DrugindexOnline^{2nd edition}

Pronouncation: (al-fa 1 PROE-teen-ase)
Class: Respiratory enzyme

Trade Names:
Aralast
- Powder for injection, lyophilized 400 mg (greater than or equal to 16 mg/mL alpha-1 proteinase inhibitor when reconstituted)
- Powder for injection, lyophilized 800 mg (greater than or equal to 16 mg/mL alpha-1 proteinase inhibitor when reconstituted)

Trade Names:
Prolastin
- Powder for injection, lyophilized 500 mg (greater than or equal to 20 mg/mL alpha-1 proteinase inhibitor when reconstituted)
- Powder for injection, lyophilized 1,000 mg (greater than or equal to 20 mg/mL alpha-1 proteinase inhibitor when reconstituted)

Trade Names:
Zemaira
- Powder for injection, lyophilized 1,000 mg

Mechanism of Action

Pharmacology

Inhibits serine proteases (eg, neutrophil elastase), which are capable of degrading protein components of the alveolar walls and which are chronically present in the lung.

Pharmacokinetics

Metabolism

Metabolic t _½ 5.9 days.

Indications and Usage

Chronic augmentation therapy in patients with congenital deficiency of alpha-1 proteinase inhibitor with clinically evident emphysema.

Contraindications

Individuals with selective IgA deficiencies (IgA level less than 15 mg/dL) who have known antibodies against IgA.

Zemaira

History of anaphylaxis or severe systemic response to alpha-1 proteinase inhibitor products.

Dosage and Administration

Adults

IV 60 mg/kg infusion once weekly. Administer at a rate not exceeding 0.08 mL/kg/min.

General Advice

• Follow manufacturer's instructions for reconstitution of powder with supplied diluent and final dilution for infusion solution.
• Do not shake or agitate vials during reconstitution or dilution.
• Inspect solution visually before administration. Do not administer if solution is cloudy, discolored, or contains more than a few small particles.
• Discard any unused product. Vials are for single-use only. Do not save medication for future use.
• Infuse prescribed dose over period of 30 min or so. Do not exceed IV infusion rate of 0.8 mL/kg/min.
• Infuse Zemaira reconstituted solution through supplied in-line filter.
• Do not administer in same IV line with other products.

Storage/Stability

Store vials in refrigerator (36° to 46°F). Do not freeze. Vials may be removed from refrigerator and stored at controlled room temperature (not to exceed 77°F). Aralast must be used within 1 mo once removed from refrigeration. Use solution within 3 h of reconstitution. Do not refrigerate solution after reconstitution.

Drug Interactions

None well documented.

Incompatibility

Do not mix with other agents or diluting solutions.

Laboratory Test Interactions

None well documented.

Adverse Reactions

Cardiovascular

Prolastin

Hypotension, tachycardia (postmarketing).

CNS

Zemaira

Headache (3%); dizziness (1%).

Dermatologic

Prolastin

Rash (postmarketing).

Zemaira

Pruritus (1%).

EENT

Aralast

Pharyngitis (2%).

Zemaira

Sinusitis (2%).

Hepatic

Aralast

ALT or AST elevations (11%).

Musculoskeletal

Zemaira

Asthenia (1%).

Pulmonary

COPD.

Respiratory

Aralast

Upper and lower respiratory tract infections (96%).

Prolastin

Dyspnea (postmarketing).

Zemaira

Upper respiratory tract infection (2%).

Miscellaneous

Prolastin

Fever (1%); flu-like symptoms, allergic-like reactions, chills (postmarketing).

Zemaira

Injection-site pain, paresthesia (1%).

Precautions

Monitor Infusion reaction Monitor patient for development of infusion reaction. If infusion reaction develops, decrease the infusion rate or temporarily stop the infusion until symptoms subside. Resume infusion at rate tolerated by patient. Vital signs Monitor vital signs and carefully observe patient throughout the infusion.

Pregnancy

Category C .

Lactation

Undetermined.

Children

Safety and efficacy not established.

Anaphylaxis

Immediately discontinue the infusion if severe anaphylactoid or anaphylactic reactions occur.

Circulatory overload

Use with caution in patients at risk of circulatory overload.

Hepatitis B

Ensure that patient receiving Prolastin is immunized against hepatitis B before beginning therapy.

Infection

Because alpha-1 proteinase inhibitor is derived from pooled human plasma, there is a risk of transmitting infectious agents (eg, viruses) and, theoretically, Creutzfeldt-Jakob disease.

Patient Information

• Advise patient, family, or caregiver that medication will be prepared and administered by a health care provider in a health care setting.
• Advise patient, family, or caregiver to report any signs or symptoms of hypersensitivity reaction (eg, hives, rash, chest tightness, wheezing, difficulty breathing, faintness).
• Advise patient, family, or caregiver to report the following symptom complex to health care provider: fever, drowsiness, chills, and runny nose followed 2 wk later by rash and joint pain.