• Mucopolysaccharridosis VI (MPS IV).

Contraindicated in:

• None.

Use Cautiously in:

• Febrile or respiratory illness
• OB: Use during pregnancy only if potential maternal benefit justifies potential fetal risk
• OB: Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant
• Pedi: Children <5 yr (safety and effectiveness not established).

CV: malaise.

Derm: facial edema.

EENT: conjunctivitis, corneal opacification, ear pain.

GI: gastroenteritis, abdominal pain.

MS: areflexia.

Neuro: SPINAL/CERVICAL CORD COMPRESSION.

Resp: dyspnea.

Misc: infusion reactions, rigors.

Drug-Drug:

• None noted.

• Solution for injection: 1 mg/mL;

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

Naglazyme

• Replaces a deficient enzyme in MPS IV. Without replacement, glycoaminoglycans accumulate resulting in cell, organ and tissue dysfunction.

• Improved walking and stair climbing.

Therapeutic Classification: replacement enzyme

Pharmacologic Classification: enzymes

Absorption: IV administration results in complete bioavailability.

Distribution: Widely distributed.

Metabolism/Excretion: Unknown.

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

(improved exercise parameters)

• Inform patient of purpose of galsulfase.
• Advise patient or caregiver to notify health care professional immediately if signs or symptoms of a hypersensitivity or infusion reaction occur during infusion or if signs or symptoms of cardiac or respiratory decompensation occur during or following an infusion. Inform patients using supplemental oxygen or continuous positive airway pressure (CPAP) during sleep to have these treatments readily available during infusion or extreme drowsiness/sleep induced by antihistamine use.
• Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding. Pregnancy can exacerbate preexisting clinical manifestations of MPS and lead to adverse pregnancy outcomes for both mother and fetus.

gal-SUL-fase

NDC Code^*

• 68135- BioMarin Pharmaceutical Inc.
  • 68135-020- NAGLAZYME
    • 68135-020-01- 1 VIAL in 1 CARTON (68135-020-01) > 5 mL in 1 VIAL