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laronidase (Rx)Brand and Other Names:Aldurazyme

 
 
 

Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

injectable solution

  • 2.9mg/5mL
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Mucopolysaccharidosis I

0.58 mg/kg IV (3-4 hours infusion) qWeek; may administer total volume 100 mL if body weight < 20 kg or 250 mL if body weight >20 kg 

Initial infusion rate 10 mcg/kg/hr; may increase q15min during 1st hour, as tolerated, to maximum 200 mcg/kg/hr

See infusion rate in Administration section

Dosage Forms & Strengths

injectable solution

  • 2.9mg/5mL
more...

Mucopolysaccharidosis I

<5 years

  • Safety & efficacy not established

≥5 years

  • <20 kg: 0.58 mg/kg IV (3-4 hr infusion) qWeek; total volume 100 mL 
  • ≥20 kg: As in adults; 0.58 mg/kg IV (3-4 hr infusion) qWeek; total volume 250 mL
  • Initial infusion rate 10 mcg/kg/hr; may increase q15min during 1st hr, as tolerated, to maximum 200 mcg/kg/hr
  • See infusion rate in Administration section
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Adverse Effects

>10%

Development of anti-laronidase antibodies (91% in one study) - clinical significance unknown

Rash (36%)

Injection site reaction (18%)

Poor venous access (14%)

Hyperreflexia (14%)

Paresthesia (14%)

Chills (20%; children 6 months to 5 years)

Otitis media (20%; children 6 months to 5 years)

Upper respiratory tract infection (32%)

Infusion reactions (49%)

  • Rash (13%)
  • Flushing (11%)
  • Pyrexia (11%)
  • Headache (9%)
  • Abdominal pain or discomfort (9%)
  • Nausea (7%)
  • Diarrhea (7%)
  • Feeling hot or cold (7%)
  • Vomiting (4%)
  • Pruritus (4%)
  • Arthralgia (4%)
  • Urticaria (4%)

1-10%

Thrombocytopenia (9%)

Corneal opacity (9%)

Chest pain (9%)

Face edema (9%)

Gravitational edema (9%)

Injection site pain (9%)

Hyperbilirubinemia (9%)

Hypotension (9%)

< 1%

Anaphylactic shock

Laryngeal edema

Fatigue

Peripheral edema

Erythema

Cyanosis

Angioedema

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Warnings

Black Box Warning

Anaphylactoid reactions, which may be life threatening, have been observed in some patients during infusions

Patients with compromised respiratory function or acute respiratory distress may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions and require additional monitoring

Contraindications

Hypersensitivity

Cautions

Consider registration in the MPS I Registry, www.MPSIregistry.com or 1-800-745-4447

Consider delaying treatment in patients with acute febrile/respiratory illness; may increase risk of infusion related reactions; premedication with antihistamines and antipyretics recommended; decrease infusion rate temporarily; manage reactions by administering additional antipyretics and antihistamines

Use caution in patients with fluid overloads or in conditions where fluid restriction is indicated; exacerbation of such conditions may occur during infusions

Not indicated for CNS manifestations of the disorder

Use caution in patients with sleep apnea; apnea treatment options should be readily available during infusion or with use of sedating antihistamines including supplemental oxygen and CPAP

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Pregnancy & Lactation

Pregnancy Category: B

Lactation: Not known if distributed into breast milk, use caution

Pregnancy Categories

A:Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

B:May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

C:Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

D:Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

X:Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

NA:Information not available.

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Pharmacology

Mechanism of Action

Recombinant alpha-L-iduronidase, an enzyme (deficient in mucopolysaccharidosis I) involved in the breakdown of glycosaminoglycans within lysosomes; walking capacity and pulmonary function improves in patients treated with this recombinant product

Absorption

Peak plasma concentration: 1.2-1.7 mcg/mL

AUC: 4.5-6.9 mcg•hr/mL

Onset: 3-6 wk (initial effect); 26 wk (max effect)

Distribution

Vd: 0.24-0.6 L/kg

Elimination

Half-life: 1.5-3.6 hr

Plasma clearance: 1.7-2.7 mL/min/kg

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Administration

IV Incompatibilities

Do not mix with other drugs in same infusion solution

IV Preparation

Dilute commercially available injection concentrate in NS containing 0.1% human albumin prior to administration

Pretreatment with antipyretics &/or antihistamines is recommended 60 min before start of laronidase infusions

Prepare solutions using PVC containers

Vial calculation

  • Appropriate number of laronidase vials should be diluted based on body weight and dose determined using the equations listed below
  • Patient's weight (kg) x 1 mL/kg of laronidase = Total # mL of laronidase
  • Total # mL/5 mL per vial = Total # of vials, rounded up to the nearest whole vial

Prepare infusion bag of NS containing human albumin 0.1% based on total infusion volume

Prior to addition of albumin to the infusion bag, remov & discard an amount of NS equal to volume of albumin

For a total infusion volume of 100 mL, add 2 or 0.4 mL of human albumin 5% or 25%; for a total infusion volume of 250 mL, add 5 or 1 mL of albumin 5% or 25%

Gently rotate to ensure proper distribution of albumin

Prior to addition of calculated volume of laronidase to infusion bag, remove & discard an equal volume of NS containing albumin 0.1%

Calculated volume of laronidase injection concentrate should be withdrawn slowly from appropriate number of vials & added slowly to infusion bag, followed by gentle rotation for even distribution; do not use filter needle since it may cause agitation & denature laronidase

IV Administration

Premedicate with antipyretics &/or antihistamines 1 hr before infusion

Infuse over 3-4 hr (see infusion rate schedule below)

Peds: <20 kg: give a total volume of 100 mL; >20 kg: give a total volume of 250 mL

Administer with PVC infusion set equipped with an inline, low-protein-binding filter with a pore diameter of 0.2 microns

Infusion rates for body Weight <20 kg

  • 2 mL/h for 15 min (10 mcg/kg/hr); if vital signs stable increase rate to
  • 4 mL/h for 15 min (20 mcg/kg/hr); if vital sign stable, increase rate to
  • 8 mL/h for 15 min (50 mcg/kg/hr); if vital sign stable, increase rate to
  • 16 mL/h for 15 min (100 mcg/kg/hr); if vital sign stable, increase rate to
  • 32 mL/h for 3 hr (200 mcg/kg/hr); continue at this rate for the remainder of the infusion

Infusion rates for body weight >20 kg

  • 5 mL/h for 15 min (10 mcg/kg/hr); if vital signs stable increase rate to
  • 10 mL/h for 15 min (20 mcg/kg/hr); if vital sign stable, increase rate to
  • 20 mL/h for 15 min (50 mcg/kg/hr); if vital sign stable, increase rate to
  • 40 mL/h for 15 min (100 mcg/kg/hr); if vital sign stable, increase rate to
  • 80 mL/h for 3 hr (200 mcg/kg/hr); continue at this rate for the remainder of the infusion

Storage

Store at 2-8°C

Protect from freezing

Do not shake

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Images

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Formulary

FormularyPatient Discounts

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The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

Tier Description
1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
NC NOT COVERED – Drugs that are not covered by the plan.
Code Definition
PA Prior Authorization
Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
QL Quantity Limits
Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
ST Step Therapy
Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
OR Other Restrictions
Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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