Zavesca is a glucosylceramide synthase inhibitor and is indicated as a monotherapy for adult patients with mild to moderate type 1 Gaucher disease. For these patients, enzyme replacement therapy is not a treatment option (for example, due to allergies, Hypersensitivity or poor venous access).
Dosage and administration
Management Instructions
Treatment should be guided by a doctor who has a good understanding of Gaucher's disease.
The recommended dose for adults with type 1 Gaucher disease is one 100 mg capsule orally three times a day. If a dose is missed, the next Zavesca capsule should be taken at the next scheduled time.
In some patients, due to adverse reactions such as tremor or diarrhea, it may be necessary to reduce the dose to 100 mg capsules once or twice a day.
Patients with renal insufficiency
For patients with mild renal insufficiency (adjusted creatinine clearance 50-70 mL/min / 1.73m²), the administration of Zavesca should be started with a dose of 100 mg twice a day. For patients with moderate renal insufficiency (adjusted creatinine clearance rate of 30-50 mL/min / 1.73m²), the administration of Zavesca should start with a daily dose of 100 mg capsules. Zavesca is not recommended for named patient basis with severe renal insufficiency (creatinine clearance <30 mL / min / 1.73m²)
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