What is porphyria? Here is the definition of a medical handbook, "porphyrin disease, porphyria - an inherited disorder of pigment metabolism with a high content of porphyrins in the blood and tissues and enhanced release of urine and feces." What lies behind these dry and not very intelligible words?
It turns out that doctors today aboutporphyria know many. It is believed that this rare form of genetic diseases plagued man from 200 thousand (according to other sources - 100 thousand). In medicine, describes 80 cases of acute congenital porphyria, the disease was incurable.
The disease is characterized by the fact that the organism is notcan produce primary blood component - red corpuscles, which in turn affects the deficiency of oxygen and iron in the blood. The blood and tissue is broken pigment exchange, and under the influence of solar ultraviolet radiation or ultraviolet rays decay hemoglobin begins.
Patients contraindicated sunlight, whichIt brings them untold suffering. Moreover, in the disease process deformed tendon that in extreme cases it leads to twisting fingers. In addition, patients strongly pale skin in the daytime they feel fatigue and lethargy, which is replaced by a sedentary life at night. We must repeat that only the symptoms characteristic of the later stages of the disease, in addition, there are many other, less horrific of its forms. As already stated above, the disease has been virtually incurable until the second half of the XX century.
Despite the fact that porphyria - not mental illness, it is, of course, an extremely destructive effect on the psyche. Yes, and in general can drive a person to suicide.
Journalist Vladimir Lagovskaya writes about the caseporphyria, the girl suffering from it for more than 15 years (name and place of residence are not called). "Daylight is deadly to Kati. The sun can burn her alive in just one minute. The girl is sick. But others are not dangerous. Katina night lasts for 15 years. From early childhood on his face and the handles, open daylight began to appear spots and rashes. The girl began to cry. We thought that this is an ordinary diathesis. Pass. But patches do not pass. On the contrary, it turned into ulcers.
- Doctors could not diagnose -said Sophia, mother Kati. - They only have determined that the child has an abnormal allergic reaction to the sun. And advised to keep my girl in the dark. The Council has proven useful. In just a few days spent without the sun, the ulcer healed almost without a trace. But they reappear each time after the girl at least for a few minutes, show up in daylight. And each time more and more. "
On the moon, the girl reacted normally. Not covered with sores and light bulbs. To Kati only deadly radiation of a certain frequency - mainly in the ultraviolet spectrum. Cream from sunburn high degree of protection to help her, but not for long: five minutes. Then unbearable pain begins. Earlier, when she was a little girl, it was transported from place to place in a closed bag. Now Kate sewed special light-tight helmet with a pump, which supplies air to breathe. Even in summer the girl wears a tight coat and gloves. In school she goes. Engaged with teachers at home in a room with windows, do not skip ultraviolet.
- Adults are spreading malicious rumors - complainsKatya's mother. - As if my daughter - a vampire. And at any moment someone to bite. One newspaper even wrote that she howls at night. Radical means no, and the available drugs only facilitate healing of the skin.
Igor Kurbatov, hematology center RAMS employee believes that Katya is indeed a potential vampire.
- The girl has a rare so-called porphyrindisease, - he says. - Its cause was discovered ten years ago. But the disease has existed before. In medieval legends about vampires described people suffering from the disease is porphyrin. Our Canadian colleagues, led by Professor Dolphin believe that it is a defect in the genes. We are not completely sure, but the point is this. Man saturated pigments so called porphyrins. They include, for example, in the blood - to hemoglobin, making it red. Patients natural circulation of porphyrins is broken. They accumulate under the skin and under the influence of sunlight become a destructive force. Begin The beaten toxic singlet oxygen, which is literally eating away the surrounding tissue. Facial features can be distorted beyond recognition, become frightening. People hide from the world and go to the street only at night. It is possible that some patients drank the blood. But not with the purpose of the devil, and as a medicine. They intuitively feel that it is necessary to make up the loss of hemoglobin. But before it could produce only human blood directly to her eating. This is to alleviate the suffering. But today all of this too, since hemoglobin is sold in pharmacies.