Strensiq

About Strensiq:

• Strensiq is a tissue nonspecific alkaline phosphatase and contains Asfotase alfa as an active substance and is designated as an orphan drug.
• HPP is a rare inherited disease that leads to early loss of teeth, malformed bones, frequent bone fractures, and difficulty breathing.
• It is caused by a TNSALP enzyme activity deficiency, which leads to elevations of several substrates like inorganic pyrophosphate (PPi). 
• Elevated levels of PPi block hydroxyapatite crystal growth which inhibits bone mineralization and causes an accumulation of unmineralized bone matrix which results in rickets and bone deformation in infants and children and as osteomalacia along with muscle weakness. 
• Replacement of the TNSALP enzyme following Strensiq treatment reduces the enzyme-substrate levels.

Strength: 

Strensiq injection is available in 18 mg/0.45 mL, 28 mg/0.7 mL, 40 mg/mL, or 80 mg/0.8 mL solution in single-use vials.

Recommended Dosage:

• The recommended dosage regimen of Strensiq for the treatment of perinatal/infantile-onset HPP is 6 mg/kg per week subcutaneously administered as:

• 2 mg/kg three times per week
• 1 mg/kg six times per week 

• The dose may be increased for lack of efficacy up to 9 mg/kg per week subcutaneously as 3 mg/kg three times per week.

Important:

• Strensiq should be administered within 1 hour after removal of the vial from refrigeration.
• Rotate the injection from the abdominal area, thigh, or deltoid to reduce the risk of lipodystrophy.
• Reddened, inflamed, or swollen areas should be avoided. 
• Strensiq vials are single-use only so, unused products should be discarded.

Warnings & Precautions:

• Hypersensitivity Reactions: Monitor and if a severe reaction occurs, discontinue treatment and initiate appropriate medical treatment.
• Lipodystrophy: Localized reactions were reported after several months of treatment; follow proper injection technique and rotate injection sites.
• Ectopic Calcifications (eye and kidneys): Monitor using ophthalmologic examinations and renal ultrasounds at baseline and periodically during treatment.

Common Strensiq Side Effects

• Injection site reactions
• Lipodystrophy
• Ectopic calcifications
• Hypersensitivity reactions

Use in Specific Population

Pregnancy: No available human data is available on Strensiq use in pregnant women to inform drug-associated risk.

Pediatric use: The safety and effectiveness of have been established in pediatric patients.

Storage and Handling

• Strensiq vials must be stored refrigerated conditions at 2°C to 8°C (36°F to 46°F) and protected from light.
• If removed from refrigeration, it should be administered within 1 hour.
• It should not be used beyond the expiry date.

Vials should not be shaken and frozen and for single-use only.