human alpha1-proteinase inhibitor: clinical use

Prescribing considerations

• Confirm documented severe deficiency and progressive emphysema through assessment by an experienced clinician.
• Ensure optimal respiratory management; strongly support smoking cessation and avoidance of environmental tobacco smoke.
• Assess suitability, competence and support before home administration, and review technique regularly.
• Consider hepatitis A and B vaccination.
• Account for sodium content in patients following a controlled-sodium diet.
• Record the product name and batch number for traceability.

Contraindications and cautions

• Hypersensitivity to human alpha1-proteinase inhibitor or an excipient.
• IgA deficiency with known anti-IgA antibodies because of the risk of severe hypersensitivity and anaphylaxis.

Monitoring

• Observe clinical status and vital signs closely during initial infusions.
• Monitor for infusion reactions; reduce or stop the infusion according to severity and provide emergency treatment for shock.
• Review home preparation, infusion technique, treatment records and adverse-reaction management.
• Monitor the underlying lung disease using specialist-selected clinical and functional measures.

Clinical pharmacology

A 51 kDa serine-protease inhibitor and normal constituent of human blood. It is the principal antiprotease against neutrophil elastase in the lower respiratory tract; replacement increases circulating and epithelial-lining-fluid concentrations.

Formulation and product differences

• Human plasma-derived powder with water for injections for intravenous infusion.
• Vial presentations use different solvent volumes but produce the same approximate concentration after reconstitution.
• Larger presentations include an administration set; all vials are single use.
• Sodium contribution varies by vial presentation.