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Xeroderma Pigmentosum: Many diseases have been linked -- usually incorrectly -- to folkloric vampirism, and here is yet another one. One question answered by the following was, "Is xeroderma pigmentosum like albinism?"
[. . .] Xeroderma pigmentosum is no crock. It is a real disease. There's a good article about the disease and its effects on one family, especially the two daughters with the affliction, in the May 14, 1990 issue of *People* magazine. A sample: '. . . The Harrison girls are very accustomed to the dark. It has been more than three years since Kim last saw Jaime [age 5] frolic in the daylight -- Sherry [age 3] has never been in direct sun . . . Jaime and then Sherry were diagnosed with xeroderma pigmentosum, or XP, a rare, often fatal genetic disorder that leaves its victims acutely vulnerable to skin and eye cancers if they are even briefly exposed to sun or any ultraviolet rays. . . . 'The critical faculty that Jaime and Sherry lack is an essential health-sustaining system. 'As it does in people, sunlight damages a cell's DNA, it's genetic material,' explains Dr. Kenneth Kraemer, a dermatologist and research scientist at the National Cancer Institute in Bethesda, MD. 'Unlike most people, XP patients lack a system to repair the damage.' The normal repair system consists of enzymes that first excise the damaged DNA in cells, then fill in the gap with undamaged DNA. Xeroderma pigmentosum, a Latin phrase meaning pigmented dry skin, is a calamity that can result when two people who happen to carry the rare, recessive gene have children. When Kim and Jim fell in love and married in 1984, neither had any idea that they both carried a gene that appears in only one in 200,000 people in the U.S.; even then, their chances of having an XP child were no more than one in four. Scientists think that finding the defective enzyme in this tiny group of XP sufferers may ultimately lead to a greater understanding of how all skin cancers occur and what role the environment may play. Consequently, although there are fewer than 1,000 XP victims in the U.S., researchers around the world are hunting for a cure for this obscure genetic disease. 'Until those efforts pay off, Kim and Jim Harrison are relying on their own preventative measures. 'If a kid is allergic to bee bites, you keep him away from bees,' says Jim, 34, and he and Kim, 30, who are emotional and determined people, have changed their entire way of life and go to extraordinary lengths to safeguard their daughters. Kim is by their side day and night, and Jim's only evenings off are spent with Bobby [12-year-old son, half-brother to the girls]. When the girls must go out in daylight to see a doctor, they are slathered with No. 50 sunblock, swathed in turtlenecks, tights and hats, shielded behind ultraviolet-proof glasses and carried to the family van, which has protective film on all its windows. In their large, rambling home, shadows predominate. Curtains are drawn tight over all windows. When an outside door needs to be opened, Kim shoos the girls to another room, and she's always on guard in case one of them gives way to temptation and peeks out under a curtain. Low-watt light bulbs are used sparingly. Otherwise the house is illuminated by candles...' The house description sounds like the homes of a few vampires I've known. <!--pagebreak--> Seriously, this is another one of those diseases, like porphyria (the CEP variety), that have been wrongly suggested as the root cause of vampire folklore. Still, XP and other diseases give those of us who write vampire fiction fertile grounds for our imaginations. As I understand it, XP differs greatly from albinism in that with the latter, undamaged DNA can still be spliced into UV-damaged cells. There are references to XP in medical books, but remember, the disease is very rare, so the information that exists is often sparse, or at least was until very recently. I've found little.
Porphyria Angel sez: Vampires suffer from a form of porphyria, a hereditary disease affecting the blood. The body fails to produce one of the enzymes necessary to make heme, the red pigment in hemeglobin. The symptoms? Extreme sensitivity to light, the lips and gums draw back from the teeth, giving a fanglike appearance, and the only way to treat it (in the Middle Ages) was by drinking large amounts of blood. Also hair may grow on exposed skin, and with the ability to go out only at night (sun exposure causes enough damage to cost the victim his nose) they may have been mistaken for werewolves. Garlic too plays a role. It stimulates heme production (a reason for its inclusion in many herbal blood tonics) and can turn a mild case of porphryia into a severe and painful one. The disease is rare, occuring once in about 200,000 people, and is recessive. Medieval inbreeding may have produced pockets of it in isolated areas. {Stolen nearly verbatim from _More_of_the_Straight_Dope_ by Cecil Adams Berkely press, ISBN 0-345-35145-2. This book contains more information than anyone wants or needs to know on the most bizarre topics imaginable.} Comments? Questions? Hope this helps, Thom. Hmmm. Yeah, I've read this theory in Cecil Adam's book, too. And what Angel says about the vampire-like symptoms is very true. It just happens to be wrong. Adams' book wasn't the first place I've come across this theory. Stephen Kaplan of the Vampire Research Center mentioned it in a newspaper interview some years ago, and the *Psychology Today* article did, too. Local newspapers in various communities recycle the so- called porphyria/vampire connection in seasonal Halloween vampire articles. Also, in her book *American Vampires*, Norine Dresser details the theory and then roundly upbraids its originator, Canadian biochemist David Dolphin. (Some wish Starkist would 'can' him.) Why? Because he's *wrong*, but more importantly, his theory has ostracized and caused the further victimization of those afflicted with porphyria, many of whom are young children. (Remember how cruel young schoolkids can be to their 'peers' who are just a little bit different?) I'm sure Angel didn't intend to further this, and in fact probably didn't know the full story. Why is the theory wrong? For many reasons, but the main two are:
<!--pagebreak--> For a child to be told that their disease makes them a vampire is unfair, and the blood-drinking aspect of it reminds me of what the Nazis used to (and still do) say about Jews. (Please don't misunderstand. I call no one here a Nazi. We all know 'Snotzies' are far worse than VAMPYRES. They simply had no taste whatsoever in clothes. Those tacky uniforms . . . no vamp would be caught *dead* in 'em.) ;-}
I propose a possible answer why to many there seems to be a porphyria/vampire connection. Until the 1960s, one of the supposedly 'sound' medical treatments for porphyria was sunbathing or treatments with sunlamps. This resulted in people afflicted with the disease walking about looking like rotting corpses, sans ears, nose -- ah, it gets grosser, friends. Picture Vincent Price as *The Abominable Doctor Phibes*, *without* the mask, and you're close. (There's a man in the Bay Area like this -- a husband, father, and a sweeter, kinder man you couldn't imagine; you'd expect worse, considering what he's endured. He's been interviewed by local television stations many times, and although the reporters *warn* you about what you're about to see, it never quite prepares you.) Submit it for your approval: one man, one Hollywood scriptwriter, vainly (veinly?) searching for the bizarre, the exciting, and the lurid for his latest vampire epic. Then, a flash of inspiration: 'Ya know, I had an Uncle Joe when I was a youngun'. He had that poori'feral disease thing, and looked just like a vampire, sure enough, the teeth and everything. It hints here in Stoker's book that vampires were weaker in the sunlight . . . hmmm. Uncle Joe couldn't walk around in the sunlight after a while. Hey! Maybe sunlight should *destroy* vampires, just like Uncle Joe! Yeah, that's the ticket.' Well, it could've happened. Truth is stranger than fiction. The point is, now that you know . . . the *rest* of the story . . . please don't continue circulating the myth (vampires have no circulation or heartbeat, anyway -- right? Originally posted to the VAMPYRES list by Adrian Darqstar in July 1991 This work is the copyright of the author. You may not copy, reproduce, distribute, publish, display, perform, modify, create derivative works, transmit, or in any way exploit any such content, nor may you distribute any part of this content, sell or offer it for sale.
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