Continuous positive airway pressure care for pediatric obstructive sleep apnea: A long-term quality improvement initiative.

Successful treatment of pediatric obstructive sleep apnea syndrome (OSAS) with continuous positive airway pressure (CPAP) is challenging due to behavioral, technical, medical, and systems factors. We undertook a quality improvement (QI) initiative involving physicians, nurses, psychologists, and respiratory therapists to improve CPAP outpatient care and processes. We aimed to: (1) increase the proportion of patients with a follow-up visit within 4 months of initiation of CPAP, (2) reduce the median time to first follow-up visit to under 4 months, and (3) increase the proportion of patients obtaining a post-initiation polysomnogram within 1 year to >50%. We also explored healthcare utilization (HCU) in a subsample of patients. Interventions focused on developing a tracking system and standardizing interdisciplinary clinical care. The proportion of patients returning to clinic within 4 months improved from 38.2% to 65.5% and median time to first follow-up visit improved from 133 to 56 days. The percentage of patients who returned for a post-initiation polysomnogram within 1 year was 71.1%. Subsample analyses showed significant reductions in the length of stay for emergency department visits from pre-CPAP initiation (Mdn = 3.00 h; interquartile range [IQR] = 7.00) to post-initiation (Mdn = 2.00 h, IQR = 5.00). The length of hospitalizations was also significantly shorter from pre (Mdn = 48.00 h, IQR = 243.00) to post-CPAP initiation (Mdn = 0.00 h, IQR = 73.00). A standardized, tracked approach to interdisciplinary outpatient CPAP care can improve follow-up care and potentially HCU.

Clinical utility of repeated positive airway pressure titrations in children with obstructive sleep apnea syndrome.

STUDY OBJECTIVES: Positive airway pressure (PAP) is the second line of treatment for obstructive sleep apnea syndrome in children. It is common practice following initiation of PAP to perform repeat titration polysomnography to re-evaluate the patient's therapeutic pressure; however, data supporting this practice are lacking. We hypothesized that repeat PAP titration would result in significant setting changes in children with obstructive sleep apnea syndrome. METHODS: We retrospectively analyzed demographic, polysomnographic, and PAP data of children with obstructive sleep apnea syndrome aged 0-18 years who were initiated on PAP and underwent 2 titration studies over a 2-year period. PAP mode and recommended pressure differences between the 2 titrations were compared. RESULTS: 64 children met inclusion criteria. The median (interquartile range) baseline obstructive apnea-hypopnea index and SpO2 nadir were 14.8 (8.7-32.7) events/h and 88.5% (85-92%), respectively. The mean differences in obstructive apnea-hypopnea index, SpO2 nadir, and % total sleep time with SpO2 < 90% between both titrations were negligible, including children with obesity, adenotonsillar hypertrophy, and trisomy 21. Additionally, there was no significant difference in mean PAP pressure between 2 separate titration studies for those on continuous PAP or bilevel PAP. CONCLUSIONS: Overall, repeat PAP titration in children with obstructive sleep apnea syndrome within the timeframe here described did not result in significant changes in PAP mode, continuous PAP pressure, or obstructive apnea-hypopnea index. Based on these data, repeat PAP titration within 2 years of an initial titration does not appear to be necessary. CITATION: Yendur O, Feld L, Miranda-Schaeubinger M, et al. Clinical utility of repeated positive airway pressure titrations in children with obstructive sleep apnea syndrome. J Clin Sleep Med. 2022;18(4):1021-1026.

Positive Airway Pressure for the Treatment of OSA in Infants.

BACKGROUND: Positive airway pressure (PAP) is a standard therapy for the treatment of OSA in children, but objective data on the effectiveness of PAP in infants are sparse. The aim of this study was to compare the effectiveness of PAP in infants younger than 6 months of age with that in school-aged children. RESEARCH QUESTION: Compared with PAP in school-aged children, can PAP be titrated as successfully in infants, and is adherence to PAP similar in both age groups? STUDY DESIGN AND METHODS: Single-center retrospective study. For consecutive infants younger than 6 months of age and school-aged children 5 to 10 years of age with OSA treated with PAP, baseline and titration polysomnography data, PAP adherence data, and parent-reported barriers to adherence were compared between groups. RESULTS: Forty-one infants and 109 school-aged children were included. Median obstructive apnea hypopnea index (OAHI) in infants was 25.7/h (interquartile range [IQR], 17.8-35.9/h) and was greater than that in school-aged children (12.1/hr; IQR, 7.6-21.5/h; P < .0001). After PAP titration, OAHI was reduced by a median of 92.1% in infants, similar to the median 93.4% reduction in school-aged children (P = .67). PAP was used in infants on 94.7% of nights, which was more than the 83% in school-aged children (P = .003). No differences were found in barriers to adherence between infants and school-aged children, with behavioral barriers being most common in both groups. INTERPRETATION: Objective data demonstrate that PAP is both highly effective at treating OSA and well-tolerated in infants. Like older patients, PAP should be considered along with other therapies for the treatment of OSA in even the youngest children.

Difficult Intubation due to Penetrating Trauma from a Crossbow Bolt.

We present the case of a patient with penetrating neck and craniofacial trauma from a self-inflicted crossbow bolt injury. This case highlights the challenges involved in prehospital airway management related to an in situ foreign object penetrating the oral cavity. We review the complications associated with such injuries and considerations for effective prehospital airway management.

Auto-titrating CPAP for the treatment of obstructive sleep apnea in children.

STUDY OBJECTIVES: In-laboratory titration polysomnography (PSG) is standard to determine optimal therapeutic continuous positive airway pressure (CPAP) in children with obstructive sleep apnea (OSA). The use of auto-titrating CPAP devices (autoCPAP) as an alternative to CPAP titration has not been well studied in children. We hypothesized that autoCPAP-derived pressures (PMEAN, PPEAKMEAN, P90) would be similar to titration PSG pressure (PPSG). METHODS: This is a retrospective study of children with OSAS initiated on autoCPAP between 2007 and 2017, who used autoCPAP for at least 2 h/night and who had adequate titration PSG were included in the analysis. AutoCPAP-derived pressures were obtained from use downloads and compared with PPSG. PPSG predictive factors were analyzed by median regression. Nonparametric methods were used for analysis. RESULTS: Of 110 children initiated on autoCPAP, 44 satisfied the inclusion criteria. Age (median (interquartile range)) was 13.01 (9.98-16.72) years, and 63.6% were obese. PPSG median (interquartile range) was 8 (7-11) cmH2O, mean autoCPAP-derived pressure (PMEAN) was 6.2 (5.6-7.6) cmH2O, peak mean pressure (PPEAKMEAN) was 9.4 (7.7-11.1) cmH2O, and average device pressure ≤ 90% of the time (P90) was 8.1 (7.2-9.7) cmH2O. AutoCPAP-derived pressures correlated with PPSG (P < .05). PMEAN was lower than the other 3 pressures (P < .0002). Median regression analysis demonstrated that after adjusting for patient characteristics such as age, sex, and obesity status, autoCPAP-derived pressures remained significant predictors of PPSG (P < .05). There were no significant interactions between these patient characteristics and autoCPAP-derived pressures. CONCLUSIONS: This study demonstrates that autoCPAP-derived pressures correlate with the titration PSG-derived pressures. These results indicate that autoCPAP can be used in the pediatric population and can determine pressures that are close to the titration pressures.

The role of polysomnography in tracheostomy decannulation of children with bronchopulmonary dysplasia.

BACKGROUND: Patients with bronchopulmonary dysplasia (BPD) may require tracheostomy for long-term mechanical ventilation. Polysomnography (PSG) may predict successful decannulation in children, however it is unclear how this success compares with children without a PSG. To better evaluate this role, we compared decannulation outcomes between tracheostomy-dependent children with BPD who underwent PSG before decannulation to those who did not. METHODS: This is a retrospective cohort study between 1 January 2007 and 1 June 2017 of tracheostomy-dependent children with BPD who were clinically considered for decannulation. Patient demographics, PSG results, and medical comorbidities were abstracted from medical records and compared between groups. Decannulation outcomes were compared between children with BPD who underwent PSG before decannulation and those who did not. RESULTS: One hundred twenty-five patients with BPD were considered for tracheostomy decannulation. Forty-six (37%) had a pre-decannulation PSG while 79 (63%) did not. Nineteen (41%) patients did not undergo decannulation within 6 months of the PSG. One (3%) patient with pre-decannulation PSG failed decannulation. Four (5%) patients without pre-decannulation PSG failed decannulation. Nineteen patients with PSG and no decannulation had significantly higher obstructive apnea-hypopnea index (OAHI) (13.62 vs 2.68 events per hour, P = 0.004), higher end-tidal CO 2 max (52.84 vs 48.03 mm Hg, P = 0.035), and were older at PSG (median age, 6.04 vs 4.04 years, P = 0.008). CONCLUSIONS: While successful decannulation can be achieved without a PSG in some patients, PSG is a valuable tool to identify BPD patients undergoing clinical evaluation for decannulation who would benefit from treatment of OSA before decannulation.

Adherence to Positive Airway Pressure for the Treatment of Obstructive Sleep Apnea in Children With Developmental Disabilities.

STUDY OBJECTIVES: To determine whether adherence to positive airway pressure (PAP) differs in children with developmental disabilities (DD) compared to typically developing (TD) children. METHODS: PAP adherence of 240 children initiated on PAP for obstructive sleep apnea (OSA) was retrospectively analyzed. Adherence between groups, expressed as percentage of nights used and hours of usage on nights used at 3 and 6 months, was compared. Predictive factors of adherence were studied using a median regression model. RESULTS: A total of 103 children with DD (median [interquartile range] age = 7.9 [3.2-13.1] years) and 137 TD (11.0 [5.5-16.1], P = .005) children were included. Percentage of nights used was significantly higher in children with DD at 3 (DD = 86.7 [33.9-97.9], TD = 62.9 [30.8-87.8] P = .01) and 6 months (DD = 90.0 [53.3-100], TD = 70.7 [29.2-90.8], P = .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 = .715; DD = 6.4 [1.8-8.3], TD = 5.7 [2.5-7.3], P = .345, respectively). This adherence measure improved over time in both groups (DD, P = .007; TD, P = .005). At 6 months, higher median neighborhood income and titration at or before 6 months were significantly predictive for percentage of nights used; higher PAP pressure was significantly predictive for hours of usage in both groups. CONCLUSIONS: Children with DD had better PAP adherence expressed as percentage of nights used than TD children. Hours of usage on nights used at 3 and 6 months were similar between groups and improved over time. Higher income and titration at or before 6 months were predictive of adherence in all children. These findings indicate that children with DD can successfully wear PAP.

Financial Analysis of an Intensive Pediatric Continuous Positive Airway Pressure Program.

Study Objectives: Continuous positive airway pressure (CPAP) is effective in treating obstructive sleep apnea in children, but adherence to therapy is low. Our center created an intensive program that aimed to improve adherence. Our objective was to estimate the program's efficacy, cost, revenue and break-even point in a generalizable manner relative to a standard approach. Methods: The intensive program included device consignment, behavioral psychology counseling, and follow-up telephone calls. Economic modeling considered the costs, revenue and break-even point. Costs were derived from national salary reports and the Pediatric Health Information System. The 2015 Medicare reimbursement schedule provided revenue estimates. Results: Prior to the intensive CPAP program, only 67.6% of 244 patients initially prescribed CPAP appeared for follow-up visits and only 38.1% had titration polysomnograms. In contrast, 81.4% of 275 patients in the intensive program appeared for follow-up visits (p < .001) and 83.6% had titration polysomnograms (p < .001). Medicare reimbursement levels would be insufficient to cover the estimated costs of the intensive program; break-even points would need to be 1.29-2.08 times higher to cover the costs. Conclusions: An intensive CPAP program leads to substantially higher follow-up and CPAP titration rates, but costs are higher. While affordable at our institution due to the local payer mix and revenue, Medicare reimbursement levels would not cover estimated costs. This study highlights the need for enhanced funding for pediatric CPAP programs, due to the special needs of this population and the long-term health risks of suboptimally treated obstructive sleep apnea.

Predictors of positive airway pressure therapy adherence in children: a prospective study.

STUDY OBJECTIVES: Children with obstructive sleep apnea are increasingly being treated with positive airway pressure (PAP), particularly if they have underlying medical conditions. Although PAP is an effective treatment, its use is challenging due to poor adherence. We hypothesized that demographic, psychosocial, and polysomnographic parameters would be related to PAP adherence. We therefore prospectively collected data potentially pertaining to PAP adherence, and correlated it with PAP use. METHODS: Fifty-six patients and their parents completed a series of psychosocial questionnaires prior to PAP initiation. Objective adherence data were obtained after 1 and 3 months of PAP use. RESULTS: The population was primarily obese; 23% had neurodevelopmental disabilities. PAP adherence varied widely, with PAP being worn 22 ± 8 nights in month-1, but mean use was only 3 ± 3 h/night. The greatest predictor of use was maternal education (p = 0.002 for nights used; p = 0.033 for mean h used/night). Adherence was lower in African American children vs other races (p = 0.021). In the typically developing subgroup, adherence correlated inversely with age. Adherence did not correlate with severity of apnea, pressure levels, or psychosocial parameters other than a correlation between family social support and nights of PAP use in month-3. CONCLUSIONS: PAP adherence in children and adolescents is related primarily to family and demographic factors rather than severity of apnea or measures of psychosocial functioning. Further research is needed to determine the relative contributions of maternal education, socioeconomic status and cultural beliefs to PAP adherence in children, in order to develop better adherence programs.

Randomized, double-blind clinical trial of two different modes of positive airway pressure therapy on adherence and efficacy in children.

STUDY OBJECTIVES: To determine the effects of bilevel positive airway pressure with pressure release technology (Bi-Flex) on adherence and efficacy in children and adolescents compared to standard continuous positive airway pressure (CPAP) therapy. We hypothesized that Bi-Flex would result in improved adherence but similar efficacy to CPAP. METHODS: This was a randomized, double-blinded clinical trial. Patients with obstructive sleep apnea were randomized to CPAP or Bi-Flex. Repeat polysomnography was performed on pressure at 3 months. Objective adherence data were obtained at 1 and 3 months. RESULTS: 56 children and adolescents were evaluated. There were no significant differences in the number of nights the device was turned on, or the mean number of minutes used at pressure per night for CPAP vs Bi-Flex (24 ± 6 vs 22 ± 9 nights, and 201 ± 135 vs 185 ± 165 min, respectively, for Month 1). The apnea hypopnea index decreased significantly from 22 ± 21/h to 2 ± 3/h on CPAP (p = 0.005), and 18 ± 15/h to 2 ± 2/h on Bi-Flex (p < 0.0005), but there was no significant difference between groups (p = 0.82 for CPAP vs Bi-Flex). The Epworth Sleepiness Scale decreased from 8 ± 5 to 6 ± 3 on CPAP (p = 0.14), and 10 ± 6 to 5 ± 5 on Bi-Flex (p < 0.0005; p = 0.12 for CPAP vs Bi-Flex). CONCLUSIONS: Both CPAP and Bi-Flex are efficacious in treating children and adolescents with OSAS. However, adherence is suboptimal with both methods. Further research is required to determine ways to improve adherence in the pediatric population.

Effects of positive airway pressure therapy on neurobehavioral outcomes in children with obstructive sleep apnea.

RATIONALE: Positive airway pressure therapy is frequently used to treat obstructive sleep apnea in children. However, it is not known whether positive airway pressure therapy results in improvements in the neurobehavioral abnormalities associated with childhood sleep apnea. OBJECTIVES: We hypothesized that positive airway pressure therapy would be associated with improvements in attention, sleepiness, behavior, and quality of life, and that changes would be associated with therapy adherence. METHODS: Neurobehavioral assessments were performed at baseline and after 3 months of positive airway pressure therapy in a heterogeneous group of 52 children and adolescents. MEASUREMENTS AND MAIN RESULTS: Adherence varied widely (mean use, 170 ± 145 [SD] minutes per night). Positive airway pressure therapy was associated with significant improvements in attention deficits (P < 0.001); sleepiness on the Epworth Sleepiness Scale (P < 0.001); behavior (P < 0.001); and caregiver- (P = 0.005) and child- (P < 0.001) reported quality of life. There was a significant correlation between the decrease in Epworth Sleepiness Scale at 3 months and adherence (r = 0.411; P = 0.006), but not between other behavioral outcomes and adherence. Behavioral factors also improved in the subset of children with developmental delays. CONCLUSIONS: These results indicate that, despite suboptimal adherence use, there was significant improvement in neurobehavioral function in children after 3 months of positive airway pressure therapy, even in developmentally delayed children. The implications for improved family, social, and school function are substantial. Clinical trial registered with www.clinicaltrials.gov (NCT 00458406).

Sleep Patterns in Children with Cystic Fibrosis.

This study examined sleep patterns and the association between sleep and perceived health for children with and without CF. Ninety families (45 CF) completed questionnaires about the child's sleep and health. Significant group differences were found for sleep patterns (bedtime, wake time, total sleep time), symptoms of sleep disordered breathing, and sleep disturbances. Poorer perceived health was associated with sleep disturbances among children with CF, but not for children without CF. This study highlights the importance of including sleep in the evaluation of children with CF, as both medical and behavioral interventions can improve the sleep of children with CF.

Pediatric polysomnography: the patient and family perspective.

STUDY OBJECTIVES: The gold-standard test used to diagnose childhood obstructive sleep apnea is polysomnography. However, this test requires an overnight stay at a sleep laboratory and the attachment of multiple sensors to the patient. The long-term impact of this testing on the child and family are not known. We hypothesized that polysomnography does not precipitate acute or chronic psychological effects in children. METHODS: A consecutive cohort of children who had undergone sleep studies 2 to 4 months prior to the interview were administered a standardized questionnaire via telephone. RESULTS: Of the 118 families that were eligible to participate, 67% could be contacted and agreed to participate; 87% of respondents reported the experience to have been satisfactory (mean Likert score of 8.6 ± 2.0 [SD] on a scale of 1-10). Similar levels of satisfaction were reported by parents of children with developmental delay or those who were younger than 3 years. The night's sleep was considered typical in 68% of cases. Sleep was less likely to be typical in children younger than 3 years (47%, p = 0.043). Eight percent of children experienced pain during the study. By caregiver report, of those children who remembered the sleep study, memories were positive in 84%. No child had evidence of serious long-term psychological issues. CONCLUSIONS: The vast majority of children and families found the polysomnography experience to be satisfactory, with no psychological sequelae. However, many children, especially those younger than 3 years, demonstrated sleep patterns different from their usual sleep. The clinical relevance of this finding merits further study. Further research evaluating the generalizability of this study is also needed.

Pulmonary capillaritis with hemorrhage due to propylthiouracil therapy in a child.

We report on a case of pulmonary capillaritis with diffuse alveolar hemorrhage in a child due to propylthiouracil (PTU). PTU treatment is a rare cause of pulmonary capillaritis in adults; we report on the first case in a pediatric patient. The treatment of pulmonary capillaritis often requires corticosteroid therapy, other immunosuppressive medications, or withdrawal of the causative agent. Our patient recovered completely after treatment with a limited course of corticosteroids and removal of PTU.

Successful transgene expression with serial doses of aerosolized rAAV2 vectors in rhesus macaques.

Bronchoscopic microspraying of recombinant adeno-associated viral (rAAV) vectors targets high doses of vector directly to pulmonary epithelium. Single-dose endobronchial gene therapy trials have been accomplished in cystic fibrosis patients; however, repeated dosing strategies are likely essential for lifetime correction. These studies address whether serial redosing with rAAV2 vectors results in an antiserotypic response and, furthermore, whether it triggers an inflammatory response prohibitive to transgene expression. Serial redosing of 9 x 10(11) infectious units of aerosolized rAAV2 vectors to rhesus macaques resulted in successful gene transfer by quantitative PCR (1.43 x 10(9) copies/g tissue) and transgene expression. Additionally, confocal microscopy and immunohistochemical analysis demonstrated in situ expression localized to the pulmonary epithelium. Although serial redosing did induce a heightened anti-neutralizing antibody response in sera, gene transfer prevailed with resultant expression. This study is the first to demonstrate successful gene transfer subsequent to repeated aerosolized doses of rAAV2 in immunocompetent nonhuman primates without associated inflammatory responses prohibitive to transgene expression.

Deposition and expression of aerosolized rAAV vectors in the lungs of Rhesus macaques.

The goals of these experiments were to efficiently deliver aerosolized adeno-associated virus (AAV) vector to the lungs of Rhesus macaques and to measure gene transfer and expression. To determine optimal lung deposition, we compared four techniques of delivering aerosolized saline admixed with the radioisotope (99m)technetium ((99m)Tc) nebulized through a mouthpiece (Neb Oral), a laryngeal airway mask (Neb LMA), or an endotracheal tube (Neb ETT), or bronchoscopically delivered by Microsprayer (PennCentury). Total lung deposition fraction, as indicated by gamma scintigraphy, averaged 0.5% (Neb Oral), 1.2% (Neb LMA), 1.8+/-0.4% (Neb ETT), and 62.3+/-11.3% (Microsprayer). Because microspraying was the most efficient method of delivery, we used it to administer saline with (99m)Tc-labeled diethylene-triamine penta-acetic acid (DTPA) admixed with 9 x 10(11) infectious units (i.u.) of AAV serotype 2 (rAAV2) vector encoding green fluorescent protein (GFP; rAAV2-GFP). Initial total and regional lung depositions were quantified by scintigraphy. We analyzed the tissue three weeks later for vector-specific DNA transduction and RNA expression. Radioisotope was detected in all lung regions, reflecting an average dose of 1.33 x 10(10)+/-9.5 x 10(9) i.u. per region. Regional data indicated an increase in expression when the dose exceeded 3 x 10(9) i.u. (P=0.030). We conclude that expression of rAAV2-GFP in lungs appears to be related to depositing a regional threshold dose greater than 3 x 10(9) i.u., easily achieved by bronchoscopic microspraying.