IV (Adults and Children ≥4 yr): 60 units/kg every other wk (range 11–73 units/kg).
US Brand Names⬆⬇
Elelyso
Action⬆⬇
Prevents the accumulation of glucocerebrosides in cells. Replaces glucocerebrosidases that are deficient in type 1 Gaucher disease.
Therapeutic effects:
Improvement in symptoms of type 1 Gaucher disease (anemia, thrombocytopenia, bone disease, splenomegaly, hepatomegaly)
Classifications⬆⬇
Therapeutic Classification: replacement enzyme
Pharmacologic Classification: enzymes
Pharmacokinetics⬆⬇
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
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.