NHF GUIDELINES Bundle

Hemophilia B without Inhibitor ➤ The treatment of choice for individuals with hemophilia B (factor IX deficiency) is recombinant factor IX or the patient's product of choice. Plasma-derived concentrate is a suitable alternative in an emergency situation when recombinant factor IX is not available. Fresh frozen plasma is no longer recommended for treatment of individuals with hemophilia B. Cryoprecipitate does not contain factor IX. ➤ When bleeding is severe, the appropriate dose of factor IX is 100-140 100-140 units/kg units/kg. This should result in a factor IX level of 80-100%. Hemophilia A or B with Inhibitor ➤ For individuals with inhibitors (antibodies to factor VIII or IX), treatment decisions may be more complicated. The care of inhibitor patients should be urgently discussed with the patient's hematologist. If an individual with an inhibitor presents in a life- or limb-threatening scenario, the safest immediate action is to prescribe recombinant factor VIIa (rFVIIa) at a dose of 90 mcg/kg or activated prothrombin complex concentrates (FEIBA) at 75-100 units/kg.* The patient or family can also provide information on response to these therapeutic bypassing agents. * Note: rFVIIa is recommended over FEIBA for acute bleeding events or procedures in hemophilia A inhibitor patients on emicizumab prophylaxis as aPCCs may cause thrombosis or thrombotic microangiopathy in those receiving emicizumab and should be avoided. * Note: In factor IX patients with a history of inhibitors and anaphylaxis, factor IX- containing products, including FEIBA should be avoided. Additional Treatment Considerations ➤ If a patient with hemophilia or other bleeding disorder or the parent of a patient with a bleeding disorder brings clotting factor concentrate with them to the emergency department, allow them to utilize it. This recommendation acknowledges many emergency rooms do not have the majority of (or any) clotting factor concentrates on formulary. Patients or family members should be permitted to reconstitute the product and administer it whenever possible. Individuals with bleeding disorders are encouraged to have an emergency dose of factor concentrate or DDAVP (in the form of intranasal Stimate ® ) in their home and to take it with them when they travel. In those situations when a patient does not bring their own clotting factor concentrate, emergency departments must be prepared to provide clotting factor replacement. Emergency departments must have ready access to factor replacement products so that they are available within one hour of the patient's arrival. In the situation where hospital formulary factor concentrate is used, in order to expedite care, emergency department providers should order unreconstituted unreconstituted factor concentrate from their pharmacy or blood bank and reconstitute the product in the emergency department. Treatment

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