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INTRODUCTION

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galsulfase (gal-sul-fase)

Naglazyme

Classification

Therapeutic: replacement enzyme

Pharmacologic: enzymes

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Indications
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Mucopolysaccharidosis VI (MPS IV).

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Action
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Replaces a deficient enzyme in MPS IV. Without replacement, glycosaminoglycans accumulate resulting in cell, organ, and tissue dysfunction. Therapeutic Effects: Improved walking and stair climbing.

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Adverse Reactions/Side Effects
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CV: malaise. EENT: conjunctivitis, corneal opacification, ear pain. Resp: dyspnea. CV: chest pain, ↑ blood pressure. Derm: facial edema. GI: gastroenteritis, abdominal pain. MS: areflexia. Misc: INFUSION REACTIONS, rigors.

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PHYSICAL THERAPY IMPLICATIONS

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Examination and Evaluation
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  • Be alert for allergy-like responses (wheezing, laryngeal edema, urticaria, other skin reactions) that occur during and after administration (infusion reactions). Report these responses to the physician or nursing staff immediately.

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

  • Monitor any changes in muscle tone or deep tendon reflexes. Report increased muscle contractions or decreased reflex activity.

  • Assess blood pressure (BP) and compare to normal values (See Appendix F). Report a sustained increase in BP (hypertension) or any chest pain or difficult, labored breathing.

  • Monitor and report any vision problems, eye inflammation, or ear pain.

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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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Patient/Client-Related Instruction
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  • Instruct patient and family/caregivers to report other troublesome side effects such as severe or prolonged malaise, facial swelling, or GI problems (gastroenteritis, abdominal pain).

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Pharmacokinetics
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Absorption: IV administration results in complete bioavailability.

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Distribution: Widely distributed.

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Metabolism and Excretion: Unknown.

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Half-life: 9 min (after 1 wk of treatment, 26 min (after 24 wk of treatment).

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TIME/ACTION PROFILE (improve exercise parameters)

ROUTE ONSET PEAK DURATION
IV unknown 24 wk unknown

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Contraindications/Precautions
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Contraindicated in: None.

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Use Cautiously in: Febrile or respiratory illness; Pedi: Children <5 yr (safety not established); OB: Pregnancy or lactation (safety being evaluated).

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Interactions
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Drug-Drug: None noted.

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Route/Dosage
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IV (Adults and Children >5 yr): 1 mg/kg once weekly.

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Availability
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Solution for IV administration (diluted prior to use): 5 mg/5 mL in 5-mL vials.

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