Cuvrior (trientine tetrahydrochloride) is a newly approved medication that's used to treat a rare genetic condition called Wilson's disease in adults. It can only be used in people with stable Wilson's disease who are able to take penicillamine (Depen).
Cuvrior (trientine tetrahydrochloride) is a chelating agent. People with Wilson's disease have high amounts of copper which can cause organ damage. Cuvrior (trientine tetrahydrochloride) works by attaching to copper in your body, preventing it from getting absorbed from your gut, and allows your body to remove it through your urine. This helps to keep your copper levels low.
The following side effects may get better over time as your body gets used to the medication. Let your healthcare provider know immediately if you continue to experience these symptoms or if they worsen over time.
Side effects that you should report to your care team as soon as possible:
Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):
No. There isn't a cure for Wilson's disease, and people with this condition will need lifetime treatment to keep copper levels under control. There are medications like Cuvrior (trientine tetrahydrochloride) that can help remove too much copper in the body due to Wilson's disease, and prevent build up of copper that can lead to serious health problems. Speak with your provider about the best treatment option for you.
Cuvrior (trientine tetrahydrochloride) can interact with many medications because of the way it works to treat Wilson's disease. It can attach to medications and supplements in your stomach and prevent them from getting absorbed into your body. This might cause certain medications and supplements to work less well for you. It's recommended to take Cuvrior (trientine tetrahydrochloride) 1 hour away from other medications you're taking by mouth. If you're taking iron, you should take Cuvrior (trientine tetrahydrochloride) at least 2 hours before or 2 hours after it. For other mineral supplements (e.g., zinc, calcium, magnesium), you should take Cuvrior (trientine tetrahydrochloride) at least 1 hour before or 2 hours after your supplement. If you have questions about this, speak with your provider or pharmacist.
If you had to stop taking penicillamine (Depen) (a first-choice treatment option for Wilson's disease) because of bothersome or life-threatening side effects, you likely aren't able to take Cuvrior (trientine tetrahydrochloride). This is because Cuvrior (trientine tetrahydrochloride) was only studied to be safe and work well in people who previously took penicillamine (Depen) for at least 1 year. Discuss with your provider if Cuvrior (trientine tetrahydrochloride) is a good option for you.
Both Syprine and Cuvrior contain the same active medication (trientine) that's used to treat Wilson's disease, but there are a few differences. For one, Syprine must be refrigerated, while Cuvrior is more conveniently stored at room temperature. Also, each medication is made slightly differently: Syprine contains trientine dihydrochloride whereas Cuvrior, a new formulation, contains trientine tetrahydrochloride. When compared to each other, Cuvrior (trientine tetrahydrochloride) is absorbed faster and in greater amounts. While Cuvrior can be used in people who don't have harmful side effects from penicillamine (Depen), Syprine can only be used in people who can't take penicillamine (Depen) because of bothersome or life-threatening side effects.
There isn't enough information available to know if Cuvrior (trientine tetrahydrochloride) is safe to take during pregnancy. But, another form of Cuvrior (trientine tetrahydrochloride) that's available as trientine hydrochloride (Syprine) has been used over several decades, and no risks for major birth defects, miscarriages, or harm to unborn babies or pregnant mothers have been found. It has, however, caused harm to unborn babies in animal studies. It's important to treat Wilson's disease during pregnancy to avoid worsening symptoms and the risk of miscarriage or other harmful effects to your baby. Discuss the risks and benefits of taking Cuvrior (trientine tetrahydrochloride) during pregnancy with your healthcare provider.
Cuvrior (trientine tetrahydrochloride) can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.
When you first start taking Cuvrior (trientine tetrahydrochloride), the copper in your body moves around until your levels are stable. This can cause worsening of your symptoms, including brain problems. Let your provider know if you start to feel worse when beginning treatment with Cuvrior (trientine tetrahydrochloride) and they might adjust your dose or even stop the medication. Your provider will monitor your copper levels when you first start taking Cuvrior (trientine tetrahydrochloride), after 3 months, and then every 6 months.
Cuvrior (trientine tetrahydrochloride) can sometimes cause copper and iron levels to get too low. Pregnant women are at a higher risk since the amount of copper they need is often greater. Women who are on their period are at a higher risk of having low iron levels. A low copper diet that's commonly recommended in people with Wilson's disease can also lead to low iron levels. Let your provider know if you experience symptoms of low copper or iron levels including muscle weakness, dizziness, feeling tired, confusion, or tingling in your feet or hands. Your provider might recommend that you take an iron supplement, but take it at least 2 hours away from Cuvrior (trientine tetrahydrochloride) to prevent any interaction.
Rash was a commonly reported side effect of Cuvrior (trientine tetrahydrochloride) in the clinical studies. If you develop a rash or other allergic reaction during treatment with Cuvrior (trientine tetrahydrochloride), contact your provider to discuss whether you should continue taking it or switch to a different medication.
Your dose will depend on the dose of penicillamine (Depen) you were taking.
The typical dose ranges from 300 mg to 3,000 mg by mouth on an empty stomach per day, split into two doses throughout the day.