Haemophilia is an inherited bleeding disorder. Blood contains many proteins, called clotting factors, of which the function is to stop bleeding. People with haemophilia have a low level or absence of one of these clotting factors in their blood.

The lack of a clotting factor causes people with haemophilia to bleed for longer periods than people whose blood factor levels are normal. People with haemophilia do not bleed faster than other people and will not bleed to death from a minor cut or injury. The main problem for people with haemophilia is internal bleeding, mainly into muscles and joints.

How is haemophilia treated?

The main treatment for haemophilia is called replacement therapy, which involves giving or replacing the clotting factor that’s missing or too low. Concentrates of clotting factor VIII (for haemophilia A) or clotting factor IX (for haemophilia B) are slowly dripped or injected into a vein.

Clotting factor concentrates can be made from human blood that has been treated to prevent the spread of diseases, such as hepatitis. With the new methods of screening and treating donated blood, the risk of developing an infectious disease from clotting factors taken from human blood is very small.

Some people may have replacement therapy on a regular basis to prevent bleeding. This is called preventive or prophylactic therapy. Or, some may only need replacement therapy to stop bleeding when it occurs. This use of the treatment, on an as-needed basis, is called demand therapy. Therapy that’s given as needed is less intensive and less expensive than preventive therapy. However, there is a risk that bleeding will cause damage before the as-needed treatment can start.

Complications of replacement therapy

Complications of replacement therapy include the following:

    • Developing antibodies, which are proteins that act against the clotting factors. Antibodies destroy the clotting factor before it has a chance to work. This is a very serious problem, because it makes the main treatment for haemophilia, i.e. replacing clotting factors, no longer effective. When antibodies develop, doctors may use larger doses of clotting factors or try different sources of the clotting factor.
    • Developing viral infections from human clotting factors. The viruses that cause AIDS (HIV) and hepatitis can be carried in clotting factors. However, there has been no documented case of these viruses being transmitted during replacement therapy for about a decade. Researchers continue to find ways to make blood products safer by careful screening of blood donors, testing and treating of blood products and vaccinating people with haemophilia for hepatitis A and B.
    • Damage to joints, muscles or other parts of the body resulting from delays in treatment.

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