Usual Adult Dose for Paroxysmal Nocturnal Hemoglobinuria

600 mg IV every week for 4 weeks, followed by 900 mg IV at week 5, then 900 mg IV every 2 weeks

Comments:

• Administer this drug IV over 35 minutes in adults.
• Administer this drug at the recommended time, or within 2 days of the recommended time.
• If an adverse reaction occurs, 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 2 hours in adults.
• The patient should be monitored for infusion reactions for at least 1 hour following the infusion.
• Monitor patients with PNH for hemolysis for at least 8 weeks after the final dose.

Use: For paroxysmal nocturnal hemoglobinuria (PNH) to reduce hemolysis

Usual Adult Dose for Hemolytic Uremic Syndrome

900 mg IV every week for the first 4 weeks, followed by 1200 mg IV at week 5, then 1200 mg IV every 2 weeks

Comments:

• Administer this drug IV over 35 minutes in adults.
• Administer this drug at the recommended time, or within 2 days of the recommended time.
• If an adverse reaction occurs, 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 2 hours in adults.
• The patient should be monitored for infusion reactions for at least 1 hour following the infusion.
• Monitor patients with aHUS for thrombotic microangiopathy (TMA) complications for at least 12 weeks after the final dose.

Use: For atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy

Usual Adult Dose for Myasthenia Gravis

900 mg IV every week for the first 4 weeks, followed by 1200 mg IV at week 5, then 1200 mg IV every 2 weeks

Comments:

• Administer this drug IV over 35 minutes in adults.
• Administer this drug at the recommended time, or within 2 days of the recommended time.
• If an adverse reaction occurs, 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 2 hours in adults.
• The patient should be monitored for infusion reactions for at least 1 hour following the infusion.

Use: For patients with generalized myasthenia gravis (gMG) who are anti-acetylcholine receptor (AchR) antibody positive

Usual Adult Dose for Neuritis

900 mg IV every week for the first 4 weeks, followed by 1200 mg IV at week 5, then 1200 mg IV every 2 weeks

Comments:

• Administer this drug IV over 35 minutes in adults.
• Administer this drug at the recommended time, or within 2 days of the recommended time.
• If an adverse reaction occurs, 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 2 hours in adults.
• The patient should be monitored for infusion reactions for at least 1 hour following the infusion.

Use: For the treatment of neuromyelitis optica spectrum disorder (NMOSD) in adult patients who are anti-aquaporin-4 (AQP4) antibody positive

Usual Pediatric Dose for Hemolytic Uremic Syndrome

2 months and older:

• Weight 5 kg to less than 10 kg: 300 mg IV for the first dose, followed by 300 mg IV at week 2, then 300 mg IV every 3 weeks
• Weight 10 kg to less than 20 kg: 600 mg IV for the first dose, followed by 300 mg IV at week 2, then 300 mg IV every 2 weeks
• Weight 20 kg to less than 30 kg: 600 mg IV every week for the first 2 doses, followed by 600 mg IV at week 3, then 600 mg IV every 2 weeks
• Weight 30 kg to less than 40 kg: 600 mg IV every week for the first 2 doses, followed by 900 mg IV at week 3, then 900 mg IV every 2 weeks
• Weight 40 kg or greater: 900 mg IV every week for the first 4 doses, followed by 1200 mg IV at week 5, then 1200 mg IV every 2 weeks

Comments:

• Administer this drug IV over 1 to 4 hours in pediatric patients.
• Administer this drug at the recommended time, or within 2 days of the recommended time.
• If an adverse reaction occurs, 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 4 hours in pediatric patients.
• The patient should be monitored for infusion reactions for at least 1 hour following the infusion.
• Monitor patients with aHUS for thrombotic microangiopathy (TMA) complications for at least 12 weeks after the final dose.

Use: For atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy

Renal Dose Adjustments

No adjustment recommended.

Liver Dose Adjustments

Data not available

Dose Adjustments

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 or MNOSD, supplemental dosing is required in the setting of concomitant plasmapheresis or plasma exchange, or fresh frozen plasma infusion (PE/PI):
SUPPLEMENTAL DOSING FOR PATIENTS RECEIVING PLASMAPHERESIS OR PLASMA EXCHANGE:

• If the most recent dose was 300 mg, administer 300 mg within 60 minutes after each plasmapheresis or plasma exchange.
• If the most recent dose was 600 mg or more, administer 600 within 60 minutes after each plasmapheresis or plasma exchange.

SUPPLEMENTAL DOSING FOR PATIENTS RECEIVING FRESH FROZEN PLASMA INFUSION (PI):

• If the most recent dose was 300 mg or more, administer 300 mg within 60 minutes prior to each infusion of fresh frozen plasma.

Precautions

The US FDA requires a Risk Evaluation and Mitigation Strategy (REMS) for eculizumab. It includes elements to assure safe use. For additional information: http://www.accessdata.fda.gov/scripts/cder/rems/index.cfm

US BOXED WARNINGS:
SERIOUS MENINGOCOCCAL INFECTIONS:

• Life-threatening and fatal meningococcal infections have been reported. Meningococcal infection may become rapidly life-threatening or fatal if not recognized and treated early. Consult with the most current Advisory Committee on Immunization Practices (ACIP) recommendations for meningococcal vaccination in patients with complement deficiencies.
• Patients should be immunized with a meningococcal vaccine at least 2 weeks prior to administering the first dose of this drug, unless the risk of delaying therapy outweighs the risk of developing a meningococcal infection.
• Patients should be monitored for early signs of meningococcal infections and evaluated immediately if infection is suspected.

CONTRAINDICATIONS:

• Unresolved serious Neisseria meningitidis infection
• Patients who are not currently vaccinated against Neisseria meningitidis, unless the risks of delaying this drug outweigh the risks of developing a meningococcal infection

• Safety and efficacy have not been established for the treatment of paroxysmal nocturnal hemoglobinuria (PNH), generalized Myasthenia Gravis (gMG), or neuromyelitis optica spectrum disorder (NMOSD) in pediatric patients younger than 18 years.
• Safety and efficacy have not been established for the treatment of hemolytic uremic syndrome (aHUS) in pediatric patients younger than 2 months.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

General:

• This drug is not indicated for the treatment of patients with Shiga toxin Escherichia coli related hemolytic uremic syndrome (STEC-HUS).
• One of the eligibility requirements for therapy with this drug is meningococcal vaccination. Patients without a history of meningococcal vaccination should be vaccinated with a polyvalent vaccine at least 2 weeks prior to receiving the first dose and be revaccinated according to national vaccination guidelines.
• This drug should only be administered as an IV infusion via gravity feed, a syringe-type pump, or an infusion pump.
• This drug should not be administered as an IV push or bolus.
• If an adverse reaction occurs during administration, the infusion may be slowed or stopped. If the infusion is slowed, the total infusion time should not exceed 2 hours.
• Patients should be monitored for at least one hour following completion of the infusion for signs of an infusion reaction.
• Atypical hemolytic uremic syndrome patients should be monitored for signs of thrombotic microangiopathy (TMA).
• This drug should be given for the duration of the patient's life, unless discontinuation is clinically indicated.

Frequently asked questions

• How does Empaveli compare to Soliris?