Hemophilia (heem-o-FILL-ee-ah) is a rare, inherited condition in which a specific blood clotting protein is lacking, thus causing a delay in clotting when bleeding occurs. Hemophilia A is a deficiency in factor VIII and Hemophilia B is a deficiency in factor IX. People with hemophilia do not bleed faster, they bleed for a longer period of time. An individual with hemophilia does not “grow out” of this condition, nor, at this time is there a cure. The severity of hemophilia varies among individuals.
The person with mild hemophilia will bleed only when injured or as a result of surgery or dental extraction. The severely affected individual bleeds as a result of minor injury, or for no apparent reason. Most problems occur because of bleeding into tissues and joints where it may not be detected before pain and swelling are evident. Although the person with hemophilia is born with normal joints, repeated bleeds cause joint deterioration and early crippling.
Effective treatment is provided through either preventative transfusion of factor concentrate into the blood stream or by infusing factor concentrate promptly when bleeding occurs. These recombinant factor concentrates, that replace the missing blood clotting proteins, are provided by the Canadian Blood Services (CBS) and are distributed by the Bleeding Disorders Program. The introduction of routine preventative therapy for severely affected children; together with the introduction of recombinant factor concentrates have led to healthy joints and a normal life expectancy.