Alpha 1-Proteinase Inhibitor Dosage

Medically reviewed by Drugs.com. Last updated on Sep 18, 2023.

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.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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