Inborn errors of metabolism:

Indications for ZEMAIRA:

For chronic augmentation and maintenance therapy in adults with alpha1-proteinase inhibitor (A1-PI) deficiency and clinical evidence of emphysema.

Adult:

Give by IV infusion at a rate of 0.08mL/kg/min. 60mg/kg once weekly. Monitor infusion rates and clinical state closely during infusion. Reduce rate or discontinue if adverse reactions occur; resume at lower rate if subside.

Children:

Not established.

Contraindications:

History of anaphylaxis or severe systemic response to A1-PI products. IgA deficiencies with known antibodies against IgA.

Warnings/Precautions:

Not for lung disease where severe A1-PI deficiency has not been established. Monitor for hypersensitivity reactions; discontinue immediately if anaphylaxis or severe reactions occur. Have epinephrine and other supportive therapy readily available. Contains human plasma; monitor for possible infection transmission (eg, viruses, Creutzfeldt-Jakob disease). Pregnancy. Nursing mothers.

Adverse Reactions:

Headache, sinusitis, upper respiratory infection, bronchitis, asthenia, cough increased, fever, inj site hemorrhage, rhinitis, sore throat, vasodilation; hypersensitivity reactions.

How Supplied:

Single-use vial—1 (w. diluent + filter transfer set)