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About CUVRIOR

CUVRIOR is a copper chelator indicated for the treatment of adult patients with stable Wilson Disease who are de-coppered and tolerant to penicillamine.1
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Meet CUVRIOR: The only FDA-approved therapy in WD in nearly 30 years.

What is CUVRIOR?

A trientine copper chelator with a dual mechanism of action that eliminates absorbed copper while also reducing copper absorption in the intestine.2 

What are some key takeaways for CUVRIOR?

CUVRIOR has on-the-go potential.

  • CUVRIOR offers convenient twice-daily dosing and doesn’t require refrigeration2
  • Supplied in a portable blister pack2
  • Take CUVRIOR on an empty stomach (at least 1 hour before meals or 2 hours after meals and at least 1 hour apart from any other food, milk, or oral medication)

CUVRIOR allows for flexible dosing.2

  • Tablets are scored so they can be divided in half if needed
  • Tablets are smaller than most other maintenance options

CUVRIOR tablets are smaller than most other WD maintenance options3-6

The starting total daily dosage of CUVRIOR in adults ranges from 300 mg to 3000 mg orally in divided doses taken just 2 times a day.2

See more about administering CUVRIOR.

CUVRIOR Clinical Trial

CHELATE: the longest and largest randomized clinical trial evaluating trientine in WD1,7

CUVRIOR has been evaluated in the first trientine head-to-head (noninferiority), prospective, randomized clinical trial against DPA1,7

Patient population1

Aged 18 to 75 years with stable WD (N=53)

Treatment1

  • After a 12-week screening period when all patients received DPA
  • Patients were randomized to either continue DPA maintenance dose (n=27) or switch to CUVRIOR (n=26) 

Primary Endpoint1

Measurement of free copper in serum by 
non-ceruloplasmin-bound copper (NCC) by speciation 
(NCC-Sp) assay at 24 weeks post-randomization 

cNCC-Ex (non-ceruloplasmin-bound by copper exchange assay) was initially used for screening and therapeutic monitoring and was intended to be used to evaluate the primary endpoint; however, it was found to be unreliable. Orphalan, with the FDA’s endorsement, created a novel 2-step method—NCC-Sp—that was used for this trial and not commercially available.1

Maintenance therapy that reduces and maintains copper levels1

CUVRIOR maintenance reduced copper levels as effectively as DPA maintenance1

Mean serum NCC by NCC-Sp assay for weeks 0 to 481

Mean difference at 24 weeks post-randomization in serum NCCa, measured by NCC-Sp assay, was –9.1µg/L (95% CI: - 24.2 to +6.1)1

Mean 24-hour urinary copper excretion (UCE) at week 24 post-randomization was lower in patients receiving CUVRIOR compared with patients receiving penicillamine1
There were no notable changes in either Clinical Global Impression of Change (CGIC) or Unified Wilson Disease Rating Scale neurological assessment from baseline (prerandomized) to weeks 24 and 481
aNCC-Ex (non-ceruloplasmin-bound by copper exchange assay) was initially used for screening and therapeutic monitoring and was intended to be used to evaluate the primary endpoint; however, it was found to be unreliable. Orphalan, with the FDA’s endorsement, created a novel 2-step method–NCC-Sp–that was used for this trial and not commercially available.1
    CHELATE was a randomized, open-label, non-inferiority Phase 3 trial to compare penicillamine with CUVRIOR as maintenance treatment for adults with WD. Key secondary outcomes include 24h urinary copper excretion, clinical stability assessment by the IAC, and Clinical Global Impression of Change (CGIC) rating scale score. Safety endpoints included AEs and SAEs using MeDRA (version 20.0) 

Safety

CUVRIOR is backed by the safety of trientine, a trusted WD therapy in use for over 35 years.2

Common adverse events 
Most common adverse events that occured in >5% of CUVRIOR-treated patients included abdominal pain (19%),b change of bowel habits (15%),c rash (12%),d alopecia (8%), and mood swings (8%)2
Similar incidence rates of treatment-emergent adverse events seen between CUVRIOR (54%) and DPA (63%) in the Phase 3 CHELATE study1
No serious adverse events were reported with CUVRIOR in the post-randomization period1

bAbdominal pain is composed of several similar terms.2
cIncludes constipation, abnormal feces, soft feces.2
dRash is composed of several similar terms.2

CUVRIOR is a conversation to have with your patient.

Treatment adherence can be an issue for your WD patients. Talk to your patients and encourage them to discuss any treatment-related questions or concerns.

Dosing & Administration2

Convenient twice-daily dosing for long-term maintenance therapy

No refrigeration provides on-the-go potential.

The starting total daily dosage of CUVRIOR in adults ranges from 300 mg to 3000 mg orally in divided doses taken just 2 times a day.

Administer CUVRIOR2 on an empty stomach:  

  • at least 1 hour before meals 
  • or 2 hours after meals 
  • at least 1 hour apart from any other food, milk, or oral medication 
  • swallow tablets without crushing, chewing, or dissolving tablets

Supplied in a portable blister pack.2 

Tablets are scored so they can be divided in half if needed.2

Tablets are smaller than most other maintenance options.3-6

Learn more about dosing 
or consider a switch1

Learn more about dosing or consider a switch to maintain your patients’ copper balance with CUVRIOR.1

REFERENCES

1. Schilsky ML, Czlonkowska A, Zuin M, et al. Trientine tetrahydrochloride versus penicillamine for maintenance therapy in Wilson disease (CHELATE): a randomised, open-label, non-inferiority, phase 3 trial. Lancet Gastroenterol Hepatol. 2022;7(12):1092-1102. 2. CUVRIOR [package insert]. Chicago, IL: Orphalan SA. 3. CUVRIOR-trientine tetrahydrochloride tablet, film coated. National Library of Medicine website. Accessed September 5, 2023. https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=f73feeae-62ad-401e-b9f7-5cb269127750 4. GALZIN-zinc acetate tablet capsule. National Library of Medicine website. Accessed September 5, 2023. https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=a0c72bff-20f3-4241-b966-34a95178d1a3 5. CUPRIMINE-penicillamine capsule. National Library of Medicine website. Accessed Septemebr 5, 2023. https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=80e736d3-2017-4d68-94b4-38255c3c59c6 6. SYPRINE-trientine hydrochloride capsule. National Library of Medicine website. Accessed September 5, 2023. https://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=c34f77a7-996b-4470-b5df-d946a7fe5dbe 7. Brewer GJ, Askari F, Lorincz MT, et al. Treatment of Wilson disease with ammonium tetrathiomolybdate: IV. Comparison of tetrathiomolybdate and trientine in a double-blind study of treatment of the neurologic presentation of Wilson disease. Arch Neurol. 2006;63(4):521-527. 8. Data on file. Orphalan, Inc.

Indication & Important Safety Information

CUVRIOR is a copper chelator indicated for the treatment of adult patients with stable Wilson Disease who are de-coppered and tolerant to penicillamine. Please see full Prescribing Information.
Important Safety Information

INDICATION

CUVRIOR is a copper chelator indicated for the treatment of adult patients with stable Wilson disease who are de-coppered and tolerant to penicillamine.

IMPORTANT SAFETY INFORMATION

CONTRAINDICATIONS

  • CUVRIOR is contraindicated in patients with hypersensitivity to trientine or to any of the excipients in CUVRIOR.

WARNINGS AND PRECAUTIONS

  • Potential for Worsening of Clinical Symptoms at Initiation of Therapy, including neurological deterioration, may occur at the beginning of CUVRIOR therapy due to mobilization of excess stores of copper. Adjust the dosage or discontinue therapy if clinical condition worsens. Evaluate serum non-ceruloplasmin copper (NCC) levels or 24-hour urinary copper excretion (UCE) when initiating treatment, after 3 months, and approximately every 6 months thereafter.
  • Copper Deficiency may develop following treatment with CUVRIOR. Periodic monitoring is required.
  • Iron Deficiency may develop following treatment with CUVRIOR. If iron deficiency develops, a short course of iron supplementation may be given.
  • Hypersensitivity Reactions, characterized by rash, have been reported with the use of trientine. Rash was reported in 12% (3/26) of CUVRIOR-treated patients, and one patient discontinued treatment due to rash. If rash or other hypersensitivity reaction occurs, consider discontinuing CUVRIOR.

ADVERSE REACTIONS

The most common adverse reactions (>5%) are abdominal pain, change of bowel habits, rash, alopecia, and mood swings.

DRUG INTERACTIONS

  • Mineral Supplements (e.g. iron, zinc, calcium, magnesium): Avoid concomitant use. If concomitant use is unavoidable, take CUVRIOR at least 2 hours before or 2 hours after iron and take CUVRIOR at least 1 hour before or 2 hours after other mineral supplements.
  • Other Drugs for Oral Administration: Take CUVRIOR at least 1 hour apart from any other oral drug.

To report SUSPECTED ADVERSE REACTIONS, contact Orphalan at 1-800-961-8320 or FDA at 1-800-FDA-1088 or 
www.fda.gov/medwatch.

Please see full Prescribing Information.

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