Inborn errors of metabolism:

Indications for STRENSIQ:

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

Adult Dosage:

Not applicable.

Children Dosage:

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.

STRENSIQ 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.

STRENSIQ Classification:

Tissue nonspecific alkaline phosphatase.

STRENSIQ 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 Drug Availability:

NO

How Supplied:

Single-use vials—1, 12