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Taliglucerase Alfa

Pronunciation

(tal i GLOO ser ase AL fa)

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

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Solution Reconstituted, Intravenous [preservative free]:

Elelyso: 200 units (1 ea) [contains polysorbate 80]

Brand Names: U.S.

  • Elelyso

Pharmacologic Category

  • Enzyme

Pharmacology

Taliglucerase alfa is an analogue of glucocerebrosidase; it is produced by recombinant DNA technology using plant (carrot) cell culture. Glucocerebrosidase is an enzyme deficient in Gaucher's disease. It is needed to catalyze the hydrolysis of glucocerebroside to glucose and ceramide, thereby reducing liver and spleen size and improving anemia and thrombocytopenia.

Distribution

Distribution: Vss: Adults: 10.7 to 11.7 L; Pediatric patients: 8.8 to 14.9 L

Half-Life Elimination

Adults: 19 to 29 minutes (dose-dependent; increased with higher doses); Pediatric patients: 33 to 37 minutes

Use: Labeled Indications

Gaucher disease: Treatment of adult and pediatric patients with a confirmed diagnosis of type 1 Gaucher disease.

Canadian labeling: Additional use (not in US labeling): Treatment of hematologic manifestations in pediatric patients 2 to 17 years of age with a confirmed diagnosis of type 3 Gaucher disease (limited data)

Contraindications

There are no contraindications listed in the US labeling.

Canadian labeling: Severe hypersensitivity to taliglucerase alfa or any component of the formulation.

Dosage

Note: Pretreatment with antihistamines, antipyretics, and/or corticosteroids can be considered for prevention of subsequent infusion reactions in patients with an infusion reaction requiring symptomatic treatment; during clinical studies, patients were not routinely premedicated prior to infusion.

US labeling:

Gaucher disease (type 1): Children ≥4 years, Adolescents, and Adults: IV: 60 units/kg every 2 weeks; dosing is individualized based on disease severity.

Conversion from imiglucerase: Initiate taliglucerase alfa using the patient’s same previous imiglucerase dose and administer every 2 weeks. Note: Conversion to taliglucerase alfa is based on a single study of patients stabilized on a biweekly imiglucerase dose for ≥6 months.

Canadian labeling:

Gaucher disease (type 1): Children ≥2 years, Adolescents, and Adults: IV: 30 to 60 units/kg every 2 weeks; dosing is individualized based on disease severity (better clinical response has been observed with 60 unit/kg dose).

Gaucher disease (type 3): Children ≥2 years and Adolescents: IV: 30 to 60 units/kg every 2 weeks; dosing is individualized based on disease severity (data are extremely limited; in a small clinical trial of pediatric patients [N=11], 2 were identified as having type 3 Gaucher disease [Zimran 2015]).

Dosage adjustment in renal impairment: There are no dosage adjustments provided in the manufacturer’s labeling.

Dosage adjustment in hepatic impairment: There are no dosage adjustments provided in the manufacturer’s labeling.

Reconstitution

Calculate the necessary dose; round up to the next whole vial when determining the number of vials needed. Reconstitute each vial with 5.1 mL of SWFI; mix gently; do not shake. Solution should be clear and colorless. Withdraw 5 mL of reconstituted solution from each vial (reconstituted vials contain 5.3 mL) and further dilute in NS to a final volume of 100 to 200 mL. For pediatric patients, use a final volume of 100 to 120 mL; for adults, a final volume of 130 to 150 mL may be used. Final volume should not exceed 200 mL for adults using ≥130 to 150 mL or more of reconstituted product. Slight flocculation may occur following dilution; this is acceptable for administration. Discard any unused product.

Administration

Administer IV over a minimum infusion time of 60 minutes (usual infusion time: 60 to 120 minutes). Administer using a low protein-binding infusion set with a 0.2 micron in-line filter.

Pediatric patients: Initiate infusion at a rate of 1 mL/minute; rate may be increased, but do not exceed 2 mL/minute based on patient tolerance.

Adult patients: Initiate infusion at a rate of 1.2 mL/minute; rate may be increased, but not exceed 2.2 mL/minute based on patient tolerance.

Compatibility

Stable in NS

Storage

Store intact vials at 2°C to 8°C (36°F to 46°F); protect from light. Do not freeze. If not used immediately, the reconstituted solution may be stored at 2°C to 8°C (36°F to 46°F) for ≤24 hours (protect from light) or at 20°C to 25°C (68°F to 77°F) for ≤4 hours (without protection from light). The Canadian labeling suggests that the reconstituted solution may be stored at room temperature for up to 12 hours when protected from light. The diluted solution for infusion may be stored at 2°C to 8°C (36°F to 46°F) for ≤24 hours; protect from light. In total, the reconstituted and diluted products can be stored for ≤24 hours. Do not freeze.

Drug Interactions

There are no known significant interactions.

Adverse Reactions

>10%:

Central nervous system: Headache (13% to 19%)

Gastrointestinal: Vomiting (children and adolescents 2 to 13 years 44%; adults 6%)

Hypersensitivity: Hypersensitivity reaction (22% to 29%; antibody-positive patients: 35%; patients switching from imiglucerase: 6%)

Immunologic: Antibody formation (adults 13% to 53%; neutralizing but effect on therapeutic response not evaluated 10%; positive at baseline 6%; children and adolescents 2 to 13 years 22%)

Neuromuscular & skeletal: Arthralgia (13%), limb pain (10%)

Respiratory: Pharyngitis (4% to 19%)

1% to <10%:

Cardiovascular: Flushing (6%)

Central nervous system: Dizziness (9%), fatigue (9%)

Dermatologic: Pruritus (6%), urticaria (6%), skin rash (≥5%)

Gastrointestinal: Nausea (9%), abdominal pain (6%)

Hypersensitivity: Fixed drug eruption (4%; type III immune-mediated; mild and intermittent), anaphylaxis (3%)

Neuromuscular & skeletal: Back pain (≥5%)

Warnings/Precautions

Concerns related to adverse effects:

• Antibody formation: The development of IgG anti-drug antibodies (ADA) has been reported; the clinical significance is unknown. Patients who develop immune or infusion reactions to taliglucerase alfa or who have had an immune response to other enzyme replacement therapies and who are switching to taliglucerase alfa should be monitored for antibody development; it is unknown if presence of antibodies is related to a higher risk of infusion reactions.

• CNS effects: Dizziness and fatigue have been observed with therapy; caution patients about performing dangerous tasks (eg, driving, operating machinery).

• Hypersensitivity/anaphylactoid reactions: Serious hypersensitivity reactions, including anaphylaxis, may occur; these reactions have occurred up to 3 hours after start of infusion. Appropriate medical support should be readily available. Base management of reaction on severity; may include slowing or temporary interruption of infusion and/or premedication (eg, antihistamine, antipyretics, corticosteroids) for mild reactions. Pretreatment may prevent subsequent reactions. Observe patient during and after infusion. If severe reactions occur, immediately discontinue infusion and initiate appropriate treatment; rechallenge with caution.

Monitoring Parameters

Hemoglobin, platelet count, angiotensin converting enzyme, tartrate-resistant acid phosphatase, chitotriosidase, IgG anti-drug antibody formation (in patients who experience, or previously experienced, immune or infusion reactions to enzyme replacement therapy); liver volume, spleen volume; bone density; ECG, echocardiogram, chest x-ray

Pregnancy Considerations

Adverse effects were not observed in animal reproduction studies. Pregnancy may exacerbate existing type I Gaucher disease or result in new symptoms. Women with type I Gaucher disease have an increased risk of spontaneous abortion if disease is not well controlled. Adverse pregnancy outcomes, including hepatosplenomegaly and thrombocytopenia (which may result in increased bleeding and postpartum hemorrhage requiring transfusion), may occur.

Patient Education

• Discuss specific use of drug and side effects with patient as it relates to treatment. (HCAHPS: During this hospital stay, were you given any medicine that you had not taken before? Before giving you any new medicine, how often did hospital staff tell you what the medicine was for? How often did hospital staff describe possible side effects in a way you could understand?)

• Patient may experience myalgia, arthralgia, asthenia, pharyngitis, rhinorrhea, rhinitis, sternutation, flu-like symptoms, painful extremities, or back pain. Have patient report immediately to prescriber dyspnea, severe dizziness, syncope, angina, edema of extremities, difficult urination, dysuria, polyuria, skin discoloration, flushing, intolerable nausea, or considerable headache (HCAHPS).

• Educate patient about signs of a significant reaction (eg, wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat). Note: This is not a comprehensive list of all side effects. Patient should consult prescriber for additional questions.

Intended Use and Disclaimer: Should not be printed and given to patients. This information is intended to serve as a concise initial reference for health care professionals to use when discussing medications with a patient. You must ultimately rely on your own discretion, experience, and judgment in diagnosing, treating, and advising patients.

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