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.
Hemophilia should be suspected in anyone who has easy
bruising, bleeding into joints and muscles,
spontaneous bleeding and/or prolonged bleeding.
The most common types of hemophilia are Hemophilia A
(classic hemophilia), which is a deficiency of factor
VIII, and Hemophilia B (Christmas disease), which is a
deficiency of factor IX. Hemophiliacs do not bleed
faster than others; they bleed longer because their
blood does not clot properly. Bleeding into joints,
muscles, and even vital organs, like the brain, can
occur. Bleeding can be life threatening. Over a
period of time, this bleeding can lead to chronic
pain, arthritis, deformity and disability. Although
hemophilia can occur in women (extremely rare), it
affects mostly males and occurs in 1 in every 7,500
male births.
Treatment of hemophilia is aimed at replacing the
deficient clotting factor. This is done via infusions
of concentrates containing the needed factor. 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 and more control over managing
their disorder. There are several product options for
people with hemophilia, including factor that is
synthetic as well as plasma derived.
The costs of treating hemophilia vary based on the
severity of the disease and the uniqueness of the
individual. The costs can range between $100,000 -
$200,000 per year, or more. Early recognition and
prompt treatment of bleeds can help keep these costs
down as well as reduce disability.
Hemophilia is a hereditary bleeding disorder that is
life-long. At present, there is no cure for
hemophilia. However, there are many resources
available to individuals living with this chronic
disorder.