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The following recommendation was approved by the Medical and Scientific Advisory Council (MASAC) on April 22, 2006, and adopted by the NHF Board of Directors on June 3, 2006.
In view of the demonstrated benefits of prophylaxis (regular administration of clotting factor to prevent bleeding) begun at a young age in persons with hemophilia A and B, MASAC recommends that prophylaxis be considered optimal therapy for individuals with severe hemophilia A and B (factor VIII or factor IX <1%).
Prophylactic therapy should be instituted early (prior to the onset of frequent bleeding), with the aim of keeping the trough FVIII or FIX level above 1% between doses. This can usually be accomplished by giving 25-50 FVIII units/kg three times per week or every other day, or 40-100 FIX units/kg two to three times weekly.
It is also recommended that individuals on prophylaxis have regular follow-up visits to evaluate joint status, to document any complications, and to record any bleeding episodes that occur during prophylaxis.
There are no clear cut guidelines as to when to stop prophylaxis. Joint bleeds with subsequent joint destruction are a lifelong problem for these individuals. Therefore, they may need to continue prophylaxis throughout their life.
As always, a careful analysis of health risks and benefits must be performed by consumers and their health care providers. As is the case with all recommendations, MASAC will periodically reexamine this recommendation as new data emerge.