What is Ondine’s Curse: Causes, Symptoms, Treatment

What is Ondine’s Curse?

Ondine’s Curse, which in medical terms is known as Congenital Central Hypoventilation Syndrome, is an extremely rare form of sleep apnea characterized by a complete and abrupt stop in breathing when the affected individual is asleep. Ondine’s Curse is a congenital condition meaning that a child is born with this condition. The problems with breathing of the child may be noticed while the infant is still in the NICU.

Sleep apnea develops as a result of the basal ganglia in the brain failing to regulate normal breathing. The main reason behind this is the high levels of carbon dioxide and low levels of oxygen in the blood. While awake this may not reach to a serious level but it becomes extremely serious when the infant is asleep.

What is Ondine’s Curse?

Why the Name Ondine’s Curse?

The story behind Central Hypoventilation Syndrome being called Ondine’s Curse, is that a curse was given by a water nymph to her unfaithful husband that he would stop breathing whenever he goes to sleep. This resulted in development of the term Ondine’s Curse referring to whenever a person completely stops breathing when asleep. In medical terminology severe form of sleep apnea is what is termed as Ondine’s Curse.

What Causes Ondine’s Curse?

Ondine’s Curse or Central Hypoventilation Syndrome is a condition, which is quite rare and there are no more than a few hundred cases of Ondine’s Curse around the world. Ondine’s Curse is a congenital condition and genetic mutation is believed to be the main cause behind the development of Ondine’s Curse or Central Hypoventilation Syndrome. The gene responsible for the development of Ondine’s Curse is the PHOX2B gene.

Due to the abnormal gene mutation the brain fails to prompt the body to breathe resulting in the affected individual stopping breathing when asleep causing Ondine’s Curse or Central Hypoventilation Syndrome.

In majority of the cases, Ondine’s Curse is present at the time of the birth of the child, but in some cases it may develop after an injury to the brain where the brainstem gets injured. In some cases dysphagia, neuroblastoma, and intestinal problems are associated with Ondine’s Curse or Central Hypoventilation Syndrome. There have been cases where various members of the same family have had Ondine’s Curse.

What Are The Symptoms Of Ondine’s Curse?

The primary presenting feature of Ondine’s Curse is complete lack of breathing at the time of birth. Shallow breathing in the newborn during sleep is the hallmark of Ondine’s Curse or Central Hypoventilation Syndrome.

In more severe cases or in cases of adult onset disease, shallow breathing will be present at the time when the individual is awake as well. There will be complications of breathing despite the patient having absolutely normal lung and airway functioning.

How Is Ondine’s Curse Treated?

Since most cases of Ondine’s Curse can be seen at the time of the birth of the child, the treatment will begin with the child being put on a ventilator. The ventilator will be connected to a tracheostomy tube whenever the child goes to sleep.

Other than this, there is no other treatment which can definitively treat Ondine’s Curse or Central Hypoventilation Syndrome. Since the treatment required for Ondine’s Curse is quite complicated with all the instruments attached to the child and thus emotional support is required for the families of the child battling with Ondine’s Curse or Central Hypoventilation Syndrome. This not only helps them to smoothly transition from a hospital setting to a home environment, but also helps them psychologically and allows them to cope up with a disease like Ondine’s Curse.

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