About Haemophilia / What Is The Difference Between Haemophilia A & B?

About Haemophilia

Haemophilia is an inherited bleeding disorder that affects mostly males. Blood contains many proteins, called clotting factors, which work to stop bleeding. In people with bleeding disorders, these clotting factors are missing or do not work as they should.

The lack of clotting factor causes people with Haemophilia to bleed for longer periods of time than people whose blood factor levels are normal or work properly. 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 bleeding internally, mainly into muscles and joints.

People with mild Haemophilia may have very few bleeding episodes, and may only need to take precautions if they are having surgery or are seriously injured. However, people with severe Haemophilia often bleed spontaneously (that is, there is no obvious cause for the bleed, it just happens). Haemophilia is a lifelong inherited genetic condition, which affects females as carriers and males who inherit the condition. About a third of new diagnoses are where there is no previous family history. It appears world-wide and occurs in all racial groups. About 6,000 people are affected with Haemophilia in the UK. There are two types of Haemophilia, the most common being Haemophilia A, in which Factor VIII is lacking. In Haemophilia B, Factor IX is lacking.

With treatment products and proper care, people with Haemophilia can live perfectly healthy lives. Without treatment, Haemophilia can cause crippling pain, severe joint damage, disability, and early death. Tragically, only about 25 percent of people with Haemophilia in the world receive adequate treatment.

The treatment for Haemophilia is usually by replacement of the missing clotting factor. This is by injecting it on a regular basis (called prophylaxis) to help prevent bleeding, or injecting at the time a bleed occurs (called on-demand therapy). Regular treatment by prophylaxis - 2 or 3 times a week - helps the blood to clot and minimises the likelihood of long term joint damage. Unfortunately, there is no permanent way of replacing or increasing the clotting factor level.

From 1986, heat and chemical treatment of blood products to eliminate blood borne viruses began. A more recent development is ‘recombinant’ clotting factor, where the missing factor is produced by genetic engineering. Since 1998 all children under 16 have been treated with recombinant. Most adults in Scotland and Wales are also now being treated with recombinant.

Before 1986, much of the clotting factor derived from donated blood had been contaminated. The Haemophilia community in the UK has been severely affected by contamination of blood products leading to widespread infection of HIV and Hepatitis C. Approximately 1,200 people have been infected with HIV. Almost all were co-infected with Hepatitis C. Tragically, over half of those infected have so far died and the emotional cost to bereaved families, who have lost sons, fathers and other relatives is a modern day tragedy.

More than 4,800 people have been infected with Hepatitis C. This disease affects the functioning of the liver.