Today, 17 April, is World Haemophilia Day, the day shining a spotlight on the global bleeding disorders community. This year, 2013, marks ’50 Years of Advancing Treatment for All’.
To quote Wikipedia, haemophilia “is a group of hereditary genetic disorders that impair the body’s ability to control blood clotting or coagulation, which is used to stop bleeding when a blood vessel is broken.” The most common type, Haemophilia A, occurs in about 1-2 in 10 000 male births, while Haemophilia B is about half as common. Both forms are more likely to occur in males than females. It is a recessive sex-linked, X chromosome disorder, and since females have two X chromosomes while men have only one, the defective gene will manifest itself in every male who carries it, while it may not manifest itself in a female carrier.
People suffering from haemophilia do not bleed more vigorously than a healthy person, but they are likely to bleed longer due to the lack of coagulation or blood clotting. Thus even a rather minor injury can result in excessive blood loss. In some injuries, such as injuries to the brain, and injuries to the insides of the joints, this can be fatal or permanently debilitating.
No cure yet exists for haemophilia, but it can be treated with regular infusion into the body of the deficient clotting factor. Sufferers also have to adapt their lifestyles and activities to minimise injury risk. Exercises to strengthen the joints, and to increase flexibility, tone and muscle strength are also recommended. There are indications that hypnosis and self-hypnosis may have some effectiveness at reducing the severity and duration of bleeding, but much investigation is still required in this regard.
As with many diseases and disorders, haemophilia impacts most severely on people living in developing countries, people who do not have access to proper care and/or treatment. It is estimated that globally 75% of people living with bleeding disorders receive very inadequate treatment, or no treatment at all, with the majority of these people living in the developing world.
Through World Haemophilia Day, it is hoped that increased awareness and support can be gained for people living with bleeding disorders, and that this can help inch us closer to the goal of quality treatment for all.