Ondine"s Curse: The Other Silent Killer
Congenital Central Hypoventilation Syndrome (CCHS), also known as Ondine's Curse is a rare but extremely serious disease that, in most cases, the untreated afflicted will die in his or her sleep due to respiratory arrest.
This ailment is so rare that only 1 in every 200,000 live born children will be diagnosed.
In 2006 there were only 200 known cases worldwide.
Most people with this illness were born with it, although some have developed the syndrome after having some sort of trauma to the brain stem.
This affects the autonomic nervous system.
This is the control center for many involuntary body functions such as heart rate, blood pressure, temperature regulation and in the case of CCHS, breathing which when is impaired is the chief symptom of the disorder.
Breathing will be disrupted although the lungs and airways are considered to be normal.
In most cases the halt in breathing, which is called apnea, occurs during sleep.
In rare cases it can transpire when the sufferer is awake as well.
Children born with this casualty will have a lifetime of apnea which can and in most cases will result in Cyanosis, a blue coloring of the skin.
In most cases this is a congenital syndrome which means it exists at birth or even before birth.
There can be several explanations such as errors in morphogenesis.
An infection in the pregnant mother can harm the unborn and cause CCHS along with a chromosome abnormality.
Diagnoses are frequently delayed because of the rarity of disease along with the overall lack of awareness among medical professionals...
especially in milder cases.
Treatment is available for those who must endure CCHS but might be considered disturbing.
While the child may breathe acceptably while awake, when sleeping or even napping a ventilator must be used.
This will require a trachea to be surgically fit.
Although this is a stifling ordeal one can continue with a fairly normal life if equipped with the correct home care.
Less than 1 percent of people diagnosed with Congenital Central Hypoventilation syndrome have been placed in an institutional venue on a long term basis.
This ailment is so rare that only 1 in every 200,000 live born children will be diagnosed.
In 2006 there were only 200 known cases worldwide.
Most people with this illness were born with it, although some have developed the syndrome after having some sort of trauma to the brain stem.
This affects the autonomic nervous system.
This is the control center for many involuntary body functions such as heart rate, blood pressure, temperature regulation and in the case of CCHS, breathing which when is impaired is the chief symptom of the disorder.
Breathing will be disrupted although the lungs and airways are considered to be normal.
In most cases the halt in breathing, which is called apnea, occurs during sleep.
In rare cases it can transpire when the sufferer is awake as well.
Children born with this casualty will have a lifetime of apnea which can and in most cases will result in Cyanosis, a blue coloring of the skin.
In most cases this is a congenital syndrome which means it exists at birth or even before birth.
There can be several explanations such as errors in morphogenesis.
An infection in the pregnant mother can harm the unborn and cause CCHS along with a chromosome abnormality.
Diagnoses are frequently delayed because of the rarity of disease along with the overall lack of awareness among medical professionals...
especially in milder cases.
Treatment is available for those who must endure CCHS but might be considered disturbing.
While the child may breathe acceptably while awake, when sleeping or even napping a ventilator must be used.
This will require a trachea to be surgically fit.
Although this is a stifling ordeal one can continue with a fairly normal life if equipped with the correct home care.
Less than 1 percent of people diagnosed with Congenital Central Hypoventilation syndrome have been placed in an institutional venue on a long term basis.
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