Medical Information
What is a Bleeding Disorder?
There are numerous bleeding disorders.
The most common ones are hemophilia and von Willebrand Disease.
Hemophilia is an inherited bleeding disorder caused by a deficiency or defect of one of the proteins necessary for blood to properly clot. These proteins are known as clotting factors. The type and severity of a person’s hemophilia depends on which protein is involved and to what degree they are deficient. The most common forms are hemophilia A (classic hemophilia), a deficiency of Factor VIII and hemophilia B (Christmas Disease), a deficiency of Factor IX. Hemophiliacs do not bleed to death from minor cuts or injuries, nor do they bleed faster; they bleed longer because their blood cannot form a firm clot. Often, bleeding is internal, into joints, and results in arthritis and crippling. Hemophilia affects mostly males and occurs in 1 of every 4000 male births.
Von Willebrand Disease is a hereditary bleeding disorder characterized by bleeding from mucous membranes (i.e. mouth, nose, throat, gastrointestinal tract) and skin surfaces. Excessive bruising, nose bleeds, heavy menstrual cycles (menorrhagia) and excessive bleeding following extraction of teeth or tonsils are the most common symptoms of von Willebrand Disease. Since it is a hereditary disorder, family history is usually positive for a bleeding tendency in other family members. Von Willebrand Disease differs from hemophilia in that it affects both males and females. It is the most common inherited bleeding disorder. About 1% to 3% of the population is affected.
Treatment
Replacing the deficient clotting factor treats hemophilia. This is done via infusions of plasma derived factor concentrates, further purified by monoclonal antibody procedures or artificially produced through recombinant DNA technology. Many patients are on a home infusion program whereby they self-infuse the clotting factor concentrate when they bleed. This ensures prompt treatment and gives them greater independence. The cost of treating severe hemophilia can run as high as $250,000 per year.
Complications
The most common clinical complication of hemophilia is damage to the joints that can occur when there is bleeding into the joints. Bleeding into the knees, elbows, ankles, shoulders, and hips can lead to chronic swelling and later deformity.
People with hemophilia are treated with blood products derived from thousands of donors. For this reason they are at risk of being exposed to viruses.
Hepatitis viruses were also often transmitted in blood products used by persons with hemophilia. These hepatitis viruses caused problems from mild chronic infections to liver failure.
The contamination of the nation’s blood supply with HIV (Human Immunodeficiency Virus) that causes
AIDS (Acquired Immune Deficiency Syndrome) in the early 1980’s was devastating to the hemophilia community. Since the mid 1980’s new screening and processing procedures have significantly decreased the threat to our nation’s blood supply.