Hemophilia is a bleeding disorder that prevents blood from clotting properly. People with hemophilia do not bleed any faster than normal, but they can bleed for a longer time. Their blood does not have enough clotting factor. Clotting factor is a protein in blood that controls bleeding. The severity of a person’s hemophilia depends on the amount of clotting factor that is missing.

Hemophilia is quite rare. About 1 in 10,000 people are born with it. Hemophilia is usually inherited, meaning that it is passed on through a parent’s genes. Sometimes hemophilia can occur when there is no family history of it. This is called sporadic hemophilia. About 30% of people with hemophilia did not get it through their parent’s genes. It was caused by a change in the person’s own genes.

When the father has hemophilia but the mother does not, none of the sons will inherit hemophilia, but all of the daughters will carry the gene.

Women who have the hemophilia gene are called carriers, and they can pass it on to their children. When the mother is a carrier and the father does not have hemophilia, for each child there is a 50% chance that a son will have hemophilia and a 50% chance that a daughter will carry the gene.

What are the signs of hemophilia?
- Big bruises;
- Bleeding into muscles and joints, especially the knees, elbows, and ankles;
- Sudden bleeding inside the body for no clear reason;
- Prolonged bleeding after a cut, tooth removal, surgery, or an accident.
- Serious internal bleeding into vital organs, most commonly after a serious trauma.

The most common type of hemophilia is called hemophilia A. This means the person does not have enough clotting factor VIII (8). A less common type is called hemophilia B. This person does not have enough clotting factor IX (9). The result is the same for hemophilia A and B; that is, they bleed for a longer time than normal.

People with hemophilia can bleed inside or outside the body. If bleeding occurs many times into the same joint, the joint can become damaged and painful. Repeated bleeding can cause other health problems like arthritis. This can make it difficult to walk or do simple activities.

Effective treatment for hemophilia is available, but as yet there is no cure. Hemophilia is treated by replacing the missing clotting factor in the blood. This is done by injecting a product that contains the needed factor into a vein. Bleeding stops when enough clotting factor reaches the spot that is bleeding.

With proper treatment, people with hemophilia live relatively normal lives. Without treatment, people with severe hemophilia may find it difficult to go to school or work regularly. They might become physically disabled and have trouble walking or doing simple activities - or die young. An estimated 400,000 people worldwide are living with hemophilia. Only 25% receive adequate treatment.

Quick treatment will help reduce pain and damage to the joints, muscles, and organs. If bleeding is treated quickly, less blood product is needed to stop the bleeding.

Other bleeding disorders:

von Willebrand disease

von Willebrand disease (vWD) is the most common inherited clotting disorder, affecting both men and women. It has been estimated that vWD affects up to one percent of the population. However, it is generally the least severe of the clotting disorders. vWD is caused by a deficiency or defect of a blood clotting protein called von Willebrand factor (vWF). The main symptoms are easy bruising, frequent or prolonged nosebleeds, heavy or prolonged menstrual bleeding, and prolonged bleeding following injury, surgery, dental work, or childbirth. vWD is treated with desmopressin (also called DDAVP) or with infusions of a clotting factor concentrate that contains vWF. DDAVP is generally only suitable for use in type 1 vWD.

Factor XI Deficiency

Factor XI deficiency (plasma thromboplastin antecedent) is an inherited bleeding disorder caused by a defect or lack of a protein in the blood (factor XI) that helps blood clot. The disorder is also known as hemophilia C. It differs from hemophilia A or B in that there is no bleeding into joints and muscles. Factor XI deficiency is the most common of the rare bleeding disorders, estimated at one in 100,000 people, and is the second most common bleeding disorder affecting women, after von Willebrand disease. Most people with factor XI deficiency experience little or no bleeding. Symptoms vary widely, even among family members. This makes it more difficult to diagnose.

Other bleeding disorders includes also: deficiency of Factor I, II, V, combined Factor V and Factor VIII deficiency, deficiency of Factor VII, X, and XIII.

For more information’s about bleeding disorders contact your local clinicians, societies of persons with bleeding disorders, or visit Web site of the World Federation of Hemophilia at www.wfh.org

Articles given here are for your information only. They cannot substitute clinical diagnostics or advices given by your local clinicians.