Inborn errors of metabolism:
Indications for: NULIBRY
To reduce the risk of mortality in patients with molybdenum cofactor deficiency (MoCD) Type A.
Adults and Children:
Initiate treatment if patient has a diagnosis or presumptive diagnosis; discontinue if presumptive diagnosis is not confirmed by genetic testing. Administer by IV infusion pump once daily at a rate of 1.5mL/min. <37 weeks (preterm neonates): initially 0.4mg/kg, then 0.7mg/kg at Month 1, and 0.9mg/kg at Month 3. ≥37 weeks (term neonates): initially 0.55mg/kg, then 0.75mg/kg at Month 1, and 0.9mg/kg at Month 3. ≥1yr: 0.9mg/kg.
NULIBRY Warnings/Precautions:
Avoid or minimize exposure to direct sun or UV light (eg, UVA or UVB phototherapy). Advise use of precautionary measures for photosensitivity (eg, protective clothing, hats, sunscreen). Pregnancy. Nursing mothers.
NULIBRY Classification:
Cyclic pyranopterin monophosphate (cPMP).
NULIBRY Interactions:
Do not mix or administer with other drugs.
Adverse Reactions:
Catheter-related complications, pyrexia, viral infection, pneumonia, otitis media, vomiting, cough/sneezing, viral upper respiratory infection, gastroenteritis, bacteremia, diarrhea; photosensitivity.
Generic Drug Availability:
NO
How Supplied:
Single-dose vial (5mL)—1
