Dosing & Uses
Dosage Forms & Strengths
injection, IV solution (single-dose vials)
- 30mg/mL
- 60mg/0.4 mL
- 105mg/0.7 mL
- 150mg/mL
Hemophilia A
Indicated for routine prophylaxis to prevent or reduce the frequency of bleeding episodes in adults with hemophilia A with or without factor VIII inhibitors
Loading dose: 3 mg/kg SC qWeek for the first 4 weeks
Maintenance dose
- 1.5 mg/kg SC qWeek, OR
- 3 mg/kg SC q2Weeks, OR
- 6 mg/kg SC q4Weeks
Selection of maintenance dose based on healthcare provider preference with consideration of regimens that may increase patient adherence
Discontinue prophylactic use of bypassing agents 1 day before starting prophylaxis
Prophylactic use of factor VIII (FVIII) products may be continued during first week of prophylaxis
See also Administration
Dosage Modifications
Renal impairment
- Mild-to-moderate (CrCl 30-89 mL/min): Pharmacokinetics are not affected
Hepatic impairment
- Mild-to-moderate (total bilirubin 1X to ≤3X ULN and any AST): Pharmacokinetics are not affected
Dosage Forms & Strengths
injection, IV solution (single-dose vials)
- 30mg/mL
- 60mg/0.4 mL
- 105mg/0.7 mL
- 150mg/mL
Hemophilia A
Indicated for routine prophylaxis to prevent or reduce the frequency of bleeding episodes in patients aged newborn and older with hemophilia A with or without factor VIII inhibitors
Loading dose: 3 mg/kg SC qWeek for the first 4 weeks
Maintenance dose
- 1.5 mg/kg SC qWeek, OR
- 3 mg/kg SC q2Weeks, OR
- 6 mg/kg SC q4Weeks
Selection of maintenance dose based on healthcare provider preference with consideration of regimens that may increase patient adherence
Discontinue prophylactic use of bypassing agents 1 day before starting prophylaxis
Prophylactic use of factor VIII (FVIII) products may be continued during first week of prophylaxis
See also Administration
Dosage Modifications
Renal impairment
- Mild-to-moderate (CrCl 30-89 mL/min): Pharmacokinetics are not affected
Hepatic impairment
- Mild-to-moderate (total bilirubin 1X to ≤3X ULN and any AST): Pharmacokinetics are not affected
Dosing Considerations
Self-administration is not recommended for children <7 years
Interactions
Interaction Checker
No Results
Contraindicated
Serious - Use Alternative
Significant - Monitor Closely
Minor
Adverse Effects
>10%
Injection site reaction (22%)
Headache (15%)
Arthralgia (15%)
Injection site erythema (11%)
1-10%
Pyrexia (6%)
Diarrhea (6%)
Injection site pruritus (4%)
Injection site pain (4%)
<1%
Rhabdomyolysis
Warnings
Black Box Warnings
Thrombotic microangiopathy and thromboembolism
- Cases of thrombotic microangiopathy (TMA) and thrombotic events reported when, on average, a cumulative amount of >100 U/kg/24 hr of activated prothrombin complex concentrate (aPCC) was administered for ≥24 hr to patients receiving emicizumab-kxwh prophylaxis
- Monitor the development of thrombotic microangiopathy and thrombotic events if aPCC is administered
- Discontinue aPCC and suspend emicizumab-kxwh if symptoms occur
Contraindications
None
Cautions
Cases of TMA reported from clinical trials; improvement was seen within 1 week following discontinuation of aPCC (see Black Box Warnings)
Thrombotic events reported from clinical trials when on average a cumulative amount of >100 U/kg/24 hr of aPCC was administered for ≥24 hr to patients receiving emicizumab-kxwh prophylaxis; no thrombotic event required anticoagulation therapy; evidence of improvement or resolution seen within one month following discontinuation of aPCC; consider benefits and risks of emicizumab-kxwh if aPCC must be used in patients receiving the drug or resuming prophylaxis following complete resolution of thrombotic event; monitor for development of thromboembolism when administering aPCC (see Black Box Warnings)
Drug interactions overview
- Clinical experience suggests that a drug interaction exists with emicizumab-kxwh and aPCC; possible for hypercoagulability with recombinant factor VIIa (rFVIIa) or Factor VIII (FVIII) with emicizumab-kxwh based on preclinical experiments
- Drug laboratory test interactions
- Intrinsic pathway clotting-based laboratory tests were affected; therefore these laboratory tests should not be used in monitoring emicizumab-kxwh; due to the long half-life of the drug, effects on coagulation essays may persist for up to 6 months after last dose
- Results affected by emicizumab: Activated aPTT; Bethesda assays (clotting-based) for FVIII inhibitor titers; one-stage, aPTT-based, single-factor assays; aPTT-based activated protein C resistance (APC-R); and activated clotting time
- Results unaffected by emicizumab: Bethesda assays (bovine chromogenic) for FVIII inhibitor titers; thrombin time; one-stage, prothrombin time-based, single-factor assays; chromogenic-based single-factor assays other than FVIII; immuno-based assays (eg, ELISA, turbidimetric methods); and genetic tests of coagulation factors (eg, factor V Leiden, prothrombin 20210)
Pregnancy
Pregnancy
No data are available regarding use in pregnant women to inform a drug-associated risk of major birth defects and miscarriage
Unknown whether treatment can cause fetal harm when administered to a pregnant woman or can affect reproduction capacity
Should be used during pregnancy only if the potential benefit for the mother outweighs the risk to the fetus
Women of childbearing potential should use contraception during treatment
Lactation
No information is available regarding the presence of emicizumab-kxwh in human milk, the effects on the breastfed child, or the effects on milk production
Human IgG is known to be present in human milk
Pregnancy Categories
A:Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B:May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.
C:Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.
D:Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.
X:Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.
NA:Information not available.
Pharmacology
Mechanism of Action
Bispecific factor IXa- and factor X-directed antibody that bridges activated factor IX and factor X in order to restore the function of missing activated factor VIII necessary for effective hemostasis
Absorption
Peak plasma concentration, steady-state: 55.1 mcg/mL (1.5 mg/kg-dose); 58.3 mcg/mL (3 mg/kg-dose); 67 mcg/mL (6 mg/kg-dose)
AUC, steady-state: 376 mcg/mL·day (1.5 mg/kg-dose); 752 mcg/mL·day (3 mg/kg-dose); 1503 mcg/mL·day (6 mg/kg-dose)
Bioavailability, 1 mg/kg dose: 80.4-93.1%
Observed similar pharmacokinetic profiles following SC administration in the abdomen, upper arm, and thigh
Distribution
Vd: 10.4L
Excretion
Clearance: 0.27 L/day
Half-life: 26.9 days
Administration
SC Preparation
Visually inspect vial for particulate matter and discoloration prior to administration; solution appears colorless to slightly yellow; do not use if particulate matter is visible or product is discolored
Do not use different vials of different concentrations when combining vials to administer prescribed dose
SC administration
SC use only
Administer doses of up to 1 mL with a 1-mL syringe (graduation 0.01 mL)
Administer doses >1 mL to ≤2 mL with a 2-mL or 3-mL syringe (graduation 0.1 mL)
Use a transparent polypropylene or polycarbonate syringe with Luer-Lok tip, sterile, for injection only, single-use, latex-free, nonpyrogenic, and commercially available in the United States
Rotate injection sites (upper outer arms, thighs, or any quadrant of abdomen) from previous injection
Do not inject into moles, scars, or areas where the skin is tender, bruised, red, hard, or not intact
Administration into the upper outer arm should only be performed by a caregiver or healthcare provider
Discard any unused drug remaining
Missed dose
- Administer as soon as possible prior to day of next scheduled dose, and then resume usual weekly dosing schedule
- Do not double doses to make up for a missed dose
Storage
Opened vials: Single-dose vials; discard any unused solution remaining after administering dose
Unopened vials
- Store in refrigerator at 2-8°C (36-46°F) in original carton to protect from light; do not freeze; do not shake
- If necessary, unopened vials may be stored at room temperature and then returned to refrigeration; temperature should not exceed 30°C (86°F) for up to 7 days
Images
Patient Handout
Formulary
Adding plans allows you to compare formulary status to other drugs in the same class.
To view formulary information first create a list of plans. Your list will be saved and can be edited at any time.
Adding plans allows you to:
- View the formulary and any restrictions for each plan.
- Manage and view all your plans together – even plans in different states.
- Compare formulary status to other drugs in the same class.
- Access your plan list on any device – mobile or desktop.
