NHF GUIDELINES Bundle

Emergency Management of Bleeding Disorders

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(factor VIII >5%) with Non- Threatening Bleeding previously responsive treatment is the same as for with hemophilia A. with a bleeding disorder triage clotting factor if: eyes or evidence of bleeding in these areas. following trauma. adhesive, or steri-strips. internal bleeding. severe anemia or volume instability. Hemophilia B without Inhibitor • Recombinant factor IX or else the patient's product of choice. • Plasma-derived concentrate is a suitable alternative in an emergency situation when recombinant Factor IX is not available. • When bleeding is severe, the appropriate dose of factor IX is 100-140 units/kg. (This should result in a factor IX level of 80-100%.) hematologist or a regional hemophilia treatment center decisions may be more complicated. The care of inhibitor patients should be inhibitor presents in a life- or limb-threatening scenario, the safest immediate activated prothrombin complex concentrates (FEIBA) at 75-100 units/kg.* The response to these therapeutic bypassing agents. in hemophilia A inhibitor patients on emicizumab prophylaxis as aPCCs may cause those receiving emicizumab and should be avoided. anaphylaxis, factor IX-containing products, including FEIBA should be avoided.

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