Early Findings on the Effectiveness of Novel Awakening Stimuli for Patients with Congenital Central Hypoventilation Syndrome

Congenital Central Hypoventilation Syndrome (CCHS) is a rare disorder that impairs autonomic breathing control, leading to alveolar hypoventilation and sometimes to central apnoea, predominantly during sleep. Patients typically require nocturnal ventilatory support and alarms to prevent life-threate...

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Main Authors: Silvia Rapella, Caterina Piazza, Francesco Morandi, Alessandro Carcano, Cinzia Arzilli, Niccolò Nassi, Igor Catalano, Francesca Formica, Emilia Biffi
Format: Article
Language:English
Published: MDPI AG 2025-03-01
Series:Sensors
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Online Access:https://www.mdpi.com/1424-8220/25/6/1759
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Summary:Congenital Central Hypoventilation Syndrome (CCHS) is a rare disorder that impairs autonomic breathing control, leading to alveolar hypoventilation and sometimes to central apnoea, predominantly during sleep. Patients typically require nocturnal ventilatory support and alarms to prevent life-threatening desaturation events. However, current alarm systems integrated into pulse oximeters do not provide adequate assistance at home. To address these limitations, we developed an assistive device with customizable multisensory stimulation that activates based on the severity and duration of desaturation episodes. In a multicenter clinical trial involving 4 children and 11 young adults with CCHS, we assessed the device’s effectiveness and the role of arousals over three nights: one baseline and two test nights. The results showed that the device significantly improved awakening rates and enabled faster recovery from desaturations in young adults. However, no such improvements were observed in children compared to the baseline. Arousal events and sleep efficiency were unaffected by the device in both groups. These findings suggest that the device can enhance the safety and autonomy of young adults with CCHS but may be more effective in alerting caregivers in pediatric cases than directly waking children. Further studies are needed to refine its application across different age groups, given the limited sample size.
ISSN:1424-8220