We never think how the respiration process is happening in our body unless we are affected by respiratory disorder. Respiration is an automatic process happening and while we need more oxygen it can also be done voluntary with our effort like running, swimming, exercises, etc. Breathing is natural process which keeps on going on living body. But there are some people who need to be awake for breathing, if felt asleep they may die. What is this all about?
Ondine’s curse also known as central hypoventilation syndrome (CHS) is a respiratory disorder that results in respiratory arrest during sleep. It is very rare disorder only about 1000 cases were known. CHS can either be congenital (by birth) or acquired later in life due to some neurological disorder. Ondine’s curse is named after a mythical story about a heartbroken water spirit who curses her unfaithful husband to stop breathing if he ever fall asleep again. It is extreme form of sleep apnea (stoppage of breathing while sleeping) so very fatal if untreated.
There is pacemaker type of cells in our brainstem (midbrain, medulla, Pons) which automatically sends the impulse to the respiratory muscles for respiration. Increase level of carbon dioxide and decrease level of oxygen somehow send the impulse to the automatic center of the brain and respiration process happening without being aware. Person with Ondine’s curse automatic center is damaged but the person has to live so, he voluntary have to active it’s respiration muscles like diaphragm for respiration.
That is ok when awake but what to do during sleep?
Most of the time the person cannot sleep; the person may die with lack of sleep rather than decrease in oxygen or increase in carbon dioxide in the body. The treatment for such patient is to provide the mechanical ventilation by machine during sleep. While the patient is awake he has to breathe by his own effort and while asleep by the mechanical ventilation. Life expectancy of ondine’s curse dependent upon severity of the disorder, timing of the diagnosis, and the success of treatment.
Pic source: Polly’s pages (aka ‘Donna Williams’)