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taliglucerase alfa (Rx)Brand and Other Names:Elelyso

 
 
 

Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

injection, lyophilized powder for reconstitution

  • 200 units/vial
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Gaucher Disease

Indicated for enzyme replacement in patients with confirmed diagnosis of type 1 (non-neuropathic) Gaucher disease

60 units/kg IV q2weeks initially

Administration

Reconstitute, dilute and administer under the supervision of a healthcare professional

Infuse IV over 1-2 hr

Adjust dose based on achievement and maintenance of each patient’s therapeutic goals; clinical trials have evaluated doses ranging from 11-73 units/kg every other week

Patients on a stable dose of imiglucerase can be switched to taliglucerase alfa at that same dose

Dosage Forms & Strengths

injection, lyophilized powder for reconstitution

  • 200 units/vial
more...

Gaucher Disease

Indicated for enzyme replacement in patients with confirmed diagnosis of type 1 (non-neuropathic) Gaucher disease

<4 years: Safety and efficacy not established

≥4 years: 60 units/kg IV q2weeks initially

Administration

Infuse IV over 1-2 hr

Adjust dose based on achievement and maintenance of each patient’s therapeutic goals; clinical trials have evaluated doses ranging from 11-73 units/kg every other week

Patients on a stable dose of imiglucerase can be switched to taliglucerase alfa at that same dose

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Adverse Effects

>10%

Vomiting, pediatrics (44%)

Hypersensitivity reactions (29%)

Headache (19%)

Arthralgia (13%)

1-10%

Fatigue (9%)

Nausea (9%)

Dizziness (9%)

Abdominal pain (6%)

Pruritus (6%)

Flushing (6%)

Vomiting (6%)

Urticaria (6%)

Serious hypersensitivity reactions, including anaphylaxis (2.8%)

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Warnings

Contraindications

None

Cautions

Anaphylaxis reported; if anaphylaxis occurs, discontinue infusion immediately and initiate appropriate treatment

Allergic and infusion reactions are common and include headache, chest pain or discomfort, asthenia, fatigue, urticaria, erythema, increased blood pressure, back pain, arthralgia, and flushing; if allergic or infusion reactions occur, decrease the infusion rate or temporarily stop the infusion; antihistamines and/or antipyretics may be administered; medical support should be readily available when taliglucerase alfa administered; observe patients closely for an appropriate period of time after administration; reactions have occurred up to 3 hr after the start of infusion

Consider risks and benefits of re-administering taliglucerase alfa in patients who have experienced severe reaction associated with taliglucerase alfa; use caution upon rechallenge; appropriate medical support should be readily available

As with all therapeutic proteins, development of IgG anti-drug antibodies (ADA) has been reported (53%); unknown if presence of ADA is associated with higher risk of infusion reactions or immunogenicity

Monitor patients that have had an immune response to other enzyme replacement therapies or who are switching to taliglucerase alfa; unknown if these patients are at higher risk of infusion reactions

Dizziness and fatigue reported; caution patients against performing dangerous tasks that require mental alertness, including driving heavy machinery

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Pregnancy & Lactation

Pregnancy Category: B

Lactation: Unknown whether distributed in breast milk; caution advised

Pregnancy 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.

more...
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Pharmacology

Mechanism of Action

Gaucher disease is caused by a deficiency of the enzyme to catalyze glucocerebrosidase resulting in lipid accumulation in the spleen, liver, kidneys, and other organs; major signs include liver or spleen damage, anemia, and thrombocytopenia

Taliglucerase alfa catalyzes the hydrolysis of glucocerebroside to glucose and ceramide which results in reduced spleen and liver enlargement, and increased RBCs and platelets

Distribution

Vd: 7.3-11.7 L

Elimination

Half-life: 18.9-28.7 minutes

Systemic clearance: 20 L/hr (for 60 units/kg dose)

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Administration

IV Preparation

Single use vial; do not use more than once

Remove required number of vials from refrigerator; do not leave at room temperature longer than 24 hr prior to reconstitution; do not heat or microwave

Using aseptic technique, reconstitute the vial with 5.1 mL sterile water for injection to yield 5.3 mL and a withdrawal volume of 5 mL

Upon reconstitution, mix vials gently (DO NOT SHAKE)

Prior to further dilution, visually inspect the solution in the vials; the solution should be clear and colorless; do not use if discolored or foreign particulate matter is present

Withdraw 5 mL (ie, 200 units/5 mL) of reconstituted solution from each vial and dilute with 0.9% NaCl to a final volume of 100-120 mL for pediatric patients or 130-15 mL for adults

Use a low-protein-binding container

IV Administration

Administered with a low-protein-binding infusion set equipped with an in-line, low-protein-binding 0.2 micrometer filter

Adults: Initial IV infusion rate of 1.2 mL/minute; may increase to 2.2 mL/minute after patient tolerability established

Pediatrics: Initial IV infusion rate of 1mL/minute; may increase to 2 mL/minute after patient tolerability established

The total volume of the infusion solution should be delivered over a period of at least 1 hour

Storage

Unopened vials

  • Refrigerate unopened vials between 2-8°C (36-46°F)
  • Protect vials from light

Reconstituted & diluted solution

  • Contains no preservative, the product should be used immediately once reconstituted; if immediate use is not possible, the reconstituted or the diluted product may be stored for up to 24 hours at 2-8°C (36-46°F)
  • Do not freeze
  • Protect from light
  • Discard any unused product
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Images

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Formulary

FormularyPatient Discounts

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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.

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.
QL Quantity Limits
Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
ST Step Therapy
Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
OR Other Restrictions
Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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