General

Pronunciation:
tali-gloo-ser-ase al-fa

Trade Name(s)

• Elelyso

Ther. Class.
orphan drugs
replacement enzyme

Pharm. Class.
enzymes

Indications

Long-term replacement therapy for patients with confirmed Type 1 Gaucher disease.

Action

Prevents the accumulation of glucocerebrosides in cells. Replaces glucocerebrosidases that are deficient in type 1 Gaucher's disease.

Therapeutic Effect(s):

Improvement in symptoms of Gaucher disease (anemia, thrombocytopenia, bone disease, splenomegaly, and hepatomegaly)

Pharmacokinetics

Absorption: IV administration results in complete bioavailability

Distribution: Unknown.

Metabolism and Excretion: Unknown.

Half-life: 18.9–28.7 min

TIME/ACTION PROFILE

Contraindication/Precautions

Contraindicated in:

• None noted.

Use Cautiously in:

• OB:   Use during pregnancy only if clearly needed
• Lactation: Use cautiously in nursing women
• Pedi:   Safety and effectiveness not established

Adverse Reactions/Side Effects

Misc: hypersensitivity reactions including anaphylaxis, infusion reactions

* CAPITALS indicate life-threatening.
Underline indicate most frequent.

Interactions

Drug-Drug

None noted.

Route/Dosage

IV  (Adults and Children)  ≥4 yr) 60 units/kg every other wk (range 11–73 units/kg).

Availability

Lyophilized powder for IV injection (requires reconstitution): 200 units/vial

Assessment

• Monitor for an improvement in symptoms including hepatomegaly, splenomegaly, anemia, thrombocytopenia, bone demineralization, and increased appetite and energy level periodically throughout therapy. Assess liver and spleen size every 6 mo to determine effectiveness of therapy.
• Assess for signs and symptoms of anaphylaxis (angioedema, dyspnea, wheezing, coughing, cyanosis, hypotension). Stop infusion immediately and treat symptomatically. Reducing infusion rate and/or premedication may prevent subsequent reactions.
• Monitor for signs and symptoms of infusion reactions (headache, chest pain, asthenia, fatigue, urticaria, erythema, increased BP, back pain, flushing). Treat by slowing infusion or with antihistamines or antipyretics. Pretreatment with antihistamines and/or corticosteroids may be used.

Potential Diagnoses

• Fatigue (Indications) 
• Deficient knowledge, related to medication regimen (Patient/Family/Teaching) 

Implementation

• Patients treated with imiglucerase can be switched to taliglucerase at the same dose.
• Remove required vials from refrigerator; use immediately, if not possible, can be stored in refrigerator for up to 24 hr or at room temperature for 4 hrs. Reconstitute each vial with 5.1 mL of Sterile Water for Injection for a volume of 5.3 mL. Mix gently; do not shake. Solution is clear and colorless; do not administer solutions that are discolored or contain particulate matter. Vials are for single use; discard solution. Diluent:  Withdraw 5 mL from each vial and dilute with 100–200 mL 0.9% NaCl.Mix gently; do not shake.
• Rate: Initial infusion rate should be 1.2 mL/min for adults or 1 mL/min for pediatric patients; if tolerated, may increase to 2.2 mL/min and 2 mL/min respectively. Infuse over 60–120 min through an in-line low protein-binding 0.2 µm filter; total infusion should be administered over at least 1 hr.

Patient/Family Teaching

• Inform patient of the purpose of this medication and the importance of treatment every 2wk. Taliglucerase helps control the symptoms but does not cure Gaucher's disease. Lifelong therapy may be required.
• Instruct patient to notify health care professional if signs and symptoms of anaphylaxis or infusion reactions occur.
• Advise female patient to notify health care professional if pregnancy is planned or suspected or if breastfeeding.
• Emphasize the importance of follow-up examinations and lab tests.

Evaluation/Desired Outcomes

Increasing hemoglobin and platelet counts and decreasing acid phosphatase levels, hepatomegaly, and splenomegaly. In pediatric patients, cachexia and wasting should diminish.