Wilson's Disease



Trientine and other Treatments for Wilson's Disease

Trientine and other treatments make Wilson's disease a very treatable condition. The goal of drug therapy in individuals with Wilson's disease is to remove excess copper from the body and prevent ongoing copper accumulation and deposition. Therapy must be life long.

DRUG THERAPY

BENEFITS

DISADVANTAGES / SIDE EFFECTS

Trientine Dihydrochloride (Trientine)

•  Well established chelating agent, now considered the initial treatment of choice for WD in symptomatic patients.

•  Effective as a maintenance treatment of WD.

•  Trientine also binds iron, and taking iron supplements can reduce the drug's effectiveness.

•  Trientine Side effects: Ageusia, Aplastic Anemia (rare); Gastritis; Sideroblastic anemia.

 

D-Penicillamine

•  The first copper chelating drug approved for use in Wilson's disease.

•  Effective as a maintenance treatment of WD.

•  D-Penicillamine is not suitable for patients with kidney disease or those who are allergic to penicillin.

•  Side effects: Ageusia, Agranulocytosis, Alopecia, Anorexia (epigastric pain, nausea, diarrhoea), Aplastic anaemia, blurred vision, Cutanous macular atrophy, Degenerative changes of the skin, depression of serum IgA levels, Diplopia, Elastosis perforans serpiginosa, Goodpasture's syndrome, Hepatotoxicity, Hyperkeratosis, Hyprgeusia, Initial hypersensitivity (rash, fever, hives), Intrahepatic cholestasis, Leukopenia, Lichen planus, Lupus-like reaction, mammary hyperplasia, Myasthenic syndrome, Nephrotic syndrome, Obliterative bronchitis, Optical axial neuritis, Oral ulcerations, Proteinuria, Ptosis, Serious retinitis, Thrombocytopenia or total aplasia.

Zinc Salts

•  Used as an initial drug therapy in presymptomatic patients and children.

•  Effective as maintenance treatment of WD.

•  Zinc works by blocking copper absorption and therefore is not suitable for symptomatic patients as initial drug therapy.

•  Issue with patient's non-compliance due to strict dosing regime.

•  Side effects: Amylase, Biochemical Pancreatitis, Gastritis, Elevation of serum alkaline phosphate, Leukopenia, Lipase and Zinc accumulation
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