Anemias:

Indications for ULTOMIRIS:

Treatment of paroxysmal nocturnal hemoglobinuria (PNH). Treatment of atypical hemolytic uremic syndrome (aHUS) to inhibit complement-mediated thrombotic microangiopathy (TMA).

Limitations of Use:

Not for treating Shiga toxin E. coli-related HUS.

Adults and Children:

Give by IV infusion; monitor for ≥1hr after completion. Give initial loading dose (LD), followed by maintenance doses (MD) 2 weeks later. PNH: <18yrs: not established. ≥18yrs (≥40–<60kg): LD: 2400mg; MD: 3000mg every 8wks; (≥60–<100kg): LD: 2700mg; MD: 3300mg every 8wks; (≥100kg): LD: 3000mg; MD: 3600mg every 8wks. aHUS: <1month: not established. Treat for a minimum of 6 months. ≥1month (≥5–<10kg): LD: 600mg; MD: 300mg every 4wks; (≥10–<20kg): LD: 600mg; MD: 600mg every 4wks; (≥20–<30kg): LD: 900mg; MD: 2100mg every 8wks; (≥30–<40kg): LD: 1200mg; MD: 2700mg every 8wks; (≥40–<60kg): LD: 2400mg; MD: 3000mg every 8wks; (≥60–<100kg): LD: 2700mg; MD: 3300mg every 8wks; (≥100kg): LD: 3000mg; MD: 3600mg every 8wks. Other dosing considerations: see full labeling.

Contraindications:

Unresolved Neisseria meningitidis infections. Individuals not vaccinated against Neisseria meningitidis, unless the risks of delaying ravulizumab therapy outweigh the risks of developing a meningococcal infection.

Boxed Warning:

Serious meningococcal infections.

Warnings/Precautions:

Increased risk of serious meningococcal infections (septicemia and/or meningitis). Vaccinate or revaccinate for meningococcal disease according to ACIP guidelines. Immunize patients without a history of meningococcal vaccination at least 2 weeks prior to treatment. Give 2 weeks of antibacterial prophylaxis if ravulizumab must be initiated immediately and vaccines administered <2 weeks before starting therapy. Monitor closely for signs of meningococcal infection; evaluate immediately if infection is suspected. Discontinue ravulizumab if undergoing treatment for meningococcal infection. Risk of other infections (eg, due to N. species [including gonococcal infections], S. pneumoniae, H. influenza); monitor closely for signs of worsening infection. PNH: risk of hemolysis and other reactions (eg, thrombosis) after treatment discontinuation; monitor closely for at least 16 weeks; if hemolysis signs/symptoms occur, consider restarting ravulizumab. aHUS: risk of TMA complications after treatment discontinuation; monitor for at least 12 months; if TMA occurs, consider reinitiating ravulizumab or organ-specific supportive measures. Pregnancy. Nursing mothers: not recommended (during and for 8 months after last dose).

Pharmacologic Class:

Complement inhibitor.

Interactions:

Administration of PE/PI (plasmapheresis or plasma exchange, or fresh frozen plasma) may reduce ravulizumab serum levels.

Adverse Reactions:

Upper RTI, headache, diarrhea, nausea, vomiting, headache, hypertension, pyrexia; meningococcal infection (may be fatal), infusion reactions (interrupt infusion if cardiovascular instability or respiratory compromise occur).

REMS:

YES

Generic Availability:

NO

How Supplied:

Single-dose vial (30mL)—1