Syprine — Medical Mutual

Wilson’s disease

Preferred products

Diagnosis of Wilson’s disease is confirmed by ONE of the following (i or ii):
i. Genetic testing results confirming biallelic pathogenic ATP7B mutations (in either symptomatic or asymptomatic individuals); OR
ii. Confirmation of at least two of the following (a, b, c, d):
a. Presence of Kayser-Fleischer rings;
b. Serum ceruloplasmin level < 20 mg/dL;
c. Liver biopsy findings consistent with Wilson’s disease;
d. 24-hour urinary copper > 40 mcg/24 hours; AND
Patient meets ONE of the following criteria (i, ii, iii, iv, v or vi):
i. Patient has tried one preferred penicillamine product and is intolerant to penicillamine therapy, according to the prescriber; OR
ii. Patient has clinical features indicating the potential for intolerance to penicillamine therapy, according to the prescriber; OR
Note: Specific clinical features include history of any renal disease, congestive splenomegaly causing severe thrombocytopenia, autoimmune tendency [documentation required]; OR
iii. Patient has a contraindication to penicillamine therapy, according to the prescriber [documentation required]; OR
iv. Patient has neurologic manifestations of Wilson’s disease; OR
v. Patient is pregnant; OR
vi. Patient has been started on therapy with trientine (generic of Syprine) and, according to the prescriber, the patient has experienced inadequate efficacy or significant intolerance; AND
The medication is prescribed by or in consultation with a gastroenterologist, hepatologist, or liver transplant physician.

1 year