Minimum Product Information
KUVAN® sapropterin dihydrochloride
Indications: Hyperphenylalaninemia (HPA) in sapropterin-responsive adult and paediatric patients with phenylketonuria (PKU) or tetrahydrobiopterin (BH4) deficiency.
Dosage and Administration: Administer with a meal to increase absorption. PKU: Start 10 mg/kg/day adjusted to 5 to 20 mg/kg/day based on responsiveness, single morning dose. BH4: Start 2 to 5 mg/kg/day adjusted to 2 to 20 mg/kg/day based on responsiveness; if necessary, distribute daily dose into 2-3 administrations. Prescribed number of tablet(s) or sachet(s) should be placed in a glass or cup of water or apple juice and stirred until dissolved. It may take a few minutes for the tablets to dissolve. To make the tablets dissolve faster they can be crushed. The solution obtained from tablets should be drunk within 15 to 20 minutes. The solution obtained from the sachets of powder for oral solution should be drunk within 30 minutes. KUVAN tablets may also be crushed and then mixed in a small amount of soft foods such as yoghurt or mashed banana.
Contraindications: Hypersensitivity to sapropterin or excipients.
Precautions: Treatment only by specialist physicians knowledgeable in the management of PKU and BH4 deficiency; treated patients must continue with a restricted phenylalanine and protein diet and undergo regular clinical assessment (such as monitoring of blood phenylalanine, tyrosine levels, nutrient intake, and psychomotor development); initiate treatment as early as possible; convulsions and exacerbations of convulsions in predisposed patients; cautious use in patients receiving concomitant levodopa; with intercurrent illness; limited data on long-term use, pregnancy and lactation; and use has not been established in patients with renal and hepatic insufficiency. Patients should be monitored for signs and symptoms of gastritis and oesophagitis.
Interactions: KUVAN may increase systemic exposure to drugs that are substrates for P-gp or BCRP. Caution with inhibitors of dihydrofolate reductase (e.g. methotrexate, trimethoprim), and concomitant use with vasodilating agents via nitric oxide metabolism or action (e.g. glyceryl trinitrate, isosorbide dinitrate, sodium nitroprusside); PDE-5 inhibitors; minoxidil; levodopa.
Adverse Effects: Headache, rhinorrhoea, pharyngolaryngeal pain, cough, nasal congestion, diarrhoea, vomiting, abdominal pain and hypophenylalaninaemia.
Overdose: Headache and dizziness have been reported, treatment of overdose should be directed to symptoms or contact the Poisons Information Centre on 131126 on management of over dosage.
Before prescribing, please review Product Information available from BioMarin Pharmaceutical Australia Pty Ltd, 1800 387 876.
Date of preparation: 20 Oct 2020. Based on approved Product Information 25 August 2020.