Kuvan is indicated to reduce blood phenylalanine (Phe) levels in patients with hyperphenylalaninemia due to tetrahydrobiopterin responsive phenylketonuria (PKU) and is to be used in conjunction with a Phe-restricted diet. To determine if there is a response to Kuvan, the recommended starting dose of Kuvan is 10mg/kg/day taken once daily for up to a month. If there is no response, the drug dose may be increased to 20mg/kg/day for up to a month. The dose may be adjusted within a range of 5mg/kg/day to 20mg/kg/day in patients who respond to Kuvan. Kuvan is developed in partnership with Merck Serono, a division of Merck KGaA, Darmstadt, Germany.
Jean-Jacques Bienaime, CEO of BioMarin, said: “The approval of Kuvan represents an important milestone for PKU patients and their families and also for BioMarin. We are extremely pleased to bring this promising treatment option to market in just a little over three years since the IND filing, and we are now ready for an immediate launch. We would like to thank all the patients, their families and physicians, our corporate partners, the FDA and BioMarin employees for their hard work and dedication in making Kuvan a reality.”