Usual Adult Dose for Wilson's Disease

750 to 1250 mg, orally, in divided doses given 2, 3, or 4 times daily
Maximum dose: 2000 mg daily

Comments:

• Increase dose for inadequate clinical response or free serum copper persistently above 20 mcg/dL.
• Re-evaluate dose every 6 to 12 months.
• Clinical experience with this drug is limited and alternate dosing regimens have not been well characterized.
• All dosing endpoints are not well defined.
• This drug is not interchangeable with penicillamine.
• Use this drug when penicillamine treatment is not possible because of intolerable or life threatening side effects.
• This drug is not recommended for cystinuria or rheumatoid arthritis.
• It is incapable of binding cystine, so it is of no use in cystinuria.
• In a small study, it was ineffective for rheumatoid arthritis.
• This drug is not indicated for treatment of biliary cirrhosis.

Usual Pediatric Dose for Wilson's Disease

13 and older:
750 to 1250 mg, orally, in divided doses given 2, 3, or 4 times daily
Maximum dose: 2000 mg daily

12 and under:
500 to 750 mg, orally, in divided doses given 2, 3, or 4 times daily
Maximum dose: 1500 mg daily

Comments:

• Increase dose for inadequate clinical response or free serum copper persistently above 20 mcg/dL.
• Re-evaluate dose every 6 to 12 months.
• Clinical experience with this drug is limited and alternate dosing regimens have not been well characterized.
• All dosing endpoints are not well defined.
• This drug is not interchangeable with penicillamine.
• Use this drug when penicillamine treatment is not possible because of intolerable or life threatening side effects.
• This drug is not recommended for cystinuria or rheumatoid arthritis.
• It is incapable of binding cystine, so it is of no use in cystinuria.
• In a small study, it was ineffective for rheumatoid arthritis.
• This drug is not indicated for treatment of biliary cirrhosis.

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Dialysis

Data not available

Other Comments

Administration advice:

• Take on an empty stomach, at least one hour before or two hours after meals.
• Take at least one hour apart from any other drug, food, or milk.
• Swallow capsules whole; do not open or chew capsules.

• Refrigerate.
• Keep container tightly closed.

• There is a potential for contact dermatitis; if exposure to capsule contents occurs, wash exposed area with water promptly.
• For the first month of treatment, have the patient take their temperature nightly, and report any fever, skin eruption, or other symptoms.