Welcome to the Crypt.

Welcome to my crypt. I am the Porcelain Vampire.

     No, I am not a roleplayer. No, I am not a life-styler. No, I do not turn into a bat. Want to know more about me? Profile is over there, go check it out. ;)


     Have you ever wanted to be a real vampire? Have you ever actually understood what that would mean? This is the story about myself. I come from a family of real vampires. We are quite literally allergic to the sun. It can burn us, blister us, & even send us to the hospital. What is this condition, you may ask? Porphyria.

     Porphyria has long been described as a harmful phototoxic reaction of the skin to the sun. There's a lot more to it than that, but we will leave this description for now. The legend of the vampire may have been largely to blame for this genetic blood disorder. That's right, a blood disorder. Porphyrins are in everyone's blood. People with this genetic blood disorder just happened to have a lot more of them react to sunlight, causing this phototoxic reaction.

     Doctors wouldn't come around to even remotely understanding this condition until the 1800's. Thankfully, people insisted that vampires must have come from recently deceased in graveyards, reawakening from the dead to feed on the living. People would have 'vampire burials', where the family member would be dug up and reburied to reassure all that they would not be returning from the grave to feed on their relatives. Vampire hunters would wear garlic around their necks to keep the smell of the dead from getting to them, later thought to 'repel vampires'. Enticing hmm?

     No wonder so many of us allergic to the sun chose to deal with it, stay quiet and suffer alone without the help of doctors until much later in history. The alternative would have been being accused of witchcraft, necromancy, or worse. Doctors today still know very little about some types of porphyria. They can agree that there are 7 different types. I'm only going to focus on my type.

     Erythropoietic Protoporphyria (EPP) is a rare inherited metabolic (blood) disorder caused by a deficiency of the enzyme ferrochelatase (FECH), which results from changes (mutations) in the FECH gene. Due to abnormally low levels of this enzyme, excessive amounts of protoporphyrin accumulate in the bone marrow, blood plasma, and red blood cells. Some patients with symptoms of EPP have a genetic change in a different gene called ALAS2.

     It will hit every apple on the way down the tree and symptoms are not always present. Unfortunately, every parent has a 50/50 chance of having a child develop EPP. EPP has been in my family for many generations. I won't bore you with how many generations (I'm one of the family historians, so I've got our genealogy written down/digitized). I'll start with my recent family members. My grandmother, my mother, myself, some cousins, my daughter, 2 of my nieces all have EPP. I even have a couple of male cousins and an uncle that has it. Oh? Did I mention women are more likely to inherit the symptoms than the men? No one knows or understands why.

Alright, off we go into this journey. Enjoy the story of my life.



Links for your enjoyment:
https://rarediseases.org/rare-diseases/erythropoietic-protoporphyria/

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