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INTRODUCTION

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laronidase (la-ron-i-dase)

Aldurazyme

Classification

Therapeutic: replacement enzymes

Pharmacologic: enzymes

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Indications
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Mucopolysaccharidosis I (MPS I; specifically Hurler and Hurler-Scheie form or Scheie form) with moderate to severe symptoms.

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Action
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Replaces the naturally occurring enzyme α-L-iduronidase which is deficient in MPS I. Without replacement, the glucosaminoglycans dermatan and heparan accumulate in tissues. Therapeutic Effects: Decreased cellular, tissue, and organ damage due to MPS accumulation resulting in improved pulmonary function and walking capacity.

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Adverse Reactions/Side Effects
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Resp: respiratory tract infections. Derm: rash. Local: injection site reactions. Misc: ALLERGIC REACTIONS, INCLUDING ANAPHYLAXIS, infusion-related reactions.

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PHYSICAL THERAPY IMPLICATIONS

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Examination and Evaluation
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  • Monitor signs of allergic reactions and anaphylaxis, including pulmonary symptoms (tightness in the throat and chest, wheezing, cough, dyspnea) or skin reactions (rash, pruritus, urticaria). Be especially alert for allergy-like responses that occur during and after administration (infusion-related reactions). Notify physician immediately if these reactions occur.

  • Assess ambulation on level surfaces and during stair climbing. Document any changes in functional ability to help determine if drug therapy is effective in maintaining or improving motor function.

  • Assess for signs of respiratory tract infections such as fever, cough, sputum production, shortness of breath, difficulty breathing, and fatigue. Notify physician if these signs occur.

  • Monitor IV infusion site for pain, swelling, and irritation. Report prolonged or excessive infusion-site reactions to the physician.

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Interventions
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  • Implement therapeutic exercises (resistive training, coordination exercises, gait training) to complement the effects of drug therapy and help achieve optimal function.

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Pharmacokinetics
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Absorption: IV administration results in complete bioavailability.

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Distribution: Unknown.

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Metabolism and Excretion: Unknown.

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Half-life: 1.5–3.6 hr.

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TIME/ACTION PROFILE

ROUTE ONSET PEAK DURATION
IV unknown unknown 1 wk

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Contraindications/Precautions
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Contraindicated in: None known.

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Use Cautiously in: Pregnancy (use only if clearly needed); Lactation (safety not established).

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Interactions
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Drug-Drug: None known.

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Route/Dosage
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IV (Adults and Children): 0.58 mg/kg once weekly, pretreat with antipyretics and/or antihistamines.

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Availability
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Solution for injection: 2.9 mg/5 mL-vial.

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