Hepatology
Wilson disease: Cuvrior, for prescribers
Cuvrior on what its first fill waits on, what the label requires you to monitor and what an insurer asks to see, with FDA decisions, label changes and open trials for Wilson disease. 3 other FDA-approved medicines for Wilson disease have no prescribing guide here yet and are listed below.
When to suspect Wilson disease and what to test →The Wilson disease prescribing guide
One page per drug with the dispensing gate, prior authorization documentation, dosing, monitoring and interactions, each line sourced.
The drug with a prescribing guide
Each cell is a summary of that drug's prescribing guide, which carries the full detail and a source on every line. Insurer thresholds are one public policy shown as an example; plans differ.
| Cuvriortrientine tetrahydrochloride | |
|---|---|
| Label dated | June 19, 2025 |
| Starting dose | Starting total daily dose from Table 1, 300mg to 3,000mg, in 2 divided doses; maximum 3,000mg a day. Table 2: 300mg is 1 tablet morning; 900mg is 2 morning and 1 evening; 1,800mg is 3 and 3; 3,000mg is 5 and 5. |
| Dispensing gate | No REMS and no boxed warning; confirm stable, de-coppered Wilson disease tolerant to penicillamine, convert the penicillamine dose by Table 1, discontinue penicillamine before day 1, and send the Navigator Start Form or an eRx to PANTHERx Rare, the only dispensing pharmacy |
| Required monitoring | Serum non-ceruloplasmin copper at initiation, 3 months and about every 6 months; 24-hour urinary copper every 6 to 12 months as an option; watch for early clinical or neurological worsening, copper deficiency, iron deficiency and rash |
| Example PA threshold | Cigna example, December 10, 2025: diagnosis by biallelic ATP7B variants or 2 of Kayser-Fleischer rings, ceruloplasmin under 20mg/dL, consistent liver biopsy and 24-hour urine copper over 40mcg; 1 of 6 reasons for trientine; GI, hepatology or transplant prescriber |
| Support line | Navigator Program, 1-877-995-ORPH (6774), Start Form by fax 1-866-716-ORPH (6774), copay as little as $0 per fill for commercial plans with an annual cap, Quick Start of a free 30-day supply plus a one-month refill after a 5-day coverage delay, and a Patient Assistance Program |
- Label dated
- June 19, 2025
- Starting dose
- Starting total daily dose from Table 1, 300mg to 3,000mg, in 2 divided doses; maximum 3,000mg a day. Table 2: 300mg is 1 tablet morning; 900mg is 2 morning and 1 evening; 1,800mg is 3 and 3; 3,000mg is 5 and 5.
- Dispensing gate
- No REMS and no boxed warning; confirm stable, de-coppered Wilson disease tolerant to penicillamine, convert the penicillamine dose by Table 1, discontinue penicillamine before day 1, and send the Navigator Start Form or an eRx to PANTHERx Rare, the only dispensing pharmacy
- Required monitoring
- Serum non-ceruloplasmin copper at initiation, 3 months and about every 6 months; 24-hour urinary copper every 6 to 12 months as an option; watch for early clinical or neurological worsening, copper deficiency, iron deficiency and rash
- Example PA threshold
- Cigna example, December 10, 2025: diagnosis by biallelic ATP7B variants or 2 of Kayser-Fleischer rings, ceruloplasmin under 20mg/dL, consistent liver biopsy and 24-hour urine copper over 40mcg; 1 of 6 reasons for trientine; GI, hepatology or transplant prescriber
- Support line
- Navigator Program, 1-877-995-ORPH (6774), Start Form by fax 1-866-716-ORPH (6774), copay as little as $0 per fill for commercial plans with an annual cap, Quick Start of a free 30-day supply plus a one-month refill after a 5-day coverage delay, and a Patient Assistance Program
Other FDA-approved medicines for Wilson disease
These have no Trial Friend prescribing guide yet, so they are not in the comparison above. Some are approved for a different form or use of the condition than the guides above, and each drug page gives its indication. The list comes from Trial Friend's audited list of FDA labels that name this condition, built October 21, 2026.
FDA decisions for Wilson disease
From the Trial Friend FDA decision calendar, which records each date from the sponsor's or the FDA's own announcement.
No pending or recent decisions on the calendar.
Recent label changes
What changed in each label revision, from the FDA's approval letters and the labels' own revision lists.
- June 2025Cuvrior. The current version of the label on DailyMed carries the date June 19, 2025. Its highlights still read revised January 2024, with no recent major changes and no boxed warning. FDA label
- April 2022Cuvrior. The dosing table, food and medicine spacing, copper test schedule and warnings in the current label read the same as in this first label. FDA label, 2022
- April 2022Cuvrior. The FDA approved Cuvrior on April 28, 2022, for adults with stable Wilson disease who are de-coppered and tolerant to penicillamine. The approval letter also asked Orphalan for a study of Cuvrior in children. FDA letter
Open trials
Questions prescribers ask about Wilson disease
Does Cuvrior require a REMS?
No. Cuvrior has no REMS. Its prescribing guide lists what does gate the first fill and its source.
What monitoring does Cuvrior require?
Serum non-ceruloplasmin copper at initiation, 3 months and about every 6 months; 24-hour urinary copper every 6 to 12 months as an option; watch for early clinical or neurological worsening, copper deficiency, iron deficiency and rash.
What do insurers require before covering Cuvrior?
This is one public insurer policy, an example rather than every plan. Cigna example, December 10, 2025: diagnosis by biallelic ATP7B variants or 2 of Kayser-Fleischer rings, ceruloplasmin under 20mg/dL, consistent liver biopsy and 24-hour urine copper over 40mcg; 1 of 6 reasons for trientine; GI, hepatology or transplant prescriber.
Which support program starts Cuvrior patients on therapy?
Navigator Program, 1-877-995-ORPH (6774), Start Form by fax 1-866-716-ORPH (6774), copay as little as $0 per fill for commercial plans with an annual cap, Quick Start of a free 30-day supply plus a one-month refill after a 5-day coverage delay, and a Patient Assistance Program.
A reference for licensed professionals, checked on November 9, 2026. Labels and plan policies change; the label on DailyMed and the plan's current policy govern. Not medical advice. For patients: the Wilson disease page and the first 90 days pages for Cuvrior.