Olipudase Alfa Dosage

Medically reviewed by Drugs.com. Last updated on Apr 16, 2024.

Applies to the following strengths: rpcp 20 mg; rpcp 4 mg

Usual Adult Dose for Sphingolipidosis

Dose Escalation Regimen:

First dose/starting dose (Day 1, Week 0): 0.1 mg/kg IV
Second dose (Week 2): 0.3 mg/kg IV
Third dose (Week 4): 0.3 mg/kg IV
Fourth dose (Week 6): 0.6 mg/kg IV
Fifth dose (Week 8): 0.6 mg/kg IV
Sixth dose (Week 10): 1 mg/kg IV
Seventh dose (Week 12): 2 mg/kg IV
Eighth dose (Week 14): 3 mg/kg IV (recommended maintenance dose)

Maintenance Dose: 3 mg/kg IV every 2 weeks

Comments:

This drug is administered via IV infusion every 2 weeks.
Dosages for dose escalation and maintenance phases are based on actual body weight (kg) for patients with BMI up to 30 and adjusted body weight (kg) for patients with BMI greater than 30.
Adjusted body weight should be calculated by squaring the patient's actual height in meters and then multiplying by 30; the manufacturer product information should be consulted.
The dose escalation regimen should be followed to reduce the risk of infusion-associated reactions or elevated transaminase levels.
The dose escalation phase includes the first 3 mg/kg dose.

Use: For treatment of non-central nervous system manifestations of acid sphingomyelinase deficiency (ASMD)

Usual Pediatric Dose for Sphingolipidosis

0 to 17 years:
Dose Escalation Regimen:

First dose/starting dose (Day 1, Week 0): 0.03 mg/kg IV
Second dose (Week 2): 0.1 mg/kg IV
Third dose (Week 4): 0.3 mg/kg IV
Fourth dose (Week 6): 0.3 mg/kg IV
Fifth dose (Week 8): 0.6 mg/kg IV
Sixth dose (Week 10): 0.6 mg/kg IV
Seventh dose (Week 12): 1 mg/kg IV
Eighth dose (Week 14): 2 mg/kg IV
Ninth dose (Week 16): 3 mg/kg IV (recommended maintenance dose)

Maintenance Dose: 3 mg/kg IV every 2 weeks

Comments:

This drug is administered via IV infusion every 2 weeks.
Dosages for dose escalation and maintenance phases are based on actual body weight (kg) for patients with BMI up to 30 and adjusted body weight (kg) for patients with BMI greater than 30.
Adjusted body weight should be calculated by squaring the patient's actual height in meters and then multiplying by 30; the manufacturer product information should be consulted.
The dose escalation regimen should be followed to reduce the risk of hypersensitivity and infusion-associated reactions or liver enzyme elevations.
The dose escalation phase includes the first 3 mg/kg dose.

Use: For treatment of non-central nervous system manifestations of ASMD

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dose Adjustments

MISSED DOSES:

A dose is considered missed when it is not administered within 3 days of the scheduled date.
At scheduled infusion after a missed dose: If the dose administered is 0.3 or 0.6 mg/kg, administer that dose twice as per the dose escalation regimen.

Escalation Phase:

For 1 missed dose:
First dose after missed dose: Administer last tolerated dose.
Second and subsequent doses after missed dose: Resume dose escalation at next infusion according to the dose escalation regimen.
For 2 consecutive missed doses:
First dose after missed dose: Administer 1 dose below last tolerated dose.
Second and subsequent doses after missed dose: Resume dose escalation according to the dose escalation regimen.
For 3 or more consecutive missed doses:
First and subsequent doses after missed doses: Resume dose escalation at 0.3 mg/kg and follow the dose escalation regimen.

Maintenance Phase:

For 1 missed dose:
First and subsequent doses after missed dose: Administer maintenance dose.
For 2 consecutive missed doses:
First dose after missed dose: Administer 1 dose below the maintenance dose.
Second and subsequent doses after missed dose: Resume the maintenance dose.
For 3 or more consecutive missed doses:
First and subsequent doses after missed doses: Restart dosing at 0.3 mg/kg and follow the dose escalation regimen.

HYPERSENSITIVITY OR INFUSION-ASSOCIATED REACTIONS:

For mild to moderate hypersensitivity reaction or mild to moderate infusion-associated reaction: Consider temporarily holding or slowing the infusion rate, and/or reducing the dose.
If dose is reduced: Re-escalate following the dose escalation regimen.
For severe hypersensitivity reaction (e.g., anaphylaxis) or severe infusion-associated reaction: Immediately discontinue administration and start appropriate medical treatment.

ELEVATED TRANSAMINASE LEVELS:

For levels above baseline and greater than 2 times the upper limit of normal before the next scheduled administration: The dose can be adjusted (prior dose repeated or reduced) or treatment can be temporarily withheld until the liver transaminases return to patient's baseline value.

Precautions

US BOXED WARNING:

HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS: Patients treated with this drug have had life-threatening hypersensitivity reactions (including anaphylaxis). Appropriate medical monitoring and support measures (including cardiopulmonary resuscitation equipment) should be readily available during administration of this drug. If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, this drug should be discontinued immediately and appropriate medical treatment should be started. A desensitization procedure to this drug may be considered in patients with severe hypersensitivity reactions.

CONTRAINDICATIONS: None

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

To avoid dosing errors (including overdosage), follow all instructions for dosage and administration.
Within 1 month before starting this drug, obtain baseline transaminase (ALT and AST) levels in all patients.
Before starting this drug, verify pregnancy status in patients of childbearing potential.
Before administration, consider premedicating with antihistamines, antipyretics, and/or corticosteroids.
Appropriate medical support measures (including cardiopulmonary resuscitation equipment) should be readily available during administration.
Before administration, inspect the syringe or infusion bag for foaming; if present, let foam dissipate before administering this drug.
Administer via IV infusion; use a low-protein binding, 0.2 micron, in-line filter during administration.
The following materials can be used: polyolefin or polyvinylchloride (PVC) with di(2-ethylhexyl) phthalate (DEHP) for infusion bags, polypropylene for syringes, polyurethane or PVC DEHP-free for infusion sets, and polyethersulfone or polytetrafluoroethylene for in-line filters.
Consult the manufacturer product information regarding infusion rates.
If no infusion-associated reactions occur, increase the infusion rate per the steps of infusion as indicated (+/- 5 minutes); each step of infusion will last for 20 minutes except for the final step which should last until completion of the infusion volume.
At the end of the infusion, flush the infusion line with 0.9% Sodium Chloride Injection, USP; use the same infusion rate that was used for the last part of the infusion.
Do not infuse this drug in the same IV line with other products.
Home Infusion:
Home administration under the supervision of a health care provider may be considered for patients on maintenance dose who are tolerating their infusion well; the decision to move patients to home infusion should be made after evaluation and recommendation by a physician.
Dose and infusion rates should remain constant for home administration and cannot be changed without supervision of a physician.
A physician should be contacted for missed doses or delayed infusion.

Storage requirements:

Unopened vials: Store refrigerated at 2C to 8C (36F to 46F).
Before reconstitution, remove the required number of vials from refrigeration and allow to reach room temperature (about 20 to 30 minutes).
Reconstituted solution: If not used immediately, store refrigerated at 2C to 8C (36F to 46F) for up to 24 hours or at controlled room temperature at 20C to 25C (68F to 77F) for up to 6 hours; discard unused solution after 24 hours (if stored refrigerated) or 6 hours (if stored at controlled room temperature).
Diluted solution: If not used immediately, refrigerate at 2C to 8C (36F to 46F) for up to 24 hours, store at room temperature at 20C to 25C (68F to 77F) for up to 12 hours (including infusion time), or discard.
Do not freeze.

Reconstitution/preparation techniques:

This drug must be reconstituted and diluted before administration.
Foaming should be avoided.
The manufacturer product information should be consulted.

IV compatibility:

Compatible: Sterile Water for Injection, USP; 0.9% Sodium Chloride Injection, USP

General:

Therapy should be directed in consultation with physicians knowledgeable in the management of ASMD.

Monitoring:

Hepatic: ALT and AST (within 1 month before starting therapy, within 72 hours before any infusion during dose escalation, or before next scheduled infusion upon resumption of therapy after a missed dose); transaminase testing (routinely during maintenance phase)
Hypersensitivity: For hypersensitivity reactions (during administration)

Patient advice:

Seek immediate medical care if signs/symptoms of hypersensitivity or infusion-associated reactions occur.
Patients of childbearing potential: Inform health care provider of a known/suspected pregnancy; use effective contraception during therapy and for 14 days after the last dose if this drug is discontinued.