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Five Quarts_ A Personal and Natural History of Blood - Bill Hayes [77]

By Root 1040 0
his fictional world, he rightly believed, the more genuine the reader’s fright. Hence his characters used such newly invented devices as a recording phonograph (an early-model tape recorder), a portable typewriter, and a Kodak camera. Likewise, a trip to Transylvania taken by one of the book’s heroes followed actual train timetables. Elsewhere, landmarks and locations were drawn from real life, as were certain events. A ship that had beached near where a vacationing Stoker wrote portions of Dracula, for example, found its way into the plot. When he was uncertain of details, he turned to experts—to his older brother, Thornley, for instance. A prominent surgeon in Ireland, Thornley Stoker vetted the final manuscript before Bram sent it to the typesetter, double-checking the blood transfusion scenes, in particular, to make sure of their accuracy.

Whereas Stoker wrapped his myth in truths, modern-day scientists have worked to expose the truths behind the myth, posing, for instance, the fascinating question, Might there have been a medical basis for the allegorical disease of vampirism? The answer is a resounding yes. It’s a blood disorder called porphyria.

In its mildest symptomatic form, porphyria is not at all vampiric—at worst, an extra sensitivity to sunlight may cause your skin to blister. But in rare cases an untreated victim may indeed look like one of the undead: Your coloring takes on a deathly pallor due to severe anemia; your lips erode and gums recede, making your teeth—the eyeteeth, in particular—appear longer, more fang-like; and sunlight turns your affected flesh caustic, causing your facial features to dissolve and fingers to be eaten away. The lesson you learn quickly is, daylight is deadly. That undiagnosed cases of porphyria may’ve first planted suspicions of vampirism hundreds of years ago, perhaps as far back as the twelfth century, was originally suggested by a Canadian biochemist in 1985. Little could Dr. David Dolphin have imagined as he stepped up to the conference podium that day in May the media monster he’d unleash. What assured he’d grab headlines was his matter-of-fact contention that victims may have been driven to drink blood to relieve their symptoms. After an initial fireball of attention, Dr. Dolphin’s hypothesis has since taken on a life of its own, especially on the Web, not unlike the legend of Elizabeth Bathory.

Porphyria is triggered by a flaw in the cellular machinery for producing heme, a crucial element of the blood’s oxygen transporter, hemoglobin. One of the steps in assembling heme involves the introduction of dark red pigments called porphyrins (from the Greek for “purple”). When the system is flawed, you end up with too much porphyrin and not enough heme. The porphyrin pigments backlog, building up in the skin, teeth, bones, and organs, causing a host of symptoms depending on where the accumulations occur. Your teeth may turn a dirty brown, for instance, and pain may settle into your limbs and back. (That sufferers can be extremely sensitive to sunlight made more sense once I learned that porphyrins are an ancestral sibling to chlorophyll, though, of course, the light-activated process of photosynthesis in plants isn’t destructive.) While toxins such as drugs, alcohol, or chemical poisoning can bring on porphyria, the illness is mainly hereditary in origin.

It’s now known that the infamous British king who reigned during America’s war for independence, George III (1738–1820), suffered from acute intermittent porphyria (AIP), one of eight distinct forms of the disease. As is typical of AIP, the king’s illness manifested most notably in neurological symptoms: seizures, hallucinations, and bouts of mania and paranoia that would last for days or weeks at a time, then vanish, with long remissions in between. That his malady was porphyria, not “madness,” as was believed during and long after his reign, would be unknown today were it not for a peculiar fact of royal life: As the monarch, George was subject to daily visits by physicians, who chronicled his every symptom. From these

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