Haemophilia Centre | Auckland | Te Toka Tumai | Te Whatu Ora

Haemophilia and other Bleeding Disorders

What is Haemophilia?

Haemophilia is a rare life-long genetic blood clotting disorder that reduces the body ability to control clotting . It is a treatable condition without a cure. 

The most common of the rare are :

• Haemophilia A (problems that affect clotting Factor VIII )
• Haemophilia B (problems that affect clotting Factor IX )

Both are classified as mild , moderate or severe , depending upon the amount of factor in the body. 

Please note they are different disorders, but both conditions can cause prolonged bleeding into spaces and areas not always visible, even from mild injuries or in fact no injury. Muscles and joints are commonly affected , which can lead to rapid damage and long- term disablilty . Some people may have several joint affected by changes in responces to bleeding , particularly where this has occured in childhood . These forms involve bleeding into the muscles and joints, which can lead to rapid damage and long-term disability.

It is thought that around 85% of all bleeding episodes occur within the muscles and joints of the arms and legs. Most of these episodes occur within the hinge joints at the knees, ankles and elbows. Joints affected by repeated bleeding episodes are called 'target' joints.

This condition may have more than one family / whānau member affected. Beware in approx 1/3 of cases it will appear without a family history . From that point onwards it will travel down affected person bloodline. In very rare events can be acqured , normally later in life , that requires a different treatment due to a different cause involving the immune system.  

Other Bleeding Disorders

It is important to remember that other types of bleeding disorder, such as Von Willebrand’s Disease , are treated by the Auckland Haemophilia Centre.

Rarer bleeding disorders such as Factor I, II, V, VII, X, XI or XIII deficiencies, platelet and fibrin disorders are also managed by the Centre. Vitamin K deficiencies and other anaemias are not . 

These disorders may affect women and men. They are not usually associated with muscle and joint bleeding except in very severe cases.


More Information about Joint Bleeds
Joint bleeds are called haemarthroses. These occur as a result of trapping and tearing of the membrane that lines the joint. This membrane is called the synovium. The synovium is a structure which normally creates the nourishing, lubricating fluid inside our joints. When this membrane is damaged, blood fills the joint. A diagram of this can be found by clicking this link.

If the bleeding into the joint is not treated quickly, iron from the blood starts to affect both the cartilage and the synovium itself. Cartilage is a very strong structure that acts like a shock absorber on the surfaces of our joints. Iron from blood reduces the stability of cartilage, which can become drier, rougher and more fragmented. Iron deposits in the synovial membrane may also trigger the release of enzymes into the joint fluid which can result in damage to the cartilage.
 

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https://www.healthpoint.co.nz/public/haematology/haemophilia-centre-auckland-te-toka-tumai/