Ondine's Curse (Congenital Central Hypoventilation Syndrome, CCHS) is a very rare, incurable and potentially fatal disorder. CCHS patients can stop breathing while asleep and often require lifelong use of ventilators. Its name is a reference to the myth of Ondine, a water nymph who had an unfaithful mortal lover. He swore to her that his "every waking breath would be a testimony of [his] love", and upon witnessing his adultery, she cursed that if he should fall asleep, he would forget to breathe. Eventually, he fell asleep from sheer exhaustion, and his breathing stopped.
CCHS is a multisystem disorder of the central nervous system where the automatic control of breathing is absent or impaired.A CCHS patient’s respiratory response to low blood oxygen saturation (hypoxia) or to CO2 retention (hypercapnia) is sluggish during awake hours and absent to varying degrees during sleep, serious illness, and/or stress.
All CCHS patients require lifelong ventilatory support.
Ventilatory support is possible by:
- invasive mechanical ventilation by connecting to a ventilator via tracheotomy tube,
- non-invasive mechanical ventilation by connecting to a ventilator via facial mask,
- breathing pacemakers (diaphragm pacers)
With appropriate home care, family support and careful medical supervision, CCHS children can lead fulfilling and productive, near-normal lives. Over 60% of the children are making normal progress in school, while another group required some or significant special educational support. With advances in home health care technology, such as portable ventilators, oximeters and ETCO2 monitors, has come optimal health management and much-improved prognoses for the infants diagnosed with CCHS.
Please go to CCHS Network @ www.cchsnetwork.org for more information about CCHS.
