taliglucerase alfa

Absorption: IV administration results in complete bioavailability

Distribution: Unknown.

Metabolism/Excretion: Unknown.

Half-Life: 18.9–28.7 min

Time/Action Profile ⬆ ⬇

ROUTE	ONSET	PEAK	DURATION
IV	unknown	end of infusion	2 wk

Contraind./Precautions ⬆ ⬇

Contraindicated in:

Use Cautiously in:

Anti–taliglucerase alfa antibodies (↑ risk of hypersensitivity reactions);
OB: Use during pregnancy only if potential maternal benefit justifies potential fetal risk;
Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant;
Pedi: Children <4 yr (safety and effectiveness not established).

Adv. Reactions/Side Effects ⬆ ⬇

Misc: infusion reactions , HYPERSENSITIVITY REACTIONS (INCLUDING ANAPHYLAXIS)

Interactions ⬆ ⬇

Drug-drug:

Route/Dosage ⬆ ⬇

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

Availability ⬆ ⬇

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 and monitor for signs and symptoms of anaphylaxis (urticaria, hypotension, flushing, wheezing, chest tightness, nausea, vomiting, dizziness). If anaphylaxis occurs, stop infusion immediately and initiate appropriate treatment. Observe patients closely for 3 hr after the start of the infusion for any reactions.
Monitor for signs and symptoms of infusion-related reactions (headache, chest pain, asthenia, fatigue, urticaria, erythema, increased BP, back pain, flushing). Treat by slowing infusion, discontinuing temporarily, and/or administering antihistamines, antipyretics, and/or corticosteroids.

Lab Test Considerations:

Monitor hemoglobin, platelet count, tartrate-resistant acid phosphatase, chitotriosidase, IgG anti-drug antibody formation. ECG, echocardiogram, and CXR. Report abnormalities.

Implementation ⬆ ⬇

Patients treated with imiglucerase can be switched to taliglucerase alfa at the same dose.
Administer antihistamine, antipyretics, and/or corticosteroids prior to infusion, if required, to prevent hypersensitivity reactions.
Administer in a health care setting with appropriate medical monitoring and supportive measures (cardiopulmonary resuscitation equipment) available during administration.
If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue immediately and initiate appropriate medical treatment (epinephrine). Consider the risks and benefits of readministering following severe hypersensitivity reactions.

IV Administration:

Intermittent Infusion: Remove required vials from refrigerator; use immediately. If unable, store in refrigerator for up to 24 hr or at room temperature for 4 hr. Reconstitution: 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 single use; discard any unused 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; if tolerated, may ↑ to max of 2.2 mL/min. Initial infusion rate should be 1 mL/min for pediatric patients <30 kg. For pediatric patients ≥30 kg, the initial infusion rate should be 1 mL/min; if tolerated, may ↑ to max of 2 mL/min. Infuse over ≥60 min through an in-line low protein-binding 0.2-micron filter.
Y-Site Incompatibility: Do not infuse in the same IV line with other products.

Patient/Family Teaching ⬆ ⬇

Explain the purpose and side effects of taligulcerase alfa to the patient and/or caregiver. Emphasize the importance of treatment every 2 wk. Taliglucerase helps control the symptoms but does not cure Gaucher disease. Lifelong therapy may be required.
Advise patient and/or caregiver to readPatient Information before starting therapy
Advise patient and/or caregiver to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other medications.
Advise patient and/or caregiver if a dose is missed to notify a health care professional.
Teach patient the signs and symptoms of an hypersensitivity reaction; if any occur, seek immediate medical care.
Teach patient and/or caregiver the signs and symptoms of infusion-related reactions.
Educate patient and/or caregiver on the importance of follow-up examinations and lab tests.
Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding.