Strensiq (asfotase alfa) — Blue Cross Blue Shield of Montana

Initial criteria

• 1. The patient has a diagnosis of either perinatal/infantile- OR juvenile-onset hypophosphatasia (HPP) AND ALL of the following:
• A. The patient was less than 18 years of age at onset [chart notes required]
• B. The patient is experiencing active disease (e.g., bone pain, fractures, gait problems)
• C. The patient has/had clinical manifestations consistent with hypophosphatasia at the age of onset prior to age 18 (e.g., vitamin B6-dependent seizures, fractures, lost teeth with roots, skeletal abnormalities such as rachitic chest deformity leading to respiratory problems or bowed arms/legs, “failure to thrive”) [chart notes required]
• D. ONE of the following [chart notes required]:
• 1. Molecular genetic testing has been completed confirming mutations in the ALPL gene that encodes the tissue nonspecific isoenzyme of ALP (TNSALP) OR
• 2. ALL of the following:
• A. The patient has/had radiographic imaging confirming the diagnosis of hypophosphatasia at the age of onset prior to age 18 (e.g., infantile rickets, alveolar bone loss, craniosynostosis)
• B. The patient has reduced activity of unfractionated serum alkaline phosphatase (ALP) in the absence of bisphosphonate therapy (i.e., below the normal lab reference range for age and sex)
• C. ONE of the following:
• 1. Elevated serum concentration of pyridoxal 5'-phosphate (PLP) in the absence of vitamin supplements within one week prior to the test OR
• 2. Elevated urine concentration of phosphoethanolamine (PEA) OR
• 3. Elevated urinary inorganic pyrophosphate (PPi)
• 2. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist, geneticist) or has consulted with such a specialist
• 3. The patient has had an ophthalmology examination and renal ultrasound at baseline (prior to starting therapy)
• 4. The patient does NOT have any FDA labeled contraindications to the requested agent
• 5. ONE of the following:
• A. The requested quantity (dose) is within FDA labeled dosing for the requested indication based on the patient’s weight OR
• B. There is support for therapy with a higher dose for the requested indication

Reauthorization criteria

• 1. The patient has been previously approved for the requested agent through the plan’s Prior Authorization process
• 2. The patient has had clinical improvement from baseline (prior to starting therapy) in at least ONE of the following [chart notes required]:
• A. Respiratory status (e.g., level of respiratory support required) OR
• B. Growth (e.g., improvement in length/height, weight, or head circumference as measured by z-scores) OR
• C. Radiographic findings (e.g., improvement in skeletal manifestations as measured by RSS or RGI-C or a decrease in fractures) OR
• D. Level of activity (e.g., improvement in motor function or activities in daily living)
• 3. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist, geneticist) or has consulted with a specialist
• 4. The patient has been monitored for signs and symptoms of ophthalmic and renal calcifications and for changes in vision or renal function
• 5. The patient does NOT have any FDA labeled contraindications to the requested agent
• 6. ONE of the following:
• A. The requested quantity (dose) is within FDA labeled dosing for the requested indication based on the patient’s weight OR
• B. There is support for therapy with a higher dose for the requested indication

Approval duration

6–12 months (6 months non-BCBSIL, 12 months BCBSIL and renewals)