My porphyria blog: EPP porphyria update

EPP porphyria update

Those of you who have read my aip porphyria story, may have read where I wrote that aip is just one type out of eight different porphyria types. There is Acute Intermittent Porphyria(AIP)- that's the one I have and it is the most debilitating one you could probably inherit because the acute attacks are life-threatening. There is Variegate Porphyria(VP), that's the skin-type with acute symptoms as well but not as life-threatening although it can be. There is Hereditary Corproporphyria(HCP), they can have acute attacks as well but harder to catch the porphyrin precursors as the attacks usually resolve quickly. There is ALAD-deficiency Porphyria(ADP) that results in an increase of ALA in the liver, all reported cases are males and it's the least common of all porphyria types, only ten documented cases. There is Porphyria Cutanea Tarda(PCT), that's the most common of the porphyrias to have, and it is essentially an acquired disorder but it can be genetically acquired also. PCT is one of the hepatic porphyrias- so is aip- anyway with PCT large amounts of porphyrins build up in the liver when the disease is becoming active. PCT becomes active when acquired factors such as iron, alcohol abuse, Hep C virus (HCV), HIV, estrogens used in oral contraceptives and pristrate cancer treatment and possibly smokinf, combine to cause a deficiency of UROD in the liver. Hemachromatosis, an iron overload disorder can also predispose individuals to PCT. PCT symptoms are confined to the skin mostly, painful blisters can develop on sun exposed areas. There is also porphyria type, Erythropoietic Protoporphyria(EPP), which is the type this post is mostly about, I'll explain soon, but let me describe EPP. EPP is characterized by sensitivity to light, it causes very painful photosensitivity . EPP sufferers can develop painful blisters from the sun that can take days to heal. There is also Congenital Erythropoietic Porphyria(CEP), CEP is also called Gunther's disease, it is very very rare and sufferer have extremely severe photosensitivity. Finally there is also Hepatoerythropoietic Porphyria(HEP), which symptoms manifest much like CEP. Us porphs use the abbreviations of these disorders when talking about them, it's much easier than trying to pronounce some of these types out loud. So now that I've explained the eight different types and your more knowledgeable about the types, I'd like to bring you an update about EPP treatment.

For years they have been working on a drug for EPP sufferers so hopefully you can go outside in the sun and not pay for it afterwards with painful blisters and scarring- not have to fear the sun.the new drug is called AFAMELANOTIDE and I've been hearing great things about it from EPP sufferers, we had some brave souls with EPP agree to volunteer to be in the clinical trials and now they are trying to get the FDA to quickly approve phase III of this drug, so that these EPP sufferers can get some damn relief as soon as possible. American Porphyria foundation has been spearheading this campaign with congress and doing very well I might add, way to go APF!! If you would like to know more about EPP, the new drug afamelanotide and how to help gain FDA approval for this new treatment so these ppl can have a sun filled life again, check out this link below, all the instructions are there-

Porphyria Purple Light Blog- EPP trial info


I got this info on the eight different porphyria types from the american porphyria foundation website, for more about the eight different porphyria types and how to diagnose, go here;
American Porphyria Foundation- About Porphyria

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