Hemophilia B


Hemophilia B - Factor IX

Hemophilia B, also called Factor IX (FIX) deficiency or Christmas disease, is a genetic disorder caused by missing or defective factor IX, a clotting protein. Most often it is an inherited bleeding disorder, passed down from parents to children, however, about 1/3 of cases are caused by a spontaneous mutation, a change in a gene. People with Hemophilia B deficiency often bleed longer than other people. Bleeds can occur internally - into joints and muscles, or externally - from minor cuts, trauma, or dental procedures. The frequency and severity of those bleeds depends on how much FIX is in the plasma, which is the straw-colored fluid portion of blood. Hemophilia B is four times less common than Hemophilia A.

The main medication to treat Hemophilia B is a concentrated FIX product, called clotting factor or simply factor. These factor therapies are infused intravenously through a vein in the arm or a port in the chest. Access to factor for the poor in developing countries is limited. Often the medical facilities simply do not have factor or it is too expensive. Persons with hemophilia living in poverty will frequently go without proper treatment and try to manage their bleeds with less effective methods such as ice, rest, and homeopathic remedies. When it is not properly treated, hemophilia can lead to severe crippling, brain damage and death.