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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MDPI AG
2025-03-01
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| Online Access: | https://www.mdpi.com/1424-8220/25/6/1759 |
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| author | Silvia Rapella Caterina Piazza Francesco Morandi Alessandro Carcano Cinzia Arzilli Niccolò Nassi Igor Catalano Francesca Formica Emilia Biffi |
| author_facet | Silvia Rapella Caterina Piazza Francesco Morandi Alessandro Carcano Cinzia Arzilli Niccolò Nassi Igor Catalano Francesca Formica Emilia Biffi |
| author_sort | Silvia Rapella |
| collection | DOAJ |
| description | 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. |
| format | Article |
| id | doaj-art-c04a738fb9814cc48d47c629b4b70a0e |
| institution | OA Journals |
| issn | 1424-8220 |
| language | English |
| publishDate | 2025-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Sensors |
| spelling | doaj-art-c04a738fb9814cc48d47c629b4b70a0e2025-08-20T01:48:54ZengMDPI AGSensors1424-82202025-03-01256175910.3390/s25061759Early Findings on the Effectiveness of Novel Awakening Stimuli for Patients with Congenital Central Hypoventilation SyndromeSilvia Rapella0Caterina Piazza1Francesco Morandi2Alessandro Carcano3Cinzia Arzilli4Niccolò Nassi5Igor Catalano6Francesca Formica7Emilia Biffi8Scientific Institute, IRCCS Eugenio Medea, 23842 Bosisio Parini, LC, ItalyScientific Institute, IRCCS Eugenio Medea, 23842 Bosisio Parini, LC, ItalyItalian Association for Congenital Central Hypoventilation Syndrome (AISICC), 50126 Florence, FI, ItalyItalian Association for Congenital Central Hypoventilation Syndrome (AISICC), 50126 Florence, FI, ItalyMeyer Children’s Hospital IRCCS, 50139 Florence, FI, ItalyMeyer Children’s Hospital IRCCS, 50139 Florence, FI, ItalyPediatric Palliative Care, Pain Service, Department of Women’s and Children’s Health, University of Padua, 35128 Padua, PD, ItalyScientific Institute, IRCCS Eugenio Medea, 23842 Bosisio Parini, LC, ItalyScientific Institute, IRCCS Eugenio Medea, 23842 Bosisio Parini, LC, ItalyCongenital 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.https://www.mdpi.com/1424-8220/25/6/1759congenital central hypoventilation syndromeassistive devicedesaturation episodesarousal |
| spellingShingle | Silvia Rapella Caterina Piazza Francesco Morandi Alessandro Carcano Cinzia Arzilli Niccolò Nassi Igor Catalano Francesca Formica Emilia Biffi Early Findings on the Effectiveness of Novel Awakening Stimuli for Patients with Congenital Central Hypoventilation Syndrome Sensors congenital central hypoventilation syndrome assistive device desaturation episodes arousal |
| title | Early Findings on the Effectiveness of Novel Awakening Stimuli for Patients with Congenital Central Hypoventilation Syndrome |
| title_full | Early Findings on the Effectiveness of Novel Awakening Stimuli for Patients with Congenital Central Hypoventilation Syndrome |
| title_fullStr | Early Findings on the Effectiveness of Novel Awakening Stimuli for Patients with Congenital Central Hypoventilation Syndrome |
| title_full_unstemmed | Early Findings on the Effectiveness of Novel Awakening Stimuli for Patients with Congenital Central Hypoventilation Syndrome |
| title_short | Early Findings on the Effectiveness of Novel Awakening Stimuli for Patients with Congenital Central Hypoventilation Syndrome |
| title_sort | early findings on the effectiveness of novel awakening stimuli for patients with congenital central hypoventilation syndrome |
| topic | congenital central hypoventilation syndrome assistive device desaturation episodes arousal |
| url | https://www.mdpi.com/1424-8220/25/6/1759 |
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