There are numerous complications that can occur as a result of the prolonged and uncontrolled bleeding associated with hemophilia, especially haemophilia A. These complications may require further treatment and prevention.
Some of the complications associated with haemophilia A include:
If hemophilia is poorly controlled, internal bleeding can occur around the joints and muscles leading to pain and stiffness. Eventually, bleeding in the joints can lead to damaged cartilage, which is the soft tissue that surrounds joints and acts as a shock absorber.
The synovium, a tissue layer inside the joint can also become damaged. This joint damage occurs more frequently in older adult patients with the condition because adequate treatment to prevent bleeding was not available in the past.
Today, children growing up with the condition can be treated with medications that will prevent joint damage.
In some cases of joint damage, surgery may be considered. For example, a damaged synovium can be removed to allow new synovial tissue to grow in its place.
If the joint damage is severe, it may be necessary to remove the entire joint, such as a hip or knee and replace it with an artificial one.
In some patients, the blood clotting factor medication used to treat hemophilia triggers the immune system to react because it starts to recognise clotting factors as foreign bodies.
The immune system starts to produce antibodies that will inhibit the effects of clotting factors, therefore rendering the medication less effective. These antibodies are referred to as inhibitors.
Estimates show that these inhibitors develop in around 20-30% of people who receive treatment for hemophilia A and 1-3% of those who are treated for hemophila B.
Inhibitors usually occur within the first year of treatment, although they can develop after this period.
In many cases, the inhibitors only occur temporarily, but the problem can be managed with immune tolerance therapy (ITT) which involves daily injections of octocog alfa (for haemophilia A).
Reviewed by Sally Robertson, BSc