Inborn errors of metabolism:
Indications for: NAGLAZYME
Mucopolysaccharidosis VI (MPS VI).
Adults and Children:
<5yrs: not recommended. ≥5yrs: Give by IV infusion over 4 hours. Initial infusion rate: 6mL/hr for the first hour, then, if tolerated, may increase to 80mL/hr for the remaining 3 hours. May extend infusion time up to 20 hours if infusion reactions occur. Pretreat with antihistamines w/wo antipyretics 30–60mins prior to starting infusion. 1mg/kg once weekly.
Discontinue if severe infusion reactions occur. Antihistamine pretreatment may increase risk of apneic episodes. Risk of acute cardiorespiratory failure in patients susceptible to fluid volume overload (eg, weighing ≤20kg, underlying respiratory illness, compromised cardiac and/or respiratory function). Consider evaluation of airway patency prior to treatment. Acute febrile or respiratory illness: delay infusion. Monitor for spinal/cervical cord compression (including back pain, limb paralysis, urinary and fecal incontinence). Pregnancy. Nursing mothers.
Recombinant human N-acetylgalactosamine 4-sulfatase enzyme.
Rash, pain, urticaria, pyrexia, pruritus, chills, headache, nausea, vomiting, abdominal pain, dyspnea; infusion reactions.
Generic Drug Availability:
Single-dose vial (5mL)—1