Epysqli Dosage

Recommended Vaccination and Prophylaxis for Meningococcal Infection

Vaccinate patients against meningococcal infection (serogroups A, C, W, Y, and B) according to current ACIP recommendations at least 2 weeks prior to initiation of EPYSQLI.

If urgent EPYSQLI therapy is indicated in a patient who is not up to date with meningococcal vaccines according to ACIP recommendations, provide the patient with antibacterial drug prophylaxis and administer these vaccines as soon as possible.

Healthcare providers who prescribe EPYSQLI must enroll in the EPYSQLI REMS.

Recommended Dosage Regimen – PNH

For patients 18 years of age and older, EPYSQLI therapy consists of:

• 600 mg weekly for the first 4 weeks, followed by
• 900 mg for the fifth dose 1 week later, then
• 900 mg every 2 weeks thereafter.

Administer EPYSQLI at the recommended dosage regimen time points, or within two days of these time points.

Recommended Dosage Regimen – aHUS

For patients 18 years of age and older, EPYSQLI therapy consists of:

• 900 mg weekly for the first 4 weeks, followed by
• 1,200 mg for the fifth dose 1 week later, then
• 1,200 mg every 2 weeks thereafter.

For patients less than 18 years of age, administer EPYSQLI based upon body weight, according to the following schedule (Table 1):

Administer EPYSQLI at the recommended dosage regimen time points, or within two days of these time points.

Recommended Dosage Regimen – gMG

​For adult patients with generalized myasthenia gravis, EPYSQLI therapy consists of:

• ​900 mg weekly for the first 4 weeks, followed by
• ​1,200 mg for the fifth dose 1 week later, then
• ​1,200 mg every 2 weeks thereafter.

​Administer EPYSQLI at the recommended dosage regimen time points, or within two days of these time points.

Dose Adjustment in Case of Plasmapheresis, Plasma Exchange, or Fresh Frozen Plasma Infusion

​For adult and pediatric patients with aHUS, and adult patients with gMG, supplemental dosing of EPYSQLI is required in the setting of concomitant plasmapheresis or plasma exchange, or fresh frozen plasma infusion (PE/PI) (Table 2).

Preparation

Dilute EPYSQLI to a final admixture concentration of 5 mg/mL using the following steps:

• Withdraw the required amount of EPYSQLI from the vial into a sterile syringe.
• Transfer the recommended dose to an infusion bag.
• Dilute EPYSQLI to a final concentration of 5 mg/mL by adding the appropriate amount (equal volume of diluent to drug volume) of 0.9% Sodium Chloride Injection, USP; 0.45% Sodium Chloride Injection, USP; or 5% Dextrose in Water Injection, USP to the infusion bag.

The final admixed EPYSQLI 5 mg/mL infusion volume is 60 mL for 300 mg doses, 120 mL for 600 mg doses, 180 mL for 900 mg doses or 240 mL for 1,200 mg doses (Table 3).

Gently invert the infusion bag containing the diluted EPYSQLI solution to ensure thorough mixing of the product and diluent. Discard any unused portion left in a vial, as the product contains no preservatives.

Prior to administration, the admixture should be allowed to adjust to room temperature [18°C to 25°C (64°F to 77°F)]. The admixture must not be heated in a microwave or with any heat source other than ambient air temperature.

Parenteral drug products should be inspected visually for particulate matter and discoloration prior to administration, whenever solution and container permit.

Administration

Only administer as an intravenous infusion.

Do not administer as an intravenous push or bolus injection.

Administer the EPYSQLI admixture by intravenous infusion over 35 minutes in adults and 1 to 4 hours in pediatric patients via gravity feed, a syringe-type pump, or an infusion pump. Admixed solutions of EPYSQLI are stable for 24 hours refrigerated at 2°C to 8°C (36°F to 46°F) and at room temperature.

If an adverse reaction occurs during the administration of EPYSQLI, the infusion may be slowed or stopped at the discretion of the physician. If the infusion is slowed, the total infusion time should not exceed two hours in adults. Monitor the patient for at least one hour following completion of the infusion for signs or symptoms of an infusion-related reaction.