Inborn errors of metabolism:

Indications for: CUVRIOR

Wilson’s disease, in patients who are de-coppered and tolerant to penicillamine.

Adult Dosage:

Swallow whole. Take on an empty stomach. Discontinue penicillamine prior to initiation. Start at 300–3000mg/day in 2 equally divided doses; max 3000mg/day. Adjust total daily dose based on clinical response and serum non-ceruloplasmin copper (NCC) levels. If the number of tabs per day cannot be equally divided, then divide the total daily dose such that the higher number of tabs is given with the first daily dose. Switching from other trientine products: see full labeling.

Children Dosage:

Not established.

CUVRIOR Warnings/Precautions:

Not substitutable on a mg-per-mg basis with other trientine products. Potential worsening of clinical symptoms (including neurological deterioration) at therapy initiation; adjust dose or discontinue if condition worsens. Assess serum NCC levels at initiation, after 3 months, and every 6 months thereafter; may also monitor periodically (every 6–12 months) with 24-hour urinary copper analysis. Monitor for iron deficiency (esp. menstruating or pregnant women) or copper deficiency (eg, in pregnancy). Evaluate and consider discontinuing if rash or other hypersensitivity reactions develop. Elderly. Pregnancy. Nursing mothers.

CUVRIOR Classification:

Chelating agent.

CUVRIOR Interactions:

Avoid concomitant mineral supplements (eg, iron, zinc, calcium, magnesium); if unavoidable, give Cuvrior at least 2hrs before or after an iron supplement, and at least 1hr before or 2hrs after other mineral supplements. Separate dosing of any other drug, food, or milk by at least 1 hour.

Adverse Reactions:

Abdominal pain, change of bowel habits, rash, alopecia, mood swings; copper deficiency, iron deficiency, lupus, hypersensitivity reactions.



Drug Elimination:

Renal. Half-life: 13.8–16.5 hours.

Generic Drug Availability:


How Supplied:

Tabs—8, 72