Sebelipase Alfa Dosage
Medically reviewed by Drugs.com. Last updated on Jun 14, 2024.
Applies to the following strengths: 2 mg/mL
Usual Adult Dose for:
Usual Pediatric Dose for:
Additional dosage information:
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.
- Seek medical attention immediately if signs or symptoms of hypersensitivity or anaphylaxis occur.
More about sebelipase alfa
- Compare alternatives
- Side effects
- During pregnancy
- Drug class: lysosomal enzymes
- Breastfeeding
- En español
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Further information
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.