Sebelipase Alfa Dosage

Medically reviewed by Drugs.com. Last updated on Jun 14, 2023.

Applies to the following strengths: 2 mg/mL

Usual Adult Dose for Wolman's Disease

Recommended dosage: 1 mg/kg as an IV infusion every other week
Patients with suboptimal clinical response:
Increase the dosage to 3 mg/kg IV every other week

Comments:

Suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers such as alanine aminotransferase (ALT), aspartate aminotransferase (AST), and/or parameters of lipid metabolism such as low-density lipoprotein cholesterol (LDL-c), or triglycerides (TG).

Use: For the treatment of lysosomal acid lipase (LAL) deficiency

Usual Pediatric Dose for Wolman's Disease

Infants with Rapidly Progressive LAL Deficiency within the first 6 months of life:

Recommended dosage: 1 mg/kg IV once a week
Patients with a suboptimal clinical response:
Increase dosage to 3 mg/kg IV once a week
Patients with continued suboptimal clinical response:
Increase dosage to 5 mg/kg once a week

Comments:

Suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers, or persistent or worsening organomegaly.

Pediatric patients over 6 months of age:

Recommended dosage: 1 mg/kg as an IV infusion every other week
Patients with suboptimal clinical response:
Increase the dosage to 3 mg/kg IV every other week

Comments:

Suboptimal clinical response is defined as any of the following: poor growth, deteriorating biochemical markers such as alanine aminotransferase (ALT), aspartate aminotransferase (AST), and/or parameters of lipid metabolism such as low-density lipoprotein cholesterol (LDL-c), or triglycerides (TG).

Use: For the treatment of lysosomal acid lipase (LAL) deficiency

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

US FDA requires a medication guide to assure safe use. For additional information: www.fda.gov/drugs/drug-safety-and-availability/medication-guides

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 1 month.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

For IV infusion only
The solution should be clear. Inspect visually for particulate matter or discoloration prior to administration. Do not use if the solution is cloudy or particles are observed.
Each vial is for single use. Discard any unused product.
Consult the manufacturer product information for recommended infusion volumes.
Infuse over at least 2 hours; consider a longer infusion time in patients receiving dosages greater than 1 mg/kg or in those who have had hypersensitivity reactions.
An infusion rate of 1 hour may be considered in patients receiving the 1 mg/kg dose who tolerate the infusion

Storage requirements:

Do not freeze or shake.
Protect from light.
Use diluted solution immediately; if cannot use immediately, may store for up to 24 hours at 2C to 8C (36F to 46F)

Reconstitution/preparation techniques:

The manufacturer product information should be consulted.

IV compatibility:

Compatible with 0.9% Sodium Chloride for dilution

Monitoring:

Hypersensitivity: Signs/symptoms of hypersensitivity reaction (during and after infusion)

Patient advice:

Seek medical attention immediately if signs or symptoms of hypersensitivity or anaphylaxis occur.
Read the Patient Information and Instructions for Use.