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Sephience
sepiapterin
Adult Dosing .
Dosage forms: PWDR: 250 mg per pkt, 1000 mg per pkt
PKU
- [7.5-60 mg/kg/dose PO qd]
- Start: 60 mg/kg/dose PO qd; Max: 60 mg/kg/day; Info: adjust dose based on phenylalanine levels; give w/ food; D/C if no decr. in phenylalanine levels at max dose x2wk
renal dosing
- [not defined]
- renal impairment: not defined
- HD/PD: not defined
hepatic dosing
- [not defined]
Peds Dosing .
- Dosage forms: PWDR: 250 mg per pkt, 1000 mg per pkt
PKU
- [1-5 mo]
- Dose: 7.5-60 mg/kg/dose PO qd; Start: 7.5 mg/kg/dose PO qd; Max: 60 mg/kg/day; Info: adjust dose based on phenylalanine levels; give w/ food; D/C if no decr. in phenylalanine levels at max dose x2wk
- [6-11 mo]
- Dose: 7.5-60 mg/kg/dose PO qd; Start: 15 mg/kg/dose PO qd; Max: 60 mg/kg/day; Info: adjust dose based on phenylalanine levels; give w/ food; D/C if no decr. in phenylalanine levels at max dose x2wk
- [12-23 mo]
- Dose: 7.5-60 mg/kg/dose PO qd; Start: 30 mg/kg/dose PO qd; Max: 60 mg/kg/day; Info: adjust dose based on phenylalanine levels; give w/ food; D/C if no decr. in phenylalanine levels at max dose x2wk
- [2 yo and older]
- Dose: 7.5-60 mg/kg/dose PO qd; Start: 60 mg/kg/dose PO qd; Max: 60 mg/kg/day; Info: adjust dose based on phenylalanine levels; give w/ food; D/C if no decr. in phenylalanine levels at max dose x2wk
renal dosing
- [not defined]
- renal impairment: not defined
- HD/PD: not defined
hepatic dosing
- [not defined]
Contraindications / Cautions .
- hypersensitivity to drug or ingredient
- caution: peds pts
Drug Interactions .
Overview
sepiapterin
phenylalanine hydroxylase activator
- antiplatelet/anticoagulant effects
- hypotensive effects
Avoid/Use Alternative
- methotrexate
- pemetrexed
- pralatrexate
- sulfamethoxazole
- sulfasalazine
- trimethoprim
Monitor/Modify Tx
- acalabrutinib
- ado-trastuzumab emtansine
- afatinib
- alteplase
- anacaulase topical
- anagrelide
- antithrombin
- apixaban
- argatroban
- asparaginase
- aspirin
- avanafil
- avapritinib
- axitinib
- bevacizumab
- binimetinib
- bivalirudin
- brentuximab vedotin
- bromelain
- bromfenac ophthalmic
- cabazitaxel
- cabozantinib
- calaspargase
- cangrelor
- caplacizumab
- carfilzomib
- celecoxib
- cilostazol
- citalopram
- clopidogrel
- collagenase clostridium histolyticum
- cysteamine
- dabigatran
- dalteparin
- danshen
- dasatinib
- deferasirox
- defibrotide
- desvenlafaxine
- deuruxolitinib
- diclofenac
- diclofenac ophthalmic
- diclofenac topical
- diflunisal
- dimethyl fumarate
- diosmin
- dipyridamole
- diroximel fumarate
- duloxetine
- edoxaban
- encorafenib
- enoxaparin
- eptifibatide
- erlotinib
- escitalopram
- ethanol
- etodolac
- evening primrose oil
- fenoprofen
- fenugreek
- fluoxetine
- flurbiprofen
- flurbiprofen ophthalmic
- fluvoxamine
- fondaparinux
- foslevodopa
- fruquintinib
- garlic
- gefitinib
- gemtuzumab ozogamicin
- ginkgo
- heparin
- hydrocortisone
- ibrutinib
- ibuprofen
- ibuprofen lysine
- icosapent ethyl
- imatinib
- indomethacin
- isosorbide dinitrate
- isosorbide mononitrate
- ketoprofen
- ketorolac
- ketorolac ophthalmic
- lenvatinib
- levodopa
- levomilnacipran
- lifileucel
- magnesium salicylate
- meclofenamate
- mefenamic acid
- meloxicam
- methyl salicylate topical
- milnacipran
- milrinone
- mitotane
- monomethyl fumarate
- mycophenolate mofetil
- mycophenolic acid
- nabumetone
- naproxen
- nattokinase
- nepafenac ophthalmic
- niacin (vitamin B3)
- nilotinib
- nintedanib
- nitric oxide
- nitrite
- nitroglycerin
- nitroprusside
- nusinersen
- omega-3-acid
- oxaliplatin
- oxaprozin
- pacritinib
- paroxetine
- pazopanib
- pegaspargase
- penicillin G
- pentosan polysulfate sodium
- pentoxifylline
- piperacillin
- piroxicam
- pirtobrutinib
- plasminogen, human
- ponatinib
- porfimer
- pralsetinib
- prasugrel
- ramucirumab
- ranibizumab
- regorafenib
- resveratrol
- reteplase
- revakinagene taroretcel
- riociguat
- rivaroxaban
- salsalate
- saw palmetto
- selpercatinib
- selumetinib
- sertraline
- sildenafil
- sirolimus albumin-bound
- sorafenib
- sotatercept
- sulindac
- sunitinib
- tadalafil
- temsirolimus
- tenecteplase
- ticagrelor
- tipranavir
- tirofiban
- tisotumab vedotin
- tivozanib
- tolmetin
- tovorafenib
- trametinib
- trazodone
- upadacitinib
- vadadustat
- valproic acid
- vandetanib
- vardenafil
- venlafaxine
- vilazodone
- vinpocetine
- vorapaxar
- vortioxetine
- warfarin
- willow bark
- zanubrutinib
- ziv-aflibercept
Adverse Reactions .
Serious Reactions
- bleeding risk
Common Reactions
- diarrhea
- headache
- abdominal pain
- hypophenylalaninemia (peds pts)
- fecal discoloration
- oropharyngeal pain
Safety/Monitoring .
Monitoring Parameters
phenylalanine at baseline, 2wk after tx start, then periodically or more frequently in peds pts
Pregnancy/Lactation .
Pregnancy
Clinical Summary
weigh risk/benefit during pregnancy; inadequate human data available; no known risk of embryo-fetal toxicity based on animal data at up to 6x and 9x MRHD
Lactation
Clinical Summary
weigh risk/benefit while breastfeeding; no human data available to assess risk of infant harm or effects on milk production
Pharmacology .
Metabolism: various tissues; CYP450: unknown; Info: active metabolite (tetrahydrobiopterin)
Excretion: feces 26.2%, urine 6.7%; Half-life: 5h
Subclass: Inherited Metabolic Disorders
Mechanism of Action
precursor of tetrahydrobiopterin (BH4), which activates phenylalanine hydroxylase, resulting in decr. phenylalanine levels
Formulary .
No Formulary Selected
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