An introduction to the causes of haemophilia

Introduction to Haemophilia

Haemophilia is a disorder that causes excessive bleeding, due to very slow clotting of the blood. This problem is a life long disorder for the sufferer. The blood is unable to clot due the absence of a specific clotting agent called `Factor VIII', one of the many different elements neccessary for the complicated process of normal coagulation of the blood. The condition is inherited with virtually every sufferer being male. Obviously menstruation in a female haemophiliac, would be a life threatening condition as bleeding would continue uncontrolled!

In affected males, bleeding commonly occurs from blood vessels that are cut or injured, so injuries to joints are a frequent source of problems to haemophiliac sufferers. In haemophilia, the blood protein, Factor VIII which is essential for the clotting mechanism, is missing, and this is due to a defective gene. Genes are, basically, messenger carriers, which convey vital information from parents onto offspring. Half of your genes come from your father and half from your mother. Each cell in your body carries more than 20,000 genes!

The majority of male haemophiliacs have a male relative with the condition, such as a brother, uncle or grandfather, however, over one third appear to have no family history of the condition!

Once a haemophiliac child starts walking, they experience trauma to their joints as they fall and tumble. Bleeding then occurs into their joints and muscles, with resultant marked pain, bruising, swelling and discomfort in the affected, injured joints usually the knees, elbows and wrists. If this bleeding is not treated promptly, marked deformities of the joints can occur. A haemophiliac child bleeds profusely, even after the mildest of injuries, and dental treatment can result in horrendous haemorrhage!

If there is a family history, the condition can even be diagnosed before birth, in the fetus in the womb, using a technique called amniocentesis. After delivery, the condition is diagnosed by measuring the blood level of Factor VIII, which is abnormally low in haemophiliacs.

As the condition is the result of low Factor VIII, regular intravenous injections of this clotting factor can prevent flare ups of the disease. Infusions of Factor VIII immediately after an injury will also prevent severe haemorrageing, and many patients have been taught to self administer their own treatment! Years ago, before the discovery of Factor VIII deficiency, most haemophiliacs died before reaching adultdhood!

The clotting Factor VIII, has to be obtained from blood that has been donated and pooled from blood transfusions from thousands of people. During the early years of the AIDS epidemic, many haemophiliacs became infected with the AIDS virus that contaminated blood transfusions given at the time, from people undiagnosed as having AIDS. As a result, some haemophiliacs and their sexual partners have died as a result of AIDS.

Now all blood is screened for the HIV virus and all Factor VIII infusions are heat treated to kill off all possible blood borne infecting viruses.

All haemophiliac children should avoid `contact sports', such as rugby, football and martial arts. Injuries should be treated as quickly as possible, and ideally easier leisure pursuits such as swimming, hiking, artistic and musical interests, should be encouraged.
IMPORTANT NOTICE : This content is from the Dr Chris Steele personal archive and is provided for convenience only. Information contained here may no longer agree with the most up to date medical advice. Please check with a medical professional before taking any action.

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