According to the article on Sciencedaily,Porphyrias refers to a group of total eight known disorders. Erythropoietic protoporphyria (EPP) is also one of those known blood disorders. People who have EPP are chronically anemic, which is why they look really tired. Since they rarely come out in the sun, because of their extreme photosensitivity, they look really pale. Their skin is so sensitive that even on a cloudy day there is enough ultraviolet light to cause blistering on the exposed body parts. Erythropoietic Protoporphyria is caused by a genetic mutation which impacts the body's ability to produce heme ( a component of the oxygen-transporting protein, hemoglobin). Protoporphyrin components start to build up in red blood cells, and plasma. Once these components are exposed to the sunlight they start creating a chemical. This chemical starts to damage the surrounding cells, which causes swelling, burning and redness in people who suffer from EPP.
According to porphyriafoundation, for the treatment of Erythropoietic Protoporphyria (EPP), a synthetic hormone called afamelanotide is used. This drug is inserted under the skin using a needle. It is released slowly over the seven days,and protects the EPP patients for about two months. The drug starts working by giving patients a tan.This tan acts like a barrier and protect them from the sun.The drug afamelanotide is allowed to use in the Europe, but is still under investigation in the united states.
This article is very interesting because it connected something fictional to something real. I love reading about new diseases, so this article caught my eye instantly. Being not able to come out in the sun and experiencing such skin conditions after being out in daylight is terrifying. However, the drug afamelanotideis helping many people and I hope it will get approved in the US too.
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