Short answer: Bleeding disorders are a group of conditions where there is a problem with the body’s clotting process, resulting in heavy and prolonged bleeding caused by factors such as surgery or injury.
Bleeding disorders are relatively rare health conditions where a person’s blood doesn’t clot properly. If someone with a bleeding disorder starts to bleed, for example as a result of injury or surgery, they might bleed for longer than other people because their blood takes longer to clot. This can occur because there is either not enough clotting factor in the blood or a disorder within how the factor is made in the plasma.
The clotting process:
A clotting factor is a protein in the blood that controls bleeding. Many different clotting factors work together in a chain of chemical reactions to stop bleeding. This is called the clotting process or haemostasis. There are over 20 different proteins and other substances involved in the clotting process.
Types of bleeding disorders:
The most common inherited bleeding disorders are Von Willebrand disease (VWD) and Haemophilia.
Von Willebrand disease (VWD): VWD is the most common inherited bleeding disorder worldwide. It affects both males and females from all racial backgrounds. People with VWD have a problem with a protein in their blood called Von Willebrand factor which normally helps control bleeding. They do not have enough of the protein, or it does not work the way it should. It is thought that up to 1 in 100 people have VWD, but most people have few symptoms. VWD is a lifelong condition and there is no cure, but there are safe and effective treatments for all types of VWD.
Haemophilia: In Australia there are more than 3,000 people diagnosed (mostly males). There are two types:
· Haemophilia A, or classical haemophilia, is the most common form and is caused by having low levels of clotting factor VIII (8).
· Haemophilia B (sometimes called Christmas Disease) is caused by having low levels of factor IX (9).
Severe haemophilia symptoms in females are very rare due to an extra X chromosome, but they can still be carriers and pass it on to their male children. However, some females have low factor levels and bleeding symptoms, and both males and females with less than 40% of the normal level of clotting factor are now recognised as having haemophilia.
Contrary to common belief, people with haemophilia do not bleed to death from a cut or scratch! Of more concern is internal bleeding episodes, which usually occur in the joints or muscles. These ‘bleeds’ may occur spontaneously without an obvious cause, or as a result of trauma or injury. Specialised treatment with clotting factors is needed to help blood clot normally. If internal bleeding is not stopped quickly with treatment, it will result in pain and swelling. Over time prolonged bleeding into joints and muscles can cause permanent damage such as arthritis, chronic pain and joint damage requiring surgery.
People with bleeding disorders are nearly always born with them, but the exceptions are acquired haemophilia and acquired VWD, which mostly occur in adulthood. These conditions are different to inherited bleeding disorders and are extremely rare.
As with many diseases and disorders, there is a psychological impact in learning to live with them. Here at WLC Medical our psychologist David Simich, or counsellors at Keystone Counselling and Belmont Counselling, can help you navigate the psychological stressors of living with a chronic blood disorder. For medical advice, support and/or referrals for adults and children, nurse practitioner Julie McLean at Perth Paediatric and Family Clinic is also here to help.