Trientine

Trientine is a prescription medication used for the treatment of Wilson's disease. 

• Trientine is available under the following different brand names: Syprine, Cuvrior.

Common side effects of Trientine include:

• skin rash
• muscle spasms or contractions
• heartburn
• stomach pain
• loss of appetite
• skin flaking, cracking, or thickening

Serious side effects of Trientine include:

• hives
• difficult breathing
• swelling of the face, lips, tongue, or throat
• fever
• skin rash
• problems with speech, balance, walking, lifting, chewing, or swallowing
• muscle pain or stiffness
• muscle weakness
• drooping eyelids 
• double vision
• symptoms of lupus (an autoimmune disorder) joint pain or swelling, headaches, confusion, chest pain, shortness of breath, skin sores, numbness, cold feeling, or pale appearance of your fingers or toes

Rare side effects of Trientine include:

• none

Seek medical care or call 911 at once if you have the following serious side effects:

• Severe headache, confusion, slurred speech, arm or leg weakness, trouble walking, coordination loss, unsteady, very stiff muscles, high fever, profuse sweating, or tremors;
• Serious eye symptoms such as sudden vision loss, blurred vision, tunnel vision, eye pain or swelling, or seeing halos around lights;
• Serious heart symptoms include fast, irregular, or pounding heartbeats; fluttering in the chest; shortness of breath; sudden dizziness, lightheadedness, or passing out.

This is not a complete list of side effects and other serious side effects or health problems that may occur because of the use of this drug. Call your doctor for medical advice about serious side effects or adverse reactions. You may report side effects or health problems to FDA at 1-800-FDA-1088.

Adult and pediatric dosage

Capsule

• 250 mg (Syprine; hydrochloride salt)

Tablet

• 300 mg (Cuvrior; tetrahydrochloride salt)

Wilson disease

Adult dosage

• Syprine
  • Indicated for treatment of Wilson disease in patients intolerant of penicillamine
  • 750–1250 mg/day orally divided every 6–12 hours
  • Increase dose if clinical response is not adequate or free serum copper persistently above 20 mcg/dL; not to exceed 2 g/day
  • Reassess long-term maintenance dose every 6–12 months
• Cuvrior
  • Indicated for treatment of adults with stable Wilson disease who are decoppered and tolerant to penicillamine
  • Dose ranges from 300-3,000 mg/day orally divided two times a day; do not exceed 3,000 mg/day.
  • Discontinue penicillamine before starting.
  • Adjust total daily dosage according to clinical assessment and laboratory monitoring of copper.
  • Recommended Cuvrior starting dose when switching from penicillamine (total daily doses).
    • Penicillamine 125 mg: Cuvrior 300 mg
    • Penicillamine 250 mg: Cuvrior 600 mg
    • Penicillamine 375 mg: Cuvrior 900 mg
    • Penicillamine 500 mg: Cuvrior 900 mg
    • Penicillamine 625 mg: Cuvrior 1,200 mg
    • Penicillamine 750 mg: Cuvrior 1,500 mg
    • Penicillamine 875 mg: Cuvrior 1,800 mg
    • Penicillamine 1,000 mg: Cuvrior 2,100 mg
    • Penicillamine 1,125 mg: Cuvrior 2,400 mg
    • Penicillamine 1,250 mg: Cuvrior 2,400 mg
    • Penicillamine 1,375 mg: Cuvrior 2,700 mg
    • Penicillamine above 1,500 mg: Cuvrior 3,000 mg

Pediatric dosage

Syprine only

• Children aged younger than 12 years: 500–750 mg/day orally divided every 6–12 hours; not to exceed 1.5 g/day
• Children aged 12 years and above: 750–1250 mg/day orally divided every 6–12 hours; not to exceed 2 g/day

Dosage Considerations – Should be Given as Follows:

• See “Dosages”

If your medical doctor is using this medicine to treat your pain, your doctor or pharmacist may already be aware of any possible drug interactions and may be monitoring you for them. Do not start, stop, or change the dosage of any medicine before checking with your doctor, healthcare provider, or pharmacist first.

• Trientine has severe interactions with no other drugs
• Trientine has serious interactions with no other drugs
• Trientine has moderate interactions with at least 27 other drugs
• Trientine has minor interactions with the following drug:
  • copper

This information does not contain all possible interactions or adverse effects. Visit the RxList Drug Interaction Checker for any drug interactions. Therefore, before using this product, tell your doctor or pharmacist about all the products you use. Keep a list of all your medications with you and share this information with your doctor and pharmacist. Check with your healthcare professional or doctor for additional medical advice, health questions, or concerns.

Contraindications

• Hypersensitivity to product or components

Effects of drug abuse

• None

Short-Term Effects

• See “What Are Side Effects Associated with Using Trientine?”

Long-Term Effects

• See “What Are Side Effects Associated with Using Trientine?”

Cautions

• Not indicated for cystinuria, RA (cf penicillamine), or biliary cirrhosis
• May cause iron deficiency anemia
• Hepatic iron overload may result from copper deficiency induced by therapy
• Monitor urinary copper and for signs of hypersensitivity (e.g., elevated body temperature)
• Worsening of clinical symptoms, including neurological deterioration, may occur when beginning therapy owing to the mobilization of excess stores of copper; adjust the dose or discontinue Trientine if the patient’s clinical condition worsens
• Drug interaction overview
  • Mineral supplements
    • Avoid coadministration with mineral supplements (eg, iron, zinc, calcium, magnesium)
    • If unavoidable, administer trientine at least 2 hours before or after iron supplements OR at least 1 hour before or 2 hours after other mineral supplements
    • Trientine has the potential to chelate non-copper cations in mineral supplements and other oral drugs and thereby decreasing systemic absorption and efficacy
  • Other oral drugs
    • Separate doses by at least 1 hour
    • Trientine may potentially bind other drugs in the GI tract and decrease their systemic absorption

Pregnancy and Lactation

• Available data on the use of trientine for the treatment of Wilson disease have not identified any drug-associated risks for major birth defects, miscarriages, or other adverse maternal or fetal outcomes
• Monitor copper levels throughout pregnancy and use the minimum effective dose
• Clinical considerations
  • Untreated Wilson disease or discontinuation of treatment during pregnancy may result in worsening neurological and hepatic symptoms, including rare reports of hepatic decompensation and liver failure
  • Untreated Wilson disease may also increase the risk of miscarriage in some symptomatic patients
  • Increased copper deposition in the placenta and fetal liver may adversely impact the fetus
• Maternal adverse effects
  • Trientine may chelate non-copper cations (eg, iron, calcium); maintain appropriate levels during pregnancy
• Fetal/neonatal adverse effects
  • Chelator-induced copper deficiency may have adverse effects on the fetus
• Lactation
  • Published data are inconsistent regarding the detection of trientine in breast milk
  • Available published literature has not reported drug-associated adverse effects in infants exposed to trientine through breast milk