(TRYE en teen)
Treatment of Wilsons disease in patients intolerant to penicillamine
Hypersensitivity to trientine or any component of the formulation
Wilsons disease: Oral: 750-1250 mg/day in divided doses 2-4 times/day; maximum dose: 2 g/day. AASLD practice guidelines suggest typical doses of 750-1500 mg/day in 2-3 divided doses with maintenance therapy of 750-1000 mg/day (Roberts, 2008).
Refer to adult dosing.
Wilsons disease: Oral:
Children <12 years: 500-750 mg/day in divided doses 2-4 times/day; maximum: 1.5 g/day. AASLD practice guidelines suggest 20 mg/kg/day rounded off to the nearest 250 mg, given in 2-3 divided doses (Roberts, 2008).
Children ≥12 years: Refer to adult dosing.
No dosage adjustment provided in manufacturer 's labeling.
No dosage adjustment provided in manufacturer 's labeling.
Administer 1 hour before or 2 hours after meals and at least 1 hour apart from any drug, food, or milk. Do not open or chew capsule, swallow whole followed by a full glass of water; notify healthcare provider of any fever or skin changes; any skin exposed to the contents of a capsule should be promptly washed with water.
Should be taken 1 hour before or 2 hours after meals and at least 1 hour apart from any drug, food, or milk.
Store at 2 ‚ °C to 8 ‚ °C (36 ‚ °F to 46 ‚ °F).
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Capsule, Oral, as hydrochloride:
Syprine: 250 mg
Antacids: May decrease the absorption of Trientine. Management: Separate trientine dosing from other oral drugs (eg, antacids) by at least 1 hour. Monitor for decreased therapeutic effects of trientine if an antacid is initiated/dose increased, or increased effects if an antacid is discontinued/dose decreased. Consider therapy modification
Calcium Salts: May decrease the serum concentration of Trientine. Trientine may decrease the serum concentration of Calcium Salts. Consider therapy modification
Carbonic Anhydrase Inhibitor Diuretics: May decrease the serum concentration of Trientine. Monitor therapy
Iron Salts: Trientine may decrease the serum concentration of Iron Salts. Iron Salts may decrease the serum concentration of Trientine. Management: Trientine manufacturer recommends avoiding concurrent use with oral iron salts due to the risk for impaired GI absorption of both trientine and the iron salt. Short courses of iron may be used; however, separate administration by at least 2 hours. Exceptions: Ferric Carboxymaltose; Ferric Gluconate; Ferric Hydroxide Polymaltose Complex; Ferric Pyrophosphate Citrate; Ferumoxytol; Iron Dextran Complex; Iron Sucrose. Consider therapy modification
Magnesium Salts: Trientine may decrease the serum concentration of Magnesium Salts. Magnesium Salts may decrease the serum concentration of Trientine. Consider therapy modification
Multivitamins/Minerals (with ADEK, Folate, Iron): May decrease the serum concentration of Trientine. Management: Trientine manufacturer recommends avoiding concurrent administration with oral minerals due to the risk for impaired GI absorption of both trientine and the mineral. Short courses of iron may be used; however, separate administration by at least 2 hours. Consider therapy modification
Multivitamins/Minerals (with AE, No Iron): May decrease the serum concentration of Trientine. Management: Trientine manufacturer recommends avoiding concurrent administration with oral minerals. The recommendation is that trientine be taken at least one hour before or two hours after meals and at least one hour apart from any drug, food, or milk. Consider therapy modification
Zinc Salts: Trientine may decrease the serum concentration of Zinc Salts. Zinc Salts may decrease the serum concentration of Trientine. Consider therapy modification
Periodic 24-hour urinary copper assessment (every 6-12 months); serum non-ceruloplasmin bound copper; LFTs; CBC; periodic ophthalmic exam
Frequency not defined.
Central nervous system: Dystonia, malaise
Dermatologic: Rash, thickening and fissuring of skin
Endocrine & metabolic: Iron deficiency
Gastrointestinal: Abdominal pain, anorexia, aphthoid ulcer, colitis, epigastric pain, gastritis, heartburn, loss of taste
Hematologic: Aplastic anemia (rare), anemia, sideroblastic anemia (reversible)
Hepatic: Iron overload
Local: Tenderness
Neuromuscular & skeletal: Muscle cramps, muscle pain, muscular spasm, myasthenia gravis, rhabdomyolysis, weakness
Miscellaneous: Fixed drug eruption, lupus-like reactions
Concerns related to adverse effects:
- Anemia: May cause iron-deficiency anemia; monitor closely.
- Copper deficiency: Induced by treatment; may lead to hepatic iron overload and/or sideroblastic anemia; reassess dose.
- Neurologic worsening: May occur with treatment initiation; less common than with penicillamine.
Other warnings/precautions:
- Hypersensitivity: Not reported with use; however, industrial workers exposed to trientine for prolonged periods have reported asthma, bronchitis, and dermatitis.
C
Teratogenic effects were observed in animal reproduction studies; fetal resorptions and abnormalities increased with decreased levels of fetal copper. Treatment of Wilson 's disease should be maintained during pregnancy; dose reduction (25% to 50% of prepregnancy dose) should be considered (Roberts, 2008).
Trientine hydrochloride is an oral chelating agent structurally dissimilar from penicillamine and other available chelating agents; an effective oral chelator of copper used to induce adequate cupriuresis
Poor
To acetyltrien (chelating activity significantly less than parent)
Urine (1% as parent; 8% as metabolite)
- Discuss specific use of drug and side effects with patient as it relates to treatment. (HCAHPS: During this hospital stay, were you given any medicine that you had not taken before? Before giving you any new medicine, how often did hospital staff tell you what the medicine was for? How often did hospital staff describe possible side effects in a way you could understand?)
- Patient may experience muscle spasms. Have patient report immediately to prescriber severe abdominal pain, severe muscle pain, severe muscle weakness, or red, blistered, or swollen skin (HCAHPS).
- Educate patient about signs of a significant reaction (eg, wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat). Note: This is not a comprehensive list of all side effects. Patient should consult prescriber for additional questions.
Intended Use and Disclaimer: Should not be printed and given to patients. This information is intended to serve as a concise initial reference for health care professionals to use when discussing medications with a patient. You must ultimately rely on your own discretion, experience, and judgment in diagnosing, treating, and advising patients.