Joe Pugliese of the Hemophilia Alliance, a not-for-profit organization of federally funded hemophilia treatment centers that is working to ensure its member have the expertise, resources and public support to sustain their integrated clinical and pharmacy services for persons with hemophilia and other bleeding disorders.
In this video, Pugliese explains the different types of hemophilia and their prevalence.
“In the United States, there’s about 20,000 people with either hemophilia A or hemophilia B. Around the world, there’s about 400,000 people affected by hemophilia,” state Pugliese, adding, “About one in five thousand live births of males. There are other bleeding disorders which similarly afflict both males and females, the most dominant one is called von Willebrand’s disease.”
Hemophilia A is a genetic disorder caused by missing or defective factor VIII, a clotting protein. Symptoms will vary and are dependent on the level factor VIII present. Mild hemophilia A, in which factor VIII levels are 6% – 49% in blood (normal is 50% – 150%), may go unnoticed unless there is an injury, surgery or tooth extraction results in prolonged bleeding. Moderate hemophilia A (1% – 5% of factor VIII in blood) tend to have bleeding episodes after injuries and these people may have spontaneous bleeding episodes. Severe hemophilia A (less 1% of Factor VIII in the blood) experience bleeding following an injury and may have frequent spontaneous bleeding episodes, often into their joints and muscles.
Treatment for hemophilia is factor VIII replenishment. Most hemophilia A patients take a recombinant factor VIII product to control bleeding and numerous products are available. Those with serious hemophilia are also likely to be on a prophylaxis regimen. Hemophilia B is due to missing or defective factor IX and like hemophilia A, this condition has different levels of severity based on the level of the clotting factor. Mild hemophilia B (6% – 49% of factor XI in blood), hemophilia B (1% – 5% of factor VIII in blood), and severe hemophilia B (less 1% of Factor VIII in the blood).
Also, like hemophilia A, treatment for hemophilia B is largely to replenish factor XI and numerous products are on the market for this community.
Treatment and symptom management for hemophilia is best controlled by persons highly trained in hemophilia. As such, specialty treatment centers are a vital part of this community.