Pyrukynd — Blue Cross Blue Shield of Montana

Initial criteria

• The patient has a diagnosis of hemolytic anemia with pyruvate kinase deficiency (PKD) AND ALL of the following:
• ONE of the following: genetic testing showing a pathogenic PKLR gene mutation OR the patient does NOT have two known pathogenic mutations in the PKLR gene AND the patient has a decrease in pyruvate kinase enzyme activity
• The patient is NOT homozygous for the c.1436G > A (p.R479H) variant
• The patient has at least 2 variant alleles in the PKLR gene, of which at least 1 is a missense variant
• The patient does NOT have two non-missense mutations
• If the patient has an FDA labeled indication, ONE of the following: 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
• The prescriber is a specialist in the area of the patient’s diagnosis (e.g., hematologist) or has consulted with a specialist in the area of the diagnosis
• The patient does NOT have any FDA labeled contraindications to the requested agent
• For members residing in Ohio with Fully Insured or HIM Shop (SG) plans, approval also requires: no FDA labeled contraindications AND ONE of the following: another FDA labeled indication, another compendia-supported indication, OR submission of two peer-reviewed medical journal articles supporting the proposed use

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 has consulted with such a specialist
• The patient does NOT have any FDA labeled contraindications to the requested agent

Approval duration

BCBSOK 36 months; BCBSIL/BCBSMT/BCBSTX 12 months; All Other Plans 6 months (initial); BCBSOK 36 months; all others 12 months (renewal)