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

Hepatic impairment
Pregnancy
Renal impairment

May not resolve penicillamine-induced systemic lupus erythematosus
Not all available brands are licensed for all age groups
Treatment to be prescribed under the supervision of a specialist
Some formulations contain propylene glycol
Avoid taking within 1 hour of other medicines
Take at a different time from iron supplement
Monitor copper levels
Monitor for neurological symptoms at initiation and adjust dose as required
Monitor liver function in patients with hepatic impairment
Monitor neurological function
Monitor patients with hepatic symptoms every 2 to 3 weeks after initiation
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
Reduce dose if gastrointestinal symptoms or haematological changes occur
Bioavailability differs with preparations;caution on changing formulations
Avoid antacids at the same time as this medicine

Monitor for manifestations of over treatment as this carries the risk of copper deficiency. Regular monitoring of clinical symptoms and copper levels are recommended at least twice a year, with more frequent monitoring during the initial phase of treatment.

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. If treatment with trientine dihydrochloride is continued during pregnancy, consider reducing the dose to the lowest effective dose and monitoring compliance. 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 dihydrochloride is contraindicated during breastfeeding.

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

Side Effects

Aplastic anaemia
Colitis
Duodenitis
Dysarthria
Dystonia
Erythema
Exacerbation of neurological symptoms of Wilson's disease
Iron deficiency anaemia
Lupus erythematosus-like syndrome
Lupus nephritis
Muscle rigidity
Nausea
Rash
Sideroblastic anaemia
Tremor
Urticaria

Presentation

Oral formulations of trientine dihydrochloride.

Drugs List

Therapeutic Indications

Uses

Wilson's disease

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

Dosage

Doses may be expressed as mg trientine base or mg trientine salt - see manufacturer's literature.

Tablets and capsules are not interchangeable due to differences in bioavailability.

Adults

Children

Contraindications

Children under 5 years
Breastfeeding

Precautions and Warnings

Hepatic impairment
Pregnancy
Renal impairment

May not resolve penicillamine-induced systemic lupus erythematosus
Not all available brands are licensed for all age groups
Treatment to be prescribed under the supervision of a specialist
Some formulations contain propylene glycol
Avoid taking within 1 hour of other medicines
Take at a different time from iron supplement
Monitor copper levels
Monitor for neurological symptoms at initiation and adjust dose as required
Monitor liver function in patients with hepatic impairment
Monitor neurological function
Monitor patients with hepatic symptoms every 2 to 3 weeks after initiation
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
Reduce dose if gastrointestinal symptoms or haematological changes occur
Bioavailability differs with preparations;caution on changing formulations
Avoid antacids at the same time as this medicine

Monitor for manifestations of over treatment as this carries the risk of copper deficiency. Regular monitoring of clinical symptoms and copper levels are recommended at least twice a year, with more frequent monitoring during the initial phase of treatment.

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. If treatment with trientine dihydrochloride is continued during pregnancy, consider reducing the dose to the lowest effective dose and monitoring compliance. 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 dihydrochloride is contraindicated during breastfeeding.

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

Counselling

Take at a different time from iron supplement.

Avoid antacids at the same time as this medicine.

Advise patient to take dose on an empty stomach at least 1 hour before meals or two hours after meals.

Advise patient to take dose at least 1 hour apart from any other medicines, food or milk.

Side Effects

Aplastic anaemia
Colitis
Duodenitis
Dysarthria
Dystonia
Erythema
Exacerbation of neurological symptoms of Wilson's disease
Iron deficiency anaemia
Lupus erythematosus-like syndrome
Lupus nephritis
Muscle rigidity
Nausea
Rash
Sideroblastic anaemia
Tremor
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

Summary of Product Characteristics: Cufence 200mg hard capsules. Univar Solutions B.V. Revised October 2021.

Summary of Product Characteristics: Trientine dihydrochloride Tillomed 250mg capsules, hard. Tillomed Laboratories Ltd. Revised August 2021.

NICE Evidence Services Available at: www.nice.org.uk Last accessed: 13 May 2022