Cuvrior (trientine tetrahydrochloride)
An approved treatment for Wilson Disease.
Drug facts
The same compound appears under different names depending on the context. Here is how to identify Trientine tetrahydrochloride wherever you encounter it, plus the key facts at a glance.
- Generic name
- Trientine tetrahydrochloride
- Brand name
- Cuvrior
- Drug class
- Copper chelator (oral)
- Manufacturer
- Orphalan
- How it's taken
- Taken by mouth as a tablet.
Cuvrior is a stable tetrahydrochloride salt of trientine, indicated for adults with stable Wilson disease who are de-coppered and tolerant to penicillamine, providing a maintenance-therapy alternative for patients who want to switch off penicillamine.
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Where Trientine tetrahydrochloride fits
Maintenance-therapy alternative for adults with stable Wilson disease who are already de-coppered and tolerant to penicillamine.
- Not approved for initial de-coppering or for pediatric patients.
- Penicillamine, original trientine formulations, and zinc remain the other established treatment options.
How Trientine tetrahydrochloride works
In Wilson disease, mutations in ATP7B prevent the liver from excreting copper into bile. Copper accumulates first in the liver and then in the brain, cornea, and other organs, where free (non-ceruloplasmin-bound) copper drives oxidative damage. Trientine is a copper-chelating molecule that binds copper in the bloodstream so the kidneys can filter it out into the urine.
The tetrahydrochloride salt formulation in Cuvrior is more chemically stable than older trientine hydrochloride formulations, allowing twice-daily dosing without strict refrigeration requirements.
Side effects and safety
Common side effects in the main trial were abdominal pain, changes in bowel habits, rash, hair loss, and mood swings. The label also warns of iron deficiency (trientine also binds iron), copper deficiency, allergic reactions such as rash, and worsening of neurologic symptoms during the first weeks of therapy in some patients with neurologic Wilson disease (so-called paradoxical neurologic worsening).
The label also notes lupus-like reactions reported with trientine hydrochloride. Cuvrior is taken at least 1 hour before or 2 hours after meals, and at least 2 hours apart from iron, since food and minerals reduce absorption.
This is not a complete list of side effects. Talk to your doctor or pharmacist about what to expect and when to seek medical attention.
Taking Trientine tetrahydrochloride
Taken by mouth as a tablet. The starting total daily dose is 300mg to 3,000mg, split into 2 doses, based on the patient's previous penicillamine dose; the total should not exceed 3,000mg a day. Take on an empty stomach, at least 1 hour before or 2 hours after meals, at least 1 hour apart from other medicines, and at least 2 hours apart from iron supplements.
The label says to avoid zinc, calcium and magnesium supplements, and if they can't be avoided, to take Cuvrior at least 1 hour before or 2 hours after them. The dose is adjusted using blood non-ceruloplasmin copper levels (checked at the start, after 3 months, and about every 6 months) and 24-hour urine copper every 6 to 12 months.
Prescribing Cuvrior? REMS, monitoring and prior authorization criteria for prescribers
Help paying for Cuvrior
With private insurance, the drugmaker's copay card can lower what you pay for Cuvrior. Drugmaker copay cards can't be used with Medicare, Medicaid or TRICARE, but charity funds often can. None of the 2 charity funds for Wilson Disease were open on October 26, 2026. Without insurance, you can apply to the drugmaker's free medicine program, which sets its own eligibility rules. Pick your insurance below to see which offers fit you.
- Copay help
Commercially insured patients with a valid prescription may pay as little as $0 per month. Terms and conditions apply.
For: private insurance · source - Bridge or quick-start supply
Quick Start Program can provide no-cost medication for a limited time if insurance is delayed. Enrollment and eligibility review required.
The official page does not say who qualifies. Ask the program. · source - Free medicine program
Patient Assistance Program for uninsured or underinsured patients; enrollment and financial assessment required.
For: no insurance, underinsured · source - Insurance and case manager help
A dedicated Care Coordinator helps with education and getting access to the medication.
The official page does not say who qualifies. Ask the program. · source
Good to know: Call a Care Coordinator at 1-877-995-ORPH (6774). CUVRIOR is dispensed through the specialty pharmacy PANTHERx per the HCP site.
Charity funds for Wilson Disease
- From a charity · Wilson Disease AssociationWDA Patient Assistance fundApply directly
Pays for: Travel and lodging (up to 7 days) for testing or treatment, for the patient and one companion, up to $2,000 per year.
The foundation says: “Status not shown on page”
How Wilson disease treatments compare
Four FDA-approved medications are available for Wilson disease, plus investigational programs at different stages. Treatment choice depends on your stage (active de-coppering versus stable maintenance), your tolerance to penicillamine, whether you have neurologic or hepatic features, and your age. This chart summarizes the practical differences between the approved options.
Wilson Disease has 4 FDA-approved treatments: Cuprimine / Depen (penicillamine, approved 1970); Syprine (trientine hydrochloride, approved 1985); Galzin (zinc acetate, approved 1997); Cuvrior (trientine tetrahydrochloride, approved 2022). Cuprimine / Depen is typically used as approved for wilson disease in general. carries a boxed warning and needs frequent blood and urine tests, twice a week in the first month on the cuprimine label..
| Drug | How it works | How it’s given | How often | Where you take it | Typical use | FDA approved |
|---|---|---|---|---|---|---|
Cuprimine / Depen penicillamine | Copper chelator that binds copper in the body so it leaves in the urine. The original Wilson disease drug. | Oral capsule or tablet, taken on an empty stomach | 1,000 to 1,500mg a day in 2 to 4 doses to start, 750 to 1,000mg for maintenance (AASLD) | Home (oral) | Approved for Wilson disease in general. Carries a boxed warning and needs frequent blood and urine tests, twice a week in the first month on the Cuprimine label. | 1970 |
Syprine trientine hydrochloride | Copper chelator that binds copper so it leaves in the urine. No trial has compared it head to head with penicillamine as a first treatment (AASLD). | Oral capsule, taken on an empty stomach (capsules require refrigeration) | 2 to 4 times daily | Home (oral) | Approved for patients who cannot tolerate penicillamine. Generic versions are available. | 1985 |
Galzin zinc acetate | Copper absorption blocker. It makes gut cells produce a protein (metallothionein) that traps copper from food before it can be absorbed. | Oral capsule, taken on an empty stomach | 3 times daily | Home (oral) | Approved for maintenance after a person has first been treated with a chelator; not for the first treatment of someone with symptoms. | 1997 |
Cuvrior trientine tetrahydrochloride You are here | Copper chelator with the same action as Syprine, in a more stable salt that is stored at room temperature and taken twice a day. | Oral tablet, taken on an empty stomach | Twice daily | Home (oral) | Approved for adults with stable Wilson disease who are de-coppered and tolerant to penicillamine; designed as a maintenance switch option. | 2022 |
This chart summarizes approved Wilson disease treatments to help you understand your options. It is not medical advice. Treatment decisions depend on your individual disease stage, prior therapies, tolerability, and lab values for copper control. Investigational programs (ALXN1840, UX701, PM577a) are not included because they are not FDA-approved. Always discuss options with your hepatologist or metabolic specialist.
Clinical trial results
Approval was supported by the global Phase 3 CHELATE trial, which randomized adults with stable Wilson disease to switch from penicillamine to either Cuvrior or to continue penicillamine. Cuvrior met its primary efficacy endpoint of non-inferiority versus penicillamine, measured by non-ceruloplasmin (free) copper concentration over 24 weeks.
Development history
Trientine has been used in Wilson disease for decades as Syprine (trientine hydrochloride, originally approved 1985) and later as generic trientine. Orphalan developed Cuvrior, a stable tetrahydrochloride salt formulation, to provide an alternative with simpler storage and twice-daily dosing. The FDA approved Cuvrior on April 28, 2022 under the 505(b)(2) pathway. It was the first new Wilson disease drug approved since zinc acetate (Galzin) in 1997.
Explore Wilson Disease trials
Other Wilson Disease treatments
Common questions about Trientine tetrahydrochloride
▸What is Trientine tetrahydrochloride (Cuvrior)?
Cuvrior is a stable tetrahydrochloride salt of trientine, indicated for adults with stable Wilson disease who are de-coppered and tolerant to penicillamine, providing a maintenance-therapy alternative for patients who want to switch off penicillamine.
▸How does Trientine tetrahydrochloride work?
In Wilson disease, mutations in ATP7B prevent the liver from excreting copper into bile. Copper accumulates first in the liver and then in the brain, cornea, and other organs, where free (non-ceruloplasmin-bound) copper drives oxidative damage. Trientine is a copper-chelating molecule that binds copper in the bloodstream so the kidneys can filter it out into the urine. The tetrahydrochloride salt formulation in Cuvrior is more chemically stable than older trientine hydrochloride formulations, allowing twice-daily dosing without strict refrigeration requirements.
▸What are the side effects of Trientine tetrahydrochloride?
Common side effects in the main trial were abdominal pain, changes in bowel habits, rash, hair loss, and mood swings. The label also warns of iron deficiency (trientine also binds iron), copper deficiency, allergic reactions such as rash, and worsening of neurologic symptoms during the first weeks of therapy in some patients with neurologic Wilson disease (so-called paradoxical neurologic worsening). The label also notes lupus-like reactions reported with trientine hydrochloride. Cuvrior is taken at least 1 hour before or 2 hours after meals, and at least 2 hours apart from iron, since food and minerals reduce absorption.
▸How is Trientine tetrahydrochloride taken?
Taken by mouth as a tablet. The starting total daily dose is 300mg to 3,000mg, split into 2 doses, based on the patient's previous penicillamine dose; the total should not exceed 3,000mg a day. Take on an empty stomach, at least 1 hour before or 2 hours after meals, at least 1 hour apart from other medicines, and at least 2 hours apart from iron supplements. The label says to avoid zinc, calcium and magnesium supplements, and if they can't be avoided, to take Cuvrior at least 1 hour before or 2 hours after them. The dose is adjusted using blood non-ceruloplasmin copper levels (checked at the start, after 3 months, and about every 6 months) and 24-hour urine copper every 6 to 12 months.
▸Is Trientine tetrahydrochloride FDA approved?
Yes, Trientine tetrahydrochloride (Cuvrior) is FDA approved (2022) for the treatment of Wilson Disease.
▸What is Cuvrior (trientine tetrahydrochloride) used for?
Cuvrior (trientine tetrahydrochloride) is a copper chelator approved for adults with stable Wilson disease who are already de-coppered and tolerant to penicillamine, giving them a maintenance-therapy alternative if they want to switch off penicillamine. It is not approved for initial de-coppering or for pediatric patients. The FDA approved Cuvrior on April 28, 2022, the first new Wilson disease drug since zinc acetate in 1997. [1]
▸How is Cuvrior taken?
Cuvrior is a tablet taken by mouth, split into 2 doses a day, with a total daily dose of 300mg to 3,000mg based on the patient's previous penicillamine dose and not exceeding 3,000mg. Take it on an empty stomach, at least 1 hour before or 2 hours after meals, at least 1 hour apart from other medicines, and at least 2 hours apart from iron supplements. The label says to avoid zinc, calcium and magnesium supplements, and if they can't be avoided, to take Cuvrior at least 1 hour before or 2 hours after them. Doctors adjust the dose using blood non-ceruloplasmin copper levels and 24-hour urine copper tests. [1]
▸What are the side effects of Cuvrior?
Common side effects of Cuvrior in the main trial were abdominal pain, changes in bowel habits, rash, hair loss, and mood swings. The label also warns of iron deficiency, copper deficiency, allergic reactions, and worsening of neurologic symptoms during the first weeks of therapy in some patients with neurologic Wilson disease. The label also notes lupus-like reactions reported with trientine hydrochloride. [1]
▸What did the Cuvrior CHELATE trial show?
The global Phase 3 CHELATE trial randomized adults with stable Wilson disease to switch from penicillamine to Cuvrior or to continue penicillamine. Cuvrior met its primary endpoint of non-inferiority versus penicillamine, measured by non-ceruloplasmin (free) copper concentration over 24 weeks, and this supported its FDA approval in 2022. [1]
Sources and references
Every factual claim on this page is drawn from the public sources listed below. Click any reference to open the original document.
- U.S. Food and Drug Administration · April 28, 2022. CUVRIOR (trientine tetrahydrochloride) prescribing information at approval. https://www.accessdata.fda.gov/drugsatfda_docs/label/2022/215760s000lbl.pdf
- Wilson Disease Association. Wilson Disease Association, Treatment Resources. https://wilsondisease.org/living-with-wilson-disease/treatment/