Hemophilia B
Hemophilia B (also called “Christmas disease”) is a deficiency in clotting factor IX. Hemophilia A is 7 times more common than hemophilia B. The incidence of hemophilia B is 1 out of 34,500 men. The outcome is good with treatment and management. Most people with Hemophilia B are able to lead relatively normal lives.
Both Hemophilia A and B are treated by infusing the missing clotting factor. The amount infused depends upon the severity of bleeding, the site of the bleeding, and the size of the patient. Some plasma concentrates are intended for home use and can be self-administered, either on a regular basis to prevent bleeding or at the first sign of bleeding. More often, they are administered three times a week, but both the dose and frequency depend on the severity of the bleeding problem. The dose is adjusted according to the results of periodic blood tests. During a bleeding episode, more clotting factors are needed.
To prevent a bleeding crisis, people with hemophilia and their families can be taught to administer factor IX concentrates at home or during outdoor activities, at the first signs of bleeding. People with severe forms of the disease may need regular prophylactic infusions, which bring factor levels higher than 1% to prevent bleeds.