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Ondine's curse: A fascinoma to medical world
Ondine (from Greek and Roman to German) was a mythical water nymph or spirit whose curse, when applied, rendered the cursed unable to breathe while sleeping. Thus, the afflicted lived without sleep. A real nightmare.
Breathing (respiration), which we all take for granted, is both complex and, obviously, fundamental. Basically, there are two control mechanisms, one voluntary, such as sprinters deliberately hyperventilating before a race start, and the other automatic. The former resides in our brains' cortex, the gray matter that does (or should do) our thinking. It is the most complex and the most recently evolved part of our brains. The automatic control of respiration resides in the brainstem, older and below the brain but above the spinal cord.
The stimulus for breathing is more the result of carbon dioxide, or CO2, buildup in the bloodstream than lack of oxygen. Chronic obstructive pulmonary disease, or COPD, is an all-too-common exception.
In COPD, characterized by chronically elevated CO2 , the regulatory mechanism responds to oxygen depletion, not CO2. Long ago, ambulance personnel, nurses and physicians learned to go light in providing oxygen. High flows can put patients with COPD to sleep, and when they wake up, if they wake up, they are not pleased. They have nasty "CO2 headaches."
Is sleep apnea related? Is sudden infant death related? These are questions for the researchers. There is a condition known as primary alveolar hypoventilation that is genetic and fatal, but, fortunately, very rare. In this instance, automatic regulation of breathing (my daughter suggested "cruise-control for respiration") is defective and the affected die. Tumors and strokes to the brainstem also can take out the cruise control.
One question emergency-room people hate begins with "Remember the patient you saw ... ?" This usually is because we get too little follow-up on the people we see and we hate surprises.
Many years ago, I received such an inquiry from a huge metropolitan medical center. The ambulance had brought in a young man after a near-drowning - blue, unresponsive, with absent respiration. We resuscitated and stabilized him and later sent him to the big city. Months later, he had normal intelligence and was alert. He had neurological deficits in face and throat and periods of apnea (nonbreathing) while sleeping. His cruise control did not respond to elevated CO2; he had Ondine's curse. He later died.
In this unique case (with normal CT brain scan), his cortex survived and his doctors were able to identify the specific clusters of brainstem nerve cells that were knocked out, adding to the foundation of medical science. The initial link in this fatal chain of events was stones thrown by alcohol-impaired friends. One stone struck his temple, causing him to collapse face-first into shallow water. His friends assumed he was joking before they pulled him out - precious minutes and a young life wasted.
www.alanfraserhouston.com Dr. Fraser Houston is a retired emergency-room physician who worked at area hospitals after moving to Southwest Colorado from New Hampshire in 1990.
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