taliglucerase alfa
taliglucerase alfa
(tali-gloo-ser-ase al-fa) taliglucerasealfa,Elelyso
(trade name)Classification
Therapeutic: orphan drugsPharmacologic: enzymes
Indications
Action
Therapeutic effects
Pharmacokinetics
Time/action profile
ROUTE | ONSET | PEAK | DURATION |
---|---|---|---|
IV | unknown | end of infusion | 2 wk |
Contraindications/Precautions
Adverse Reactions/Side Effects
Miscellaneous
- allergic reactions including anaphylaxis
- infusion reactions
Interactions
Drug-Drug interaction
None noted.Route/Dosage
Availability
Nursing implications
Nursing 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.
- 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 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; 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.
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.