ALPHA1-PROTEINASE INHIBITOR (HUMAN)
ALPHA1-PROTEINASE INHIBITOR (HUMAN)  (pro'ten-ase)  Prolastin, Aralast, Zemaira Classifications: enzyme inhibitor; Therapeutic: enzyme inhibitor Pregnancy Category: C |
Availability
Prolastin: 25 mg/mL; Aralast: 16 mg/mL; Zemaira: 50 mg/mL
Action
Alpha1-proteinase inhibitor (alpha1-PI; alpha1-antitrypsin) is extracted from plasma and used in patients with panacinar emphysema who have alpha1-antitrypsin deficiency. Alpha1-antitrypsin deficiency is a chronic, hereditary, and usually fatal autosomal recessive disorder that results in a slowly progressive, panacinar emphysema.
Therapeutic Effect
Prevents the progressive breakdown of elastin tissues in the alveoli, thus slowing panacinar emphysema progression.
Uses
Indicated for chronic replacement therapy in patients with alpha1-antitrypsin deficiency and demonstrable panacinar emphysema.
Contraindications
Individuals with selective IgA deficiencies; pregnancy (category C); lactation.
Cautious Use
Patients with significant heart disease or other conditions that may be aggravated with slight increases in plasma volume. Safety and efficacy in children are not established.
Route & Dosage
| Panacinar Emphysema Adult: IV 60 mg/kg once/wk |
Administration
| Intravenous
PREPARE: IV Infusion: ??Warm unopened diluent and concentrate to room temperature.??Use the supplied, double needle transfer device to reconstitute with sterile water for injection (supplied by manufacturer) to yield a concentration of 20 mg/mL. ADMINISTER: IV Infusion: ?? Give within 3 h after reconstitution.??Give alone, without mixing with other agents.??Administer at rate of 0.08 mL/kg/min or more slowly as determined by response and comfort of the patient.?? Note: The recommended dosage takes about 30 min to administer to a 70 kg person. |
- Store unreconstituted drug at 2°–8° C (35°–46° F). Do not refrigerate after reconstitution. Discard unused solution.
Adverse Effects (≥1%)
Hematologic: Leukocytosis. CNS: Dizziness, fever (may be delayed). Respiratory: Upper and lower respiratory tract infections. Other: Hepatitis B if not immunized.Pharmacokinetics
Distribution: Crosses placenta; distributed into breast milk. Metabolism: Undergoes catabolism in the intravascular space; approximately 33% is catabolized per day. Half-Life: 4.5–5.2 d.Nursing Implications
Assessment & Drug Effects
- Administer with caution in patients at risk for circulatory overload. Monitor cardiac status.
- Monitor respiratory status (rate, dyspnea, lung sounds) throughout therapy.
- Lab tests: Monitor serum alpha1-PI level (minimum serum concentration level should be 80 mg/mL); periodic pulmonary functions and ABGs.
Patient & Family Education
- Avoid smoking and notify physician of any changes in respiratory pattern.
Common adverse effects in italic, life-threatening effects underlined; generic names in bold; classifications in SMALL CAPS; 
Canadian drug name; Prototype drug