Haemophilia disease is hereditary and observed in boys; It is a condition in which patients' blood does not clot normally. The disease is observed quite rarely. If you have hemophilia, you may bleed longer than others after an injury. Or you may bleed internally, especially in the knee, ankle and elbow, without injury or trauma. This bleeding can damage your organs or tissues and be life-threatening.
Haemophilia is usually hereditary. In other words, the disease is genetically inherited from mother and father to their children. Individuals with hemophilia have deficiency or deficiency in clotting factors 8, 9 or 11. These clotting factors are a protein necessary for normal blood clotting. There are several types of clotting factors. These proteins work with blood platelets to help blood clot.
Normal blood clotting cannot be achieved without clotting factors. Some people with hemophilia need injections of a clotting factor or factors to stop bleeding. There are two basic types of hemophilia. If you have hemophilia A, your level of clotting factor VIII is low or you do not have this factor in your blood. The majority of hemophilia patients have hemophilia A. If you have a deficiency of clotting factors 9, then you have Hemophilia B. Very rarely, clotting factor 11 deficiency is called hemophilia C.
Hamophilia can be mild, moderate or severe, depending on the level of clotting factor in the blood. The majority of patients have severe hemophilia. It makes more sense to explain this as follows; Let's assume the factor level of a normal individual is 100%. Your factor level for severe hemophilia disease is below 1, and the course of the disease is directly related to these levels.
The main signs and symptoms of hemophilia disease are excessive bleeding and easy bruising.
Vaccineri Bleeding
Amount of bleeding type of hemophilia and depends on its severity. In children with mild hemophilia Symptoms may not be observed except in cases of work attempt, accident or surgery. Men with severe hemophilia may experience heavy bleeding after circumcision. The majority of patients are diagnosed with excessive bleeding during the circumcision attempt.
Bleeding can occur on the surface of the body (external bleeding) or inside it (internal bleeding).
Some of the symptoms of excessive external bleeding:
-Bleeding in the mouth as a result of cuts and bites, or tooth cutting or extraction
-Her nosebleed from both nostrils
- Severe bleeding from a small cut
-A cut that bleeds again shortly after it stops
-Signs of internal bleeding, blood in the urine (bleeding in the kidney or bladder) and blood in the stool (bleeding in the intestine or stomach).
Hemophilia patients are usually diagnosed with excessive bleeding that occurs during circumcision. If patients bleed excessively during circumcision, care should be taken in case of hemophilia. When hemophilia is suspected or you have a bleeding problem, your doctor will take your personal and family medical history. It will reveal whether you or anyone in your family has a history of frequent and/or heavy bleeding or bruising. Since the disease is inherited via the X chromosome, the same disease is usually present in the maternal side of hemophilia patients, especially in their maternal uncles.
You will also be given a physical examination and blood tests to diagnose hemophilia. Blood tests are performed to investigate the following:
-The clotting time of your blood
-Whether you have low levels of any of the clotting factors in your blood
-The complete absence of any of the clotting factors in your blood
Test results will show whether you have hemophilia, if so, which type you have, and how severe it is.
The severity of symptoms may overlap between categories. Example For example, in some people with mild hemophilia, the frequency or severity of bleeding may be as much as in people with moderate hemophilia.
Severe hemophilia can cause serious bleeding problems in babies. Therefore, children with severe hemophilia are usually diagnosed within the first year of life. Those with milder forms of hemophilia may not be diagnosed until adulthood.
How is Hemophilia Treated?
The basic treatment of hemophilia is called replacement therapy, which replaces the missing factor. Concentrates of clotting factor VIII (for hemophilia A) or clotting factor IX (for hemophilia B) are dripped or injected slowly into a vein. These treatments help replace absent or low amounts of clotting factors. Patients can perform this treatment in a hospital setting or can apply it to themselves at home.
Coagulation factor concentrates can be obtained from human blood that has been processed to prevent the transmission of diseases such as hepatitis. With current methods such as screening and processing of donor blood, the risk of transmitting infectious diseases from human clotting factors is very low. To further reduce this risk, you or your child can receive a clotting factor concentrate that does not use human blood. These are called recombinant clotting factors.
You can receive regular prophylaxis treatment to prevent bleeding. This is called preventive treatment. Or you may only need replacement therapy to stop bleeding when it occurs. Using treatment in this way when necessary is called need treatment.
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