Strensiq Prior Authorization Criteria – Medicare Part D

Prior Authorization Criteria for Approval

Initial Evaluation

Strensiq will be approved when ALL of the following are met:

1. The patient has ONE of the following diagnoses:

   1. Perinatal or infantile-onset hypophosphatasia
      OR
   2. Juvenile-onset hypophosphatasia
      AND

2. The patient has documentation (i.e., medical records) of clinical manifestations to support the diagnosis of hypophosphatasia at the age of onset prior to age 18 (e.g., vitamin B6-dependent seizures, skeletal abnormalities such as rachitic chest deformity leading to respiratory problems or bowed arms/legs, “failure to thrive”)
   AND

3. The patient has documentation (i.e., medical records) of radiographic imaging to support the diagnosis of hypophosphatasia at the age of onset prior to age 18 (e.g., infantile rickets, alveolar bone loss, craniosynostosis, fractures)
   AND

4. The patient has documentation (i.e., medical records) of confirmed mutation(s) in the ALPL gene that encodes the tissue non-specific isoenzyme of alkaline phosphatase (TNSALP)
   AND

5. The patient has documentation (i.e., medical records) of a measured total serum alkaline phosphatase (ALP) level that is below the normal lab reference range for age and sex
   AND

6. The patient has documentation (i.e., medical records) of ONE of the following:

   1. Elevated urine concentration of phosphoethanolamine (PEA)
      OR
   2. Elevated serum concentration of pyridoxal 5'-phosphate (PLP) in the absence of vitamin supplements within one week prior to the test
      OR
   3. Elevated urinary inorganic pyrophosphate (PPi)
      AND

7. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist or geneticist with expertise in metabolic bone diseases) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis
   AND

8. The requested dose is within FDA labeled dosing (based on the patient’s weight) for the requested indication

Length of Approval: 12 months

Renewal Evaluation

Strensiq will be approved when ALL of the following are met:

1. The patient has been previously approved for the requested medication through the plan’s Prior Authorization criteria
   AND

2. The patient has ONE of the following diagnoses:

   1. Perinatal or infantile-onset hypophosphatasia
      OR
   2. Juvenile-onset hypophosphatasia
      AND

3. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist or geneticist with expertise in metabolic bone diseases) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis
   AND

4. There is documentation (i.e., medical records that the patient has had a decrease from baseline (before treatment with the requested medication) in at least ONE of the following levels:

   1. Urine concentration of phosphoethanolamine (PEA)
      OR
   2. Serum concentration of pyridoxal 5'-phosphate (PLP) in the absence of vitamin supplements within one week prior to the test
      OR
   3. Urinary inorganic pyrophosphate (PPi)
      AND

5. The patient has documentation (i.e., medical records) of clinical improvement and/or stabilization with the requested medication (e.g., improvement in respiratory status, growth, pain, radiographic findings, other symptoms associated with the disease)
   AND

6. The requested dose is within FDA labeled dosing (based on the patient’s weight) for the requested indication

Length of Approval: 12 months