Emicizumab

Indications

Emicizumab is used for: Hemophilia A

Adult Dose

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 Hepatic impairment Mild-to-moderate (total bilirubin 1X to ?3X ULN and any AST): Pharmacokinetics are not affected

Child Dose

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

Renal Dose

Renal impairment Mild-to-moderate (CrCl 30-89 mL/min): Pharmacokinetics are not affected

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)

Contra Indications

Precautions

Cases of TMA reported from clinical trials; improvement was seen within 1 week following discontinuation of aPCC 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

Pregnancy-Lactation

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

Interactions

Adverse Effects

Side effects of Emicizumab : >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

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