Trientine (CUVRIOR) is a copper chelator, with trientine tetrahydrochloride as its main component. It is used for the treatment of adult patients with Wilson's disease who have achieved copper depletion and are tolerant to penicillamine.

How to Use Trientine (CUVRIOR)

Recommended Dosage Regimen

The usual dosage for adult patients is 750-1500 mg per day, administered orally in 2-4 divided doses.

The specific dosage should be converted based on the penicillamine dosage previously used by the patient.

For example, convert 125 mg of penicillamine to 300 mg of CUVRIOR, 250 mg of penicillamine to 600 mg of CUVRIOR, and so on, until 1,500 mg or a higher dosage of penicillamine is converted to 3,000 mg of CUVRIOR.

The total daily dosage should not exceed 3,000 mg.

Important Administration Instructions

CUVRIOR should be taken on an empty stomach, at least 1 hour before a meal or 2 hours after a meal, and should be separated from other foods or milk by at least 1 hour.

Tablets should be swallowed whole; they must not be crushed, chewed, or dissolved.

For patients with swallowing difficulties, scored tablets can be split into halves for administration. However, tablets from an opened blister pack should not be stored for later use.

Dosage Adjustment of Trientine (CUVRIOR)

Adjustment Based on Clinical Evaluation

After initiating CUVRIOR treatment, the dosage should be adjusted based on clinical evaluation and serum non-ceruloplasmin copper (NCC) levels.

It is recommended to monitor serum NCC levels at the start of CUVRIOR treatment, 3 months after the start of treatment, and approximately every 6 months thereafter.

The 24-hour urinary copper excretion (UCE) can also be measured regularly (every 6 to 12 months) as an auxiliary monitoring indicator.

Precautions for Trientine (CUVRIOR) Use in Special Populations

Patients with Hepatic Impairment

Currently, there is a lack of specific research data on the use of CUVRIOR in patients with hepatic impairment, so it should be used with caution.

Patients with severe hepatic impairment should avoid using CUVRIOR.

Patients with Renal Impairment

Patients with mild renal impairment (creatinine clearance 51-80 mL/min) do not require dosage adjustment.

Patients with moderate renal impairment (creatinine clearance 31-50 mL/min) should use CUVRIOR with caution.

The use of CUVRIOR is not recommended for patients with severe renal impairment (creatinine clearance < 30 mL/min) or those requiring dialysis.

Pregnant Women

Existing data have not found an association between trientine and major birth defects, miscarriage, or other adverse maternal-fetal outcomes.

However, animal studies have shown that trientine can cause embryo-fetal loss and fetal abnormalities when copper is not supplemented.

Copper levels should be monitored during pregnancy, and the lowest effective dosage should be used.

Lactating Women

Existing data on the detection of trientine in breast milk are inconsistent.

No drug-related adverse effects have been reported in infants exposed to trientine through breast milk.

The developmental and health benefits of breastfeeding should be weighed against the mother's clinical need for CUVRIOR.