NHF Administration of Inhibitor Bypassing Agents in the Home for Patients with Hemophilia and Inhibitors Guideline Summary

Recommendations

PwH and inhibitors should be prescribed the appropriate inhibitor bypassing agents to be administered in the home to treat or prevent bleeds associated with a personalized bleeding management plan.
PwH and inhibitors and/or their family members should be instructed in how to mix and administer inhibitor bypassing agents in a sterile manner in the home.
PwH and inhibitors and/or their family members should be educated about signs and symptoms of serious bleeding episodes and when to call their HTC care providers.
PwH and inhibitors and/or their family members should be educated about potential adverse effects of bypassing agents.
PwH and inhibitors and/or their family members should be given emergency contact numbers for HTC staff during regular hours and for 24-hour emergency on-call staff after hours.
Any adverse reaction should be reported to the HTC. In addition, adverse reactions may be reported to the pharmaceutical company and to the FDA at www.fda.gov/medwatch.

Persons with hemophilia A (factor VIII deficiency) and hemophilia B (factor IX deficiency) have frequent bleeding episodes that can be treated or prevented by infusion of the missing clotting factor (factor VIII or factor IX, respectively) or prevented with a non-factor replacement agent such as emicizumab. The most serious adverse event for patients with hemophilia A or B is the development of inhibitors, which are antibodies that neutralize infused factor VIII or factor IX so that the factor concentrate cannot stop the bleeding. This can lead to serious, even fatal bleeding episodes.
Management of Bleeding using an inhibitor bypassing agent (BPA), namely FEIBA (Anti-Inhibitor Coagulant Complex) or recombinant FVIIa (rFVIIa) product (NovoSeven RT or SEVENFACT) may be indicated in the setting of acute injury, bleeding, and around the time of surgery. These products may also be used for prophylaxis either alone or in combination with immune tolerance induction (ITI). These agents promote thrombin generation even in the absence of FVIII or FIX activity. Table 1 lists the individual product characteristics. Please review MASAC Document #272 for more details on dosing.

Administration of Inhibitor Bypassing Agents in the Home for Patients with Hemophilia and Inhibitors