Hemophilia

2024 Author: Rachel Wainwright | [email protected]. Last modified: 2023-12-15 07:39

The tendency of the human body to prolonged hemorrhage and bleeding is called hemophilia and this disease belongs to the group of hemorrhagic diathesis (from “hemorrhage” - bleeding and “diathesis” - predisposition).

Bleeding with hemophilia occurs completely spontaneously, for no apparent reason, and is difficult to stop with conventional means. The first historical information about the hemophilia disease is in the Talmud, which describes the deaths of boys as a result of ritual circumcision in the second century BC.

Causes of hemophilia

Scientists attribute hereditary factors to the main reasons provoking the development of hemophilia. Genetically defective blood clotting is inherited from generation to generation, and the carrier of the defective gene is exclusively the female body, and the patient with hemophilia, as a rule, is a man. In fairness, it should be noted that there are scientifically described cases of hemophilia in women, but these cases are extremely rare and occur when both parents of a sick girl are carriers of the damaged gene.

Passing the disease on to her children, the female “conductor” herself remains healthy, her sons are doomed to hemophilia, and her daughters also become carriers of the hidden gene until they pass it on to their children. Therefore, there is only one way to interrupt the pathological chain, to use the rather cruel advice of geneticists and carefully plan the birth of future offspring. In accordance with the recommendations of modern geneticists, female carriers should not have children at all, and only sons should be born in the families of men with hemophilia and healthy women, pregnant girls are invited to undergo an artificial termination of pregnancy.

Currently, scientists have not yet found a way to eliminate the cause of hemophilia, today, unfortunately, it is not yet possible, because it is embedded in the human genetic code. A patient with hemophilia has no choice but to learn how to live with this disease and get used to the fact that his body needs special precautions and special care every day.

Diagnosis of hemophilia

An attentive parent can notice the first signs of hemophilia in children at a very early age. Symptoms of hemophilia become pronounced when the baby begins to stand up and independently explore the space around him. During this period, the inevitable time of the first bruises and abrasions begins, without which neither the first steps nor the skills of handling unfamiliar objects can do.

A characteristic feature of hemophilia disease, which makes it possible to diagnose it in a timely and unambiguous manner, is the inadequacy of the child's body response to the injury. Even a minor bruise or a tiny scratch causes profuse and prolonged bleeding with hemorrhage into the intermuscular space and the formation of huge hematomas. Bruises and hematomas with hemophilia do not dissolve for a long time, gradually acquiring shades from bluish-blue to yellow-green.

Symptoms of hemophilia

With hemophilia, bleeding, their nature and duration differ significantly from bleeding in a healthy person. Minor tissue damage, even during conventional injections, can bleed for a long time (up to several weeks), forming subcutaneous hematomas.

A common occurrence in hemophilia is severe nosebleeds, which are difficult to stop using the usual methods in such cases. Symptoms of hematuria may also appear - the appearance of blood in the urine. In newborns with hemophilia, during childbirth, extensive hematomas form in the head area (cephaloquematomas), later umbilical cord bleeding occurs later, then when the first teeth erupt. All the symptoms of hemophilia described above must necessarily draw the attention of parents to the pathological condition of the child and force them to turn to specialists in time.

Hemophilia treatment

As mentioned above, there is no cure for hemophilia that will permanently save the patient from the disease. But there is the possibility of constant control over its course with the help of constant injections of the missing blood coagulation enzymes, produced from donor material.

The disease of hemophilia is extremely dangerous, especially in childhood and adolescence, when often the correct diagnosis has not yet been made and without medical support there is a real risk of death. The timeliness of going to the doctor with hemophilia in childhood is a measure of the favorable course and stabilization of the child's body.

Qualified treatment of hemophilia and constant monitoring of the course of the disease should be carried out only by an experienced specialist - hematologist. Each patient with hemophilia needs to know and remember that the following drugs are categorically contraindicated for him: aspirin (acetylsalicylic acid), analgin, indomethacin, brufen and butazolidines.

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The information is generalized and provided for informational purposes only. At the first sign of illness, see your doctor. Self-medication is hazardous to health!