Establishing a telehealth model addressing paediatric sleep health in remote and rural Northern Territory Australia: Overcoming the distance barrier.

AIM: This study examined the outcomes of a telehealth model for sleep health assessment among Indigenous and non-Indigenous children residing in remote and regional communities at the Top End Northern Territory (NT) of Australia. METHODS: Video telehealth consultation, that included clinical history and relevant physical findings assessed virtually with an interstate paediatric sleep physician was conducted remotely. Polysomnography (PSG) and therapeutic interventions were carried out locally at Darwin, NT. The study participants were children referred between 2015 and 2020. RESULTS: Of the total 812 children referred for sleep assessment, 699 underwent a diagnostic PSG. The majority of patients were female (63%), non-Indigenous (81%) and resided in outer regional areas (88%). Indigenous children were significantly older and resided in remote or very remote locations (22% vs. 10%). Referral patterns differed according to locality and Indigenous status - (non-Indigenous via private (53%), Indigenous via public system (35%)). Receipt of referrals to initial consultation was a median of 16 days and 4 weeks from consult to PSG. Remote children had slightly longer time delay between the referral and initial consult (32 vs. 15 days). Fifty one percent were diagnosed to have OSA, 27% underwent adenotonsillectomy and 2% were prescribed with CPAP therapy. CONCLUSIONS: This study has demonstrated that a telehealth model can be an effective way in overcoming logistical barriers and in providing sleep health services to children in remote and regional Australia. Further innovative efforts are needed to improve the service model and expand the reach for vulnerable children in very remote communities.

Differences in Outcomes between Early and Late Diagnosis of Cystic Fibrosis in the Newborn Screening Era.

OBJECTIVES: To evaluate children with cystic fibrosis (CF) who had a late diagnosis of CF (LD-CF) despite newborn screening (NBS) and compare their clinical outcomes with children diagnosed after a positive NBS (NBS-CF). STUDY DESIGN: A retrospective review of patients with LD-CF in New South Wales, Australia, from 1988 to 2010 was performed. LD-CF was defined as NBS-negative (negative immunoreactive trypsinogen or no F508del) or NBS-positive but discharged following sweat chloride < 60 mmol/L. Cases of LD-CF were each matched 1:2 with patients with NBS-CF for age, sex, hospital, and exocrine pancreatic status. RESULTS: A total of 45 LD-CF cases were identified (39 NBS-negative and 6 NBS-positive) with 90 NBS-CF matched controls. Median age (IQR) of diagnosis for LD-CF and NBS-CF was 1.35 (0.4-2.8) and 0.12 (0.03-0.2) years, respectively (P <.0001). Estimated incidence of LD-CF was 1 in 45 000 live births. Compared with NBS-CF, LD-CF had more respiratory manifestations at time of diagnosis (66% vs 4%; P <.0001), a higher rate of hospital admission per year for respiratory illness (0.49 vs 0.2; P = .0004), worse lung function (forced expiratory volume in 1 second percentage of predicted, 0.88 vs 0.97; P = .007), and higher rates of chronic colonization with Pseudomonas aeruginosa (47% vs 24%; P = .01). The LD-CF cohort also appeared to be shorter than NBS-CF controls (mean height z-score -0.65 vs -0.03; P = .02). CONCLUSIONS: LD-CF, despite NBS, seems to be associated with worse health before diagnosis and worse later growth and respiratory outcomes, thus providing further support for NBS programs for CF.

Sleep quality and obstructive sleep apnoea in Indigenous and non-Indigenous Australian children.

BACKGROUND: Literature pertaining to the prevalence of obstructive sleep apnoea (OSA) and sleep quality among Indigenous Australian children is sparse. This study assessed various sleep related parameters and outcomes between Indigenous and non-Indigenous Australian children. METHODS: Children referred to the sleep health service in the Northern Territory of Australia for a clinically suspected sleep disorder between 2015 and 2021 were included in this study. Self-reported sleep measures alongside polysomnography data were assessed and compared between these two diverse ethnic population. RESULTS: Of the 671 sleep studies assessed, 121 (18%) were from Indigenous children. The majority of patients were male (61%), with a median age of 5.7 (3.5, 8.9) years, and body mass index (BMI) in the normal range (57%). Indigenous children were significantly older (median 7.2 years (4.5, 11.9), with a higher BMI (p = 0.005) and a greater proportion living in very remote locality (14% vs. 6% non-Indigenous, p = 0.001). Indigenous children had higher Paediatric Daytime Sleepiness Scale scores (p = 0.001), higher screen use before bed (p = 0.005), later bedtimes (p = 0.001) and reduced total sleep time (p = 0.034) compared to non-Indigenous children. Prevalence of OSA was higher in Indigenous children (55% vs. 48%) and with greater severity compared to non-Indigenous children. CONCLUSIONS: In this study, OSA was more prevalent and more severe in Indigenous children than their non-Indigenous peers. However, this may not necessarily be extrapolated to the general Indigenous paediatric population. Sleep hygiene and sleep quantity was also decreased further impacting adequate sleep. This highlights the importance of identifying and managing these addressable parameters and for targeted interventions.

Comparison of polysomnographic characteristics between low birthweight and normal birthweight children in the Northern Territory of Australia: A case-control study.

OBJECTIVES: To describe the sleep architecture of pediatric patients according to whether they were born low birthweight (birthweight <2500 g, LBW) or normal birthweight (birthweight >2500 g). DESIGN: Case control study. SETTING: Pediatric sleep laboratory in the Northern Territory of Australia during a 5-year study period (2015- 2020). PARTICIPANTS: Pediatric patients (aged <18 years) referred to the specialist sleep service for assessment of clinically suspected sleep disorders. MEASUREMENTS: Sleep onset latency, rapid eye movement (REM) sleep latency, wake time after sleep onset, total sleep time, sleep efficiency, non-rapid eye movement stages N1/N2/N3, and REM sleep duration, total/spontaneous/respiratory/limb related arousal indexes, total/non-rapid eye movement/REM obstructive apnea-hypopnea index and oxygen saturation. RESULTS: One hundred and seventy-two pediatric patients had birthweight data available of whom 19 were LBW. LBW patients showed significantly greater sleep disruption and higher prevalence of poor sleepers (<80% efficiency). In multivariate regression models, increasing birthweight was associated with significantly greater sleep efficiency and total sleep time. After accounting for gestational age LBW was associated with increased odds of obstructive sleep apnea. CONCLUSIONS: Among pediatric patients LBW is associated with increased sleep disruption and reduced sleep efficiency. This is attenuated by gestational age, though both gestational age and LBW significantly influence odds of obstructive sleep apnea. This sleep health deficit may contribute to development of chronic disease in this vulnerable population, and should be monitored to provide avenues for early intervention.

Nocturnal respiratory failure in a child with congenital myopathy - management using average volume-assured pressure support (AVAPS).

This is a case report of the effective use of bi-level positive airway pressure support (BPAP) using the volume-assured pressure support feature in a pediatric patient with a congenital myopathy and significant nocturnal hypoventilation. Our patient was started on nocturnal nasal mask BPAP but required high pressures to improve her oxygen saturations and CO2 baseline. She was then trialed on a BPAP machine with the volume-assured pressure support feature on. The ability of this machine to adjust inspiratory pressures to give a targeted tidal volume allowed the patient to be on lower pressure settings for periods of the night, with the higher pressures only when required. She tolerated the ventilation well and her saturations, CO2 profiles, and clinical condition improved. This case report highlights the benefits of the volume-assured pressure support feature on a BPAP machine in a child with a neuromuscular disorder.