Jack, 7

Discontinued penicillamine

Diagnosed at age 6 with symptoms of proteinuria, shuffling gait and emotional lability. Serum ceruloplasmin
5 mg/dL.

TREATMENT HISTORY:
Started treatment with penicillamine and developed degenerative skin changes and leukopenia.

CURRENT TREATMENT:
CLOVIQUE 750 mg/d.

Cathy, 11

Intolerant to penicillamine

Diagnosed at age 8 based on abnormal transaminases.

TREATMENT HISTORY:
Because of intolerance of penicillamine, she started therapy with trientine hydrochloride.

CURRENT TREATMENT:
CLOVIQUE 1500 mg/d.

Tyler, 22

Noncompliant with treatments and diet.

Diagnosed at age 10 when older brother was diagnosed.

TREATMENT HISTORY: Started on trientine hydrochloride because of intolerance of penicillamine; however, he was not compliant with treatment and missed several doses per day.

CURRENT TREATMENT:
CLOVIQUE 1500 mg/d.

Sarah, 38

Maintenance therapy with zinc was not effective, so she was switched to a chelating agent.

Diagnosed at age 18 after experiencing slurred speech, drooling and fatigue. Kayser-Fleischer rings noted on exam; 24-hour urine confirmed Wilson's disease.

TREATMENT HISTORY:
Started on penicillamine and switched to trientine hydrochloride because of side effects. Moved to zinc maintenance therapy; however, zinc alone was not effective.

CURRENT TREATMENT:
CLOVIQUE 2000 mg/d.

Not actual patients.

important safety Information

Wilson's disease (WD), the most common inherited disorder of copper metabolism, results from a failure of the copper excretory pathway. This leads to toxic accumulation of copper in the liver and eventually other organs.1 The worldwide prevalence of WD is estimated to be one in 30,000 individuals.2

The condition can be treated with a low copper diet and the use of chelating agents that bind copper to facilitate its excretion from the body. CLOVIQUETM (trientine hydrochloride) is a chelating agent indicated for treatment of patients with WD who are intolerant of the first-line treatment, penicillamine.3

Indications and Usage

CLOVIQUETM may be used to treat Wilson's disease in patients who cannot take the medication penicillamine. Clinical experience with trientine hydrochloride is limited and alternate dosing regimens have not been well-characterized; all endpoints in determining an individual patient's dose have not been well defined. CLOVIQUETM and penicillamine cannot be considered interchangeable. CLOVIQUETM should be used when continued treatment with penicillamine is no longer possible because of intolerable or life endangering side effects.

Unlike penicillamine, CLOVIQUETM is not recommended in cystinuria or rheumatoid arthritis. The absence of a sulfhydryl moiety renders it incapable of binding cystine and, therefore, it is of no use in cystinuria. In 15 patients with rheumatoid arthritis, trientine hydrochloride was reported not to be effective in improving any clinical or biochemical parameter after 12 weeks of treatment.

CLOVIQUETM is not indicated for treatment of biliary cirrhosis.

Important Safety Information

  • CLOVIQUETM is contraindicated in patients hypersensitive to CLOVIQUETM or any components of the formulation. Patients should be observed closely for signs of possible hypersensitivity.
  • Patients receiving CLOVIQUETM should remain under regular medical supervision throughout the period of drug administration. Patients (especially women) should be closely monitored for evidence of iron deficiency anemia.
  • The treatment can be monitored by the determination of free copper in the serum. Therapy may be monitored with a 24-hour urinary copper analysis periodically (i.e., every 6-12 months).
  • CLOVIQUETM contains a color additive FD&C Yellow #5 (tartrazine) that may cause allergic-type reactions (including bronchial asthma) in certain people. It is frequently seen in patients who also have aspirin hypersensitivity.
  • Patients should be directed to take CLOVIQUETM on an empty stomach, at least one hour before meals or two hours after meals and at least one hour apart from any other drug, food, or milk. The capsules should be swallowed whole with water and should not be opened or chewed. For the first month of treatment, the patient should have his temperature taken nightly, and he should be asked to report any symptom such as fever or skin eruption.
  • In general, mineral supplements should not be given since they may block the absorption of CLOVIQUETM.
  • CLOVIQUETM should be used during pregnancy only if the potential benefit justifies the potential risk to the fetus.
  • The following adverse reactions have been reported in a clinical study using trientine hydrochloride: iron deficiency, systemic lupus erythematosus. In addition, dystonia, muscular spasm, myasthenia gravis have been reported in marketed use.
  • CLOVIQUETM carton should be kept refrigerated at 2°C - 8°C (36°F - 46°F).
  • For patient convenience, individual blister pack (or tray) may be stored for a maximum single period of 30 days at or below room temperature (25°C (77°F)) with protection from sources of heat and humidity. Capsules stored at room temperature should be discarded after 30 days.

Please see full Prescribing Information for CLOVIQUETM Capsules.

You are encouraged to report negative side effects of prescription drugs to FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088. You may also contact Kadmon Pharmaceuticals, LLC at 1-877-377-7862 to report negative side effects.

References:
  1. Gaffney D, Fell GS, O'Reilly DS. ACP Best Practice No 163. Wilson's disease: acute and presymptomatic laboratory diagnosis and monitoring. J Clin Pathol. 2000;53(11):807-812.

  2. European Association for Study of Liver. EASL clinical practice guidelines: Wilson's disease. J Hepatol. 2012;56(3):671-685.

  3. Clovique. Package Insert. Kadmon Pharmaceuticals, LLC; 2018.

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