Vampires and porphyria

Cecil's Columns/Staff Reports
1

http://www.straightdope.com/classics/a990507.html

This is in response to your assertion that the myth of vampirism has nothing to do with porphyria; frankly, I don’t buy it. To address your points:

1: you say that 200 diagnosed cases is too few for the spreading of a belief in vampires – but picture the social environment of feudal Transylvania. With a relatively small gene pool of nobles, a low population density and poor transport connections, there would often be cases of inbreeding. These are exactly the conditions that lead to noble families in which very rare genetic diseases become widespread; look at hemophilia in the Battenbergs, or the Habsburg abnormalities. Families would quickly realise that many of their children were being born with these problems, and learn to keep them out of sunlight and to do whatever else had shown good effects on others similarly afflicted. In effect, the afflicted families would quickly beome experts in treating the particular type of prophyria from which they suffered.

2: This is unconvincing: we have a “standard” vampire legend that agrees well with observed symptoms, but such myths are passed on by hearsay. People will embellish and change the stories, particularly if they don’t think they have any basis in fact. I’ll stick to the most common vampire myth, and I see good parallels with porphyria.

3: True, the heme that they need will be broken down by the digestive system, but what about the raw iron? In the modern world, porphyria sufferers can take simple chemical iron tablets to combat their anemia – but medieval transylvanians would have needed to get the iron in one form or another. Bear in mind that they would have seen this as some sort of curse, or in another way imbued it with religious connotations. Making the link between porphyria and blood is not impossible, especially as these people would have so many cases of the disease to go on.

As for the other points, I don’t think there’s any need to postulate further. How does this sound for a hypothesis:
In the inbred environment of the Romanian mountain nobility, the gene for a particular porphyria became common. The rise of dual recessives is the classic inbreeding depression. The noble houses found that they needed to keep their children away from the sun to prevent them from suffering terrible burns, but eventually realised that blood could alleviate their suffering. In a medical system dominated by the theory of the four humours, it would be one of the first things they would try. They also hushed it up, not telling their subjects.
The people in the villages became suspicious. Powerful forces were at work in the castle. There were counts whose faces were never seen, who were mad, long fanged, pale skinned; who craved blood, and would come to them looking for it (for indeed, wouldn’t the serfs who belonged to a lord be the logical source for the lifeblood he needed?). Nobles would generally be physically more powerful than their undernourished subjects, and any who took in large doses of protein rich blood would have the capacity to grow yet stronger, thus at least partially accounting for vampires’ extra powers. What is more, a man who lived his life by night and was accustomed to stealth, perhaps with an ability to climb rocks and trees (strength and need to do so) might be seen to have the ability to disappear. Leave the rest to a healthy dash of class struggle, suspicion of the unknown, and fearmongering.

This is wild conjecture, but it is completely plausible. A medical condition has been linked to a myth, both in cause (inbreeding) and symptoms (light sensitivity and iron appetite). Perhaps it isn’t true, but it seems more likely than people happening to make something up that was so similar to a possible reality. If it is, one must pity the real Counts Dracula of the past, unwell, hated by all, and locked away for their lives – possibly even believing that they were accursed of God.

I’m not for a moment suggesting that people with porphyria are vampires. I’m sure it’s a very nasty condition, and it’s always unpleasant to be taunted for something that is not one’s fault. However, the blame lies with those who abuse sufferers, not with those who point out a fascinating medical possibility for the rise of one of our culture’s strongest folk rumours. I don’t believe that political correctness should have any place in the evaluation of scientific theories – and I find this theory, in a modified form, fascinating and believable.

Rupert Griffiths
Trinity College
Cambridge
CB2 1TQ
UNITED KINGDOM
email/msn: swyves@hotmail.com

2

Your guess

Your guess

Still guessing

According to Cecil, flat untrue

Your guess

Your guess

Yup

3

As I say, i know this is my guess. What I’m asking is, where do the guesses seem unlikely? Cecil has stated that this theory is unconvincing; I find it plausible, and I find his debunking of it flawed.
As for the “flat untrue” comment, take a look at what we both said: he points out that heme can’t be taken in across the digestive system. I agree. I point out that iron is a key component of the blood, stripped by porphyria and replaced by any form of iron intake. Until Cecil cares to comment on whether he agrees or not, we can’t be at loggerheads.

rupert

4

Just so you know, there are many different types of hereditary porphyria. Some are autosomal recessives, but others are dominant.

This is a common misconception. Hemophilia A, the disease that appeared in some of the male descendants of Queen Victoria, is not really the result of inbreeding. It is an X-linked trait, so males receive only one copy of the defective gene (from their mothers). For male offspring, it doesn’t matter how closely the mother and father are related, because the father’s genes have nothing to do with Hemophilia A. But if a male hemophiliac and a female carrier should mate, their daughters could have Hemophilia A, provided they get one copy of the defective gene from each parent. This is rare even in inbred families, because hemophiliacs often died before they could father children. There have been no female hemophiliacs among Victoria’s descendants, as far as I know.

Please provide a citation showing that iron supplements can be used to treat porphyrias. There are many types of porphyria, but is my understanding that iron deficiency is not the problem. Porphyrias are caused by excess production of porphyrins or a inability to convert precursors into heme, or some other disorder of porphyrin or heme metabolism. I fail to see how iron supplements would help. Indeed, aggratation of symptoms of one of the most common types, porphyria cutanea tarda, is associated with excessive iron intake or exessive iron stores.

Overfed lay-about nobles were physically stronger than hard-working field workers? I have to admit that I have my doubts.

5

Sure – for instance, http://www.cpf-inc.ca/diet_guide.htm states “those with erythopoietic porpoyria may become deficient in iron, and may need to follow a diet high in iron”. From what I understand, iron is very easy to come by in current western society by comparison to medieval central europe, supplements being also readily available and hugely rich in the mineral. I do wonder whether drinking an excess of blood might even have worsened our putative porphyria symptoms – a case of “if some is good, more must be better” going wrong?
As for the strenth thing, think about medieval warfare – strong, well armoured, high-born knights able to mow down swathes of footsoldiers. Combine that with archeaological evidence from skeletons of the period and you get a picture of serfs suffering from severe malnutrition. While in our society more excercise makes you stronger, the same is not true for people without access to sufficient energy and protein in the diet. Serfs were generally significantly smaller than nobles, almost certainly as a result of malnutrition.

6

Vampire myths appear in a variety of cultures throughout Eurasia. They are not confined to an isolated region of Europe nor to nobility. You would have to extend your assumptions to a much, much larger area and also to all strata of society.

If you found this column, check into his column on the various ways to kill a vampire. You’re not going to find yourself in Transylvannia anymore.

7

The problem here, swyves, is that you are comparing only a small number of symptoms of an extremely rare genetic disorder to a superficial Hollywood depiction of vampires and not how vampires were thought of in the original folklore. You can’t make a statement about where the story came from unless you understand the original stories.

8

But nobles ate a diet rich in meat anyway.

A question of equipment and tactics, not diet. The heavy lancer ruled the battlefield from the invention of the stirrup until the invention of the longbow.

9

It is not at all obvious that this would have been the case. For one thing, most people who inherit the porphyria gene never develop any symptoms and even among those who do, those symptoms are rarely acute. The problem with porphyria is often not that its symptions are obvious but rather that they can be so subtle that, even today, it is frequently misdiagnosed. Recognising a common pattern would have been exceptionally unlikely. This is why it was not identified as a specific condition until the late nineteenth century. In the case of what are now the most famous and best documented historical sufferers, George III and his family, it is clear that they and their doctors did not even come close to grasping what it was they were dealing with.

10

Why would you assume that Vampires should be strong, noble, and transylvanians? Vampirism (in the broad sense of ghosts sustaining themselves by feeding on the livings’ “energy”, not necessarily blood) is an extremely widespread myth all over the world. With huge differences from place to place. You’re only trying to compare Dracula’s peculiarities as he appears in the novel/movies with the symptoms of a disease. Pick some genuine legend, and then only come back comparing the description of these vampires with the condition of people suffering from porphyria.

11

What I meant is that you don’t need a medical explanation for the origin of vampire’s myth. It’s a so common theme everywhere that it would be surprising not to find it somewhere in some form or another. And if you think that these sort of myths must have been related to some real condition, then you must come up too with a similar explanation for every sort of life-sucking ghosts. Meanwhile, the most sensible assumption is that they all originated in the same source : the human psyche.

12

The “most common vampire myth” you describe isn’t a myth at all, it’s a modern literary/cinematic character type. As others have pointed out, there’s no real reason other than Dracula to link vampires exclusively with Transylvania or Romanian nobility. There is also nothing in European vampire lore to suggest that vampires are vulnerable to sunlight. The notion that they are seems to have originated no earlier than 1922 with the film Nosferatu, and even then it isn’t sunlight alone that can harm a vampire.

*Funny definition of “likely” and “possible reality” that you’re using there. Since we’re all creatures of the world with limited mental abilities anything we can imagine is going to have some similarities to possible reality. The scenario you’ve invented has some similarities to possible reality, and might make a fun novel, but it’s not at all plausible in real-world terms.

For a very interesting look at the possible real-life origins of the global vampire myth, I recommend Paul Barber’s excellent Vampires, Burial, and Death. Barber’s theory is that the folkloric idea of the vampire derives in part from pre-modern concepts (and misunderstandings) about disease, death, and decomposition.

13

The entire sentence from the source you quote reads:

You left out the second part

I think your cite is (intentionally?) inaccurate regarding iron supplementation. From Up To Date:

Anemia in CEP is caused by premature destruction of red blood cells (hemolysis). No iron is lost in the process. The body recycles it. That’s what plasma iron turnover means. Excessive iron supplementation risks causing iron overload.

Even if iron were a miracle cure for the anemia associated with CEP (and it’s not), iron supplementation wouldn’t ameliorate the most severe symptom of the disease, photosensivity. Minimal sun exposure causes painful burn-like reactions. Scarring of chronically sun-exposed areas is inevitable. Scarring produces functional deficits of the hands and often the face. Would someone with a disease like this care that their hematocrit was 35 instead of 25 (again insert caveat that iron supplementation does not produce this change)?