Inborn errors of metabolism:

Indications for ZAVESCA:

Mild to moderate type 1 Gaucher disease when enzyme replacement therapy is not feasible.


≥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.


<18yrs: not recommended.


Pregnancy (Cat.X).


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). Use effective methods of contraception during therapy; men should maintain contraception for 3 months after treatment. Elderly. Labor & delivery. Nursing mothers: not recommended.

Pharmacologic Class:

Glucosylceramide synthase inhibitor.


May antagonize imiglucerase.

Adverse Reactions:

Diarrhea, weight loss, tremor, flatulence, abdominal pain, GI disturbances, weakness, headache, dizziness, paresthesia, cramps, thrombocytopenia, reduced spermatogenesis, peripheral neuropathy.

How Supplied:

Caps—18, 90

Pricing for ZAVESCA

90 capsules of 100mg dose pack (Qty: 1)
Appx. price $9123