AdultPediatricDosage Forms & Strengths lyphilized powder for reconstitution 0.5g/vial (Aralast NP) 1g/vial (Aralast NP, Zemaira) 4g/vial (Zemaira) 5g/vial (Zemaira) ready-to-use IV solution 1g/50mL (Glassia) 1g/20mL (Prolastin C) Alpha-1 Antitrypsin Deficiency Indicated for chronic augmentation and maintenance therapy in adults with emphysema caused by congenital alpha-1-proteinase inhibitor deficiency 60 mg/kg IV infusion qWeek IV infusion rate Aralast NP, Prolastin C, Zemaira: Not to exceed 0.08 mL/kg/min Glassia: 0.04 mL/kg/min Diabetes Mellitus, Type-1 (Orphan) Glassia (IV): Treatment of recent onset (<15 yr) with type 1A diabetes mellitus with residual beta-cell function Prolastin C: Orphan designation for treatment of type 1 diabetes mellitus patients with residual beta-cell function Orphan sponsors Glassia: Kamada, Ltd.; Kiryat Weizmann, Science Park; Ness-Ziona 74036 Israel Prolastin C: Grifols Therapeutics, Inc; 8368 US Business Hwy 70 West; Clayton, NC 27520 Alpha-1 Proteinase Inhibitor Deficiency (Orphan) Inhalation therapy for treatment of congenital deficiency of alpha-1 proteinase inhibitor Orphan sponsor Kamada, Ltd.; Kiryat Weizmann, Science Park; Ness-Ziona 74036 Israel Bronchopulmonary Dysplasia (Orphan) Prevention of bronchopulmonary dysplasia Orphan sponsor Arriva Pharmaceuticals, Inc; 1010 Atlantic Avenue; Alameda, CA 94501 Graft vs Host Disease (Orphan) Glassia: Treatment of GVHD Orphan sponsor CSL Behring LLC; 1020 First Ave, PO Box 61501; King of Prussia, PA Cystic Fibrosis (Orphan) Orphan Sponsor CSL Behring LLC; 1020 First Avenue; King of Prussia, PA 19406 Arriva Pharmacueticals, Inc; 1010 Atlantic Avenue; Alameda, CA 94501 PPL Therapeutics (Scotland) Limited; Roslin, Edinburgh EH259PP, Scotland, United Kingdom Safety and efficacy not established >10% Increases ALT, AST >2 times upper limit or normal (11%) 1-10% Infusion Reactions (9.6%) Headache (0.7%) Pharyngitis (1.6%) Increased cough (0.6%) <1% Headache (0.3%) Somnolence (0.3%) Chills and fever (0.1%) Chest pain (0.1%) Dizziness (0.1%) Increased cough (0.1%) Pruritus (0.1%) Rash (0.1%) Vasodilation (0.1%) Contraindications Hypersensitivity Selective IgA deficiencies (IgA <15 mg/dL) with known IgA antibodies Liver disease associated with alpha 1-proteinase inhibitor deficiency Cautions Patients at risk of circulatory overload D/C immediately if anaphylactic or severe anaphylactoid reaction Theoretical risk of pathogen transmission Report suspected infection Aralast to Alpha Therapeutic Corp. 1-888-675-2762 (US) or 1-323-225-9735 (international) Prolastin to Bayer Corp 1-800-288-8371 Zemaira to ZLB Behring 1-800-504-5434 Pregnancy Category: C Lactation: not known if distributed into breast milk, use cautionPregnancy Categories A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk. B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk. C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done. D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk. X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist. NA: Information not available. Mechanism of Action Purified human alpha-1-proteinase inhibitor (alpha-1-antitrypsin, ATT) inhibits serine proteases such as neutrophil elastase in lungs; alpha1-antitrypsin deficiency leads to the development of emphysema resulting from increased elastic damage in the lung Source: Human plasma donors Pharmacokinetics Half-life elimination: 5-6 days Vd: 3.5 L Peak plasma time: 1 hr (3 weeks to achieve threshold levels) Metabolism: Undergoes catabolism in the intravascular space; 33% of AAT is catabolized per day IV Preparation Use aseptic technique Reconstitution requires no more than 5 min for 0.5-g or 1-g vials and no more than 10 min for 4-g or 5-g vials Gently swirl vial; do not shake Do not invert vial until ready to withdraw contents When reconstitution procedure is strictly followed, a few small particles may remain; will be removed by microaggregate filter IV Administration For IV infusion only IV infusion rate Aralast NP, Prolastin C, Zemaira: Not to exceed 0.08 mL/kg/min Glassia: Not to exceed 0.04 mL/kg/min Storage Unopened vials Aralast NP, Prolastin C, Zemaira: Temperatures not to exceed 25°C (77°F); avoid freezing (may damage diluent vial) Glassia: 2-8 °C (36-46 °F); do not freeze Opened vials Store at room temperature Use within 3 hr of entering/reconstituting the vials to avoid the potential ill effect of any inadvertent microbial contamination No images available for this drug. No images available for this drug.A Patient Handout is not currently available for this monograph.Print FormularyPatient DiscountsAdding plans allows you to compare formulary status to other drugs in the same class.To view formulary information first create a list of plans. Your list will be saved and can be edited at any time.Create Your List of PlansAdding plans allows you to:View the formulary and any restrictions for each plan.Manage and view all your plans together – even plans in different states.Compare formulary status to other drugs in the same class.Access your plan list on any device – mobile or desktop. The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information. View explanations for tiers and restrictions Tier Description 1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs. 2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs. 3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs. 4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products. 5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products. 6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products. NC NOT COVERED – Drugs that are not covered by the plan. Code Definition PA Prior Authorization Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription. 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