Taliglucerase Alfa

Pronunciation: TAL-i-GLOO-ser-ase AL-fa
Class: Enzyme replacement

Trade Names

Elelyso
- Injection, lyophilized powder for solution 200 units

Pharmacology

Catalyzes the hydrolysis of glucocerebroside to glucose and ceramide.

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Pharmacokinetics

Distribution

Vd at steady state is 7.3 to 11.7 L.

Elimination

The median terminal half-life is 18.9 to 28.7 min. Cl ranges from 20 to 30 L/h.

Indications and Usage

Long-term enzyme replacement therapy for adults with confirmed diagnosis of type I Gaucher disease.

Contraindications

None well documented.

Dosage and Administration

Adults

IV 60 units/kg once every 2 wk. Adjust dose based on achievement and maintenance of therapeutic goals.

Conversion

Patients currently treated with a stable dose of imiglucerase may be switched to taliglucerase at the same dose.

General Advice

• For IV infusion only.
• Reconstitute vial with 5.1 mL of sterile water for injection. Mix vial gently; do not shake. Withdraw 5 mL of reconstituted product and further dilute with sodium chloride 0.9% to a final volume of 100 to 200 mL. Mix gently; do not shake. Use immediately after reconstitution. Slight flocculation (translucent fibers) may occur after dilution. Prepare using low–protein-binding containers.
• Administer diluted solution with a low–protein-binding infusion set equipped with an in-line low–protein-binding 0.2 micrometer filter by IV infusion at an initial infusion rate of 1.3 mL/min. After patient tolerability to the infusion rate is established, infusion rate may be increased to 2.3 mL/min. Total volume of infusion solution should be delivered over a period of no less than 1 h.
• Consider pretreatment with antihistamines and/or corticosteroids to prevent subsequent infusion reactions in those cases in which symptomatic treatment was required.

Storage/Stability

Store vials at 36° to 46°F; protect from light. Do not heat or microwave. Reconstituted or diluted product may be stored for up to 24 h at 36° to 46°F. Do not freeze. Protect from light. Discard any unused product.

Drug Interactions

None well documented.

Adverse Reactions

CNS

Headache (19%); asthenia, dizziness, fatigue, insomnia, paresthesia (more than 2%).

Dermatologic

Erythema, pruritus, rash, skin irritation (more than 2%).

EENT

Pharyngitis/throat infection (19%); pharyngolaryngeal pain, throat irritation (more than 2%).

GI

Abdominal pain, diarrhea, nausea (more than 2%).

Genitourinary

UTI/pyelonephritis (11%).

Hepatic

ALT increased (more than 2%).

Local

Infusion-site pain (more than 2%).

Musculoskeletal

Arthralgia (13%); back pain, extremity pain (11%); bone pain, muscle spasms, musculoskeletal discomfort, musculoskeletal pain (more than 2%).

Respiratory

Upper respiratory tract infection/nasopharyngitis (22%); dyspnea (more than 2%).

Miscellaneous

Infusion reaction, including angioedema, arthralgia, asthenia, back pain, chest pain or discomfort, coughing, cyanosis, dyspnea, erythema, fatigue, flushing, headache, increased BP, urticaria and wheezing (46%); influenza/flu (13%); chest discomfort, flushing, pain, peripheral edema (more than 2%); anaphylaxis.

Precautions

Monitor Monitor patients for infusion reactions. Monitor patients who develop infusion or immune reactions with taliglucerase and those who have had an immune reaction to other enzyme replacement therapies for the development of antibodies to taliglucerase.

Pregnancy

Category B .

Lactation

Undetermined.

Children

Safety and efficacy not established.

Hypersensitivity

Anaphylaxis has been reported.

Immunogenicity

Immunoglobulin G antibodies to taliglucerase have been reported.

Infusion reactions

Infusion reactions, including allergic reactions, were the most common adverse reaction reported. Pretreatment with antihistamines and/or corticosteroids may prevent subsequent reactions in those cases in which symptomatic treatment was required.

Overdosage

Symptoms

No data available.

Patient Information

• Advise patients that medication will be prepared and administered by a health care provider in a health care setting and that the infusion usually takes 60 to 120 min.
• Advise patients that medication may cause severe allergic reactions or infusion reactions. Counsel patients that they must be carefully reevaluated for treatment with taliglucerase if serious allergic reactions occur. Also counsel patients that infusion reactions can usually be managed by slowing the infusion rate; treatment with medications such as antihistamines, antipyretics, and/or corticosteroids; and/or stopping and resuming treatment with decreased infusion rate. Inform patients that pretreatment with antihistamines and/or corticosteroids may prevent subsequent reactions.
• Advise patients to report any adverse reactions while receiving taliglucerase treatment.

Copyright © 2009 Wolters Kluwer Health.