Home Mechanical Ventilation CH 02-HMV Considerations

Sheila Kun
Nurse Case Manager Pediatric Pulminology
skun@chla.usc.edu

CCHS
Congenital Central Hypoventilation Syndrome
congenital central hypoventilation syndrome (CCHS) or primary alveolar hypoventilation, is a respiratory disorder that is fatal if untreated. Persons afflicted with Ondine's curse classically suffer from respiratory arrest during sleep.

Persons who have CCHS get it at birth, or develop it due to severe neurological trauma/damage to the brainstem. The diagnosis may be delayed because of variations in the severity of the manifestations or lack of awareness in the medical community, particularly in milder cases. (Chin, 2006).[1]

This very rare and serious form of central sleep apnea involves an inborn failure of autonomic control of breathing. About 1 in 200,000 live born children have the condition. In 2006, there were only about 200 known cases worldwide. In all cases, episodes of apnea occur in sleep, but in a few patients, at the most severe end of the spectrum, apnea also occurs while awake.

A persons gender or race is not a determining factor when dealing with susceptibility to CCHS. Males and females are both affected equally and a person's ethnicity, as of this point, has been not been coincided a variable to the disease.

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