LARONIDASE

LARONIDASE
(la-ron'i-dase)
Aldurazyme
Classifications: enzyme replacement therapy;
Therapeutic: enzyme replacement therapy

Prototype: Pancrelipase
Pregnancy Category: B

Availability

2.9 mg/5 mL injection

Action

Laronidase is a recombinant form of human alpha-L-iduronidase used for enzyme replacement therapy in individuals with mucopolysaccharidosis I (MPSI). This is an inherited lysosomal storage disease caused by deficiency of the enzyme alpha-L-iduronidase.

Therapeutic Effect

Replacement therapy for individuals lacking the enzyme alpha-L-iduronidase in mucopolysaccharidosis.

Uses

Treatment of Hurler and Hurler-Scheie forms of mucopolysaccharidosis I (MPS I); treatment of moderate to severe Scheie form of MPS I.

Contraindications

Hypersensitivity to laronidase; children <5 y.

Cautious Use

Renal or hepatic dysfunction; history of allergies, asthma; hypersensitivity to drugs, especially recombinant forms; pregnancy (category B), lactation.

Route & Dosage

Mucopolysaccharidosis
Adult/Child (>5 y): IV 0.58 mg/kg infused over 3–4 h once/wk

Administration

Intravenous
  • Pretreatment with antipyretics and/or antihistamines 60 min prior to infusion is recommended.

PREPARE: IV Infusion: Determine volume of infusion based on the patient's body weight (100 mL if ≤20 kg or 250 mL if >20 kg). Prepare IV infusion of 0.1% albumin (human) in NS injection as follows: 1) remove and discard a volume of NS injection equal to the volume of albumin (human) to be added to the IV bag (for 100 mL infusion use 2 mL of 5% albumin of 0.4 mL or 25% albumin, for 250 mL infusion use 5 mL of 5% albumin or 1 mL of 25% albumin); 2) add the appropriate volume of albumin to the IV bag and gently rotate; 3) add the albumin to IV bag and gently rotate to mix; 4) withdraw and discard a volume of the 0.1% albumin in NS injection from the IV bag equal to the volume of laronidase concentrate to be added; 5) slowly withdraw the required amount of laronidase from vials (avoid excessive agitation), then slowly add laronidase to the IV solution. Use immediately.  

ADMINISTER: IV Infusion: Infuse initially at 10 mcg/kg/h; may increase q15min during first h, as tolerated, to a max rate of 200 mcg/kg/hr. Maintain max for remainder of the infusion (2–3 h).  

INCOMPATIBILITIES Solution/additive: No compatibility data available. Do not recommend mixing or infusing with other drugs.

  • Store at 2°–8° C (36°–46° F). Do not freeze or shake. Discard any unused drug.

Adverse Effects (≥1%)

Body as a Whole: Infusion reactions (flushing, fever, headache, rash), injection site pain, hypersensitivity reactions. CNS: Hyperreflexia, paresthesias. CV: Chest pain, hypotension, edema. Hematologic: Thrombocytopenia. Respiratory: Upper respiratory tract infection. Skin: Rash.

Pharmacokinetics

Half-Life: 1.5–3.6 h.

Nursing Implications

Assessment & Drug Effects

  • Monitor for infusion-related reactions. Slow or stop infusion and notify physician for any of the following: cough, bronchospasm, dyspnea, urticaria, angioedema, pruritus, or other signs of hypersensitivity.
  • Lab tests: Periodic platelet count.

Patient & Family Education

  • Report promptly difficulty breathing, rash, or itching.

Common adverse effects in italic, life-threatening effects underlined; generic names in bold; classifications in SMALL CAPS; Canadian drug name; Prototype drug

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