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Sebelipase Alfa Dosage

Medically reviewed on June 24, 2016.

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

1 mg/kg IV every other week

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

Usual Pediatric Dose for Wolman's Disease

1 month or older:
1 mg/kg IV every other week

Rapidly progressive LAL deficiency presenting within first 6 months of life:
-Initial dose: 1 mg/kg IV once a week
-Maintenance dose: May increase to 3 mg/kg IV once a week if optimal clinical response is not achieved

Use: For the treatment of LAL deficiency

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

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
-Administer the diluted solution using a low protein binding infusion set with an in-line, low protein binding 0.2 micron filter.
-Infuse over at least 2 hours; consider a longer infusion time in patients receiving the 3 mg/kg dose or those who have had hypersensitivity reactions.
-May consider an infusion rate of 1 hour in patients receiving the 1 mg/kg dose who tolerate the infusion
-Consult the manufacturer product information for recommended infusion volumes.

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.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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