alpha1-proteinase inhibitor, human

Therapeutic Classification: pulmonary replacement enzyme inhibitor

Pharmacologic Classification: enzyme inhibitors

Indications ⬆ ⬇

REMS

Replacement therapy (chronic) in patients with emphysema associated with alpha₁-antitrypsin deficiency.

Action ⬆ ⬇

Prevents the destructive action of elastase on alveolar tissue in patients who have alpha₁-antitrypsin deficiency.

Therapeutic effects:

Slowing of the destructive process on lung tissue.

Pharmacokinetics ⬆ ⬇

Absorption: Following IV administration, absorption is essentially complete.

Distribution: Achieves high concentration in epithelial fluid of the lungs.

Metabolism/Excretion: Broken down in the intravascular space.

Half-Life: 4.5–5.9 days.

Time/Action Profile ⬆ ⬇

(↑ serum levels of alpha₁-proteinase inhibitor)

ROUTE	ONSET	PEAK	DURATION
IV	2–6 days	within 3 wks	unknown

Contraind./Precautions ⬆ ⬇

Contraindicated in:

Hypersensitivity to polyethylene glycol (Aralast);
Emphysema associated with alpha₁-antitrypsin deficiency, where risk of panacinar emphysema is small (PiMZ and PiMS phenotype). ;
Selective IgA deficiency with known anti-IgA antibody (↑ risk of allergic reactions).

Use Cautiously in:

Patients at risk for volume overload;
OB: Safety not established.

Adv. Reactions/Side Effects ⬆ ⬇

GI: ↑liver enzymes

Hemat: transient leukocytosis

Neuro: dizziness, headache

Resp: cough, respiratory tract infection, sore throat

Misc: delayed fever

Interactions ⬆ ⬇

Drug-drug:

None known.

Route/Dosage ⬆ ⬇

IV (Adults ): 60 mg/kg once weekly.

Availability ⬆ ⬇

Injection: 500 mg/vial (Aralast, Aralast NP); 1000 mg/vial (Aralast, Aralast NP, Glassia, Prolastin-C, Zemaira)

Assessment ⬆ ⬇

Monitor respiratory status (rate, lung sounds, dyspnea) prior to and weekly during therapy.
Assess for fever, chills, and dizziness. May occur up to 12 hr after infusion and disappear over 24 hr. Treat symptomatically.
Monitor for signs of fluid overload (dyspnea, rales/crackles, hypertension, jugular venous distention) in patients at risk for volume overload.
If adverse events occur during the infusion, decrease rate or discontinue until reactions resolve.

Lab Test Considerations:

Monitor serum alpha₁-proteinase inhibitor levels to determine response to therapy. Minimum serum concentration should be 80 mg/dL.
- May cause transient mild ↑ in leukocytes.

Implementation ⬆ ⬇

IV Administration:

IV Push: Bring drug and sterile water vials to room temperature. Follow manufacturer’s instructions for vacuum transfer using transfer device/needle. Reconstitute Aralast- NP, Prolastin-C and Zemaira with manufacurer-provided 20 mL vial of sterile water for injection. Swirl to mix; do not shake; may take 5 min for 500 mg and 10 min for 1000 mg vials until powder is dissolved. Glassia comes ready to use. Concentration: 500 mg vial: 25 mg/mL; 1000 mg vial: 50 mg/mL. Do not administer solutions that are discolored or contain particulate matter. Do not refrigerate after reconstitution. Use within 3 hr. Discard unused solution.
Rate: Administer IV push at a rate of 0.08 mL/kg/min Aralast- NP, Prolastin-C or Zemairaover 15–30 min or 0.04 mL/kg/min over 60–80 min Glassia. Use the large volume 5-micron conical filter provided for administration of Zemaira.
Incompatibility: Administer separately. Do not mix with other drugs or diluents.

Patient/Family Teaching ⬆ ⬇

Explain purpose of medication and need for long term weekly therapy to patient and family. Patient should avoid smoking and notify health care professional of any changes in breathing pattern or sputum production.
Inform patient that fever may occur within 12 hr after infusion and will resolve by 24 hr. Fever may be treated symptomatically.
Advise patient of need for periodic pulmonary function tests to determine disease progression and response to therapy.
Explain purpose of hepatitis B vaccine prior to beginning therapy. A small risk of hepatitis is caused by the manufacturing process, and vaccination is recommended.

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Pronunciation ⬇

Classifications ⬆ ⬇