Pyrukynd taper pack — Blue Cross Blue Shield of Oklahoma

Initial criteria

• The patient has a diagnosis of hemolytic anemia with pyruvate kinase deficiency (PKD) AND ALL of the following:
• A. ONE of the following:
• 1. Genetic testing showing a pathogenic PKLR gene mutation OR
• 2. The patient does NOT have two known pathogenic mutations in the PKLR gene, AND the patient has a decrease in pyruvate kinase enzyme activity AND
• B. The patient is NOT homozygous for the c.1436G > A (p.R479H) variant AND
• C. The patient has at least 2 variant alleles in the PKLR gene, of which at least 1 is a missense variant AND
• D. The patient does NOT have two non-missense mutations AND
• The patient’s age is within FDA labeling for the requested indication, OR there is support for using the requested agent for the patient’s age for the requested indication AND
• The prescriber is a specialist in the area of the patient’s diagnosis (e.g., hematologist) or has consulted with a specialist AND
• The patient does NOT have any FDA labeled contraindications to the requested agent

Reauthorization criteria

• The patient has been previously approved for the requested agent through the plan’s Prior Authorization process
• The patient has had clinical benefit with the requested agent
• The prescriber is a specialist in the area of the patient’s diagnosis (e.g., hematologist), or the prescriber has consulted with a specialist
• The patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

36 months (BCBSOK); 12 months (others)