Nulibry (Fosdenopterin)

Nulibry (Fosdenopterin)

Nulibry

Fosdenopterin

For injection: 9.5 mg of fosdenopterin as a lyophilized powder or cake in a single-dose vial for reconstitution.

BridgeBio Pharma, Inc.

Medical Use

Nulibry, also known as cyclic pyranopterin monophosphate (cPMP), is used to lower the risk of death in patients with molybdenum cofactor deficiency (MoCD) Type A.

Recommended Dosage:

  • -Initiation: Begin Nulibry therapy upon diagnosis or presumptive diagnosis of MoCD Type A. Confirm the diagnosis promptly after starting treatment. If genetic testing does not confirm MoCD Type A, discontinue Nulibry.
  • -Administration: Nulibry should be administered by a healthcare professional. If deemed appropriate, a caregiver may administer it at home. It is for intravenous infusion only, using non-DEHP tubing with a 0.2 micron filter. Do not mix with other medications or infuse with other drugs.
  • -Infusion Rate: Administer Nulibry via an infusion pump at 1.5 mL/minute. For doses less than 2 mL, use a slow intravenous push. Administer within four hours of reconstitution.

Dosage for Patients Less Than One Year of Age (by gestational age):

  • -Preterm Neonates (Gestational Age < 37 Weeks):
    • -Initial: 0.4 mg/kg once daily
    • -Month 1: 0.7 mg/kg once daily
    • -Month 3: 0.9 mg/kg once daily
  • -Term Neonates (Gestational Age ≥ 37 Weeks):
    • -Initial: 0.55 mg/kg once daily
    • -Month 1: 0.75 mg/kg once daily
    • -Month 3: 0.9 mg/kg once daily

-Dosage for Patients One Year of Age or Older:

  • -For patients aged 1 year and above, the recommended dose is 9.5 mg/kg of fosdenopterin, based on actual body weight. This should be given as an intravenous infusion once daily.

Missed Dose Instructions:

  • -If a dose of Nulibry is missed, administer it as soon as possible. Ensure that the next scheduled dose is given at least six hours after the missed dose.