taliglucerase alfa(tali-gloo-ser-ase al-fa) ,
Pregnancy Category: B
ClassificationTherapeutic: orphan drugs
Long-term replacement therapy in adults with confirmed Type 1 Gaucher disease.
Prevents the accumulation of glucocerebrosides in cells. Replaces glucocerebrosidases that are deficient in type 1 Gaucher’s disease.
Improvement in symptoms of Gaucher disease (anemia, thrombocytopenia, bone disease, splenomegaly, and hepatomegaly)
Absorption: IV administration results in complete bioavailability
Metabolism and Excretion: Unknown.
Half-life: 18.9–28.7 min
|IV||unknown||end of infusion||2 wk|
Contraindicated in: None noted.
Use Cautiously in: Obstetric: Use during pregnancy only if clearly needed Lactation: Use cautiously in nursing women Pediatric: Safe and effective use in children has not been established
Adverse Reactions/Side Effects
- allergic reactions including anaphylaxis
- infusion reactions
Drug-Drug interactionNone noted.
Intravenous (Adults) 60 Units/kg every other week (range 11–73 Units/kg)
Lyophilized powder for IV injection (requires reconstitution): 200 Units/vial
- 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.
- 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 Nursing DiagnosesFatigue (Indications)
Deficient knowledge, related to medication regimen (Patient/Family Teaching)
- Patients treated with imiglucerase can be switched to taliglucerase at the same dose.
- Remove required vials from refrigerator; do not leave at room temperature for longer than 24 hr. 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 1.3 mL/min; if tolerated, may increase to 2.3 mL/min. Infuse over 60–120 min; total infusion should be administered over at least 1 hr.
- 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.
- Increasing hemoglobin and platelet counts and decreasing acid phosphatase levels, hepatomegaly, and splenomegaly. In pediatric patients, cachexia and wasting should diminish.