Inborn errors of metabolism:

Indications for STRENSIQ:

Treatment of perinatal/infantile- and juvenile-onset hypophosphatasia.

Adult:

Not applicable.

Children:

See full labeling. Give by SC inj in the abdomen, thigh, deltoid, or buttocks; rotate inj sites. 6mg/kg per week given as 2mg/kg 3 times weekly or 1mg/kg 6 times weekly. Perinatal/infantile-onset: if ineffective, may increase dose up to 9mg/kg per week given as 3mg/kg 3 times weekly. <40kg: do not use 80mg/0.8mL vial.

Warnings/Precautions:

Do not administer to areas that are reddened, inflamed, or swollen. Discontinue if severe hypersensitivity reaction occurs; monitor for reoccurrence if repeat treatment. Perform ophthalmic exams and renal ultrasounds at baseline and periodically during treatment. Monitor for ectopic calcifications and changes in vision or renal function. Possible immune-mediated clinical effects. Pregnancy. Nursing mothers.

Pharmacologic Class:

Tissue nonspecific alkaline phosphatase.

Interactions:

May cause erroneous results in lab assays that utilize alkaline phosphatase (ALP)-conjugated test system. Do not rely on serum ALP measurements for clinical decision making due to expected high levels.

Adverse Reactions:

Inj site reactions, lipodystrophy, ectopic calcifications, hypersensitivity reactions.

Generic Availability:

NO

How Supplied:

Single-use vials—1, 12