Abstract
Purpose
Congenital Central Hypoventilation Syndrome (CCHS) requires lifelong ventilatory support during sleep. Subjects with CCHS are vulnerable to sleep disturbances associated with treatments, monitoring alarms, and care they receive. We hypothesized that sleep would be disrupted in patients with CCHS due to ventilatory support and other treatments at night.
Methods
An anonymous survey of patients with CCHS, age up to 17 years was conducted through REDCAP. Subjects were recruited in person, by flyer, email, and social media. Data collected included demographics, PHOX2B genotype, ventilatory support, treatments, nursing, and sleep parameters.
Results
We received 23 responses (35% female, 8.1 years ± 5.6). PHOX2B genotypes were 20/24 PARM (2), 20/25 PARM (4), 20/26 PARM (2), 20/27 PARM (9), ≥ 20/28 PARM (2), and NPARM (2). Two subjects did not indicate the PHOX2B genotype. 13/23 were ventilated by PPV via tracheostomy, 7 by NIPPV, 2 by diaphragm pacing, and 1 did not indicate. Additional treatments received at night included suctioning (9), aerosol (1), G-tube feeding (2), and none (11). Only 9 received nursing at night. 13 used pulse oximetry for monitoring, and 9 used both pulse oximetry and end tidal CO2 monitor. 17/23 rarely woke up due to ventilator or monitor alarms. 11/23 usually or sometimes woke up at least once a night; only 2/11 woke up due to alarms. 5/17 who rarely woke up to the alarms had night nursing.
Conclusion
Most subjects with CCHS did not awaken to ventilator or monitoring alarms and a majority of these patients did not have nighttime nursing. (Mathur et al. in Sleep 43(Supplement_1):A333, 2020)
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Funding
No funding was received for conducting the study or with the preparation of the manuscript. The REDCap platform used in this study was supported by grants UL1TR001855 and UL1TR000130 from the National Center for Advancing Translational Science (NCATS) of the U.S. National Institutes of Health. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
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Shreya Mathur, Thomas G. Keens, and Iris Perez contributed to the study conception and design. Material preparation, data collection and analysis were performed by Shreya Mathur, Eric Laifman, Thomas G. Keens, Sheila Kun, Sally L. D. Ward and Iris A. Perez. The first draft of the manuscript was written by Shreya Mathur and Iris Perez and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.
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The questionnaire and methodology for this study was approved by the Institutional Review Board of the Children’s Hospital Los Angeles (IRB#: CHLA-19–00249). Informed consent was obtained from all individual participants included in the study.
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N/A. Patients were notified in the consent form that they will not be identified in publications of the research results.
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The authors declare no competing interests.
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Mathur, S., Laifman, E., Keens, T.G. et al. Children with Congenital Central Hypoventilation Syndrome Do Not Wake up to Ventilator Alarms. Sleep Breath 26, 1277–1280 (2022). https://doi.org/10.1007/s11325-021-02452-7
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DOI: https://doi.org/10.1007/s11325-021-02452-7