Porphyry refers to the category of rarest diseases. Unfortunately, while incurable. How does people have people suffering from this ailment? From this article you will learn about the history of one girl with a diagnosis «Porphyry».
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What is Porfíry? Here is the definition from the medical director: «Porphyrin disease, porphyry - hereditary impaired pigment exchange with elevated content of porphyrins in the blood and tissues and enhanced by their release with urine and feces». What is hidden behind these dry and not very understandable words?
It turns out that the doctors today about Porphyry know a lot. It is believed that one person from 200 thousand (according to other data - out of 100 thousand) suffers that this rare form of gene pathology. The medicine describes about 80 cases of acute congenital porphyria, when the disease was incurable.
The disease is characterized by the fact that the body cannot produce the main component of blood - red tales, which in turn is reflected on the deficiency of oxygen and iron in the blood. In the blood and tissues a pigment exchange is broken, and the decay of hemoglobin begins under the influence of solar ultraviolet radiation or ultraviolet rays.
Patients are contraindicated with sunlight, which brings them unbearable suffering. Moreover, in the process of the disease, tendons are deformed that in extreme manifestations leads to twisting of the fingers. In addition, patients are very pale skin, during the daytime they feel the decline of strength and lethargy, which is replaced by a more mobile lifestyle at night. It is necessary to repeat that such symptoms are characteristic only for late stages of the disease, in addition, there are many other, less terrifying forms. As already written above, the disease was practically incurable until the second half of the XX century.
Despite the fact that Porphyria - the disease is not mental, it, naturally, is extremely destructively affects the psyche. Yes, and generally can bring a person to suicide.
Journalist Vladimir Lagovskaya writes about the case of Porphyria in the girl who has been ill for more than 15 years (surname and place of residence are not called). «Daylight fatal for katy. The sun can burn her alive in just one minute. Girl hospital. But for others is not dangerous. Katina night lasts 15 years. From early childhood on the face and handles, open daylight, spots and rashes began to appear. The girl started screaming. Thought that this is ordinary diathesis. Pass. But the stains did not pass. On the contrary, turned into ulcers.
- Doctors could not make a diagnosis - Sophia says, Mom Kati. - They only determined that the child has an abnormal allergic reaction to the sun. And advised to keep my girl in the dark. The Council was helpful. For a few days spent without the sun, ulcers healed almost without trace. But they appeared again every time the girl at least for a few minutes was shown in daylight. And every time everything is stronger».
On the moon, the girl reacted normally. Not covered with ulcers and with light light bulbs. For kati, only the radiation of a certain frequency is murderous - mostly ultraviolet spectrum. Kremai from a high degree of protection helps her, but not long: five minutes. Then the unbearable pain begins. Previously, when the girl was small, she was transported from place to place in a closed bag. Now the kate sewed a special light-tight helmet with a pump that serves air for breathing. Even in the summer, a girl wears a dense coat and gloves. She does not go to school. Engaged with teachers at home in a room with glasses that do not miss ultraviolet.
- Adults spread evil rumors, - Catina Mom complains. - As if my daughter is a vampire. And at any time he can bite someone. One newspaper even wrote that at night she would. There is no radical tool, and the existing drugs only facilitate skin healing.
Igor Kurbatov, an employee of the hematological center of the RAMS, believes that the attitude and in fact a potential vampire.
- The girl has a rare so-called porphyrine disease, "he says. - Its reason was discovered ten years ago. But the disease itself existed before. In medieval legends of vampires described people who suffered from porphyrin disease. Our Canadian colleagues under the guidance of Professor Dolphin believe that it causes a defect in genes. We are not fully sure, but the essence of this is. Man is saturated with pigments, so-called porphyrins. They enter, for example, the composition of blood - in hemoglobin making it red. In patients, natural circulation of porphyrins is broken. They accumulate under the skin and under the action of sunlight acquire destructive power. Begin to isolated a poisonous atomic oxygen, which literally corps surrounding fabrics. Face features can be distorted beyond recognition, to become frightening. People hide from light and go out on the street only at night. It is possible that some patients drank blood. But not with the devilish goal, but as a medicine. They intuitively felt that it was necessary to fill the loss of hemoglobin. And before it could only be mined from human blood, directly using it. It facilitated suffering. But today it is all unnecessary, because hemoglobin is sold in pharmacies.
