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Diseases Related to Vampirism
SUBJECT: Xeroderma Pigmentosum
DATE: July 26, 1991
Vamps,
(The following excerpt is from a posting of mine dated April 23,
1991. Many diseases have been linked -- usually incorrectly -- to
folkloric vampirism, and here is yet another one. Names have been
removed to protect the vampish. *Note: One question answered by the
following was, 'Is xeroderma pigmentosum like albinism?')
[. . .] 'Hemophilia + Xeroderma Pigmentosum = Vampire?' I don't think
so, but it has been suggested (like that other nasty disease, poor-
old-something) ;-} Sometimes I think real vampires are spreading
these rumors about 'various diseases = vampirism' to throw us poor
mortals off the scent.
[. . .] 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.
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.
SUBJECT: Porphyria
DATE:July 27, 1991
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:
*1. So-called 'real' vampires, as related in Eastern European
folklore and elsewhere, were not destroyed by sunlight. (See Dragon's
earlier posting about Stoker's *Dracula*, which shows old Drac only
weakened by sunlight.) Yet if a porphyria victim ventured into the
sunlight, they'd have developed one helluva sunburn. (These days
porphyria is treatable, but not curable.)
According to Anne Rice and others, the sunlight method of vampire
'disposal' was an invention of Hollywood, probably with the first
version (silent) of the movie *Nosferatu*. (*Nosferatu* was creepy as
hell, but a bad rip-off of Stoker's novel. The scriptwriter(s) even
used the same names for the movie's characters: Count Dracula,
Johnathan Harker, Lucy, Van Helsing, etc.) After that, horror writers
and Hollywood kept amplifying upon the sunlight weakness until they
arrived at the modern 'shake & bake' school of vampirism we see today
in vamp movies and books. (You've got to admit, though: for all it's
inaccuracies, it's one *sweet* little weakness. I, for one, am
unwilling to give it up.)
*2. Porphyria victims lack heme in their blood. Very true; but
drinking all the blood in the world would do them no good. Human
beings cannot assimilate heme from blood by drinking it. Our
digestive systems were simply not designed to handle this. Porphyria
victims do not possess this ability, either. So if drinking blood was
indeed the treatment for porphyria in the Middle Ages -- which I doubt
-- it couldn't have worked. (Who would have arrived at this
*brilliant* blood-drinking treatment deduction, and with what tools or
methodology? Could these have been the same people who prescribed
bloodletting for various ailments? Maybe medieval barbers bled one
patient and fed the 'offal' to the next!)
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? ;-). A father or mother or
child somewhere will thank you.
Adrian Darqstar