Five Quarts: A Personal and Natural History of Blood (19 page)

BRAM STOKER TOOK GREAT CARE TO CLOAK HIS VAMPIRE TALE IN THE guise of realism. The more authentic and contemporary 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 surviving documents, modern-day British researchers have gleaned conclusive evidence for a posthumous diagnosis of AIP. Aside from jottings regarding the characteristic mental paroxysms, which began when George was in his twenties, the clincher was this notation: “His Majesty has passed . . . bloody water,” by which was meant discolored urine, elsewhere described as “bluish,” dark and “bilious,” and having left “a pale blue ring” around the specimen flask. All are telltale signs of excessive porphyrin production, as are severe abdominal pain and muscle weakness. The medical records also show how royal protocol must have frustrated the doctors, who could never speak unless first spoken to. When King George was at the peak of his delirium, entire visits passed in silence, as on one day in January 1812: “His Majesty appears to be very quiet this morning, but not having been addressed we know nothing more of His Majesty’s condition of mind or body than what is obvious in his external appearances.” Perhaps this explains the preoccupation with the kingly pee.

Having exonerated George III of madness, the British researchers then posed the next logical question: Since AIP is always hereditary, who else in his bloodline carried the disease? By combing through historical accounts and medical records—a search abetted, once again, by the fastidious description of urine samples—they were able to trace the disorder through thirteen generations, spanning more than four hundred years. Among his ancestors, fifteen were identified as sufferers and/or carriers, beginning with his father and going back to Mary, Queen of Scots (1542–1587), where the porphyria paper trail ended. As with George, retrospectively diagnosing Mary with the illness allows for a radically revised view of her reign. Mary, whom the researchers called “one of the great invalids of history,” was so sick so often that her opponents accused her of using hypochondria, to borrow the modern term, as a political ploy. Conversely, her enemies were accused on one occasion of poisoning her, an episode now ascribed not to foul play but to the fickleness of genetics.

Such a rare disease as porphyria would never have manifested in so many family members were it not for the strict controlling of the bloodline through intermarriages, a phenomenon not unique to royal dynasties. A similar kind of inbreeding occurred within isolated and remote communities during the Dark Ages, for example, and, in these shallow gene pools, recessive traits could flourish. Hence, as biochemist and medical writer Nick Lane postulates, a type of porphyria that is among the rarest today—congenital erythropoietic porphyria (CEP), the disfiguring, vampiric form of the disease described earlier—may’ve once been relatively common in spots of Eastern Europe now recognized as the cradle of vampire myths, the valleys of Transylvania. Assuming this to have been the case, it’s easy to imagine how the corpse-like appearance and odd behavior of sufferers may have given rise to whispers of vampirism; how, within these enclaves, certain folk remedies would’ve been embraced; and how, over time, the rumors and remedies would have gradually evolved into legend. Garlic is a good example. It’s now well known that certain chemicals in garlic can exacerbate porphyria symptoms, a lesson that Transylvanian sufferers may have had to learn through painful experience. Little agility is needed to make the next leap, to imagine how a sufferer’s way of averting a flareup could mutate into a superstition among the healthy for preventing the disease and then into a means of warding off a vampire attack. Likewise, the genuine need to avoid the sun could have transmuted into the dramatic literary convention of the bright light of day turning a vampire to toast.

In view of all this, the hypothesis put forth by David Dolphin doesn’t sound so far-fetched: that, hundreds of years ago, victims of this most heinous form of porphyria may have self-medicated by drinking human blood. In a sense, this is evocative of earlier thinking, such as the belief in ancient Rome that a swallow of gladiator blood could cure epilepsy. Provocative, yes, but scientific, no. In Dr. Dolphin’s theory, however, science concedes. The severe anemia caused by CEP leaves sufferers with dangerously low levels of heme in their circulatory systems. In medical terms, a heme deficiency is an iron deficiency, which is why the modern treatment for this rare form of porphyria is regular transfusions of blood. Though not recommended, a patient could instead be given a straw. The heme molecule is robust enough to survive digestion and will make its way into the bloodstream.

When I consulted a nutritionist on this final notion, I was so preoccupied by the repulsive thought of ingesting blood that I was startled when she stated the obvious: “We eat blood all the time. It’s in our meat, in all the animals we kill for food.” Mary Kay Grossman is a registered dietician and coauthor of the bestseller
The Insulin-Resistance Diet.
“In our culture,” she continued, with the exception of kosher diets, “we don’t drain the blood off. If you cook it, it’s not unhealthy to eat blood, and it doesn’t lose its nutritional value.” In fact, Grossman explained, some cultures, such as the Masai of Kenya and Tanzania, subsist entirely on blood and milk—cow’s blood, that is. “They milk the cows, then puncture the throat, and drain the blood off.” (The cows survive, by the way.) The Masai then mix the two and drink it fresh, she added, or give it a few days to ferment. “They live in an extremely dry climate where it’s almost impossible to grow anything, so the blood supplies iron and the milk is a major source of protein.”

Raw animal blood is a central part of the diet of other pastoral groups in eastern Africa, I later learned, including the Karimojong of Uganda, but, globally, cooked animal blood as a main ingredient in traditional dishes is far more common. The Inuits with their seal’s blood soup, for example. The Tibetans with yak’s blood cubes, a snack of reduced yak’s blood served with sugar and hot butter. And the English with their black pudding, a baked then fried concoction of pig’s blood, bread cubes, skim milk, beef suet, barley, oatmeal, and mint. You can even taste your way through every region of France through local interpretations of
boudin noir,
“blood sausage.”
Larousse Gastronomique,
the classic French encyclopedia of Continental cuisine, describes sixteen variations, following the basic recipe of equal parts onions, pork fat, and pork blood.

King George III was likely never fed such savory dishes when he was ill. Rather, according to historians, he was often straitjacketed, tied to a bed or chair, and subjected to a varying regimen of vomits, purges, blisterings (the placing of hot coals on the skin in order to draw “bad humors” to the surface), cuppings, bloodlettings, and leechings. Heaven help the king. Perhaps some degree of solace can be found, however, in the fact that the last three forms of treatment would have, in theory at least, helped the ruler. While anemia can be relieved through adding blood, removing blood will quickly reduce the level of porphyrins in circulation. In fact, some types of porphyria are currently treated through phlebotomy, the modern-day counterpart to bloodletting, as is the more common hereditary blood disease, hemochromatosis, in which a dangerous excess of iron in the blood must be decreased through regular blood draws. (A curious side note: Once this heme-heavy blood is collected, it’s routinely destroyed, even though—assuming the donor with hemochromatosis is otherwise disease-free—its iron-richness is exactly what many ER patients need.)

Four of King George’s sons are believed to have had porphyria, including the heir apparent, George IV, whose wife (a first cousin) and daughter, Charlotte, were also afflicted. It’s quite possible the illness later led to Princess Charlotte’s death during childbirth at age twenty-one (her son was stillborn), a tragedy that precipitated a regency crisis: The king, who by this point was blind, enfeebled, and nearly eighty, now had no legitimate heir beyond George IV. Moving swiftly, he arranged marriages for his three eldest sons, and each produced a child in the year 1819, one of whom, Victoria, would be crowned queen of England at age eighteen. Although Victoria, who also married a first cousin, Albert, was spared porphyria, she introduced another devastating blood disease into the British royal family, one that would eventually taint the ruling houses of Spain, Germany, and Russia: hemophilia.

T
EN

Shemophilia

BLOOD NATURALLY SEPARATES. REMOVED FROM THE constant “stirring” of the circulatory system and collected in, say, a test tube, blood settles into our tricolor hematological flag, amber, white, and red. The band at the top is plasma, the liquid in which the cells of the blood are normally suspended. Next is the narrowest stripe and the palest, a blend of white cells and platelets. And beneath these, the bog of burgundy-colored red cells, the heaviest of which, the deep red, almost black corpuscles laden with waste, have sunk like sediment at the bottom of a pond. Curiously, what some would call the defining quality of blood—its redness—does not in fact contain the defining quality of humanness, DNA; red cells are “dumb” cells, devoid of a cellular “brain,” a nucleus.

The color blue is not part of the mix, although the long-lived phrase
blue blood
deserves a little deconstruction. Its etymology begins with
sangre azul,
a term that arose out of a medieval case of xenophobia. During the Moorish occupation of Spain, members of the oldest and staunchly Christian families of the Castile region claimed they were superior for never having intermarried with the darker-skinned Muslim invaders of their country. The proof of their blood’s purity was as near as a forearm. A Castilian need only point to the tributaries of blue visible through his or her white skin. What they called
sangre azul
we’d now call an optical effect, deep purple blood seen through pale purplish veins seen through an epidermal scrim.

By the time
blue blood
crossed into the English language in the 1830s, concurrent with the beginning of Queen Victoria’s reign, it had shed the racial connotation and become synonymous with society’s upper crust. Within this rarefied meringue of British blue-bloodedness were further distinctions: the gentry class, the aristocracy, and, at the top, the royal family. Royalness, too, had its own degrees. To be of “morganatic blood,” for example, meant that one of your parents was of pure royal extraction—that is, “of the blood,” the bluest of the blue—and the other was not. Perhaps your mother and father had married not as a dynastic stratagem but out of love—what folly! Entering into a morganatic marriage came with a price: the forfeiture of your children’s right to succession. Compared to other sovereigns of her era, Queen Victoria was far more accepting of such unions. A sterling example of this magnanimity came in the spring of 1866. When informed that an obscure Austrian prince wished to marry one of her cousins, Victoria dismissed the many objections regarding the gentleman’s unequal birth and gave her full blessing. What’s more, upon first laying eyes on this tall, strapping man, the queen saw not the penniless military officer but a solution to a problem, one the prince’s physical presence brought to the fore. What the queen would never admit publicly was her deep concern about the quality of her family’s blood, made, as she described it, “so lymphatic,” generation after generation, by all “that constant fair hair and blue eyes.” Prince Teck was all things but with jet-black hair and dark good looks. Shortly after meeting him, the queen wrote to her eldest daughter, Vicky, a lifelong confidante, who was grown with fair-haired children of her own and ensconced as the crown princess of Prussia. Oh, Victoria lamented, “I do
wish
one could find some more black eyed Princes or Princesses for
our
Children!”

It’s hard to imagine Vicky’s reaction as she read the rest of her mother’s words, which, in just a handful of sentences, move from despair to envy to a rising franticness (note how even the underscorings escalate): “I can’t help thinking what dear Papa [the queen’s deceased husband] said—that it was in fact a blessing when there was some little
imperfection
in the
pure Royal
descent and that some fresh blood was infused.” Here the queen paused for a moment, asking her daughter to excuse this “somewhat odd letter,” before diving back in: “It is
not
as
trivial
as you may think, for darling Papa—
often
with vehemence said: ‘We
must have some strong dark blood
.’ ”

This letter reads as if a mother’s intuition had sharpened in grandmotherhood, but at the time Victoria would’ve had no idea of the troubles to come. The hemophilia that would eventually touch sixteen family members had, in 1866, manifested solely in Victoria’s youngest son, thirteen-year-old Leopold. A strange aspect of the disease is that while females carry the defective gene that prevents proper clotting, in general only males develop the disorder. In other words, it stays hidden in a woman until it shows up in a son. By scanning Queen Victoria’s family tree, medical historians have established that two of Leopold’s five sisters, Alice and Beatrice, were carriers. There’s also no doubt that his mother introduced hemophilia into the royal bloodline.

How Victoria got it is something of a puzzler. Tracing back her ancestry, no red branchings of the disease appear, which leads to three possibilities. The traditionally held view is that it was caused by a spontaneous mutation (this occurs in about 30 percent of hemophilia cases). Second, that, against the odds, Victoria’s mother, maternal grandmother, maternal great-grandmother, and so on were carriers whose sons never suffered the disorder. Or, third, the most sensational possibility, that Victoria was illegitimate. Genetics has fueled this particular speculation. Given that every daughter of a male hemophiliac (and a normal female) is a carrier, perhaps then, as a pair of British scientists postulated in the mid-1990s, Edward, Duke of Kent, was not her biological father. (As a
Newsweek
headline blared, “Was Queen Victoria a bastard?”) Well, maybe, maybe, and maybe, although I’m leaning toward (1), spontaneous mutation.

To her dying day the queen refused to believe that the disease came from her side of the family. It is also considered unlikely that she was ever fully briefed on the cause and patterns of the disorder, even though a fairly sound clinical description had been established at the dawn of the nineteenth century. In Leopold’s early childhood, no obvious flags went up. He was her most “delicate” son, Victoria admitted, born tiny, and less graceful than his three older brothers. She blamed his frequent bruising on clumsiness and, in her ignorance of Leo’s true condition, was ofttimes impatient and critical. Sickly, pallid, and frail, the boy was an embarrassment. At the time of his fifth birthday, though, a family walk, a skinned knee, and a cut that would not stop bleeding forced Victoria to face the reality that her son was a “bleeder.” With that, the queen made an emotional turnaround, and a well-intended overprotectiveness set in. She drafted an all-staff bulletin of sorts regarding her son. Forthwith and henceforth, all active play with other boys would be denied the lad, and “all the
essentially
English
notions
of
’manliness’
must be put out of the question.” His tutor must never leave Leo unattended, and a long list of activities became restricted. But of course, tell a child “don’t” and he’ll naturally be tempted to “do.” When Leopold was eight, to cite one example, he somehow managed to ram a steel pen through the roof of his mouth.

Leopold, age nine, with his mother, Queen Victoria, April 1862

Stitches didn’t work well for a hemophiliac because, obviously, they just introduced more holes. The alternative was cauterizing, a method that essentially melted closed a wound, using either a caustic substance or a red-hot brand. I can only hope that Leopold was well anesthetized when this treatment was inflicted. When Victoria described this incident in a letter to her daughter Alice, her words seemed to stumble, as if numbed: “The fear was—the bleeding could not be stopped and then—you know he could not have lived.”

That Leopold survived into his early thirties is medically remarkable. Most bleeders in his day never made it into adolescence. In fact, individuals with severe hemophilia faced a similar mortality rate well into the 1960s. To answer definitively why Prince Leopold lived as long as he did, however, would require something that simply no longer exists: a sample of his blood. With it, a modern hematologist would be able to find exactly what was missing. Without it, a good guess is still possible. But first, some basics are in order.

The simplest way to describe coagulation is to say it’s a complex process in which blood turns from a liquid to a solid. As many as twenty different blood proteins take part in this coordinated effort—what one scientist with a touch of the poet named “the clotting cascade.” Thirteen of these blood proteins are called factors, and a deficiency of any one results in a different clotting or bleeding disorder. To picture the prevalence in the general population, imagine a twenty-thousand-seat sports stadium filled to capacity and split evenly between the sexes. Four hundred attendees, men and women alike, would have the most common bleeding disorder, von Willebrand’s disease, in which a deficiency of the von Willebrand factor keeps platelets from clumping properly at the site of an injury. Just two attendees, men only this time, would have hemophilia A, or classic hemophilia, which is caused by a lack of factor VIII. To find a single gentleman with hemophilia B, two additional stadiums are needed (one in thirty thousand men have this deficiency in factor IX). Now on to a bigger challenge. To find a woman with classic hemophilia, one should probably scrap the whole stadium idea and consider instead the entire population of the United States. One in one hundred million citizens is this rarest of the rare, a female hemophiliac. Its infrequency in women is simply a matter of genetics, the female XX versus the male XY. The recessive gene for hemophilia rides the X chromosome. In a female, if one X is defective, the other can normally compensate. It would take the pairing of two defective X’s for the condition to develop.

In males, the first sign of a serious bleeding disorder often comes when a baby boy is circumcised, a danger that has been recognized since ancient times. In the Babylonian Talmud, the collection of Jewish rabbinical laws written between the third and sixth centuries, it was declared that a newborn son would be excused from circumcision if two brothers had previously died from the procedure. From a modern standpoint, this law calls to mind a common misconception that should be stanched forthwith: that a hemophiliac with, say, a minor wound will never stop bleeding. The lack of factor VIII or IX doesn’t mean your blood won’t ever coagulate; the woodwinds may be absent, so to speak, but the orchestra still plays. Other components of the clotting cascade continue to do their job. The problem is, you clot more slowly and hence bleed longer. How long depends largely upon how much clotting factor is present in your blood. Analyses of blood can calculate the speed of your clotting as well as pinpoint which factor is deficient and in what quantity. A person with mild hemophilia, for instance, will have, at best, only half the clotting activity of a healthy person. By contrast, in severe hemophilia, the amount is less than one one-hundredth of normal. Once these calculations are determined, treatment is fairly straightforward, at least in theory. You simply inject or infuse the missing substance. And of course the earlier in a person’s life that these levels are assessed, the better.

Regarding Leopold, it is possible to piece together details of his condition through his words, for the young man’s personal correspondence lays bare his suffering. A well-understood medical consequence of severe hemophilia is spontaneous internal bleeding into the joints and muscles, which balloon with blood, becoming excruciating and crippling. Leopold clearly had this. In a letter to his sister Louise begun June 6, 1870, he barely got past the “Dearest Loo” before having to stop, so fierce was the pain. He couldn’t continue until four days later: “. . . At this moment I am in agonies of pain; my knees get worse daily and I get more desperate.” Despite the limited relief offered by the treatments of the day—bed rest, ice packs, and, only as a last resort, morphine—Leopold, seventeen at the time, leavened his note with a bit of gallows humor. “If this continues long I shall soon be driven to Bedlam [by which he meant the loony bin], where I shall be fortunately able to terminate a wretched existence by knocking out my brains (if I have any) on the walls; that is the brightest vision that I can picture to myself as a future. . . .”

Signed, “Your wretched brother, Leopold.”

If humor helped him rise above the pain, an intense pursuit of academics also provided an escape. By this point in his life, the Scholar Prince, as he would be known, had become well versed in Shakespeare and fluent in several languages. Over his mother’s objections, Leo attended Oxford University, the first in the family to do so. His gumption is all the more remarkable when you factor in that he also had epilepsy. After graduation he became one of the queen’s most trusted political advisers, gaining the title Duke of Albany. At the same time, he wanted to establish a life for himself apart from his mother and longed to marry. In 1882, at age twenty-nine, Leopold made both himself and the queen happy by wedding Princess Helena of Waldeck, sister of the Dutch queen. The happiness would last just two years. Shortly before the birth of his second child, Leopold took a spill—what for a healthy person would’ve merely meant a bump on the head. He died a few hours later from a brain hemorrhage. Upon receiving the news of her son’s loss to hemophilia, Queen Victoria, now sixty-five, set down in her journal three devastated words, “Am utterly crushed.”