Positive Airway Pressure for the Treatment of the Childhood Obstructive Sleep Apnea Syndrome

Abstract

In this review, we have summarized the benefits of treatment with positive airway pressure for the childhood obstructive sleep apnea syndrome and presented a socio-ecological framework to enhance our understanding of positive airway pressure adherence predictors and important targets of comprehensive positive airway pressure treatment models across different pediatric populations. Although positive airway pressure is clearly a beneficial treatment for pediatric obstructive sleep apnea syndrome, additional research is needed to evaluate how socio-ecological factors may interact to predict positive airway pressure adherence, with more attention to the impact of the broader healthcare setting and on treatment approaches and outcomes in special pediatric populations.

The obstructive sleep apnea syndrome (OSAS) in the general pediatric population is common, occurring in 1-4% of individuals across the pediatric age range ¹. However, its prevalence is higher in special populations. For example, the prevalence of OSAS has been estimated at 2% in a primarily non-obese adolescent population², but as high as 13% in obese adolescents ³; and between 45 and 55% in children with Down syndrome (DS) ^4-6. Untreated OSAS is associated with significant complications, such as behavioral and learning difficulties, systemic hypertension, pulmonary hypertension and metabolic derangements⁷. In children, the primary treatment is adenotonsillectomy (AT)⁸. However, some children require positive airway pressure (PAP) therapy, the second-line treatment, if they are not surgical candidates or if OSAS persists after AT. In fact, even children with low risk factors have residual OSAS rates up to 29%. Those with high risk factors, such as obesity and DS, have an estimated residual rate greater than 70% ^7,9. This review will summarize the benefits of PAP treatment for pediatric OSAS and the use of a socio-ecological framework to understand factors linked to PAP adherence¹⁰. We will also present a comprehensive model of PAP treatment to promote adherence and discuss the use of PAP in special populations.

Benefits of Positive Airway Pressure Treatment for OSAS

Continuous (CPAP), Bi-level (BPAP), or Auto-titrating (AutoPAP) positive airway pressure therapy involves wearing a snug-fitting mask every night that is attached a machine via tubing that delivers pressurized air in order to maintain upper airway patency. When used consistently, PAP has been shown to be highly effective at resolving polysomnographic manifestations of OSAS in children and adolescents ^11,12. Studies indicate that PAP also reduces symptoms of OSAS and improves child school performance, behavior, daytime sleepiness and quality of life ^11,13,14. For example, Beebe and Byers evaluated self-reported academic grades, self- and caregiver-reported academic quality of life, and objectively-measured attention before and 4 months after initiation of PAP in a sample of 13 obese adolescents with OSA ¹⁵. Based on adherence data, the treated group was divided into PAP users (n = 6) and non-adherent participants (n = 7). Non-adherent participants showed worse functioning over time, while PAP users showed stable or improved functioning. Additionally, Marcus et al. evaluated neurobehavioral functioning before and after 3 months of PAP therapy in a heterogeneous group of 52 children and adolescents. Attention deficits, sleepiness, behavior, and caregiver- and child- reported quality of life improved. There was also a significant correlation between decreased sleepiness and increased adherence, although no associations emerged between other behavioral outcomes and adherence. While there was suboptimal adherence use overall (mean use, 170 ± 145 [SD] minutes per night), there was significant improvement in neurobehavioral function in children after 3 months of PAP, including in those with neurodevelopmental delays.

A growing body of literature suggests improvements in cardiometabolic risk following PAP treatment in obese youth, including improved heart rate, insulin resistance, blood pressure lipids, and increase in arousal-related sympathetic-parasympathetic balance ^16-18. In a study that assessed cardiometabolic functioning before and 1 year after PAP initiation, clinically relevant improvements were seen in insulin resistance and systolic blood pressure load ¹⁶. Additionally, total cholesterol and low-density lipoproteins improved in a study of 18 obese adolescents treated with PAP in the absence of a change in BMI ¹⁷. Early use of PAP in extremely low birth weight infants has also demonstrated an improvement in growth and weight ¹⁹. Thus, PAP is effective in treating pediatric OSAS as well as its neurobehavioral and physiological consequences. Although more research is needed on the longitudinal impacts of pediatric PAP use, PAP use in adults ameliorates OSAS-related and general mortality ^20,21, suggesting that promoting PAP adherence in childhood could benefit downstream neurobehavioral and physical health outcomes in adulthood.

Prescribing and Titrating Positive Airway Pressure

CPAP is the most frequently prescribed mode of PAP therapy for the treatment of pediatric OSAS and is effective in most cases. Often, pediatric patients are empirically prescribed a low pressure, such as 4-5 cm H₂O in order to acclimate them to CPAP until a titration PSG can be performed; and optimal pressure identified and then prescribed. For patients over the age of 6 years and especially for adolescents, AutoPAP is becoming a more commonly prescribed mode.²² For some patients, however, bi-level PAP (BLPAP) is initially prescribed. Frequent Indications for the prescription of BLPAP, include sleep-related hypercapnia or hypoventilation diagnosed on the baseline PSG (for example associated with neuromuscular disorders), and presence of a hypoventilation syndrome [e.g, congenital central hypoventilation syndrome (CCHS) or rapid-onset obesity with hypothalamic dysregulation, hypoventilation, and autonomic dysregulation (ROHHAD)].

In order to identify adequate treatment pressures, a titration polysomnogram (PSG) is performed. Pressure is titrated according to standardized protocol during an overnight PSG. PAP is typically started at home settings and then titrated by increasing pressure to correct for obstructive apneas, obstructive hypopneas, snoring, stridor, and paradoxical breathing. Pressure may also be weaned if no events are observed on prescribed pressure. The goals for PAP titration are: 1) fewer than two obstructive events per hour, 2) SpO₂ ≥ 94%, 3) minimal paradoxical breathing and flow limitation, 4) ETCO₂ <55 mm Hg and not greater than 50 mm Hg for longer than 10 minutes unless otherwise ordered, and 5) adequate waveforms on ETCO₂ and corresponding TcCO₂ values, excluding transient elevations related to movement, arousal, sighs, etc.

If OSA persists beyond CPAP of 16 cm H₂O or other ordered parameter, then the mode is switched to BLPAP prior conversation with the sleep physician on-call. Most children with OSA are titrated according to the same protocols. It is our practice to repeat titrations every 12-18 months approximately in preschool, school-aged and adolescent children to reassess pressures or within a shorter time-frame if another intervention is performed that would influence the status of OSAS (e.g., surgery, oral procdure/device, weight loss, etc). In infants, repeat titrations are often performed every 3-6 months given rapid changes in growth and development.

Applying a Socio-Ecological Framework to Understand Adherence to Positive Airway Pressure

Unsurprisingly, adherence to this relatively uncomfortable treatment is a major obstacle to care, thereby impacting potential short- and long-term benefits. Effectiveness of PAP is limited by poor adherence across the age spectrum. Studies have shown that as many as one third of pediatric patients drop out of PAP treatment ¹¹ and in those who continue to use PAP, nightly, use is suboptimal ^11,12. Definitions of adherence vary across studies with reported rates of adherence to PAP of 24 to 87% and average wear of 4.0-5.3 hours/night ^23,24.

Although numerous studies have been conducted to evaluate and improve PAP adherence in adults ^25-33, there have been very few studies in the pediatric age group, especially in adolescents and those with special considerations, including infants and those with genetic or neurodevelopmental conditions, and/or craniofacial anomalies ^24,34,35. However, available research examining predictors of pediatric PAP has identified several salient child and family factors,^24,36-40 which can be understood through the application of a socio-ecological framework.¹⁰ A socio-ecological framework, which has been applied to understand predictors of medical and behavioral sleep outcomes across the lifespan^41-44, posits that there are multiple, dynamic and interacting factors and processes at the individual child (microsystem), family and school/work (mesosystem), and broader community and socio-cultural levels (macrosystem) that influence outcomes. PAP adherence is a complex behavior, particularly in childhood when caregivers or other family members may be more involved in medical care and health behaviors relative to later in adulthood. Thus, a socio-ecological framework (Figure 1) is especially useful in understanding and identifying predictors of pediatric PAP adherence as well as targeted treatment approaches.

Figure 1.

Socio ecological Framework for PAP Adherence

At the individual child level, recent meta-analytic research and reviews have identified female sex, non-Latinx White racial/ethnic background, younger age, the presence of neurodevelopmental delays, and greater baseline AHI as factors more consistently associated with higher adherence ^24,36. Of note, as race is a socio-political construct,⁴⁵ racial/ethnic background at the individual level is conceptualized here as a proxy for experiences of systemic racism in the across socio-ecological levels, such as in the healthcare setting; rather than as a biological factor influencing adherence. With regard to family factors, higher maternal education has also been linked to better adherence³⁶, with additional evidence suggesting that caregiver health beliefs, including the perceived benefits of PAP and self-efficacy, can influence adherence^24,36,38-40. In addition, qualitative research conducted with adolescents on PAP and their caregivers has highlighted the importance of family-level organizational and interpersonal processes, including structured bedtime routines and adaptive caregiver-child communication and problem-solving, as being facilitators of optimal adherence^38,39. Research on other socio-ecological factors beyond the immediate child and family levels is incredibly limited and is an important direction for future research. For example, given that PAP is a prescribed medical treatment, there are likely healthcare setting and broader socio-cultural factors that interact with child/family socio-demographic characteristics to influence PAP initiation and adherence. Differential access to medical and specialty care for families of lower-socioeconomic status or of racial/ethnic minority backgrounds could impact consistent access to and engagement in PAP follow-up care⁴⁶. In addition, experiences of healthcare bias and discrimination among families of racial/ethnic minority backgrounds have been linked to poorer patient-provider communication and less collaborative decision-making, and should be explored in relation to PAP adherence^47,48. Overall, more research assessing the interaction of socio-ecological factors in predicting pediatric PAP adherence is needed.

A Comprehensive Treatment Model to Support PAP Initiation and Adherence

Given the complex interplay between medical, behavioral, and technical aspects of OSAS and treatment with PAP, an interdisciplinary approach that considers multiple socio-ecological factors related to PAP implementation and maintenance is recommended. A team of medical (e.g., physicians, nurses, and nurse practitioners), behavioral health providers (e.g., psychologist, social worker), and respiratory therapists (RT) specializing in sleep disorders and PAP may best support children and families in successful implementation and maintenance PAP for the treatment of OSAS ^23,49-51. These team members can address different aspects of the individual child (e.g., medical and neurodevelopmental comorbidities; physiological and behavioral consequences of untreated OSAS), the child’s care (e.g., access to and engagement in medical specialty services), and the child’s broader family context (e.g., family health beliefs and behaviors). Evidence also suggests that adherence improves over time, which may be due to children and families needing time to acclimate to PAP usage, integrate PAP into routines, and engage in PAP adherence-related problem-solving ^23,34,37,51; therefore, frequent follow up is recommended, particularly in the months after PAP initiation.

The interdisciplinary team provides education about what OSAS is and the potential risks of not treating it, as well as what PAP is and potential benefits and challenges. The medical team and RT then evaluate craniofacial characteristics and offers options for masks and headgear and as fit the child with the interface. A behavioral health assessment of family sleep routines, structure, and PAP-related health beliefs (e.g., benefits of OSAS treatment; self-efficacy around PAP usage), behavioral health concerns, and child characteristics such as sensory sensitivities can be assessed to address psychosocial barriers and identify child and family factors that could impact treatment. In addition, this assessment includes the identification of stressors that the family may face that could be directly related to PAP initiation and adherence, as well as those that could impact engagement in medical care (e.g., financial insecurity; transportation needs to attend follow-up visits). The team can also demonstrate to the family how to put on and use the equipment, as well as model approaches to reduce stress and resistance of the child by assisting the family in placing the PAP mask on the child during the visit.

Based on the behavioral health assessment and observations of the child and family during the clinic demonstration, an individualized family-centered implementation plan is developed. Often the plan includes a period of daytime practice and desensitization while having the child or teen engaged in an enjoyable and distracting activity, such as watching a show, playing video games or using a tablet. PAP is then incorporated into the bedtime routine with the initial goal of the child falling asleep with it on and then the next goal of the child sleeping all night with it. Positive parenting and reinforcement, as well as potential reward systems (e.g., token economy, PAP Fairy visiting and leaving a prize under the pillow if the child is wearing it) may also be discussed, and young children and those with developmental differences may also benefit from a PAP social story. Close follow up by members of the team via phone or visits is essential in supporting known PAP adherence facilitators^38,39, including family engagement and collaborative problem-solving to address barriers and modify the PAP treatment plan over time. These follow-up interactions also help to monitor side effects, such as skin breakdown, nasal or eye irritation, midface growth, and mask fit given children are growing. A titration PSG is usually required to determine adequate therapeutic pressures, but may not be in those who are treated with AutoPAP ²².

Treatment with positive airway pressure in special pediatric populations with OSAS

Children with developmental disabilities (DD) have increased prevalence of OSAS and many of them require PAP after AT to effectively treat OSAS. However, there are very few studies reporting improvement associated with PAP and/or PAP adherence in this population. In general, research has shown that children with DD have similar or better PAP adherence compared to typically developing children (TD). A study of 56 children of whom 23% had DD, reported that the adherence of the group was not influenced by DD⁵². Hawkins et al showed that children with DD were very likely to be adherent to CPAP with an odds ratio of 2.55 (p=0.007)⁵³. Authors attributed these results to increased dependence on caregiver support, increased parental perception of PAP necessity, and decreased ability of the patient to remove the interface. Kang et al conducted a retrospective study of 103 children with DD and 137 TD to compare differences in adherence patterns expressed as percentage of nights used and hours of usage on nights used at 3 and 6 months³⁴. The study showed that children with DD, with adequate support, have better PAP adherence than typically developing children. In this study, the percentage of nights used was significantly higher in children with DD at 3 (DD, Median [IQR] =86.7 [33.9-97.9], TD=62.9 [30.8-87.8] p=0.01) and 6 months (DD=90.0 [53.3-100], TD=70.7[29.2-90.8], p=0.003). Hours of usage on nights used at 3 and 6 months were similar between groups (DD=5.0 [1.4-7.9], TD=4.6 [1.9-7.2], p=0.715; DD=6.4 [1.8-8.3], TD=5.7 [2.5-7.3], p=0.345, respectively). Importantly, the latter adherence measure improved over time in both groups (DD, p=0.007; TD, p=0.005). However, adherence over the 6-month observation period increased linearly in the DD group only. The TD group’s adherence decreased from month 1 to month 3 and then increased from month 3 to month 6. At 6 months, non-Latinx White racial/ethnic background and higher PAP pressure were significantly predictive for percentage of nights used; higher PAP pressure was significantly predictive for hours of usage in both groups.

Children with DS

PAP research in this population has mostly been examined under the umbrella of children with DD. Trucco et al conducted one of the few studies in this population⁵⁴. They reported 27 children with DS and OSAS diagnosed via cardiorespiratory polygraphy, 25 of whom received PAP. At almost 2-year follow up, 46% were using PAP for 8 hours per night. Another retrospective study of 26 children with DS-OSAS followed for 2 years showed that at 18-24 months after initiation, the group’s percentage of nights used was 66[29.0-97.4]% and minutes of usage on nights used was 219.0[62.0-474.5]mins⁵⁵. This highlights the fact that many children with DS can successfully be treated with PAP. Future prospective studies including functional outcomes, such as the ability to participate in activities of daily living (e.g., school), as well as family-centered outcomes, which are changes or benefits that are meaningful to the specific child and/or his/her family, are needed.

Infants

OSAS is frequently treated with PAP in infants with craniofacial and airway anomalies, and neuromuscular diseases ^56,57. Several case series have reported successful PAP use in infants with OSAS ^58,59 but overall, there is a paucity of data on this topic. Cielo et al recently published a retrospective study comparing 41 infants < 6 months to 109 children aged 5-10 years³⁵. Both groups were treated with PAP for OSAS. Median age at PAP initiation was 2 months for infants and 7.6 years for the school-age group. Both age groups were approximately two-thirds male. Twenty infants (48.8%) had an underlying craniofacial abnormality compared to 10 (9.2%) school-age children (p<0.0001). PAP was used in infants on 94.7% of nights, which was greater than 83% in school-age children (p=0.003). Reported barriers to PAP use were similar between the infant and school-age groups. Child behavior, including the child crying or refusing to wear PAP was the most common barrier in both age groups. There was no difference in reported problems related to mask fit, which was a barrier in 5 (12.2%) infants and 11 (10.1%) of school-age children. There were no differences in barriers related to skin or nasal irritation with both being uncommon in both age groups. This study showed that PAP is highly effective in treating OSAS and is well-tolerated in infants, suggesting that PAP should be routinely considered as an OSAS treatment in early development.

High-flow nasal cannula as an alternative to PAP

High-flow nasal cannula (HFNC) is typically used in the inpatient setting as respiratory support in many respiratory acute diseases. Since the use of HFNC results in positive airway pressure, its use has been expanded to the ambulatory setting mostly in patients with OSAS who have had trouble tolerating PAP. A detailed review of the mechanisms by which HFNC can improve obstructive breathing and ventilation are beyond the scope of this article. A handful of studies have shown that HFNC reduces respiratory events, improves oxygenation, reduces heart rate, and does not disturb sleep quality in pediatric patients with moderate to severe OSA.^60,61 Studies with relatively small sample sizes have shown adequate tolerance of HFNC in children with OSAS and developmental disabilities, such as DS, who have been intolerant to PAP;⁶² and recently a retrospective study focusing in infants and young children showed that HFNC can effectively alleviate obstructive events and gas exchange abnormalities in this population.⁶³ However, further prospective research is needed to establish the role of HFNC compared to PAP in the treatment of OSAS.

Summary

In this review, we have summarized the benefits of PAP treatment for pediatric OSAS and presented a socio-ecological framework to enhance our understanding of PAP adherence predictors and important targets of comprehensive PAP treatment models across different pediatric populations. Although PAP is clearly a beneficial treatment for pediatric OSAS, additional research is needed to evaluate how socio-ecological factors may interact to predict PAP adherence, with more attention to the impact of the broader healthcare setting and on treatment approaches and outcomes in special pediatric populations.

Data Availability Statement:

Data sharing is not applicable to this article as no new data were created or analyzed in this study.