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        Diagnosis of Nocturnal Hypoventilation in Pediatric Neuromuscular Disorders: A Survey of Clinical Practice in Australia and New Zealand

        Adelaide L Withers,Jenny Downs,Andrew Wilson,Graham Hall 대한수면연구학회 2023 Journal of sleep medicine Vol.20 No.1

        Objectives: Nocturnal hypoventilation is a complication of neuromuscular disorders. There are various recommendations for measuring pCO<sub>2</sub> during polysomnography and numerous national and international definitions of hypoventilation that could contribute to significant variations in clinical practice. We therefore aimed to determine clinical practices implemented by Australasian pediatric sleep physicians.Methods: Pediatric sleep physicians completed an electronic survey for information regarding pCO<sub>2</sub> measurements and definitions of hypoventilation that are followed for children with neuromuscular disorders.Results: It was found that transcutaneous measurement of pCO<sub>2</sub> was performed in all centers, with 25% of the centers simultaneously performing capnography. Twelve definitions of hypoventilation were used, including published definitions from the American Academy of Sleep Medicine (AASM) manual and recommendations of the pediatric Australasian Sleep Association/Australasian Sleep Technologists Association. The most commonly used definition of hypoventilation (9/17, 53%) was the 2012 pediatric AASM definition (pCO<sub>2</sub> >50 mmHg for >25% of the total sleep time). There was a discrepancy between centers and individuals within the same center when defining hypoventilation. Answers stating the use of the Australasian definitions (rise in pCO<sub>2</sub> ≥10 mmHg from wake to sleep, average rise in pCO<sub>2</sub> ≥3 mmHg from non rapid eye movement to rapid eye movement sleep) were more frequent when asked specifically via a checkbox (yes/no) compared to free text.Conclusions: These results confirm the heterogeneity and lack of standardization of clinical practice within Australasia when measuring pCO<sub>2</sub> during polysomnography and defining hypoventilation. The Australasian definitions were not used as frequently as anticipated.

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        Respiratory Health, Sleep Dysfunction, and Mental Health in Children and Adolescents With a Neuromuscular Disorder: A Descriptive Qualitative Study

        Meg Huston,Adelaide L Withers,Jenny Lam,Andrew Wilson,Jenny Downs 대한수면연구학회 2023 Journal of sleep medicine Vol.20 No.1

        Objectives: To explore how respiratory health and sleep problems affect mental health in children and adolescents with neuromuscular disorders (NMDs).Methods: A qualitative descriptive study was carried out with content analysis of in-depth interviews conducted with children with NMDs and their parents. Data were collected during semi-structured interviews with children with NMDs and their parents. A total of 14 families were recruited (17 parents and 7 children and adolescents with NMD aged 5–17 years). Each interview was conducted with 1–3 family member(s).Results: Engagement in community activities, meaningful relationships, feeling well, and achieving independence each contributed positively to children’s mental health. Additional challenges to mental health during periods of poor respiratory and/or sleep health included: 1) challenges to behavioral regulation, 2) changes to mood, and 3) challenges to thinking. Parents and children reported nurturing and coping self-management strategies when their physical and mental health were dually challenged in addition to medical management strategies such as non-invasive ventilation (NIV).Conclusions: This study supports the notion that changes in mental health may indicate poor physical health in children with NMDs. Treating respiratory and sleep problems, such as by early NIV implementation, may improve the mental health of children with NMDs and thus support their overall wellbeing and health outcomes.

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