Hemophilia is a lifelong, hereditary disorder. In 1 out of 3 cases, there is no history in the family; the cause is a new genetic mutation. Thus hemophilia can affect any family. The two types of hemophilia, A and B, affect 3,000 Canadians.
The blood of people with hemophilia doesn’t clot normally. They don’t bleed more profusely or more quickly than others…but for a longer period if not treated. External wounds are usually not serious.
Far more important is internal bleeding into joints and muscles. Untreated, this is excruciatingly painful and leads to severe crippling. When bleeding occurs in a vital organ, especially the brain, it can be fatal.
Clotting factors and other drugs are usually effective in treating people with hemophilia. Children can grow up to lead full lives. But these drugs are not a cure! And in about 30% of people with severe hemophilia, the immune system rejects the clotting factors infused to stop or prevent bleeding. This complication, called an inhibitor, can be very serious.
The most severe forms of hemophilia affect almost only males. Women who are carriers, however, often have symptoms and can have bleeding problems that affect their quality of life.
Von Willebrand Disease (VWD)
Von Willebrand disease (VWD) is the most common bleeding disorder. One in 100 Canadians (300,000 people) carry the gene for von Willebrand disease. Symptoms affect an estimated 30,000 Canadians, both male and female. Many of these people have yet to be properly diagnosed. Life-threatening hemorrhaging can occur after childbirth, surgery or trauma. A woman’s quality of life can be more seriously affected. Heavy menstrual bleeding can lead to hysterectomies. These can be avoided if the woman is properly diagnosed and treated. Effective treatments exist for von Willebrand disease.
Rare Factor Deficiencies
A small number of Canadians, fewer than 1,000, suffer from rare factor deficiencies. These people have low levels of a specific blood protein, either factor I, II, V, VII, X, XI, or XIII. Like hemophilia and VWD, these are genetic conditions for which there are treatments but no cures.
Platelet Function Disorders
There are many different kinds of platelet function disorders. In these disorders, blood platelets do not function normally, resulting in blood not clotting properly. In some individuals, bleeding can be severe.
Some people have no symptoms at all until they have a serious injury or surgery. As with von Willebrand disease, many cases go undiagnosed for decades.