What is Hemophilia?
What is Hemophilia? Hemophilia is an inherited bleeding disorder that almost always affects males. A person with hemophilia has low or non-existent levels of blood clotting protein called factor. Coagulation factor is necessary for the clotting mechanism in our bodies to work.
There are 13 blood clotting proteins (coagulation factor) along with platelets and fibrin necessary for clotting blood. These 13 blood clotting proteins normally combine to form a clot. If one factor is missing or present at low levels, this causes blood clotting problems and a proper clot will not form.
Types of Hemophilia and other bleeding disorders
Hemophilia A: This person has low levels of or is missing completely factor 8 (Also called FVIII or factor VIII deficiency) 80% of people with hemophilia have Type A Hemophilia. Factor VIII deficiency usually only manifests in males.
Hemophilia B: This person has low levels of or is missing completely factor 9 (Also called FIX or factor IX deficiency) 20% of people with hemophilia have Type B Hemophilia. Factor IX deficiency usually only manifests in males.
Hemophilia C: This person has low levels of or is missing completely factor 11 (Also called FXI or factor XI deficiency) Hemophilia C is 10 times rarer than type A. Factor XI deficiency is different because it can show up in both males and females.
Von Willebrands Disease: a bleeding disease similar to Hemophilia that affects both males and females equally. Von Willebrand factor circulates attached to factor VIII and is necessary to form a clot.
Read more about types of hemophilia and other bleeding disorders here.
What is Hemophilia severity?
Severe 0% - 1% factor level. About 80% of hemophilia sufferers are considered severe.
Moderate 1% - 5% factor level. About 10% of hemophilia sufferers are considered moderate.
Mild 5% - 50% factor level. About 10% of hemophilia sufferers are considered mild.
Severity levels do not change, as in get better or worse. These levels are genetic and tend to run the same in a given family. "Normal" levels of clotting protein are 50 - 150%.
Note: Severity levels are just a benchmark. Many people with hemophilia have bleeding patterns that reflect the severity level above or below the actual diagnosis. It is believed that this is caused by how well all the other components of the blood clotting cascade are working.
What is Hemophilia Treatment?
Hemophilia is a life-long inherited bleeding disorder. However, since the mid 1990s , prophylaxis or "Prophy," as it is commonly called, has become the standard hemophilia treatment for people with severe and moderate hemophilia. Instead of infusing just when they have a prolonged bleeding time, people on prophy infuse 3 times a week in order to keep a sustainable amount of factor in their system.
Read more about the treatment of hemophilia here.
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