Adult Dosing

Hyperphenylalaninemia (HPA) caused by tetrahydrobiopterin-(BH4–) responsive phenylketonuria (PKU)

• 5-20 mg/kg PO per day
• Start 10 mg/kg PO daily
• Adjust dose based on Phe levels
• Max: 20 mg/kg/day (discontinue if Phe levels do not decrease at max dose for 1 month

Pediatric Dosing

Hyperphenylalaninemia (HPA) caused by tetrahydrobiopterin-(BH4–) responsive phenylketonuria (PKU)

Child >4 yrs

• 5-20 mg/kg PO per day
• Start 10 mg/kg PO daily
• Adjust dose based on Phe levels
• Max: 20 mg/kg/day (discontinue if Phe levels do not decrease at max dose for 1 month
• Note: Give with food

[Outline]

• Adult Dosing
• Pediatric Dosing

• Hyperphenylalaninemia (HPA) caused by tetrahydrobiopterin-(BH4–) responsive phenylketonuria (PKU)

• Hypersensitivity to any of the ingredients of the product

• N/A

Renal Dose Adjustment

• Renal impairment: Dose adjustments not defined. Caution advised

Hepatic Dose Adjustment

• Hepatic impairment: Dose adjustments not defined. Caution advised

• It is used in conjunction with a Phe-restricted diet
• Monitor blood Phe Levels During therapy. Identify non responders by monitoring blood Phe levels. Discontinue treatment if blood Phe does not decrease after 1 month of treatment
• Caution advised when co-administering sapropterin and medications known to inhibit Folate Metabolism because these drugs can decrease BH4 levels by inhibiting the enzyme dihydropteridine reductase
• Use with caution when co-administering sapropterin and drugs known to affect nitric oxide-mediated vasorelaxation because both may induce vasorelaxation
• Caution advised when co-administering sapropterin and levodopa as patients with underlying neurologic disorders experienced convulsions, exacerbation of convulsions, over-stimulation, or irritability during co-administration of levodopa and sapropterin

Cautions: Use cautiously in

• Hepatic impairment
• Co-administration of drugs which inhibit folate metabolism
• Co-administration of PDE- 5 inhibitors
• Co-administration of levodopa
• Hx of seizure disorder
• Geriatric population

Pregnancy Category:C

Breastfeeding: It is not known whether sapropterin is excreted in human milk. Due to the potential for serious adverse reactions in nursing infants from sapropterin and because of the potential for tumorigenicity (animal studies), a decision should be made whether to discontinue nursing or to discontinue the drug, taking into account the importance of the drug to the mother.

• CNS: Headache
• RESP: URTI, rhinorrhea, cough
• EENT: Pharyngolaryngeal pain, nasal congestion
• GI: Nausea, vomiting, abdominal pain, diarrhea
• HEMA:: Neutropenia
• MISC: Pyrexia, contusion

• Enzyme cofactor (BH4); decreases phenylalanine levels by acting as a synthetic form of the cofactor (BH4) for the enzyme phenylalanine hydoxylase (PAH) which converts phenylalanine to tyrosine
• Well absorbed following oral administration; food increases absorption
• Metabolism: Unknown
• Excretion: Unknown
• Half-life: 6.7 hrs

US Trade Name(s)

• Kuvan

US Availability

Kuvan

• TABS: 100 mg

Canadian Trade Name(s)

• Kuvan

Canadian Availability

Kuvan

• TABS: 100 mg

UK Trade Name(s)

• Kuvan

UK Availability

Kuvan

• TABS: 100 mg

Australian Trade Name(s)

• N/A

Australian Availability

• N/A

[Outline]

Endocrine/Metabolic

Metabolic Agents

Miscellaneous

Drug Name: Kuvan 1 MG/ML Oral Solution

Ingredient(s): Sapropterin

Imprint: 177

Color(s): White

Shape: Round

Size (mm): 10.00

Score: 1

Inactive Ingredient(s): ascorbic acid / crospovidone / dibasic calcium phosphate / mannitol / riboflavin / sodium stearyl fumarate

Drug Label Author:
BioMarin Pharmaceutical Inc.

DEA Schedule:
Non-Scheduled