the start of PAP, Alex’s mother reported improved energy, improved attention, and improved behavior for Alex during the day. According to the modem downloads, Alex’s adherence substantially improved across time both in percentage of nights used and in hours of usage (Figs. 16.2 and 16.3). Alex’s number of naps and duration of naps progressively improved until she stopped napping completely by 9 months after starting PAP. Alex was seen in clinic 5 months later. According to the modem download, Alex had excellent adherence as the download revealed 97% for an average of 8 hours and 22 minutes. Her mother reported that Alex continued to be rewarded with iPad time and noted improvements in energy, daytime sleepiness, and less irritability during the day, as well as no snoring and improved restless sleep at night. They were encouraged to follow up in clinic in 6 months.

Emerging areas and conclusions Although PAP has been used to treat pediatric OSAS for a few decades, rates of use/adherence are not well understood, and barriers and facilitators are still largely unknown. Through this comprehensive review of 100 90

Percentage

80 70 60 50 40 30 20 10 0 Months 0-3

Months 3-6

FIGURE 16.2

Months 6-9

Months 9-12 Months 12-18

Percentage of days used.

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Average Minutes of Use

600 8 hours, 32 minutes

500 400 5 hours, 51 minutes

6 hours, 19 minutes

300 200 3 hours, 10 minutes 100 0 Months 0-3

Months 3-6

FIGURE 16.3

Months 6-9

Months 9-12

Months 12-18

Minutes of use on nights used.

literature related to PAP use and adherence, factors influencing PAP use, and interventions to improve PAP adherence, it can be concluded that (1) the optimal duration of nightly PAP use in children needs to be established to inform appropriate adherence parameters; (2) large data sets/ multisite studies are needed to gain a comprehensive understanding of rates of PAP use and adherence in pediatric populations; (3) factors influencing PAP use need to be examined in larger studies to reveal robust relationships; and (4) interventions to improve adherence are (i) lacking theoretical rationale, (ii) do not appear to be designed based on findings of prior studies but rather are designed based on current clinical judgment and psychological principles, and (iii) have not been systematically evaluated.

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