Canadian pharmaceutical company, Medunik Canada announced that Pheburane (a tasteless oral formulation of sodium phenylbutyrate), is now available for distribution in Canada. Pheburane is the first drug to be approved in Canada for the chronic management of urea cycle disorders (UCD) after receiving market authorisation on January 27th, 2015.

"Most patients with UCD are required to use medication 3-4 times daily to prevent severe complications from their disease, so taste is critical. Increased compliance to the tasteless formulation of Pheburane allows for better management of the condition, thereby improving the overall quality of life of UCD patients," says Dr. Simerpal Gill, director of medical affairs at Medunik Canada.

"Our urea cycle disorder families and medical professionals are very excited to have Pheburane as a treatment choice that will lessen the burden of taking a previously unpalatable medication that so many struggle with on a daily basis. If a UCD patient cannot take all their medication, the disease can spiral out of control, causing brain damage or even death. We believe having access to Pheburane will help improve compliance, leading to better stability, fewer hyperammonemic crises, and improved outcomes for patients," says Tresa Warner, mother of a girl with ornithine transcarbamylase deficiency and president of the National Urea Cycle Disorders Foundation.

Pheburane (a tasteless oral formulation of sodium phenylbutyrate) is indicated as adjunctive therapy in the chronic management of urea cycle disorders, involving deficiencies of carbamylphosphate synthetase, ornithine transcarbamylase or argininosuccinate synthetase. Pheburane should be used with dietary protein restriction and, in some cases, dietary supplements (e.g., essential amino acids, arginine, citrulline, and protein-free calorie supplements).

Pheburane is indicated in patients with neonatal-onset presentation (complete enzyme deficiencies, presenting within the first 28 days of life). It is also indicated in patients with late-onset disease (partial enzyme deficiencies, presenting after the first month of life) who have a history of hyperammonemic encephalopathy.

Pheburane, developed by Lucane Pharma, is under exclusive distribution in Canada through Medunik Canada.

Medunik Canada's vision is to improve the health and quality of life of Canadians living with rare diseases.