Inborn errors of metabolism:
Indications for: ZAVESCA
Mild to moderate type 1 Gaucher disease when enzyme replacement therapy is not feasible.
Adult Dosage:
≥18yrs: 100mg 3 times daily; may reduce to 100mg once or twice daily if diarrhea or tremor occurs. Mild renal impairment (CrCl 50–70mL/min): 100mg twice daily. Moderate renal impairment (CrCl 30–50mL/min): 100mg once daily. Severe renal impairment: not recommended.
Children Dosage:
<18yrs: not established.
ZAVESCA Warnings/Precautions:
Perform neurological evaluations at baseline, then periodically at 6-month intervals. Reevaluate and consider discontinuation if signs/symptoms of peripheral neuropathy develop (eg, numbness, tingling). GI disease (eg, inflammatory bowel). Monitor platelets. Renal impairment. Elderly. Pregnancy. Nursing mothers: not recommended.
ZAVESCA Classification:
Glucosylceramide synthase inhibitor.
ZAVESCA Interactions:
May antagonize imiglucerase.
Adverse Reactions:
Diarrhea, weight loss, tremor, flatulence, abdominal pain, GI disturbances, weakness, headache, dizziness, paresthesia, cramps, vision problems, thrombocytopenia, back pain, dry mouth, menstrual changes; reduced spermatogenesis, peripheral neuropathy.
Generic Drug Availability:
YES
How Supplied:
Caps—18, 90