For Fear of Porphyria
Carpathian Mountain ice chills the bones, but something far more sinister curdles the blood. It is a name that must not be spoken, even when exhumation of the “undead” reveals scalp and facial hair grown longer, fingernails fit for a wolf’s paw, and an unholy glean to the skin.
Premature burial, you say. Oh, yes, it happens. No freak accident, however, or erred pronouncement of death can exculpate the sawbones, tame peasant superstition, or ground flights of fancy.
The Count has risen, and the dawn’s solar flares are no match for another force of nature, one that bides time in the crypts of body cells while bedtime stories, ornate crucifixes, and silver bullets promulgate a myth. He walks among us, and, for those unknowing and hapless victims whose blood has been tainted by Count Porphyria, the quotidian suddenly becomes a race against the disfigurement, skin blistering, swelling, forehead hair growth, and utter havoc wrought by his growing legions.
How could this have happened? If God exists, how could He have given full sway to a creature ingenuously entombed in a permeable, protective vault? Why have Professor Van Helsing and his modern-day counterparts been powerless to halt the scourge?
Perhaps protoplasm and her offspring, ectoplasm and endoplasm, like native soil and a coffin’s nurturing bosom, shield the beast in some way. We can only surmise that the story must go something like this. Be forewarned, reader. It is not pretty!
Back in 1985, when cool wits and steady nerves did not prevail, a biochemist and fearless vampire hunter, named David Dolphin, theorized a connection between the physical appearance of patients suffering from porphyria and the traits of folkloric creatures of the night. He postulated that porphyria, a group of rare, largely hereditary blood diseases, may have afflicted the so-called vampires of yesteryear, in other words, ordinary people whose resulting facial scarring, mutilated noses and fingers, receding lips and gums, and subsequent elongated canines could be directly attributed to the malady. While holy water and a bell’s toll did little to halt the death march of these hapless hordes, an extraordinary sensitivity to sunlight became their constant adversary, forcing them underground and restricting activity to nightly jaunts.
If that were not enough, other characteristics inherent to porphyria provided further food for thought, or perhaps for fertile imaginations. For example, garlic was found to worsen the symptoms of porphyria, and, while injections of blood products treat the illness today, centuries ago its victims may have sought relief drinking blood.
A link to stress has also been expounded. We know that porphyria is inherited, but perhaps a stress of some sort may have been required to unleash its symptomatology. Just imagine, in ages gone by, a stricken sibling or other family member biting you to quench a thirst for blood. Suddenly, that stress would jumpstart your own latent porphyria, and, lo and behold, you would grow your own set of matching fangs.
Nonsense, you say! Let’s debunk the myth and shed light on the argument, no pun intended. Porphyria comprises a series of seven disorders, only the rarest of which, called congenital erythropoietic porphyria, causes skin blistering, itching, swelling, and disfigurement. Just 200 cases have been documented, far too few to account for the widespread belief in vampires. Enter the imaginative element of human nature. Furthermore, porphyria victims do not crave blood, and even if they were to drink blood, digestion in the stomach would render it useless. And garlic, an ally in the fight against the unholy host? That has never been proven.
So, what are we left with, scientifically speaking and keeping medical jargon to a minimum? In congenital or acquired porphyria, body cells lack the basic enzymes required to process porphyrins, which are components of heme, in turn a component of blood hemoglobin. Because they are neither degraded nor biosynthetically processed, porphyrins accumulate in the body and are toxic to tissue in high concentrations. Throw alcohol, antibiotics, certain foods, sunlight, and fasting into the recipe, and you have concocted a surefire way to trigger an attack and befriend the night.
It does not end there, however. Count Porphyria’s little brother, Prince Catalepsy, may have a hand in the matter. Catalepsy affects the central nervous system of epilepsy patients, freezing muscles, slowing heart rate and respiration, and giving new meaning to the term “living dead,” for those of us who inadvertently stumble upon a cataleptic “corpse.”
We must stake a conclusion here. When the horrors of everyday living take their toll, when we seek scapegoats to cover our own evil tendencies, and when choosing between right and wrong is tempered by shades of gray and the color of money, we must find the courage of those prematurely buried. A return to consciousness, a brush of dirt from our clothes, and a return home demonstrate, to the surprise of our adversaries, that we are not down for the count. Unless, of course, long hair and fingernails, reddish mouths and teeth, receding skin, expanding and engorged abdomens, and the pungent smell of decomposition indicate broken springs in our biological time clocks. At that point we can expect a knock on the door from our parish priests or a new generation of Van Helsings.
© 2010, Albert M. Balesh, M.D. All rights reserved.
posted by Dr. Al at
8/15/2010 08:34:00 AM
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