FDA Approves Cuvrior to Treat Wilson’s Disease
Key takeaways:
In May 2022, the FDA approved Cuvrior (trientine tetrahydrochloride) to treat Wilson’s disease in adults. It’s the first medication approved for Wilson’s disease in over 50 years.
Cuvrior is a possible alternative to penicillamine (Cuprimine, Depen). Penicillamine is currently the go-to option for treating Wilson’s disease.
Cuvrior is expected to be available in the U.S. by early 2023. Its retail cost hasn’t been announced yet.
Copper plays a hidden role in many aspects of our lives. It’s found in electrical wires throughout our homes, it coats the outside of pennies, and a version of it is even the foundation of a certain type of birth control — the copper IUD.
But these aren’t the only uses. Copper is also a mineral that our bodies need for health purposes. Our diets typically give us a sufficient amount of copper every day. However, too much of it can be problematic. This risk is well known to people living with Wilson’s disease — an inherited (genetic) disorder that makes it hard to get rid of copper. If this condition is left untreated, copper levels can build up and cause serious health issues.
To help with this, the FDA recently approved Cuvrior (trientine tetrahydrochloride) to treat Wilson’s disease in adults. It’s the first new medication approved for Wilson’s disease in more than 50 years. Here, we’ll talk about Cuvrior’s ability to treat Wilson’s disease and why its approval is notable.
What is Cuvrior?
Cuvrior is an oral tablet medication that can treat Wilson’s disease in adults.
However, Cuvrior isn’t meant for all adults with Wilson’s disease. It should only be used if your copper levels are under control and you want an alternative option to penicillamine (Cuprimine, Depen), which is the first-choice medication for treating the condition.
Cuvrior is made by Orphalan, a French pharmaceutical company.
How does Cuvrior work to treat Wilson’s disease?
Cuvrior is a type of chelator. Chelators are medications that attach to unwanted substances in your body and help you to get rid of them. Cuvrior acts like a chelator by attaching to copper in your body, reducing your gut’s absorption of it, and getting rid of it through your urine.
This treatment strategy isn’t unique to Wilson’s disease. Chelators are also used for other health conditions. For instance, if you have far too much iron in your body, your healthcare provider may give you a medication like deferasirox (Exjade, Jadenu). Deferasirox can attach to iron in your blood and get rid of it when you go to the bathroom.
How is Cuvrior different from other forms of trientine?
This difference is pretty nuanced. Cuvrior contains trientine tetrahydrochloride. But another type of trientine — trientine hydrochloride — has actually been approved in the U.S. for decades. Trientine hydrochloride is available as a generic product, and it’s also available as name-brand Syprine.
These two versions of trientine haven’t been compared in head-to-head clinical studies, so we don’t know for sure if one is more effective than the other. However, they’re meant to be used in slightly different situations.
They’re both approved as alternative options to penicillamine for Wilson’s disease. But trientine hydrochloride (the older medication) should only be used only if penicillamine causes bothersome side effects that can’t be tolerated. Cuvrior, the new medication, is a maintenance medication that you can take even if penicillamine doesn’t cause harmful side effects.
Cuvrior’s chemistry also makes it more stable than trientine hydrochloride. Because of this, Cuvrior can be stored at room temperature. Trientine hydrochloride’s storage is less convenient, as it has to be refrigerated.
How is Cuvrior dosed?
Cuvrior is a 300 mg oral tablet. It can be taken by mouth 2 times daily to control Wilson’s disease over time. But each time you take a dose, there’s a good chance you’ll be taking several pills at a time.
The specific dose you’ll take depends on how much penicillamine you were taking. For instance, if you were taking 875 mg of penicillamine every day, you’ll likely take three Cuvrior tablets twice daily (1,800 mg total). But if you were taking 1,250 mg of penicillamine every day, you’ll likely take four Cuvrior tablets twice daily (2,400 mg total). You shouldn’t take more than 3,000 mg of Cuvrior in a given day.
And regardless of how often you take it, make sure to take it on an empty stomach (1 hour before or 2 hours after eating). Don’t crush or chew the tablet, either. If need be, you can cut it in half along the scored line. The pills also need to remain in their original packaging, so you shouldn’t store them in a pill box (if you use one).
How effective is Cuvrior?
Most of what we know about Cuvrior’s effectiveness comes from a small, international clinical trial called the CHELATE study. The study took place over about 8 months and analyzed about 50 adults living with Wilson’s disease.
In this study, Cuvrior’s effectiveness was compared to penicillamine. Everyone in the study started off by taking penicillamine, but after about 3 months, half of the group was switched to Cuvrior. At the end of the study, the two medications were found to control copper levels to a similar degree.
With these results in mind, researchers determined that Cuvrior is non-inferior to penicillamine. This means Cuvrior is a possible alternative option to penicillamine, the current go-to treatment for Wilson’s disease.
What are the known side effects of Cuvrior?
Like with all medications, Cuvrior can cause side effects. The most common side effects consist of:
Stomach pain
Constipation or other bowel changes
Skin rashes
Hair loss
Mood changes
These aren’t the only side effects. Rare but serious side effects are also possible. This includes anemia, certain short-term neurological problems, and lupus. It’s also possible to develop a copper deficiency.
If you’re experiencing any side effects that are concerning or intolerable, make sure to reach out to your healthcare provider right away.
Does Cuvrior interact with other medications?
Yes, it can interact with many medications — especially oral medications. Because of how it works to treat Wilson’s disease, Cuvrior can also bind to other medications when they’re in your gastrointestinal tract at the same time. This makes them less effective.
Because of this interaction, you should try to separate Cuvrior from any other oral medications by at least 1 hour. For example, if you take a Cuvrior dose at 8:00AM, you shouldn’t take anything between 7:00AM and 9:00AM. But if you need to take mineral-based supplements, like iron supplements, it’s better to give it at least 2 hours of separation.
If you use any non-oral medications — such as injectable medications or medications you apply to your skin — this risk doesn’t apply.
How much will Cuvrior cost?
Cuvrior isn’t available quite yet, and its manufacturer hasn't announced the price yet. So, at this time, we don’t know how much it’ll cost. But if it’s anything like its older brother, trientine hydrochloride, it’ll likely be expensive.
For example, for an average 30-day supply of generic trientine hydrochloride 250 mg capsules, the average retail cost is over $34,500. A free GoodRx coupon can drop this down to as low as $944.
However, this is just a prediction. Make sure to check back with GoodRx’s Cuvrior page for price updates and ways to save as we get closer to the medication’s launch date.
When will Cuvrior be available?
Orphalan, Cuvrior’s manufacturer, expects the new medication to be available in the U.S. by early 2023. A more specific timeframe hasn't been announced yet. We’ll likely get more details as the new year gets closer.
Once it’s available, Cuvrior will be a new option for U.S. adults with Wilson’s disease. But it’s not quite as new for other countries, as it has been approved in the European Union since 2017.
The bottom line
Cuvrior is an oral medication that’s now FDA-approved to treat Wilson’s disease in adults. It works as a chelator, helping to control copper levels in your body. It’s a possible alternative to penicillamine. But there are a lot of things to keep in mind, such as it can cause a lot of drug interactions, you’ll likely take multiple pills every per day, and it won’t be available in the U.S. until 2023.
If you think Cuvrior is a possible option for you or a loved one, reach out to a pharmacist or healthcare provider for details.
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