People suffering haemophilia don’t have enough of, or are missing, one of the blood-clotting proteins naturally found in blood.
In those with haemophilia A, the clotting factor VIII is not present in sufficient amounts or is absent. They don’t bleed faster or more profusely than usual, but longer.
Clotting factors (designated by Roman numerals I through XIII) are blood proteins that work together to clot blood.
When the body detects bleeding, clotting factors are switched on in a specific order, each activating the next factor in the 'blood-clotting cascade' to ultimately form a fibrin clot, which acts like a net, holding the platelets together to create a firm blood clot.
Factor VIII is one of the clotting factor proteins that help produce the fibrin clot. In haemophilia A sufferers, whose bodies do not make sufficient factor VIII, firm blood clots don’t form in wounds, and thus bleeding is not easily stopped.
Without enough factor VIII, patients can experience spontaneous, uncontrolled internal bleeding that is painful, debilitating and damaging to the joints.
Signs and Symptoms
• Large bruises
• Bleeding in the muscles and joints, particularly in the knees, elbows and ankles
• Spontaneous bleeding
• Bleeding for a long time after getting a cut, removing a tooth or having surgery
• Serious internal bleeding into vital organs, most commonly after serious trauma
There is currently no cure for haemophilia. However, infusion therapy, commonly known as factor VIII replacement therapy, is used to treat it.
The therapy works by replacing the missing or non-functioning clotting factor VIII protein. When the clotting factor is administered after an injury, the body begins to use it immediately to form a clot to stop bleeding.
More info on HAEMOPHILIA A here.