Tuesday 4 February 2014

ONDINE'S CURSE - Congenital Central Hypoventilation Syndrome (CCHS); Primary Alveolar Hypoventilation (PAH) - a very rare form of sleep apnea




This is a fatal respiratory disorder that causes failure of automatic respiration. People with this disorder are not capable of breathing spontaneously - they must consciously and voluntarily force themselves to breathe. They must do this even while sleeping if not they will die off

 Ondine's curse, medically known as Congenital Central Hypoventilation  Syndrome (CCHS) or Primary Alveolar Hypoventilation (PAH), is a very rare and severe form of sleep apnea

Just as the name implies, Ondine's curse is exhibited typically as a congenital disorder, i.e. children are born with it. It is most likely due to genetic mutation. However, in rare circumstances, it can also result from severe brain stem or spinal cord injury caused by an automobile accident, stroke or as a complication arising from neurosurgery.

Signs and symptoms of Ondine's curse include
  • Respiratory arrests during sleep
  • Hirschsprung disease
  • Dysphalgia (Difficulty in swallowing)
  • Darkening of the skin due to inadequate amounts of oxygen
  • Drowsiness
  • Fatigue
  • Headaches
  • Inability to sleep at night


Treatment of this condition involves the use of a ventilator* connected to a tracheotomy tube* at the front of the throat whenever the affected individual goes to sleep, even during naps. 

*Ventilator: This is a machine that mechanically moves breatheable air into and out of the lungs, to provide the mechanism of breathing for a patient who is physically unable to breathe, or breathing insufficiently.

 *Tracheotomy tube: This is a tube that enables the ventilator to blow air into the windpipe and lungs. One end of the tube is attached to the ventilator while the other end is attached to the windpipe. This is done by surgically cutting the anterior part of the neck through a process known as Tracheotomy



Other potential treatment options include oxygen therapy and medicines for stimulating the respiratory system




ETYMOLOGY/ORIGIN OF THIS DISEASE:


Ondine was a nymph who was breathtakingly beautiful and possessed of a streak of independence, truly what we call a 'free spirit'.

Like all nymphs and mermaids, she was wary of men. If a nymph ever falls in love with a man and bears his child, she will begin to age like a mortal woman, losing her eternal youthfulness and everlasting life. 



Nonetheless, when Ondine saw the handsome young Palemon she was smitten and began to watch for him on his daily walks. When Palemon noticed her, he was taken by her incredible beauty and came back frequently to try to get a glimpse of her again.  

Eventually they talked and fell in love. He then broke his engagement with the young noblewoman Berta and, in time, convinced Ondine to marry him. When they exchanged their wedding oaths, Palemon vowed that "My every waking breath shall be my pledge of love and faithfulness to you."



But it was not to be.
 


The following year Ondine gave birth to their son. From that moment on, her beauty began to fade, her body suddenly susceptible to the effects of age. As her youthful attractiveness gave way to a more mature beauty, Palemon's eye began to wander to the younger women he met at court.



One fateful day Ondine was out walking on their estate when she heard the sound of Palemon's familiar snoring. Planning to take him back home so he could finish his nap, the amused Ondine entered the stables to wake him.
The scene she encountered filled her with great sorrow. Discarded garments littered the floor and her beloved Palemon lay sleeping in the haystack, his arms wrapped around his former fianceé Berta. Having sacrificed her immortality for this man, she was filled with anger and regret.

Kicking her sleeping husband, she woke him and uttered her curse. "You pledged faithfulness to me with your every waking breath and I accepted that pledge. So be it. For as long as you are awake, you shall breathe. But should you ever fall into sleep, that breath will desert you."

Ondine still retained some of her magic . . . enough to make the curse come true. And so it was. Palemon would never sleep again.
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