Drugs List

Therapeutic Indications

Uses

Wilson's disease

Treatment of Wilson's disease in patients intolerant to penicillamine therapy.

Contraindications

Children under 5 years
Breastfeeding

Precautions and Warnings

Pregnancy
Renal impairment

May not resolve penicillamine-induced systemic lupus erythematosus
Treatment to be prescribed under the supervision of a specialist
Advise patient to take on an empty stomach 1 hr before or 2 hrs after food
Monitor copper levels
Monitor neurological function
Monitor renal function in patients with renal impairment
Neonate exposed in utero: Monitor serum copper concentrations
Pregnancy: Monitor maternal serum copper concentrations
Iron supplements may be required if iron deficiency occurs
Bioavailability differs with preparations;caution on changing formulations
Avoid antacids at the same time as this medicine
Avoid iron supplements within 2hrs of dose

Caution is advised when switching a patient from another trientine formulation. Tablets and capsules are not interchangeable due to differences in bioavailability.

Monitor for manifestations of overtreatment as this carries the risk of copper deficiency.

It is recommended to monitor patients closely for signs and symptoms of neurological deterioration in order to optimise the dose.

Pregnancy and Lactation

Pregnancy

Use trientine dihydrochloride with caution during pregnancy.

The manufacturer recommends trientine tetrahydrochloride is not used in pregnancy unless the potential benefit to the mother outweighs the potential risk to the foetus. Animal studies have shown reproductive toxicity, although likely to be a result of trientine-induced copper deficiency. At the time of writing there is limited human data available. Risks are unknown. If use cannot be avoided the pregnancy should be monitored in order to detect possible foetal abnormality and to assess maternal serum copper levels throughout the pregnancy. The dose of trientine should be adjusted to maintain serum copper levels within the normal range. Babies born to mothers being treated with trientine should be monitored for serum copper and ceruloplasmin levels where appropriate.

Lactation

Trientine tetrahydrochloride is contraindicated during breastfeeding.

The manufacturer advises that the patient either discontinues trientine tetrahydrochloride or discontinues breastfeeding. The presence of trientine tetrahydrochloride in human breast milk and the effects on exposed infants are unknown. Copper deficiency, however, is a potential toxicity.

Side Effects

Colitis
Duodenitis
Erythema
Iron deficiency anaemia
Nausea
Pruritus
Rash
Urticaria

Presentation

Oral formulations of trientine tetrahydrochloride.

Drugs List

Therapeutic Indications

Uses

Wilson's disease

Treatment of Wilson's disease in patients intolerant to penicillamine therapy.

Dosage

The doses are expressed as mg of trientine base (ie. not in mg of the trientine tetrahydrochloride salt).

Adults

Children

Contraindications

Children under 5 years
Breastfeeding

Precautions and Warnings

Pregnancy
Renal impairment

May not resolve penicillamine-induced systemic lupus erythematosus
Treatment to be prescribed under the supervision of a specialist
Advise patient to take on an empty stomach 1 hr before or 2 hrs after food
Monitor copper levels
Monitor neurological function
Monitor renal function in patients with renal impairment
Neonate exposed in utero: Monitor serum copper concentrations
Pregnancy: Monitor maternal serum copper concentrations
Iron supplements may be required if iron deficiency occurs
Bioavailability differs with preparations;caution on changing formulations
Avoid antacids at the same time as this medicine
Avoid iron supplements within 2hrs of dose

Caution is advised when switching a patient from another trientine formulation. Tablets and capsules are not interchangeable due to differences in bioavailability.

Monitor for manifestations of overtreatment as this carries the risk of copper deficiency.

It is recommended to monitor patients closely for signs and symptoms of neurological deterioration in order to optimise the dose.

Pregnancy and Lactation

Pregnancy

Use trientine dihydrochloride with caution during pregnancy.

The manufacturer recommends trientine tetrahydrochloride is not used in pregnancy unless the potential benefit to the mother outweighs the potential risk to the foetus. Animal studies have shown reproductive toxicity, although likely to be a result of trientine-induced copper deficiency. At the time of writing there is limited human data available. Risks are unknown. If use cannot be avoided the pregnancy should be monitored in order to detect possible foetal abnormality and to assess maternal serum copper levels throughout the pregnancy. The dose of trientine should be adjusted to maintain serum copper levels within the normal range. Babies born to mothers being treated with trientine should be monitored for serum copper and ceruloplasmin levels where appropriate.

Lactation

Trientine tetrahydrochloride is contraindicated during breastfeeding.

The manufacturer advises that the patient either discontinues trientine tetrahydrochloride or discontinues breastfeeding. The presence of trientine tetrahydrochloride in human breast milk and the effects on exposed infants are unknown. Copper deficiency, however, is a potential toxicity.

Side Effects

Colitis
Duodenitis
Erythema
Iron deficiency anaemia
Nausea
Pruritus
Rash
Urticaria

Overdosage

It is strongly recommended that the UK National Poisons Information Service be consulted on cases of suspected or actual overdose where there is doubt over the degree of risk or about appropriate management.

The following number will direct the caller to the relevant local centre (0844) 892 0111

Information may be obtained if you have access to ToxBase the primary clinical toxicology database of the National Poisons Information Service. This is available via password on the internet ( www.toxbase.org ) or if this is unavailable at the backup site ( www.toxbasebackup.org ).

Further Information

Last Full Review Date: September 2019

Reference Sources

Drugs in Pregnancy and Lactation: A Reference Guide to Fetal and Neonatal Risk, 10th edition (2015) ed. Briggs, G., Freeman, R. Wolters Kluwer Health, Philadelphia.

Summary of Product Characteristics: Cuprior 150 mg film-coated tablets. GMP-Orphan United Kingdom Ltd. Revised September 2017.

NICE Evidence Services Available at: www.nice.org.uk Last accessed: 08 April 2022.