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Porphyria consists of a group of 8 different disorders, that are of significantly different, one from another. A main aspect of all, are the accumulation of "porphyria precursors". The precursors are found normally within the body, but not to the extent of mass accumulation. Within each type of porphyria, there is a specific enzyme deficiency - enzymes being mainly involved in the synthesis of haem/heme, which is a substance important to various bodily functions. It is found abundantly within bone marrow, red blood cells, and the liver, though how it functions within the liver, is very different from its functions within bone marrow and blood. Which type of porphyria it is, is dependent upon which enzyme is deficient. Inherited porphyrias are either autosomal dominant (passed from one parent), or autosomal recessive (passed from both parents).
Autosomal genes always occur in pairs. Those individuals who are autosomal dominant have received one gene which is paired with a normal gene, which is then passed down to the eventual offspring. The odds a child from such a parent will develop symtoms, are 2 to 1, whereas in those individuals who are autosomal recessive, the odds are 1 in 4 will develop symptoms in their lifetime. There is genetic testing , however, to determine if a child has the disease, or will be prone/ pre - disposed, even though they will most likely not get symptoms in - utero.
Symptoms of porphyria are lameness, blistering and severe burning of skin, due to sun sensitivity, which is caused by excess porphyrins in blood plasma, which, depending on the type of porphyria, have originated from either the liver, or bone marrow. UV light interacts with porphyrin in such a way, that it damages skin tissues, and can cause scarring. Urine will turn various shades of red, blue, and purple, and sufferers can also experience mental derangement. Those with porphyria also experience what appear to be elongated canines, but aren't - it is simply that the gums have receded from the teeth, exposing more of them, and giving the appearance of being elongated..There are also incidences in which sufferers have been known to drink blood. This has given the impression that porphyria sufferers, are simply modern day vampires. These individuals are not vampires. This, according to them, is a means of alleviating the painful symptoms caused by porphyria. People who have porphyria can expect longevity when they take on suggested precautionary measures (or, learn how to cope, and take care of themselves properly). Progressive deterioration, and death are very rare within the most acute porphyrias.
Porphyria Variegata, it is believed, can be traced as far back as Mary, Queen of Scots. It travelled all through the line of the Stuarts, Hanoverians, through King George III, Queen Victoria, her daughter Feodora, and granddaughter Charlotte, William of Gloucester, and now Windsors, most recently, Princess Margaret. Though Margaret had porphyria variegata, it is believed that the disease did not cause her death, but most certainly, it added a heavy degree of suffering to her life.
Copyright ~ Silver Rainbolt 2004 ~ All Rights Reserved
You may not legally copy any part of this document without prior written consent of the author. Doing so, infringes upon copyright, and is punishable under the law.
Copyright 2003/2004 held by Silver Rainbolt/In Medias Res, and Damien Daville/Vampire Church
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