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What is Haemophilia?

Article Summary: Haemophilia is a blood condition in which an essential clotting factor is either partly or completely missing. This causes a person with haemophilia to bleed for longer than normal. Cuts and grazes are not great problems as a little pressure and a plaster are usually enough to stop bleeding. The main problem is internal bleeding into joints, muscles and soft tissues. 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.

Haemophilia is a hereditary condition. This means that it is passed on from mother to child at the time of birth. The blood of a person with hemophilia does not clot normally. He does not bleed more profusely or more quickly than other people; however, he bleeds for a longer time.
The Society was established in 1950 and is the national charity in the UK of and for people with haemophilia, von Willebrand's and related bleeding disorders. We have over 4000 members and supporter as well as a network of 16 local groups throughout the UK. We provide information, advice and support services and we advocate and campaign to secure the best possible care and treatment.

Haemophilia in European royalty featured prominently and thus is sometimes known as "the royal disease". Queen Victoria passed the mutation to her son Leopold and, through several of her daughters, to various royals across the continent, including the royal families of Spain, Germany, and Russia. Tsarevich Alexei Nikolaevich, son of Nichola II, was a descendant of Queen Victoria and suffered from hemophilia. It was claimed that Rasputin was successful at treating the Tsarevich Alexei of Russia's hemophilia.

Hemophilia (heem-o-FILL-ee-ah) is a rare, inherited bleeding disorder in which your blood doesn’t clot normally. If you have hemophilia, you may bleed for a longer time than others after an injury. You also may bleed internally, especially in your knees, ankles, and elbows. This bleeding can damage your organs or tissues and, sometimes, be fatal.

The lack of clotting factor causes people with haemophilia to bleed for longer periods of time than people whose blood factor levels are normal. 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.

Haemophilia is used to describe continuous bleeding post-injury. This can lead to extensive haemorrhaging, typically in the joints and muscles, following injury and, in severe cases, without any recognisable signs. Joint bleeding is painful and, if left untreated, can lead to persistent joint damage, possibly culminating in permanent destruction of the joints. Muscle injuries also have widespread repercussions in haemophiliacs.

Bleeding is mostly internal. The deficiency in clotting factors is associated with a wide range of bleeding episodes, usually into the joints or muscles. These bleeding episodes, or 'bleeds', may occur spontaneously (apparently without any cause), or as a result of trauma (injury). Bleeding can be stopped by infusion of the appropriate clotting factor by intravenous injection. If internal bleeding is not quickly stopped with treatment, it will result in pain, swelling and damage to the tissues. Over a period of time, bleeding into joints and muscles causes permanent damage (such as arthritis in the joints) and chronic pain.

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Peter Hutch
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