Today – Wednesday, April 17 – is World Haemophilia Day and landmarks around the world will tonight be lit up in red to raise awareness and understanding of haemophilia and other bleeding disorders.
Haemophilia is an incurable bleeding disorder affecting more than 2,800 Australians (mostly male), which prevents the blood from clotting properly.
It is an inherited condition and up to two thirds of those diagnosed have a previous family history of the disorder. Interestingly, while men only pass the genes on to their daughters, women carrying the gene can pass it on to both their sons and daughters, and in most cases, sons will subsequently develop haemophilia.
It is caused by a deficiency (<40 per cent production) of one of two key clotting factors, Factor VIII (FVIII) and Factor IX (FVIX), both of which play a major role in clot formation and control of bleeding.
FVIII deficiency is known as Haemophilia A and is the most common form of the bleeding disorder, accounting for between 80-85 per cent of cases. FIX deficiency is less common and is known as Haemophilia B, or Christmas Disease.
MYTH: people with haemophilia bleed to death if they get scratched
Although there is a common misconception that people living with haemophilia will bleed to death from a cut or scratch, it is in fact internal bleeds that are their biggest concern.
Without treatment, people living with haemophilia may experience spontaneous bleeds (i.e. bleeding for no apparent reason), particularly into their joints and muscles.
Up to 80 per cent of muscle and joint bleeds occur in ‘hinged’ joints such as the knees, ankles and elbows which, over time, can result in permanent damage such as arthritis and chronic pain.
In severe cases, internal bleeds into sensitive areas such as the neck, head and the gastrointestinal region require immediate medical attention and can be life-threatening.
There have been many recent developments in the treatment of haemophilia, with new treatments continuing to come to market. The treatments currently available in Australia allow those with haemophilia to have a normal life-span.
In some cases, Australians living with the bleeding disorder are able to administer their treatment themselves at home, affording them more control and flexibility in preventing bleeds.
This World Haemophilia Day, people are being urged to reach out to those who may be living with an undiagnosed, and/or untreated bleeding disorder to join the haemophilia community and ensure they are receiving the best support and care available.
If you, or anyone you know is living with a bleeding disorder and requires support or would like more information, visit the Haemophilia Foundation Australia.