Hemophilia by Ray Sahelian, M.D.

 

Hemophilia is an inherited bleeding disorder. Blood contains many proteins, called clotting factors, which work to stop bleeding. People with hemophilia have a low level or absence of one of these clotting factors in their blood. The lack of clotting factor causes people with hemophilia to bleed for longer periods of time than people whose blood factor levels are normal. People with hemophilia do not bleed faster than other people, and will not bleed to death from a minor cut or injury. The main problem for people with hemophilia is bleeding internally, mainly into muscles and joints.

What is the difference between hemophilia A and hemophilia B?
There are two types of hemophilia: hemophilia A (sometimes called classical hemophilia) and hemophilia B (sometimes called Christmas disease). Both are caused by a low level or absence of one of the proteins in the blood (called factors) that control bleeding. Hemophilia A is caused by a deficiency of factor VIII, and hemophilia B is caused by a deficiency of factor IX. There is no difference between the two types of hemophilia, except that hemophilia B is about five times less common than hemophilia A.

 

Clotting Factor Concentrates

There is insufficient evidence from randomized controlled trials to determine whether prophylactic clotting factor concentrates decrease bleeding and bleeding-related complications in hemophilia A or B, compared to placebo, on-demand treatment, or prophylaxis based on pharmacokinetic data from individuals. 

     Factor VIII or IX replacement in a prophylactic manner is utilized for many patients with moderate to severe hemophilia A or B. Studies have shown it to be effective in reducing or preventing degenerative joint disease in many but not all patients. However, many unanswered questions still exist and optimization of this expensive treatment regimen is needed.

 

Advate for Hemophilia A

Advate (Antihemophilic Factor (Recombinant), Plasma/Albumin Free Method (rAHF-PFM), Baxter) A new 2000 IU (5 ml) ultra-high dosage is available for the prevention and control of bleeding episodes in people with hemophilia A.