World Hemophilia Day
15.04.2016
The aim of World Hemophilia Day, held annually on 17 April, is to strengthen the awareness of this rare and severe disease and everyday challenges that people living with it face.
Hemophilia is a rare disease that occurs due to congenital deficiency or reduced activity of blood clotting factors. Clotting factors are proteins in blood that participate in the formation of a clot, designated by Roman numerals (factor I, factor II and so on). Each clotting factor has a dedicated function and, in order to form a clot, they have to act together and in a very precise manner, according to the specific order. If any of those factors is missing or its function is disrupted, the result is abnormal bleeding.
The most common form of hemophilia (80% of all cases) is hemophilia A or classic hemophilia, caused by the deficiency or defect of clotting factor VIII, also known as antihemophilic globulin (AHG). The second most common form of hemophilia is hemophilia B, caused by the deficiency of another protein necessary for clotting, factor IX. In line with this, the most common symptom of this disease is prolonged internal or external bleeding, which is difficult to stop. Persons with a severe form of hemophilia, characterized by less than 1% of normal activity of clotting factors, bleed frequently, sometimes once or twice a week - often spontaneously (without data on injury), with frequent spontaneous bleedings in joints which are more active and exposed to stress. Bleeding in joints and muscles causes severe pain and disability, whereas bleeding in vital organs such as brain can result in death.
Approximately one in every 10,000 people become ill with hemophilia. In most cases it is a hereditary disease that men become ill with, whereas women (mothers) are carriers, and become ill with hemophilia very rarely. Moreover, there are children ill with hemophilia whose mothers are not hemophilia gene carriers. Namely, one-third of babies born with hemophilia come from families with no known history of this disease, and in these cases hemophilia is caused by a spontaneus genetic mutation - a genetic error which occured very early in baby's cells.
Persons with hemophilia can maintain a high quality of living with adequate replacement therapy containing the clotting factor that is missing or the function of which is disrupted, taken intravenously in order to stop the bleeding quickly.
HALMED urges all citizens ill with hemophilia to adhere to advice by healthcare professionals and take the prescribed therapy.
World Hemophilia Day was first held in 1989, on the date of birth of Frank Schnabel - the founder of World Federation of Hemophilia, which advocates adequate treatment and care, as well as enabling the highest possible quality of living for all persons ill with hemophilia. On the occasion of World Hemophilia Day, the World Federation of Hemophilia also attempts to strengthen awareness of other bleeding disorders, such as Von Willebrand disease.
