Hemophilia patients with inhibitors faced the constraint of inadequate treatment for several years before the era of
recombinant factor VIIa (rFVII). Initially,
rFVIIa was used in the compassionate-use programs. After a worldwide license was issued, more than 1.5 million doses were administered.
Bleeding of joints and muscles was controlled effectively by means of an early home treatment program, with either a standard dose of 90 μg/kg every 2 to 3 hours for a few doses or a single dose of 270 μg/kg. For more serious
bleeding episodes or
minor surgery, an initial dose of 90 μg/kg was given every 2 hours for 24 to 48 hours followed by increased intervals of 3 to 6 hours according to the severity of
bleeding and efficacy of
bleeding control. In cases of major surgery such as
orthopedic procedures, the same regimen can be applied except for a higher initial dose of 120 to 180 μg/kg. However, increasing the dose should be considered if there are unexpected
bleeding complications since the half-life and clearance of
rFVIIa differ between individuals. In addition, prophylaxis is administered to a small number of patients. Finally, the reported thromboembolic events found in
hemophilia patients with inhibitors receiving
rFVIIa are extremely low, much less than 1%.