Hemophilia

Hemophilia is an inherited bleeding disorder in which the blood cannot clot properly due to a deficiency or absence of clotting factors in the blood. Because the gene that causes the disease is located on the X-chromosome, only boys are affected, while girls are carriers. The severity of hemophilia a person has is determined by the level of factor in the blood. The lower the factor level, the more likely it is that bleeding will occur, which can lead to serious life-threatening health problems. Depending on the level of deficiency of these factors, patients may present with spontaneous bleeding or bleeding that cannot be stopped after injury or surgery. The diagnosis is made by determining the deficient factor and its level in the blood. The main treatment of the disease today is to replace the missing factor. If we use the medication regularly 2-3 times a week from the first year of life, it is called Primary Prophylaxis. Secondary prophylaxis if we use it in case of target joint development after bleeding develops. If the patient uses factor drugs intermittently, he/she prefers the “Treatment as it bleeds” method. The treatment performed by giving radioactive material to the target joints is called Radioactive Synevectomy. Gene therapy options have also been used in the last 10 years. However, gene therapy costs are still very high and only a very small percentage of patients can access this treatment.