The impact of obstructive sleep apnea in children with sickle cell disease and asthma.

INTRODUCTION: Asthma and obstructive sleep apnea (OSA) are chronic diseases that disproportionately affect children with sickle cell disease (SCD). The literature describes the negative impact that both conditions have on children with SCD separately; however, the effect of OSA on asthmatic children with OSA is less specific. We hypothesized that the presence of OSA in children with SCD and asthma is associated with specific hematologic markers, worse clinical outcomes, and greater healthcare utilization. METHODS: We retrospectively evaluated children with both SCD and asthma who underwent polysomnography (PSG). We assessed their demographic information, PSG data, hematologic indices, and healthcare utilization based on the concurrent presence of OSA. RESULTS: Fifty-nine percent of the cohort had OSA with a lower oxygen saturation (SpO2 ) nadir (87% vs. 93%, p < 0.001) and a lower median daytime SpO2 (96.5% vs. 98.5%, p < 0.05); those with OSA were more likely to have the hemoglobin SS genotype (86% vs. 46.5%, p = 0.03). Additionally, those with OSA had a higher mean corpuscular volume (87 vs. 77.2 fL, p = 0.03) and reticulocyte count (10.1% vs. 5.5%, p < 0.01). There was no difference in asthma severity or healthcare utilization between those with OSA and those without OSA. DISCUSSION: Overall, children with SCD and asthma might be at increased risk for developing OSA, and screening for sleep-disordered breathing should be incorporated as part of their routine care.

A social media intervention for the families of young Black men with obstructive sleep apnoea.

Objective: To address positive airway pressure (PAP) adherence in adolescents diagnosed with obstructive sleep apnoea (OSA) by pilot testing a novel, online, facilitated, peer-support and health education programme for families. Design Setting and Methods: Families participated in separate Facebook peer-groups (adolescent [n=6] and parent [n=6]) for four weeks, followed by face-to-face interviews. Participants received OSA and PAP educational videos and posts, engaged with questions and polls, and viewed de-identified postings of peer PAP use data. Results: Adolescent participants were young Black males aged 13-17 years (n=6) with obesity (n=5), severe sleep apnoea (100%) and 4-15 months of prior PAP use. Parent participants were mothers (n=4) and fathers (n=2). Four of six young males increased their mean PAP use during the intervention period. Overall, parents were more engaged with the Facebook group page than adolescents, but interviews revealed the online group/peer-support and education provided was highly regarded and appreciated by families. Parents were particularly appreciative of being involved in care and diagnosis in this way. Conclusion: Results of this pilot trial provide important data regarding intervention design, content, and delivery approaches to be considered in the development of future interventions aiming to engage families and improve adolescent PAP adherence.

Pediatric pulmonary year in review 2021: Sleep medicine.

Pediatric pulmonology publishes original research, review articles, and case reports on a wide variety of pediatric respiratory disorders. In this article, we summarized the past year's publications in sleep medicine and reviewed selected literature from other journals in this field. We focused on original research articles exploring aspects of sleep-disordered breathing in patients with underlying conditions such as cystic fibrosis, asthma, and sickle cell disease. We also explored sleep-disordered breathing risk factors, monitoring, diagnosis, and treatment; and included recent recommendations for drug-induced sleep endoscopy and ways to monitor and improve PAP adherence remotely.

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.

Sleep Deficiency in Adolescents: The School Start Time Debate.

Adolescence is commonly accepted as a challenging time for sleep, with multiple factors contributing to sleep deficiency in adolescents. These include physiologic changes with shifts in their circadian rhythm; medical sleep disorders; and social, cultural, and environmental factors. Early school start times negatively affect sleep in adolescents as well, with poorer outcomes in their overall health, wellbeing, and performance. This article highlights the different contributing factors for sleep deficiency in adolescents and the consequences of sleep deficiency. In addition, the authors discuss the impact of delayed school start times in improving adolescents' sleep and overall function.

Age and weight considerations for the use of continuous positive airway pressure therapy in pediatric populations: an American Academy of Sleep Medicine position statement.

This position statement provides guidance for age and weight considerations for using continuous positive airway pressure therapy in pediatric populations. The American Academy of Sleep Medicine commissioned a task force of experts in pediatric sleep medicine to review the medical literature and develop a position statement based on a thorough review of these studies and their clinical expertise. The American Academy of Sleep Medicine Board of Directors approved the final position statement. It is the position of the American Academy of Sleep Medicine that continuous positive airway pressure can be safe and effective for the treatment of obstructive sleep apnea for pediatric patients, even in children of younger ages and lower weights, when managed by a clinician with expertise in evaluating and treating pediatric obstructive sleep apnea. The clinician must make the ultimate judgment regarding any specific care in light of the individual circumstances presented by the patient, accessible treatment options, patient/parental preference, and resources. CITATION: Amos L, Afolabi-Brown O, Gault D, et al. Age and weight considerations for the use of continuous positive airway pressure therapy in pediatric populations: an American Academy of Sleep Medicine position statement. J Clin Sleep Med. 2022;18(8):2041-2043.

Benefit of pulmonary subspecialty care for children with sickle cell disease and asthma.

OBJECTIVE: Asthma is a recognized comorbidity in children with sickle cell disease (SCD). It increases the risk of acute chest syndrome (ACS), vaso-occlusive episodes, and early mortality. We aim to determine whether evaluation and management of children with SCD and asthma by a pulmonologist reduce rate of asthma exacerbation and ACS. METHODS: The study included 192 patients with SCD (0-21 years) followed at Children's Hospital of Philadelphia Hematology between January 1, 2015, and December 31, 2018, with a diagnosis of asthma, wheeze, or cough. Patients were placed in two groups: those evaluated by a pulmonologist (SCD-A-P) and those not (SCD-A). Rates of emergency department (ED) visits and hospitalizations for asthma exacerbation and ACS were compared between groups and over time. RESULTS: SCD-A-P patients (n = 70) were predominantly SCD type SS with lower hemoglobin and hematocrit compared to SCD-A patients (n = 122). SCD-A-P started with a higher average rate of hospital visits for asthma exacerbation and ACS per year (2.69 [1.02-4.37]) compared to SCD-A (0.43 [0.24-0.63]), (p < 0.001). For SCD-A-P patients with at least one hospital visit (n = 48), the average rate decreased from 3.93 (1.57-6.29) to 0.85 (0.48-1.23) following pulmonary consultation (p = 0.014) and was comparable to the SCD-A rate by study end. CONCLUSION: SCD-A-P was mainly SCD type SS and had higher ED/hospitalization rates for asthma exacerbation and ACS compared to SCD-A, but the rates significantly decreased following pulmonology consultation. These findings support the pulmonologist's role in the multidisciplinary care of SCD patients and highlight the need for evidence-based asthma guidelines for children with SCD.

Pediatric pulmonology year in review 2019: Sleep medicine.

Pediatric Pulmonology publishes original research, review articles as well as case reports on a wide variety of pediatric respiratory disorders. In this article, we summarize the past year's publications in sleep medicine and we review selected literature from other journals within this field. Articles highlighted are topics on risk factors of sleep-disordered breathing, diagnosis, and treatment of obstructive sleep apnea as well as the utility of polysomnography in various complex conditions.

Positive airway pressure adherence in pediatric obstructive sleep apnea: A systematic scoping review.

Positive airway pressure (PAP) therapy is a commonly prescribed treatment for pediatric obstructive sleep apnea (OSA). Negative health consequences associated with untreated OSA make understanding the utilization of PAP therapy imperative. The aim of this review was to describe PAP use in children and adolescents with OSA, explore factors that influence use, and describe published scientific or clinical approaches to improve use. Among 20 studies, average PAP adherence was 56.9% (range, 24-87%). PAP use averaged 4.0 h (SD = 3.1) to 5.2 h (SD = 3.4) per night. Cautious consideration of summary estimates of PAP use is necessary as studies were heterogeneous and adherence definitions widely varied across studies. Age, sex, and developmental delay were the only factors associated with PAP use in more than one study. The majority of approaches to improve use were program evaluations rather than scientifically tested interventions. This review identified critical gaps in the existing literature and sets forth a research agenda for the future.

Pediatric polysomnography-A review of indications, technical aspects, and interpretation.

Polysomnography is an elaborate diagnostic test composed of numerous data-collecting sensors working concomitantly to aid in the evaluation of varied sleep disorders in all age groups. Polysomnography is the study of choice for the assessment of pediatric sleep-disordered breathing, including obstructive sleep apnea syndrome, central apnea, and hypoventilation disorders, and is used to help determine treatment efficacy. Beyond the purview of snoring and breathing pauses, polysomnography can elucidate the etiology of hypersomnolence, when associated with a multiple sleep latency test, and abnormal movements or events, whether nocturnal seizure or complex parasomnia, when a thorough patient history cannot provide clear answers. This review will highlight the multitudinous indications for pediatric polysomnography and detail its technical aspects by describing the multiple neurophysiologic and respiratory parametric sources. Knowledge of these technical aspects will provide the practitioner with a thoughtful means to understand the limitations and interpretation of polysomnography.

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.

Updates on management of pediatric obstructive sleep apnea.

Obstructive sleep apnea (OSA) affects about 1%-5% of the pediatric population. The consequences of untreated OSA in children include neurocognitive deficits, behavioral problems, poor school performance as well as systemic and pulmonary hypertension. The treatment options for pediatric OSA are numerous with a variety of surgical and non-surgical interventions. As our understanding of the complexities of OSA grows, the options for management have continued to expand as well. The objectives of this review are to describe the commonly prescribed treatments for pediatric OSA including adenotonsillectomy as well as use of positive airway pressure. We also highlight other surgical and non-surgical interventions available. In addition, we provide updates on current research focusing on newer diagnostic and experimental treatment modalities.

Pulmonary hypertension and chronic hypoventilation in ROHHAD syndrome treated with average-volume assured pressure support.

INTRODUCTION: Rapid-onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) syndrome is an exceptionally rare clinical entity with significant morbidity and high mortality with challenging-to-treat hypoventilation. CASE PRESENTATION: An 11-year-old morbidly obese Chinese female presented with a putative diagnosis of ROHHAD associated with a left psoas ganglioneuroma. Initial polysomnography showed severe obstructive sleep apnea and hypoventilation. She was not adherent to prescribed non-invasive positive pressure ventilation (NIPPV). Echocardiography demonstrated evidence of pulmonary hypertension, likely secondary to chronic hypoventilation. With behavioral modification and trial of average volume-assured pressure support (AVAPS), adherence improved with eventual improvement of her pulmonary hypertension. CONCLUSION: AVAPS may improve ventilation and NIPPV adherence in central hypoventilation disorders such as ROHHAD, reducing risk of morbidity and mortality.

Kleine-Levin Syndrome.

Kleine-Levin syndrome (KLS) is a rare disorder characterized by discrete episodes of hypersomnia associated with cognitive and behavioural abnormalities, as well as normal alertness and function between episodes. The prevalence of KLS may be underestimated as it is often misdiagnosed and managed as another sleep disorder, neurological disorder or psychiatric condition. KLS is more typically seen in adolescence than at other ages, and is more common in males than in females. There are currently neither standard biomarkers nor specific imaging study findings, making the diagnosis of KLS a challenge. Furthermore, there are no consistently effective therapies. The prognosis, however, is felt to be overall favorable, as episodes become progressively milder and less frequent before resolving entirely in most patients.