• Paroxysmal nocturnal hemoglobinuria (PNH).
• Atypical hemolytic uremic syndrome (aHUS).

Contraindicated in:

• Unresolved Neisseria meninigitidis infection
• Patients not vaccinated against Neisseria meninigitidis (unless risk of delaying treatment outweighs risks of developing a meningococcal infection)
• Lactation: Lactation.

Use Cautiously in:

• OB: Use during pregnancy only if potential maternal benefit justifies potential fetal risk
• Pedi: Children <1 mo (safety and effectiveness not established).

CNS: headache, dizziness.

GI: abdominal pain, diarrhea, nausea.

Resp: upper respiratory tract infection.

MS: arthralgia.

Misc: MENINGOCOCCAL INFECTIONS, fever, Haemophilus influenzae infection, infusion reactions, Neisseria gonorrhoeae infection, Streptococcus pneumoniae infection.

Drug-Drug:

• None reported.

• Solution for injection: 10 mg/mL;

Paroxysmal Nocturnal Hemoglobinuria

• IV (Adults 100 kg): Loading dose — 3,000 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 3,600 mg every 8 wk.
• IV (Adults 60–99 kg): Loading dose — 2,700 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 3,300 mg every 8 wk.
• IV (Adults 40–59 kg): Loading dose — 2,400 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 3,000 mg every 8 wk.

Atypical Hemolytic Uremic Syndrome

• IV (Adults and Children 1 mo and 100 kg): Loading dose — 3,000 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 3,600 mg every 8 wk. Treatment should continue for 6 mo.
• IV (Adults and Children 1 mo and 60–99 kg): Loading dose — 2,700 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 3,300 mg every 8 wk. Treatment should continue for 6 mo.
• IV (Adults and Children 1 mo and 40–59 kg): Loading dose — 2,400 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 3,000 mg every 8 wk. Treatment should continue for 6 mo.
• IV (Adults and Children 1 mo and 30–39 kg): Loading dose — 1,200 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 2,700 mg every 8 wk. Treatment should continue for 6 mo.
• IV (Adults and Children 1 mo and 20–29 kg): Loading dose — 900 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 2,100 mg every 8 wk. Treatment should continue for 6 mo.
• IV (Children 1 mo and 10–19 kg): Loading dose — 600 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 600 mg every 4 wk. Treatment should continue for 6 mo.
• IV (Children 1 mo and 5–9 kg): Loading dose — 600 mg single dose (if switching from eculizumab, give loading dose 2 wk after last eculizumab dose); Maintenance dose (initiated 2 wk after loading dose) — 300 mg every 4 wk. Treatment should continue for 6 mo.

Ultomiris

• Ravulizumab is a monoclonal antibody that binds to complement protein, C5. This binding inhibits complement activation, a necessary step in the initiation of hemolysis due to PNH

• Decreased hemolysis associated with PNH.
• Decreased complement-mediated thrombotic microangiopathy in aHUS.

Therapeutic Classification: hemostatic agents

Pharmacologic Classification: complement.inhibitors, monoclonal antibodies

Absorption: IV administration results in complete bioavailability.

Distribution: Distributed to tissues.

Metabolism/Excretion: Unknown.

Half-life: 50 days.

(inhibition of serum free C5)

• Explain purpose of ravulizumab to patient. Advise patient to read Medication Guide before starting and periodically during therapy in case of changes.
• Caution patients about risk of meningococcal infection/sepsis. Advise patient to notify health care professional immediately if symptoms of meningitis or other infections occur. Inform patient of need for vaccination before starting therapy. May increase risk of infections caused by Streptococcus pneumoniae, Haemophilus influenzae, and gonorrhea. Advise patient to consult health care professional if at risk for gonorrhea infection, about gonorrhea prevention, and regular testing.
• Inform patient of REMS program. Provide REMS educational materials and ensure patients are vaccinated with meningococcal vaccines. Enrollment in the Ultomiris REMS and additional information are available by telephone: 1-888-765-4747 or at www.ultomirisrems.com.
• Advise patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and consult health care professional before taking any new medications.
• Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected and to avoid breast feeding and for at least 8 mo after last dose of ravulizumab.
• Instruct patient to carry Ultomiris Patient Safety Card that describes symptoms and patient to immediately seek medical evaluation if symptoms occur, with them at all times during and for at least 8 mo after therapy.

rav-ue-LIZ-ue-mab

NDC Code^*

• 25682- Alexion Pharmaceuticals Inc.
  • 25682-022- Ultomiris
    • 25682-022-01- 1 VIAL, GLASS in 1 CARTON (25682-022-01) > 30 mL in 1 VIAL, GLASS