Nexviazyme

Long-term enzyme replacement therapy for patients w/ Pompe disease (acid α-glucosidase deficiency).

IV infusion 20 mg/kg once every 2 wk. Begin at initial rate of 1 mg/kg/hr & gradually increase every 30 min until max rate of 7 mg/kg/hr (for 20 mg/kg dose) & 10 mg/kg/hr (for 40 mg/kg dose). Infantile-onset Pompe disease (IOPD) patient who experience lack of improvement or insufficient response in cardiac, resp &/or motor function in 20 mg/kg Increase dose to 40 mg/kg every other wk. Patient who does not tolerate avalglucosidase α at 40 mg/kg every other wk Decrease dose to 20 mg/kg every other wk.

Life-threatening hypersensitivity when re-challenge was unsuccessful.

Discontinue immediately if severe hypersensitivity or anaphylaxis; severe infusion-associated reactions (IARs) occur. Slow or temporarily stop infusion rate if mild or moderate hypersensitivity reactions & IARs occur. IAR during &/or w/in few hr after infusion & w/ higher infusion rates. Administration of general anaesth for placement of central venous catheter or other surgical procedures in patients w/ IOPD w/ cardiac hypertrophy; cardiac arrhythmia including ventricular fibrillation & tachycardia, & bradycardia resulting in cardiac arrest. Patients w/ cardiac hypertrophy & significantly compromised resp function; acute underlying illness at the time of infusion; advanced Pompe disease w/ compromised cardiac & resp function; susceptible to fluid vol overload. Give antihistamines, antipyretics, &/or corticosteroids to prevent or reduce IARs. Consider anti-drug Ab (ADA) testing if patients do not respond to therapy; adverse-event-driven immunologic testing including IgG & IgE ADA in patients who have risk for allergic reaction or previous anaphylactic reaction to avalglucosidase α. May affect ability to drive & use machines. Hepatic & moderate or severe renal impairment. Pregnancy & lactation. Childn ≤6 mth.

Hypersensitivity; headache; nausea; pruritus, rash. Anaphylaxis; dizziness, somnolence, tremor, burning sensation; ocular & conjunctival hyperaemia, eye pruritus, eyelid oedema; tachycardia; HTN, flushing, cyanosis, hot flush, hypotension, pallor; cough, dyspnoea, resp distress, throat irritation, oropharyngeal pain; diarrhoea, vomiting, lip swelling, swollen tongue, abdominal & upper abdominal pain, dyspepsia; urticaria, erythema, erythematous & pruritic rash, hyperhidrosis, palmer erythema, skin plaque; muscle spasms, myalgia, pain in extremity, flank pain; fatigue, chills, chest discomfort, pain, flu-like illness, infusion site pain, pyrexia, asthenia, face oedema, feeling cold & hot, sluggishness; increased BP & body temp, decreased O₂ saturation.

Other Agents Affecting Metabolism

A16AB22 - avalglucosidase alfa ; Belongs to the class of enzymes. Used in the treatment of alimentary tract and metabolism problems.

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