Nursing Considerations for Emicizumab Kim Drucis, BSN, MSN, CRNP Hemophilia Nurse Coordinator Johns Hopkins Department of Hematology

Emicizumab E meaning "blessing" (恵) or "picture" (絵) + MI meaning"beauty" (美).  Emi also means "(a) smile," in Japanese. 2018 Genentech, INC.

Patient Considerations: First Subcutaneous injection available for patients with Hemophilia A with and without inhibitors 3 dosing options: weekly, bimonthly, monthly Eliminates need for central lines in children, reducing infection risk and anguish associated with IV access. Limited data on surgical planning Limits use of certain lab tests (aPTT, Bethesda assay inhibitor titers, APC-R, ACT) Long half-life (~30 days)

Transitioning patients to Emicizumab ∙Discontinue aPCCs at least 24 hours before starting Emicizumab ∙Educate patients regarding use of an aPCC while on Emicizumab and limiting dose to 100u/kg/day ∙ Continue prophylaxis for some period of time during the first 1-4 weeks of Emicizumab while getting to steady state to prevent bleeding ∙Plan ahead for weekly visits as appropriate April 30, 2019

Side Effect Profile: Erythema, tenderness, warmth, pruritus, pain at injection site (22%) Headache (15%) Joint pain (15%) Fever (6%) Diarrhea (6%) April 30, 2019 https://www.gene.com/medicalprofessionals/medicines/hemlibra

Side Effects to Report: TMA Thrombosis ∙Confusion ∙Stomach (abdomen) or back pain ∙Weakness ∙Nausea/vomiting ∙Swelling of the arms/legs ∙Feeling sick ∙Yellowing of the skin/eyes ∙Decreased urination ∙Swelling in the arms/legs ∙Feel faint ∙Pain/redness in your arms/legs ∙Headache ∙Shortness of breath ∙Numbness in your face ∙Chest pain/tightness ∙Eye pain or swelling, trouble seeing ∙Fast heart rate ∙Coughing up blood April 30, 2019 https://www.gene.com/medicalprofessionals/medicines/hemlibra

Lab monitoring for TMA: D Dimer Platelet Count Serum creatinine LDH Peripheral smear to look for schistocytes April 30, 2019 https://www.gene.com/medicalprofessionals/medicines/hemlibra

Preparation and injection 4 vial sizes: 2 concentrations ∙Ready to use, do not mix concentrations ⋅Consider vial sized prescribed to reduce waste/cost ∙Instruct patient on transfer needle for preparation and SQ needle for injection https://www.gene.com/medicalprofessionals/medicines/hemlibra

Subcutaneous Injection Rotate sites, 2 ml max injection Draw up prescribed volume, no need to round or use +/-10% April 30, 2019

Additional Considerations: Store in refrigerator, take out 15 minutes prior to injection, may be stored up to 7 days out of fridge Check weights quarterly and update dosing regularly Confirm home supply of factor for bleed treatment prior to initiation Involve HTC for surgical planning due to challenges with concomitant factor/bypassing agent use and lab monitoring If discontinuing Hemlibra, it may take up to 6 months for plasma levels to completely clear April 30, 2019

Treatment of Bleeds: Inhibitor patients: Non Inhibitor Patients: Watchful waiting rVIIa (90 micrograms/kg) when treatment is recommended Avoid use of aPCC except under supervision of hematologist (50 units/kg/dose) Non Inhibitor Patients: Factor VIII replacement at routine doses Caution with tolerized inhibitor patients Follow subjective response April 30, 2019

Anti Drug Antibodies: ADA’s to emicizumab are separate from the development of inhibitors to F8 Monitor patient for clinical changes/increased bleeding Lab monitoring for anti-drug antibodies a prolonged conventional aPTT assay and/or a low factor VIII activity (by clot-based assay or chromogenic assay using human reagents) should be a useful initial evaluation April 30, 2019