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

Initial criteria

• The patient has a diagnosis of either perinatal/infantile- or juvenile-onset hypophosphatasia (HPP) AND all of the following:
• • The patient was less than 18 years of age at onset [chart notes required]
• • The patient is experiencing active disease (e.g., bone pain, fractures, gait problems)
• • 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]
• • ONE of the following [chart notes required]:
• – Molecular genetic testing has been completed confirming mutations in the ALPL gene that encodes the tissue nonspecific isoenzyme of ALP (TNSALP) OR
• – ALL of the following:
• • Radiographic imaging confirming the diagnosis of hypophosphatasia at onset prior to age 18 (e.g., infantile rickets, alveolar bone loss, craniosynostosis)
• • Reduced serum alkaline phosphatase (ALP) activity below normal range for age and sex in absence of bisphosphonate therapy
• • ONE of the following: elevated pyridoxal 5'-phosphate (PLP) without recent vitamin supplements, elevated urinary phosphoethanolamine (PEA), or elevated urinary inorganic pyrophosphate (PPi)
• The prescriber is a specialist in or has consulted a specialist in endocrinology or genetics
• The patient has had ophthalmology exam and renal ultrasound at baseline (prior to therapy)
• The patient has no FDA labeled contraindications to Strensiq
• ONE of the following: the requested dose is within FDA labeled dosing based on weight OR support for higher dose for requested indication

Reauthorization criteria

• The patient has been previously approved for Strensiq through the plan’s prior authorization process
• The patient has had clinical improvement from baseline (prior to therapy) in at least ONE of the following [chart notes required]: respiratory status, growth, radiographic findings, or level of activity
• The prescriber is a specialist in or has consulted a specialist (e.g., endocrinologist, geneticist)
• The patient has been monitored for ophthalmic and renal calcifications and for changes in vision or renal function
• The patient has no FDA labeled contraindications to Strensiq
• ONE of the following: requested dose is within FDA labeled dosing for indication based on weight OR support for higher dose for requested indication

Approval duration

12 months