sodium phenylbutyrate — Medica

Initial criteria

• Diagnosis was confirmed by ONE of the following (i or ii):
• i. Genetic or enzymatic testing confirmed a urea cycle disorder; OR
• ii. The patient has hyperammonemia diagnosed with an ammonia level above the upper limit of the normal reference range for the reporting laboratory (reference ranges are dependent upon patient’s age).
• The medication is prescribed in conjunction with a protein-restricted diet.
• Patient will not be receiving concurrent therapy with another phenylbutyrate product (examples include sodium phenylbutyrate [Buphenyl, generic], Pheburane, Olpruva, and Ravicti).
• The medication is prescribed by or in consultation with a metabolic disease specialist (or specialist who focuses in the treatment of metabolic diseases).

Reauthorization criteria

• Reauthorization may be granted if the diagnosis remains confirmed by genetic or enzymatic testing (previously established) and the patient continues to meet initial criteria including dietary management, non-concurrent phenylbutyrate therapy, and specialist oversight.

Approval duration

Approve for 1 year if genetic or enzymatic testing confirmed a urea cycle disorder; approve for 3 months if hyperammonemia diagnosis only.