Pyrukynd — Blue Cross Blue Shield of Texas

Hemolytic anemia with pyruvate kinase deficiency (PKD)

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
If the patient has an FDA labeled indication, then ONE of the following: A. The patient’s age is within FDA labeling for the requested indication for the requested agent OR B. 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 the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND
The patient does NOT have any FDA labeled contraindications to the requested agent

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

BCBSOK: 36 months; BCBSIL, BCBSMT, BCBSTX: 12 months; All Other Plans: 6 months; Renewal: BCBSOK: 36 months; All other plans: 12 months