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Congenital Central Hypoventilation Syndrome (CCHS): When sleep becomes a death sentence

http://blacksuperherofan.com/wp-trackback.php Some hours after his birth, little James appeared to have bluish skin, and the doctors observed that his eyes didn’t look quite right. Moments later, his breathing became shallow and inconsistent.

Kavadarci The doctors had to admit him to the neonatal intensive care unit for close monitoring and further tests.

can i order Pregabalin online Later, these procedures confirmed that little James had a rare disorder called Congenital Central Hypoventilation Syndrome (CCHS).

Phatthaya What this syndrome means

Congenital Central Hypoventilation Syndrome is a rare disorder of the organ of respiration, the lung, which begins at childhood, and can lead to death if not detected and treated early.

Breathing is an involuntary activity that is coordinated by the brain and nerves. That’s why we still breathe when we are asleep. However, in CCHS, the brain and nerves don’t seem to control this activity as they should.  Consequently, the child’s breathing is sluggish and uncoordinated, and could even slow to a stop during sleep, causing death.

Signs and symptoms

Congenital Central Hypoventilation Syndrome could be mild, moderate, or severe. People with mild cases may not show any symptoms at all, and may not even know they have a problem with their lungs. In moderate and severe CCHS, people may experience respiratory difficulties that could lead to an abrupt cessation of breathing.

Common symptoms of CCHS include:

  1. Shallow and reduced breathing.
  2. Bluish appearance or darkening of the skin color as a result of poor oxygenation of blood in the lungs.
  3. Sleep difficulties
  4. Tiredness and drowsiness.
  5. Headaches.
  6. Abnormal pupil, the dark-colored opening at the center of the eye
  7. Feeding difficulties
  8. Occasional episodes of heavy sweating.
  9. Respiratory arrest, that is, cessation of breathing during sleep.

Cause of Congenital Central Hypoventilation Syndrome

Congenital Central Hypoventilation Syndrome is caused by a mutation in the genes that control the development and maturation of nerves. Once the nerves are dysfunctional, they will not coordinate breathing as they should, which would result in breathing problems.

Treatment

There is currently no cure or medicines for congenital central hypoventilation syndrome, but doctors can treat it by placing patients on mechanical ventilation, which provides an artificial source of breathing, for the patient – 24 hours a day. The disadvantage of this is that patients are susceptible to deadly chest infections, such as pneumonia. Patients with mild symptoms may only need help with breathing at night and may not need artificial respiration all day long.

History of Congenital Central Hypoventilation Syndrome

There’s a German tale to support this medical abnormality. In the tale; a nymph called Ondine places a conditional curse on her mortal husband, that if he is ever unfaithful, he shall die.

Shortly after their wedding, her husband gets reunited with his first love, and this sets off the curse. He couldn’t sleep for fear of dying and was made to always think before breathing.