Hemophilia - what is it, and how to avoid critical complications?

Understand the hemophilia - that this is very important, because this ailment is very insidious. This pathology is hereditary. This disease is very dangerous: it is important to identify it at the initial stage of development, so as not to bring the situation to a critical situation.

Hemophilia - what is it?

To understand what this illness is, the meaning of the term used will help. From the Greek language, "haima" means "blood," and "philia" means "addiction." This disease is characterized by low blood coagulability. They suffer from men, but there are exceptions (type C). They get bad blood clotting from the mother: she is the carrier of the disease and transfers the "bonus" to her sons. The history contains the names of great women who were carriers of the hemophilia gene. For example, Queen Victoria and the Duchess of Hesse.

How is haemophilia inherited?

The gene of this pathology is recessive. It is located in the X chromosome. The inheritance of a pathological gene is associated with sex. In order to start manifesting the disease, you need two X-chromosomes with a mutation. This is a female "set". Men also have X and Y chromosomes. However, in practice everything happens differently. When a woman becomes pregnant with a girl who has two mutating X chromosomes, a miscarriage occurs for 4 weeks. This is due to the fact that the developing fetus is not viable.

A girl can be born with one mutating X chromosome. In this case, the disease itself does not manifest itself: the dominant healthy gene does not recessively trigger the triggering mechanism of the disease. Inheritance of hemophilia can be observed in boys. In the male body, there is no dominant gene in the Y chromosome, and the X-set with the mutation is intensively developing. For this reason, boys inherit this disease, and hemophilia has a recessive trait.

What threatens bad blood clotting?

This pathological condition is very dangerous. The emergence of subsequent problems is inextricably linked with the stage of the disease. This is how dangerous the bad coagulability of blood is:

1. It provokes detachment of the placenta in late pregnancy.
2. May cause profuse bleeding during labor.
3. Dental extraction, surgical operations and severe injuries provoke blood loss.

Hemophilia - Species

Blood clotting is a protective reaction of the body. In this process, fibrinogens, platelets and plasma factors take an active part. The deficiency of one of these substances causes a disruption in the operation of the entire coagulation system. Depending on the missing factor, these types of hemophilia are distinguished:

• A;
• B;
• C.

According to the degree of severity of the course of the disease, these degrees of pathology are distinguished:

1. Easy - with her bleeding happens rarely. In addition, they are low-intensive.
2. Medium-severe - for it is characterized by moderate severity of hemorrhagic manifestations.
3. Severe - if, for example, poor coagulation of the blood in a newborn, this is manifested by bleeding from the umbilical cord, the presence of hematomas on the head, melena and so on. In adulthood, such a disease is no less dangerous than in infancy.

Hemophilia A

For this type of disease is characterized by a deficit of antihemophilic globulin - factor VIII. This type of ailment is considered classic and the most common. It is diagnosed in 85% of cases when the patient has a hemophilia gene. This type of disease is accompanied by the most severe bleeding. For this reason it is important to understand: hemophilia - what is it and what is fraught with.

A distinctive feature of this type of ailment is a violation of the plasma phase of hemostasis. In simple terms, bleeding does not always occur immediately after getting injured. This is because the platelet and vascular phases function properly. After the same day after receiving the injury, heavy bleeding can begin, which is very difficult to stop. This problem can not be solved in one day.

Hemophilia B

The second name for this disease is Christmass disease. The disease is characterized by a deficiency of factor IX. This enzyme component activates the Stuart-Prower complex. This type of hemophilia in children occurs in 20% of cases with the diagnosis of weak blood coagulability. Such a pathology is observed in 1 out of 30,000 newborn boys.

Hemophilia C

This subspecies has its own clinical manifestation. It is very different from the classic type of disease. This subspecies is even excluded from the modern classification. For this reason it is important to understand hemophilia - what is it. It has a distinctive feature that does not occur in other types of disease. Such hemophilia in women is observed as often as men. This is due to the fact that gene transfer is carried out by a dominant feature. There is also a national susceptibility in the pathological state. This disease affects Ashkenazi Jews more often.

Signs of hemophilia

In diseases of types A and B, a similar clinical picture. In this case, poor blood clotting symptoms are as follows:

1. The presence of various hematomas on the body. They appear even after a small bruise. Such hematomas have a wide range of distribution. When pressing on the place of a bruise there is a strong pain.
2. In 80% of cases, hemarthrosis occurs. The joint becomes inflamed, hot, and the skin over it blushes. With repeated hemorrhages, osteoarthritis begins to develop. Simultaneously with it there can be a curvature of pelvic bones and a vertebral column, atrophy of muscles, deformation of feet.
3. Bleeding that occurs after surgery, cuts, tooth extraction and other injuries.
4. In 5% of cases (more often in patients at a young age) there is a hemorrhagic stroke .
5. Hemorrhage in the mesentery - is accompanied by acute pain and other signs similar to the clinic of peritonitis.
6. In 20% of cases, hematuria is observed. This pathological condition can be accompanied by attacks of renal colic and cause the development of pyelonephritis.
7. The formation of gangrene , provoked by giant hematomas.
8. Intestinal bleeding, which is observed in 8% of cases in those who suffer from poor coagulation. Black feces are often accompanied by weakness and dizziness.

Signs of poor blood clotting in hemophilia type C in most patients are weakly expressed or not manifested at all. Some people have bleeding, menorrhagia and the appearance of bruises on the body. Even in one family, whose members suffer from this ailment, the severity of clinical manifestations varies greatly. However, unlike diseases of types A and B, hemophilia C spontaneous hemorrhages in the muscles are very rare. Hemarthroses occur only on condition of rough traumatization of joints.

Bad coagulability of blood - what to do?

If you have at least one sign of a disease, you should immediately seek medical help. To confirm the diagnosis, the doctor will prescribe to the patient an examination that includes such laboratory methods:

• determination of the amount of coagulation factors present in the blood;
• prothrombin index;
• determination of the time during which blood clotting takes place;
• mixt;
• the level of fibrogen in the blood and so on.

Hemophilia is considered irrelevant - the treatment of this disease is reduced to maintenance therapy. In addition, not the least role is given to nutrition. During the period of exacerbation of the disease, its treatment should be carried out in the hospital of the medical institution. Experts of such clinic know hemophilia - what a pathology it is. The patient who has contacted the patient must have a "Book of the patient" with him. This accompanying document contains information about the human blood group, its Rh factor. It also indicates the severity of the disease.

How to stop blood with poor blood coagulability?

When a person has hemophilia, he needs to be especially careful. You can not take lightly of your health! Principles of behavior in this case are as follows:

1. If the wounds are deep, you need to suture and conduct replacement therapy. In addition, ice should be applied to the damaged area (it should not come in contact with the skin, so it must be wrapped with a paper towel or a thin towel before use).
2. In the wound and scratches can form large clots. Of them, the blood oozes. Because of such clots, wounds increase. To avoid this, you need to carefully remove the "lumps". Further, this area should be washed with a solution of penicillin. After that, a bandage impregnated with a hemostatic composition is applied to the skin. This accelerates wound healing.

Medication with poor blood coagulability

Therapy for hemophilia looks like this:

1. The patient is injected with missing clotting factors. The number of such procedures can vary from 4 to 8 per day.
2. Assign injections of preparations made on the basis of plasma.
3. To remove from the patient's body antibodies to coagulation factors, the doctor can prescribe plasmapheresis .
4. Hereditary hemophilia provides for the drip of a solution of glucose, Reamberin or Polyglucin.

With hemarthroses, the puncture of the joint bag is additionally performed. Aspiration of bloody contents and its enrichment with hormonal preparations is carried out. Without fail, the affected limb should be immobilized during therapy. In the future, after rehabilitation, therapeutic physical training and physiotherapeutic manipulations are prescribed.

What to eat with poor blood clotting?

Nutrition has a strong effect on the condition of patients. To prevent the occurrence of bleeding, patients with hemophilia should enrich their diet with such food:

• fats of animal origin (butter, lard, cream);
• liver (brains, kidneys, liver);
• buckwheat porridge;
• berries of violet color (blueberry, blueberry, blackberry and others);
• bananas and mangoes;
• red pepper, radish, red cabbage and other vegetables;
• walnuts;
• beans, peas, soybeans and other beans;
• dill, parsley, basil;
• white bread.