Usual Adult Dose for Alpha-1 Proteinase Inhibitor Deficiency

60 mg/kg intravenously once a week

Comments:

• Dose ranging studies using efficacy endpoints have not been performed.

Use: Chronic augmentation and maintenance therapy in adults with clinical evidence of emphysema due to hereditary deficiency of Alpha 1-Proteinase Inhibitor (alpha 1-antitrypsin deficiency)

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

CONTRAINDICATIONS:

• IgA deficient patients with antibodies against IgA (severe hypersensitivity risk)
• Anaphylaxis or other severe systemic reaction to alpha 1-proteinase inhibitor

Safety and efficacy have not been established in patients younger than 18 years.

Report all infections thought by a physician to possibly be from this drug to the manufacturer or the FDA at www.fda.gov/medwatch.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• Visually inspect for particulate matter and discoloration prior to administration; the product may contain a few protein particles; do not use if discolored or cloudy.
• Use an inline filter for administration; the manufacturer product information should be consulted.
• Consult the manufacturer product information for infusion speed.

Storage requirements:

• Refrigerate; do not freeze
• Do not re-refrigerate once the product has been stored at room temperature.

Reconstitution/preparation techniques:

• The carton and label on each vial show the actual amount of alpha 1-proteinase in milligrams (as determined by the capacity to neutralize porcine pancreatic elastase).
• Allow unopened vial to warm up to room temperature.
• Visually inspect for particulate matter and discoloration prior to administration; the product may contain a few protein particles; do not use if discolored or cloudy.
• Pool drug from several vials to achieve the intended dose into an empty, sterile intravenous solution container using aseptic technique.
• Keep at room temperature; administer within 3 hours.

IV compatibility:

• Do not mix with other agents or diluting solutions.

Monitoring:

• Monitor the infusion rate closely during administration and observe the patient for infusion related reactions.

Patient advice:

• Inform patients about the signs of hypersensitivity including pruritus, generalized urticaria, flushing, swollen lips/tongue/uvula, wheezing, tightness of chest, dyspnea, hypotension, and syncope.
• Inform patients this product is made from human plasma and has a risk of transmitting infectious agents that can cause disease, although this risk is reduced by donor screening, testing plasma for certain viruses, and inactivating or removal during manufacturing.
• This product raises plasma Alpha 1-proteinase levels, but effects on pulmonary exacerbations and the progression of emphysema have not been demonstrated in adequately powered, randomized, controlled clinical trials.