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Zelvysia
sapropterin
Adult Dosing .
Dosage forms: PWDR: 100 mg per pkt, 500 mg per pkt
PKU
- [5-20 mg/kg/dose PO qd]
- Start: 10-20 mg/kg/dose PO qd; Info: adjust dose based on phenylalanine levels; give w/ food; D/C tx if no decr. in phenylalanine levels at 20 mg/kg/day dose x1mo
renal dosing
- [not defined]
- renal impairment: not defined
- HD/PD: not defined
hepatic dosing
- [not defined]
Peds Dosing .
- Dosage forms: PWDR: 100 mg per pkt, 500 mg per pkt
PKU
- [1 mo-6 yo]
- Dose: 5-20 mg/kg/dose PO qd; Start: 10 mg/kg/dose PO qd; Info: adjust dose based on phenylalanine levels; give w/ food; D/C tx if no decr. in phenylalanine levels at 20 mg/kg/day dose x1mo
- [7 yo and older]
- Dose: 5-20 mg/kg/dose PO qd; Start: 10-20 mg/kg/dose PO qd; Info: adjust dose based on phenylalanine levels; give w/ food; D/C tx if no decr. in phenylalanine levels at 20 mg/kg/day dose x1mo
renal dosing
- [not defined]
- renal impairment: not defined
- HD/PD: not defined
hepatic dosing
- [not defined]
Contraindications / Cautions .
- hypersensitivity to drug or ingredient
- caution: pts <7 yo (20 mg/kg/day dose)
Drug Interactions .
Overview
sapropterin
phenylalanine hydroxylase activator
- hypotensive effects
Monitor/Modify Tx
- avanafil
- cilostazol
- dipyridamole
- isosorbide dinitrate
- isosorbide mononitrate
- levodopa
- methotrexate
- milrinone
- nitric oxide
- nitrite
- nitroglycerin
- nitroprusside
- phenobarbital
- pyrimethamine
- riociguat
- sildenafil
- tadalafil
- trimethoprim
- valproic acid
- vardenafil
Adverse Reactions .
Serious Reactions
- hypersensitivity rxn
- anaphylaxis
- esophageal ulcer
- esophageal stricture
- gastric ulcer
- GI bleeding
Common Reactions
- headache
- rhinorrhea
- pharyngolaryngeal pain
- diarrhea
- vomiting
- rhinitis
- cough
- nasal congestion
- esophagitis
- gastritis
- hyperactivity
Safety/Monitoring .
Monitoring Parameters
phenylalanine at baseline, 1wk after tx start, then periodically
Pregnancy/Lactation .
Pregnancy
Clinical Summary
benefits outweigh risks during pregnancy; no known risk of fetal harm based on limited human data; no known risk of teratogenicity based on animal data at 3x MRHD, though risk of holoprosencephaly at 10x MRHD
Lactation
Clinical Summary
weigh risk/benefit while breastfeeding; no known risk of infant harm based on limited human data; no human data available to assess effects on milk production
Pharmacology .
Metabolism: unknown; CYP450: unknown
Excretion: unknown; Half-life: 6.7h
Subclass: Inherited Metabolic Disorders
Mechanism of Action
synthetic form of tetrahydrobiopterin (BH4) activates residual phenylalanine hydroxylase, resulting in decr. phenylalanine levels
Formulary .
No Formulary Selected
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