Porphyrias at the Movies


Some days ago I wrote a post with a question for those of you that like movies

 

 

Here is the answer…Porphyrias.

 

Porphyrias form a group of rare diseases that result as a consequence of disorders of the synthesis of Hem group. Unfortunately. patients with porphyria are frequently misdiagnosed, and when the diagnosis is made, sometimes it occurs after  months or years of looking for help.

 

Porphyrias are in normal life, as infrequent as frequent they are in movies.

 

In the movies included in the question, there are characters inspired (or allegedly inspired) by patients with Porphyria.

 

Imaging a patient very pale, whose teeth are pink, whose urine is frequently red, that can show some psychological abnormalities,  that interact very few with the neighbors, …only seen at night, and that develops blisters in the skin when exposed to the sun…imaging also that this kind of patients, in a very ancient time, could have been assisted by healers that recognized in their pallor  the same sign showed by persons that have lost a lot of blood, imaging that the healers recommended this kind of patients to drink fresh blood from animals for treatment of the pallor and weakness… Too much imagination?  What does this description remind you?… Maybe this?

 

 

 

Different authors have related the legend of vampires with some of the signs of the unfortunate patients suffering of porphyria. 

 

We will discuss soon these and other issues that have made porphyrias the common factor that links vampires, King George, Vincent Van Gogh, Gaston Leroux’s character in Phantom of the Opera, and the children in the movies “The others”.

 

Do not forget to return soon to see the series “Porphyrias in the movies”.

 

Certainly you will learn some trivia, but I hope that you will also learn important issues that can help you to do the diagnosis in these unfortunate patients and to help them to avoid their pilgrimage through numerous physicians until they find that doctor that recalls this Biochemistry topic!

 

 

 

 

9 thoughts on “Porphyrias at the Movies

  1. Dear cinemabuzz:

    Do not jump to conclusions so quickly. It is true that some signs are shared, but
    Porphyria patients are just normal persons with a disease, and they do not have a particular tendency to attack people, by day or at night.

    If you are attacked in the middle of the night by a pale person that intends to drink your blood, I suggest that you assume that it is a vampire.

    If you want to be sure, and you have the chance (some vampires like to talk with the victims before biting them), I suggest that you do in your attacker the following tests:

    1. – The Cross test.

    It is known that vampires go back when they see a cross.

    2. – The Holy Water test.

    If it happens that you have a small bottle of Holy water in your pocket (I always have one, just in case), you can spill some drops in your attacker. If he/she yells and an ugly blister appears immediately in his /her skin, then it is a sign that you are in the presence of a vampire.

    The main problem with tests (1) and (2) is that they lack sensitivity. Nowadays is frequent that some of the vampires that arrive to the Western hemisphere were not raised in a Christian family while they were humans, and these former tests can not work properly in vampires raised in Muslim, Buddhists or other religions environment, so you can obtain false negatives results with the two former tests.

    More sensitivity is shown by the following tests:

    3. – The Garlic test

    It is a very sensible test. Vampires, by definition, are allergic to garlic. This characteristic was demonstrated by Dr. Abraham Van Helsing (Van Helsing, A et al: Anaphylactic Shock induced by garlic antigens in Transylvania species. The Journal of Vampires, Werewolves and Witches, 1897).
    Lack of air, wheezes, and other breathing difficulties, that vampires develop when they are in contact with garlic, can indicate that you are in front of “one of them”. Gabriel Van Helsing Jr. has published recently an article describing a confirmatory test called Vampilisa, an Elisa for detection of anti-garlic IgE in vampires.

    4. – The Mirror test

    This is the simplest test for detecting vampires. It is simple and cheap. It only requires a small mirror that you can carry with you (it is accepted nowadays that metro sexual men carry also mirrors with them).
    For some reasons not very well understood, mirrors do not reflect a vampire figure. I am sure you know that, since this fact appears in books and movies about vampires. This is an interesting quote of Anna, a female vampire about this fact:

    “Vampires get such a crummy deal. Not only do we have to sleep in worm-eaten old coffins and wear these smelly old clothes, but we can’t even look in the mirror when we want to fix ourselves up a little bit”
    (Movie: The vampire moves in”, 1985)

    Dear Cinemabuzz, I hope that the use of the tests described above will allow you to detect a vampire if you are attacked at night for somebody trying to suck your blood. If you are interested in knowing something more about real Vampires Biochemistry, please, stay tuned. In the meantime, I will continue discussing porphyrias at the movies. I am sure you will find some issues that you can use in your blog.

  2. Well then I must not be a vampire (as there is no such thing except in the active imagination of writers), as one of the projects for the community I live in is saving the remains of a Catholic church, which includes a 7 foot cross. I did the required sign of the cross with holy water every time I entered church for mass.

    As for garlic, too much garlic does tend to rob my blood cells of oxygen due to the sulfur that is generated from the chemical breakdown of garlic in my digestive system. This will make me feel under the weather for a few days, but as long as I don’t have too much.

    And as for the mirror test, my husband says I spend too much time in the mirror. But covering the sores I get from too much time in the sun, requires careful application of makeup.

    By the way, I have HCP, which is one of the mixed-type porphyrias. There have been very few cases of CEP and even fewer HEP, which manifest in infancy. If you are unfamiliar with these terms, do a little research and then let me know if being so flip about the association between porphyria and vampires is appropriate.

    You will only receive from the world what you give to it.

  3. Dear Henrischmidt:

    Please, review this link to other page in the same site, where it appears more detailed information about Porphyria and links to websites with important information about this disease.

    https://biochemistryquestions.wordpress.com/biochemistry-at-the-movies-page/

    I am sorry that you considered that I took lightly this disease. I think I took light a comment of other blogger about being attacked by a vampire, and not the disease itself.

    I am aware of the importance of inform about this disease and other rare diseases, since sometimes medical students (and physicians) focus in high yield concepts and forget some rare diseases. I have seen some undiagnosed patients with PKU and other metabolic disorders in special institutions in undeveloped countries, and last year I knew about an American patient with Porphyria that was treated as a withdraw syndrome since she had been using drugs for some time. After some months of study, a physician decided to study Hem metabolites in the patient and diagnosed a Porphyria. I wonder also how many patients can be hospitalized in psychiatric hospitals in different parts of the world, because of this kind of diseases ( I intend to continue this topic yet by recalling the theory that Van Gogh suffered from Accute Intermitent Porphyria)

    I hope that in some way this discussion reminds everybody of the different ways in which this disease can appears,

    Once again, I am sorry if you felt that I took this topic lightly and I apologize if you felt offended. Please, read the information in the link above and let me know your thoughts.

    Best wishes

  4. Pingback: There is Biochemistry also in “Twilight”! « The Biochemistry Questions Site

  5. I have porphyria, I have no side effects besides a little depression that I’m managing without meds. This is the first time I really googled this and I am amazed at all these comments and websites! Really, people walking around in daylight do not even know of porphyria, let alone vampires connected to it…Well, I must say, if I could, there’s quite a few people who’d I’d like to give a scare… got the pale skin…mmm

  6. As a person with porphyria (AIP), I have seen quite a few films about people with porphyria although I may be one of the few who actually realized that the person who was being portrayed in the film had porphyria in real life. The King’s Speech comes to mind but the cast of real-life people also includes Vincent Van Gogh, John Adams, Alexander the Great, Charles Darwin, Edgar Allen Poe, Mary Queen of Scots, James I, Mozart. These are not names one readily associates with vampires

    • By referencing The King’s Speech, i presume you mean King George VI had porphyria. Very likely because his genetic line has porphyrics. His brother the Duke of Gloucestor had a son who was definitely dx’d with it. See a wonderful YouTube presentation, Prince William of Gloucester. Prince William was an otherwise normal and handsome young man, and the YouTube doesnt concentrate on his illness but mentions it. George VI was a direct descendant of George III, Mary Queen of Scots, James I, and Queen Victoria/Prince Albert. He did not act like a vampire.
      In fact he was not one. I too have Porphyria and I am not a vampire and a point the finger of shame at those who spread such rumors. Nasty people! Much worse than vampires!

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