Opfolda — Blue Cross Blue Shield of Montana

Initial criteria

• ONE of the following: A. requested agent eligible for continuation of therapy AND prescriber states patient has been treated (not sample) within past 90 days and is at risk if therapy changed OR B. ALL of the following:
• • Diagnosis of late-onset Pompe disease (acid maltase deficiency [AMD], glycogen storage disease type II [GSDII]) confirmed by ONE of: A. genetic analysis confirming biallelic mutation in GAA gene OR B. deficient acid alpha-glucosidase enzyme activity in dried blood spots, leukocytes, skin fibroblasts, or skeletal muscle tissue
• • Patient is not improving on current enzyme replacement therapy (ERT)
• • Patient weight ≥ 40 kg
• • Requested agent will be taken in combination with Pombiliti
• • If FDA labeled indication: ONE of the following: A. patient’s age within FDA labeling OR B. support exists for using agent for patient’s age