Congenital Central Hypoventilation Syndrome (CCHS)- Ondine’s Curse - Seeker's Thoughts

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Congenital Central Hypoventilation Syndrome (CCHS)- Ondine’s Curse

What is Congenital Central Hypoventilation Syndrome (CCHS)?
Central hypoventilation syndrome is a respiratory
disorder that results in respiratory arrest during sleep or, CCHS is a disorder of the nervous system in which the cue to breathe is lost when the patient goes to sleep.
This results in a lack of oxygen and a build-up of carbon dioxide in the body, which can sometimes turn fatal.
 There are less than 1,000 known cases all over the world.
Though the name describes the disorder as congenital, some forms of the disease may also be present in adults.
In fact, adult onset is far more common than congenital presentation; there have been many adult cases reported in medical journals over the years.
acquired  Central hypoventilation syndromeACHS can develop as a result of severe injury or trauma to the brain or brainstem.
Ondine’s Curse
The disease is also known as Ondine’s Curse. Ondine, a nymph in French mythology, had cursed her unfaithful husband that he would forget to breathe the moment he fell asleep.

The Reason for this disease
The mutation of a gene called PHOX2B, which is crucial for the maturation of nerve cells in the body, can cause CCHS.
It can also be genetically acquired, which is when it is congenital. However, sudden mutation is more common than a transmission of the mutated gene from parent to child.

Treatment
People generally require tracheostomy and lifetime mechanical ventilation on a ventilator in order to survive. However, it has now been shown that biphasic cuirass ventilation can effectively be used without the need for a tracheotomy. Other potential treatments for Ondine's curse include oxygen therapy and medicine for stimulating the respiratory system. Currently, problems arise with the extended use of ventilators, including fatal infections and pneumonia.
Most people with CCHS (unless they have the Late Onset form) do not survive infancy, unless they receive ventilatory assistance during sleep. An alternative to a mechanical ventilator is diaphragm pacing