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

## TL;DR

A new device with customizable alarms was tested to help patients with a breathing disorder, showing better results in young adults than children.

## Contribution

A novel assistive device with multisensory stimulation was developed and tested for CCHS patients.

## Key findings

- The device improved awakening rates and recovery from desaturation in young adults.
- No significant improvements were observed in children compared to baseline.
- Arousal events and sleep efficiency were unaffected in both age groups.

## Abstract

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.