Ondine's curse, also known as congenital central hypoventilation syndrome (CCHS), is an unusual, extreme type of sleeping disorder in which a person stops breathing entirely while sleeping.
One of the most enchanting legendary tales in the world of medicine is Ondine’s curse. Ondine was an immortal water spirit who became human after falling in love, marrying, and having a child with a man. When she found her husband sleeping with another woman, she cursed him to stay alive in order to control his own breathing, according to one tale. He was unable to breathe while sleeping. For the remainder of his life, her husband was never allowed to sleep peacefully for fear of dying. The unusual condition caused by lack of circulatory breath control while voluntary respiration remains intact was called “Ondine’s curse” during the 19th century. Ondine’s curse is now most often concerned with congenital central hypoventilation syndrome.
Causes and Symptoms
Ondine’s curse affects only one in every 30 million individuals, implying that only a few hundred people worldwide are affected. As a result, it is considered a very rare condition. The root cause tends to be a genetic defect. It’s believed to be when the brain doesn’t prompt breathing, which can also happen with insomnia.
Ondine’s curse is present from birth, it can cause trouble swallowing, digestive complications known as Hirschsprung’s disease, or tumours known as neuroblastoma. Shallow breathing while resting, cyanosis in the fingers or toes, epilepsy, cardiac disturbances, and neurological problems are all signs of insufficient oxygen during sleep, which can be caused by both congenital and acquired forms. The congenital condition is nearly invariably present when a child is born, while the non-congenital form appears later in life (for example, after spinal cord surgery or with brainstem tumours or strokes).
While cases have been diagnosed in the womb, the majority of those affected begin to show symptoms shortly after birth. When anaesthesia or sedatives are used, symptoms can occur in milder situations.
People with CCHS breathe shallowly, which results in a lack of oxygen and a buildup of carbon dioxide in the blood.
Acid reflux and reduced upper gastrointestinal secretion are other symptoms, which include nausea, discomfort, dysphagia (swallowing difficulty), and vomiting.
Despite advancements in diagnosis, the key elements of Ondine’s curse treatment remain respiratory assistance, including mechanical ventilation and, in some cases, intubation. Life expectancy is expected to be reduced depending on the seriousness of the disease, while positive interventions are extending longevity even for those who are most severely affected.
Although most people die as a result of Ondine’s curse, one English teen beaten the odds. Liam Derbyshire was only expected to survive six weeks after being diagnosed with Ondine’s curse as a child. Derbyshire, who was born in 1999, is now alive thanks to 24-hour treatment and a ventilator at home.