Laronidase
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From MedlinePlus, U.S. National Library of Medicine
Specialty pharmacy: therapy management checklist, storage and cold chain, dosing calendar, Medicare Part D coverage.
Boxed warning
WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS, and ACUTE RESPIRATORY COMPLICATIONS ASSOCIATED WITH ADMINISTRATION WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS, and ACUTE RESPIRATORY COMPLICATIONS ASSOCIATED WITH ADMINISTRATION See full prescribing information for complete boxed warning . Appropriate medical monitoring and support measures, including cardiopulmonary resuscitation equipment, should be readily available. If a severe hypersensitivity reaction occurs, discontinue ALDURAZYME immediately and initiate appropriate medical treatment.
( 5.1 ) Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions and require additional monitoring. Appropriate respiratory support should be available during infusion. ( 5.2 ) Hypersensitivity Reactions Including Anaphylaxis Patients treated with ALDURAZYME have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Appropriate medical monitoring and support measures, including cardiopulmonary resuscitation equipment, should be readily available during ALDURAZYME administration.
If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue ALDURAZYME immediately and initiate appropriate medical treatment. In patients with severe hypersensitivity reactions, a desensitization procedure to ALDURAZYME may be considered [see Warnings and Precautions (5.1) ] . Acute Respiratory Complications Associated with Administration Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions and require additional monitoring [see Warnings and Precautions (5.2) ] .
Hydrolytic Lysosomal Glycosaminoglycan-specific Enzyme
Dosing in kidney impairment
This label has no separate kidney-impairment dosing section. Check warnings and clinical pharmacology in the full label.
Preparation and administration
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Recommendations Prior to ALDURAZYME Treatment Premedication Prior to ALDURAZYME administration, consider premedicating with antihistamines, with or without antipyretics, 60 minutes before the start of infusion [see Warnings and Precautions (5.1 , 5.4) ]. Medical Support Appropriate medical monitoring and support measures, including cardiopulmonary resuscitation equipment, should be readily available during ALDURAZYME administration.
Recommended Dosage and Administration The recommended dosage of ALDURAZYME is 0.58 mg/kg (actual body weight) administered once weekly as an intravenous infusion. ALDURAZYME injection must be diluted with 0.9% Sodium Chloride Injection to a final volume of 50 mL, 100 mL or 250 mL as determined by the patient's body weight and cardiopulmonary condition: Patients with a body weight equal to or greater than 2 kg and less than 4 kg should receive a total volume of 50 mL; patients with a body weight equal to or greater than 4 kg and up to 20 kg should receive a total volume of 100 mL; and those patients with a body weight greater than 20 kg should receive a total volume of 250 mL [see Dosage and Administration ].
For patients with underlying cardiac or respiratory compromise and weighing up to 30 kg, physicians may consider diluting ALDURAZYME in a volume of 100 mL and administering at a decreased infusion rate [see Dosage and Administration ]. The initial infusion rate of ALDURAZYME is 10 mcg/kg/hr and may be increased every 15 minutes during the first hour, as tolerated, to a maximum infusion rate of 200 mcg/kg/hr.
The maximum rate is then maintained for the remainder of the infusion (2 to 3 hours) [see Dosage and Administration , Warnings and Precautions (5.2 , 5.3) ] . If one or more doses are missed, restart ALDURAZYME treatment as soon as possible and maintain the 1-week interval between infusions thereafter. Do not double a dose to compensate for a missed dose.
Administration Modifications due to Hypersensitivity or Infusion Associated Reaction In the event of a severe hypersensitivity reaction (e.g., anaphylaxis) or severe infusion-associated reaction (IAR), immediately discontinue ALDURAZYME administration and initiate appropriate medical treatment. For additional recommendations in the event of a severe hypersensitivity reaction, [ see Warnings and Precautions ].
In the event of a mild to moderate hypersensitivity reaction or a mild to moderate IAR, consider temporarily holding the infusion for 15 to 30 minutes, or slowing the infusion rate by 25% to 50% [ see Dosage and Administration ] , and initiating appropriate medical treatment [see Warnings and Precautions (5.1 , 5.4) ] .
If symptoms persist despite holding or slowing the infusion, stop the infusion and monitor the patient. Consider re-initiating the infusion within 7 to 14 days using the incremental rate steps table [see Dosage and Administration ] , up to 25% or 50% of the rate at which the reaction occurred with appropriate premedication.
If symptoms subside after holding the infusion, resume infusion at a 25% to 50% reduced rate as tolerated. Alternatively, if symptoms subside after slowing the infusion, complete infusion at the reduced rate as tolerated. Starting with next infusion, increase the infusion rate by increments of 25% as tolerated until the recommended infusion rate is reached.
Closely monitor the patient.
Preparation Instructions Prepare ALDURAZYME using low-protein-binding containers. There is no information on the compatibility of diluted ALDURAZYME with glass containers. Dilute ALDURAZYME in the following manner using aseptic technique: Determine the infusion bag volume and number of ALDURAZYME vials to be diluted based on actual body weight in kg and the recommended dose [see Dosage and Administration ] .
Round the number of vials up to the next whole number. Remove the appropriate number of ALDURAZYME vials from the refrigerator and allow the vials to reach room temperature 20°C to 25°C (68°F to 77°F) before use. Do not heat or microwave the vials. Visually inspect the solution in each vial for particulate matter and discoloration.
The ALDURAZYME solution should be clear to slightly opalescent and colorless to pale yellow. Some translucency may be present in the solution. Discard if the solution is discolored or if visible particulate matter is present. Withdraw and discard a volume of the 0.9% Sodium Chloride Injection from an infusion bag, equal to the volume of ALDURAZYME to be added.
Slowly withdraw the calculated volume of ALDURAZYME from the appropriate number of vials using caution to avoid excessive agitation. Do not use a filter needle, as this may cause agitation. Agitation may denature ALDURAZYME, rendering it biologically inactive. Discard any unused solution remaining in the vial. Slowly add the ALDURAZYME solution to the 0.9% Sodium Chloride Injection solution through the port of the infusion bag and avoid agitation.
Do not use a filter needle. Gently rotate the infusion bag to ensure proper distribution of ALDURAZYME. Do not shake the infusion bag. 2.5. Storage Instruction for the Diluted Solution If the diluted ALDURAZYME solution is not used immediately: Refrigerate the diluted solution at 2°C to 8°C (36°F to 46°F) for up to 36 hours.
Discard any unused ALDURAZYME diluted solution after 36 hours. Do not store the diluted solution at room temperature. The solution must be infused within 8 hours after removal from the refrigerator, inclusive of the total infusion time, or discarded.
Administration Instructions Use an infusion set equipped with a low-protein-binding, 0.2 micron, in-line filter to administer the diluted ALDURAZYME solution. The total volume of infusion [see Dosage and Administration ] should be administered over approximately 3 to 4 hours as tolerated per the infusion rate steps outlined in Table 1 below.
At the end of the infusion, flush the infusion line with 0.9% Sodium Chloride Injection, using the same infusion rate as the one used for the last part of the infusion. Do not infuse ALDURAZYME in the same intravenous line with other products. [See table below.] Start infusion at rate in Step 1. In the absence of infusion-associated reactions after vital sign assessment, increase infusion rate sequentially per the steps in Table 1 every 15 minutes to reach the target rate in Step 5.
Continue Step 5 until infusion is completed. The total infusion time is approximately 3 to 4 hours.
| Patient Weight Range | Total Infusion Volume | Step 1 10 mcg/kg/hr | Step 2 20 mcg/kg/hr | Step 3 50 mcg/kg/hr | Step 4 100 mcg/kg/hr | Step 5 200 mcg/kg/hr |
|---|---|---|---|---|---|---|
| Infusion Rate in mL/hour | ||||||
| ≥2 to <4 kg | 50 mL | 1 | 2 | 4 | 8 | 16 |
| ≥4 to <20 kg | 100 mL | 2 | 4 | 8 | 16 | 32 |
| >20 kg | 250 mL | 5 | 10 | 20 | 40 | 80 |
Drug interactions
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- Laronidase
Contraindications
None. None. ( 4 )
Safety data
Reported adverse events (FAERS)
Liver injury (LiverTox)
LiverTox has no chapter for laronidase. Check the label's hepatic warnings.
Pregnancy and lactation
Pregnancy
8.1 Pregnancy Risk Summary Available data from the MPS I Registry pregnancy sub-registry, published case reports, and the global pharmacovigilance database with ALDURAZYME use in more than 30 pregnant women have not identified a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes. The continuation of treatment for MPS I during pregnancy should be individualized to the pregnant woman. Untreated MPS I may result in adverse pregnancy and infant outcomes ( see Clinical Considerations ).
No evidence of fetal harm has been observed in rats when laronidase was administered during organogenesis at doses up to 6.2 times the recommended human dose (see Data ). The background risk of major birth defects and miscarriage for the indicated population is unknown. All pregnancies have a background risk of birth defect, loss, or other adverse outcomes. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2% to 4% and 15% to 20%, respectively.
Clinical Considerations Disease-Associated Maternal and/or Embryo-Fetal Risk Pregnancy can exacerbate preexisting clinical manifestations of MPS and lead to adverse pregnancy outcomes for both mother and fetus.
Data Animal Data When laronidase was administered to pregnant female rats during organogenesis (gestation days [GD] 7-17) at doses of 0, 0.036, 0.36 or 3.6 mg/kg/day intravenously (equivalent to 7.3, 73.1, 730.8 units/kg/day) decreased maternal body weight gains and food consumption were observed with no corresponding effects on reproductive and litter parameters including number and distribution of corpora lutea, implantations and early and late resorptions at doses up to 3.6 mg/kg/day (6.2 times the recommended human dose of 0.58 mg/kg on a mg/kg basis). Laronidase has not been evaluated for effects on embryo-fetal development in any other species.
Breastfeeding (LactMed)
Limited information from on mother receiving laronidase for mucopolysaccharidosis type I indicates that the drug is not detectable in breastmilk and her breastfed infant suffered no adverse reactions or adverse developmental effects from the drug in milk. The lack of penetration into milk is consistent with its high molecular weight of 82,000 Da. If laronidase is required by the mother, it is not a reason to discontinue breastfeeding. Until more data are available, laronidase should be used with careful monitoring during breastfeeding.
Supply and cost
Drug shortage status
Recalls (last 24 months)
Therapeutic equivalence (Orange Book)
Price ladder
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