Laronidase

Trade Names:
Aldurazyme
- Injection 2.9 mg laronidase/5 mL

Pharmacology

Provides exogenous enzyme for uptake into lysosomes and increases the catabolism of glycosaminoglycans.

Pharmacokinetics

Absorption

Mean AUC 4.5 to 6.9 mcg·h/mL.

Distribution

Mean Vd 0.24 to 0.6 L/kg.

Elimination

Mean plasma Cl 1.7 to 2.7 mL/min/kg and elimination t _½ 1.5 to 3.6 h.

Indications and Usage

Treatment of patients with Hurler and Hurler-Scheie forms of mucopolysaccharidosis I (MPS I); patients with Scheie form who have moderate to severe symptoms.

Contraindications

Standard considerations.

Dosage and Administration

IV 0.58 mg/kg once/wk delivered over 3 to 4 h.

General Advice

• For IV administration only. Not for intradermal, IM, subcutaneous, or intraarterial administration.
• Follow manufacturer's instructions for diluting the concentrated solution for infusion.
• Prepare infusion solution using PVC containers and administer with a PVC infusion set equipped with an in-line, low protein-binding 0.2 mcm filter.
• Do not administer if solution is cloudy, discolored, or contains particulate matter.
• Do not shake or agitate vials during dilution. Do not use filter needles during preparation of infusion.
• Discard any unused product. Vials are for single-use only. Do not save medication for future use.
• Initial IV infusion rate should be no more than 10 mcg/kg/h. The initial infusion rate of 10 mcg/kg/h may be incrementally increased every 15 min during first hour, as tolerated, until a max infusion rate of 200 mcg/kg/min is reached. The max rate is then maintained for remainder of the infusion.
• Do not administer in same IV line with other products.

Storage/Stability

Store vials of concentrated solution in refrigerator (36° to 46°F). Do not freeze or shake. Use diluted infusion solution immediately. If immediate use is not possible, store the diluted infusion solution in refrigerator (36° to 46°F). Do not store diluted infusion solution longer than 36 h from time of preparation to completion of infusion.

Drug Interactions

None well documented.

Incompatibility

Do not mix with other medicinal products in the same infusion.

Laboratory Test Interactions

None well documented.

Adverse Reactions

Cardiovascular

Vein disorder (14%); hypotension (9%); dependent edema (9%).

CNS

Hyperreflexia (14%); paresthesia (14%).

Dermatologic

Rash (36%).

EENT

Corneal opacity (9%).

Hematologic

Thrombocytopenia (9%).

Hepatic

Bilirubinemia (9%).

Respiratory

Upper respiratory tract infection (32%).

Miscellaneous

Anaphylaxis; injection-site reaction (18%); injection-site pain (9%); infusion-related reactions (eg, flushing) (32%); chest pain (9%); abscess (9%); facial edema (9%); positive antibodies for laronidase (91%).

Precautions

Pregnancy

Category B .

Lactation

Undetermined.

Children

Safety and efficacy not studied in children younger than 5 yr of age.

Hypersensitivity/Infusion reactions

Infusion-related hypersensitivity reactions may occur. Because of increased prevalence of coronary artery disease in patients with MPS I, use epinephrine with caution. Prior to infusion, patients should receive antipyretics and/or antihistamines. If an infusion reaction occurs, decreasing the infusion rate, temporarily stopping the infusion, and/or administration of additional antipyretics and/or antihistamines may ameliorate the symptoms.

Patient Information

• Advise patient, family, or caregiver that medication will be prepared and administered by a health care provider in a health care setting.
• Advise patient, family, or caregiver that an antipyretic (eg, acetaminophen) and antihistamine (eg, diphenhydramine) will be administered before each treatment to prevent or reduce severity of infusion reactions.
• Advise patient, family, or caregiver to report any signs or symptoms of infusion reaction (eg, flushing, fever, headache, rash).
• Encourage patient to enroll and participate in the MPS I (mucopolysaccharidosis I) registry as noted in the manufacturer's product information.

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