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Congenital Central Hypoventilation syndrome
How is it treated?
The treatment of CCHS involves ensuring adequate ventilation when the child is unable to breathe on their own. No medication has been shown to be effective and mechanical ventilation is needed. This blows air/oxygen at pressure intermittently through a tracheostomy (see entry) or face mask into the lungs (positive pressure ventilators). For those with daytime ventilation, special pacemakers can be implanted to stimulate the nerves to the diaphragm, the main breathing muscle.
As children with CCHS may under-breathe or stop breathing as soon as they fall asleep, it is important that they receive continuous observation or monitoring so that ventilation may be started at the beginning of sleep. Oxygen monitoring is required as a minimum, although giving additional oxygen to breathe would be inadequate treatment alone. Hospital discharge usually takes place once patients are set up with their home ventilator and monitors and are stable. Parents and any other carers at home need training in how to look after the child's ventilation. The biggest delays to hospital discharge include alterations to housing and employment of carers - these can take many months to achieve.
As children grow, their need for ventilation may change and hence regular assessment is required. The condition appears to be life-long, as no patient with CCHS has been documented to outgrow this disorder. Despite this, many children do well, and may attend a regular school and have a normal lifestyle while awake. They should be wary of swimming under water, as they do not have a normal sense of asphyxia.
How is it diagnosed?
| Inheritance patterns and prenatal diagnosis 
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