Haemophilia & You

If you have haemophilia, information contained within this website section may help you to continue to live a normal, healthy life.

Just because you have haemophilia doesn’t mean it has to affect how you live your life. With correct and careful treatment, it is possible to ensure it has very little impact on your life

What is haemophilia?

Haemophilia is a condition that affects the body’s ability to clot blood. When someone experiences a cut or injury, their blood vessels are damaged, which causes them to bleed. When this happens, clotting factors that circulate in the blood kick-start a reaction that results in the formation of a blood clot made of a substance called fibrin that helps to seal the blood vessel and stop it from bleeding.

A person with haemophilia has lower levels of a certain clotting factor in their blood. The less clotting factor they have, the more severe their haemophilia will usually be. People with haemophilia A have less factor VIII and people with haemophilia B have less factor IX than people who don’t have the condition. This means that when a person with haemophilia bleeds, the fibrin blood clot doesn’t form properly in the blood vessel so they continue to bleed.

Factor VIII and factor IX are particularly important for forming fibrin clots in the blood vessels of joints and muscles. It is for this reason that bleeding into joints and muscles is the most serious problem for people with haemophilia

How is haemophilia diagnosed?

If there is a family history of haemophilia, a woman may decide to find out whether she is a carrier of the haemophilia gene before starting a family. Foetal tests for haemophilia are only carried out under certain circumstances during the pregnancy.

Children with severe haemophilia are usually diagnosed before they are one year old, but those with a milder form of the condition may not be diagnosed until they are much older. Young children with severe haemophilia may develop dark bruises that seem lumpy – a tell-tale sign that they may have haemophilia. They may also experience a joint or muscle bleed at this time.

If a doctor thinks that a child has haemophilia, they will do a blood test to check how much factor VIII or IX they have in their blood. If they are diagnosed with haemophilia, this test will also establish whether they have mild, moderate or severe haemophilia.

Prophylaxis vs. On-demand Therapy

Over the last few years you may have been receiving factor VIII treatment for your haemophilia as an infusion several times a week. This is known as prophylaxis which aims to prevent bleeds from happening. This differs from on-demand therapy, in which the missing factor is given once a bleed has started. This works best if the bleed is treated as soon as it starts. It is however better to prevent bleeds with propylaxis than to treat the after they occur.

However, once a bleed has occurred within a joint, the joint is more likely to experience further bleeds while it is recovering. The joint may become a ‘target joint’ and may experience repeated bleeds, which can result in long-term joint damage. It is however not only target joint that get arthropathy.

The UK Haemophilia Centre Doctors Organisation (UKHCDO) recommend that people with severe haemophilia receive prophylaxis to prevent these bleeds from happening, because this has been shown to cause less pain and better quality of life than on-demand therapy.1

Prophylaxis and sports
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References

  1. Richards M et al. Br J Haematol 2010;149(4):498-507.
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