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

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

3. 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)

4. 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

5. Side Effect Profile:
Erythema, tenderness, warmth, pruritus, pain at injection site (22%)
Headache (15%)
Joint pain (15%)
Fever (6%)
Diarrhea (6%)
April 30,

6. 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,

7. Lab monitoring for TMA:
D Dimer
Platelet Count
Serum creatinine
LDH
Peripheral smear to look for schistocytes
April 30,

8. 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

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

10. 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

11. 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

12. 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