Strensiq - NC Standard

Restricted Product(s):

• Strensiq® (asfotase alfa)

FDA Approved Use:

• Indicated for the treatment of patients with perinatal/infantile- and juvenile-onset hypophosphatasia (HPP).

Criteria for Approval of Restricted Product(s):

Initial Criteria for Approval:

1. The onset of the disease was perinatal/infantile or juvenile and started at 18 years of age or less; AND
2. The patient has a confirmed diagnosis of ONE of the following (medical record documentation required):
   1. Perinatal/infantile-onset hypophosphatasia (HPP); OR
   2. Juvenile-onset hypophosphatasia; AND
3. The patient has genetic mutation in ONE of the following confirmed thru genetic testing (medical record documentation required):
   1. Alkaline phosphatase liver/bone/kidney (ALPL) gene; OR
   2. Tissue-nonspecific alkaline phosphatase (TNSALP) gene; AND
4. The patient has low serum alkaline phosphatase (ALP) activity; AND
5. The patient has radiographic imaging to support the diagnosis of hypophosphatasia (e.g. infantile rickets, alveolar bone loss, craniosynostosis) (medical record documentation required); AND
6. The patient has at least ONE of the following baseline results documented (medical record documentation required):
   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); AND
7. Strensiq will be dosed according to FDA-labeling using weight-based dosing; AND
8. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., endocrinologist, metabolic bone disease specialist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis; AND
9. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies.

Duration of Approval: 180 days (6 months)

Continuation Criteria for Approval:

1. The patient has been previously approved with Blue Cross and Blue Shield of North Carolina (Blue Cross NC) or would have met initial criteria for approval upon the start of therapy; AND
2. The patient has a confirmed diagnosis of ONE of the following (medical record documentation required):
   1. Perinatal/infantile-onset hypophosphatasia (HPP); OR
   2. Juvenile-onset hypophosphatasia; AND
3. There is information supporting that the patient has had a decrease from baseline with Strensiq in at least ONE of the following (medical record documentation required):
   1. Serum concentration of pyridoxal 5’-phosphate (PLP) in the absence of vitamin supplements within one week prior to the test; OR
   2. Urine concentration of phosphoethanolamine (PEA); OR
   3. Urinary inorganic pyrophosphate (PPi); AND
4. The patient has shown positive response to treatment with Strensiq as evidenced by an improvement or stabilization (e.g., respiratory status, growth, or radiographic findings) from baseline (medical record documentation required); AND
5. Strensiq will be dosed according to FDA-labeling using weight-based dosing; AND
6. The patient continues to be managed by or in consultation with a specialist in the area of the patient’s diagnosis (e.g., endocrinologist, metabolic bone disease specialist).
7. For formularies that exclude (non-formulary) the requested medication, Non-formulary Exception Criteria applies.

Duration of Approval: 365 days (1 year)

References:

All information referenced is from FDA package insert unless otherwise noted below.

Policy Implementation/Update Information:

February 2025: Criteria change: Updated age requirement to allow coverage for patients > 18 who had disease onset prior to 18 years of age.

July 2023: Criteria change: Added age indication. Added medical record documentation required for diagnosis in initial and continuation criteria. Added genetic mutation confirmation thru genetic testing, serum alkaline phosphatase (ALP) and baseline tests/levels, and radiographic imaging to support diagnosis. Added the prescriber must be a specialist in the area of the patient’s diagnosis.

March 2020: Criteria update: Reformatted criteria.

October 2018: Annual review; No change.

October 2017: Annual review; No change.

April 2016: Original utilization management criteria issued.