Version May 2024
Molybdenum cofactor deficiency (MoCD) type A: Note: Initiate therapy with confirmed or presumptive diagnosis of MoCD type A. Confirm presumptive diagnosis as soon as possible; discontinue treatment if genetic testing does not confirm diagnosis.
GA <37 weeks: IV: Initial: 0.4 mg/kg/dose once daily for 1 month, then increase to 0.7 mg/kg/dose once daily for 2 months, then increase to target dose of 0.9 mg/kg/dose once daily.
GA ≥37 weeks: IV: Initial: 0.55 mg/kg/dose once daily for 1 month, then increase to 0.75 mg/kg/dose once daily for 2 months, then increase to target dose of 0.9 mg/kg/dose once daily.
Molybdenum cofactor deficiency (MoCD) type A: Note: Initiate therapy with confirmed or presumptive diagnosis of MoCD type A. Confirm presumptive diagnosis as soon as possible; discontinue treatment if genetic testing does not confirm diagnosis.
Infants: IV: Initial: 0.55 mg/kg/dose once daily for 1 month, then increase to 0.75 mg/kg/dose once daily for 2 months, then increase to target dose of 0.9 mg/kg/dose once daily.
Children and Adolescents: IV: 0.9 mg/kg/dose once daily.
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
(For additional information see "Fosdenopterin: Drug information")
Molybdenum cofactor deficiency type A: IV: 0.9 mg/kg once daily (using actual body weight).
Missed doses: If a dose is missed, administer the missed dose as soon as possible; the next scheduled dose should be administered at least 6 hours after the missed dose.
There are no dosage adjustments provided in the manufacturer's labeling (has not been studied).
There are no dosage adjustments provided in the manufacturer's labeling (has not been studied).
Animal studies suggest that use of fosdenopterin may result in sensitivity to sunlight and UVA or UVB phototherapy.
Mechanism: Unknown
Risk factors:
• Exposure to direct sunlight or artificial UV light without the use of precautionary measures (eg, use of protective clothing and hats, broad spectrum sunscreen with a high protection factor [SPF], sunglasses).
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.
Frequency not defined:
Dermatologic: Maculopapular rash
Gastrointestinal: Abdominal pain, diarrhea, gastroenteritis, vomiting
Hematologic & oncologic: Anemia
Infection: Bacteremia, influenza, viral infection
Local: Catheter complication (catheter site complications)
Nervous system: Agitation, seizure
Ophthalmic: Swelling of eye
Otic: Otitis media
Respiratory: Cough, lower respiratory tract infection, oropharyngeal pain, pneumonia, sneezing, tonsillitis (viral), viral upper respiratory tract infection
Miscellaneous: Fever
There are no contraindications listed in the manufacturer's labeling.
Concerns related to adverse effects:
• Photosensitivity: May increase sensitivity to sunlight; avoid or minimize exposure to direct sunlight and artificial UV light exposure (including UVA or UVB phototherapy), and utilize precautionary measures (eg, protective clothing, hat, sunglasses); use a broad spectrum sunscreen with high sun protection factor in patients ≥6 months. If photosensitivity occurs, patients should seek medical attention immediately with consideration of dermatological evaluation.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling. [DSC] = Discontinued product
Solution Reconstituted, Intravenous, as hydrobromide:
Nulibry: 9.5 mg (1 ea)
Solution Reconstituted, Intravenous, as hydrobromide [preservative free]:
Nulibry: 9.5 mg (1 ea [DSC]) [latex free]
No
Solution (reconstituted) (Nulibry Intravenous)
9.5 mg (per each): $1,785.60
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Parenteral: IV: If reconstituted solution is refrigerated following preparation, allow solution to come to room temperature prior to administration. Do not heat; do not refreeze. Administer with non-DEHP tubing through a 0.2 micron filter via infusion pump at a rate of 1.5 mL/minute. Dose volumes <2 mL may require slow IV push via syringe. Administration must be completed within 4 hours of preparation.
Missed dose: If a dose is missed, administer the dose as soon as possible and then administer next dose ≥6 hours after administration of missed dose.
IV: If reconstituted vial has been refrigerated, allow to come to room temperature (by hand warming for 3 to 5 minutes or exposing to ambient air for ~30 minutes) before administration; do not heat. Administer by IV infusion at 1.5 mL/minute through a 0.2 micron filter with non–di-2-ethylhexyl phthalate (DEHP) tubing; complete infusion within 4 hours after reconstitution. Do not mix or administer with other medications. May be administered by caregiver/patient if deemed appropriate following necessary training and instruction.
Store intact vials frozen at −25°C to −10°C (−13°F to 14°F) in original carton to protect from light. Following reconstitution, may store at room temperature (15°C to 25°C [59°F to 77°F]) or refrigerated (2°C to 8°C [36°F to 46°F]) for up to 4 hours, including infusion time; discard any unused portion. Do not heat or refreeze reconstituted solution.
To reduce the risk of mortality in patients with molybdenum cofactor deficiency (MoCD) type A (FDA approved in all ages).
None known.
There are no known significant interactions.
Animal reproduction studies have not been conducted.
Monitor for rash or development of photosensitivity reactions (redness, burning sensation of skin, blisters); if this occurs seek medical attention immediately.
Fosdenopterin is a cyclic pyranopterin monophosphate that undergoes conversion to molybdopterin, which is further converted to molybdenum cofactor. Molybdenum cofactor is required for molybdenum-dependent enzyme activation, including sulfite oxidase, which reduces levels of neurotoxic sulfites (eg, S-sulfocysteine).
Note: Pharmacokinetic data is based on a single infusion administered to healthy adult subjects.
Distribution: Vd: ~300 mL/kg.
Protein binding: 6% to 12%.
Metabolism: Primarily via nonenzymatic degradation processes to compound Z, an inactive oxidation product of endogenous cyclic pyranopterin monophosphate.
Half-life elimination: 1.2 to 1.7 hours.
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