Prior Authorization and Quantity Limit Criteria for Approval

Initial Evaluation

Trientine hydrochloride capsules will be approved when ALL of the following are met:

1. The patient has a diagnosis of Wilson’s disease confirmed by ONE of the following: 
   1. Confirmation of genetic mutation of the ATP7B gene OR 
   2. The patient has TWO or more of the following: 
      1. Presence of hepatic abnormality (e.g., acute liver failure, cirrhosis, fatty liver) 
      2. Presence of Kayser-Fleischer rings 
      3. Serum ceruloplasmin level less than 20 mg/dL 
      4. Basal urinary copper excretion greater than 40 mcg/24 hours or the testing laboratory’s upper limit of normal 
      5. . Hepatic parenchymal copper content greater than 40 mcg/g dry weight
      6. Presence of neurological symptoms (e.g., dystonia, hypertonia, rigidity with tremors, dysarthria, muscle spasms, dysphasia, polyneuropathy, dysautonomia) AND 
2. ONE of the following: 
   1. The patient has tried and had an inadequate response to penicillamine OR 
   2. The patient has an intolerance or hypersensitivity to penicillamine OR 
   3. The patient has an FDA labeled contraindication to penicillamine AND 
3. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., gastroenterologist, hepatologist, neurologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND
4. ONE of the following: 
   1. The requested quantity (dose) does NOT exceed the program quantity limit OR 
   2. BOTH of the following: 
      1. The requested quantity (dose) is greater than the program quantity limit AND 
      2. The prescriber has provided information in support of therapy with a higher dose for the requested indication 

Length of Approval: 12 months

Renewal Evaluation

Trientine hydrochloride capsules will be approved when ALL of the following are met:

1. The patient has been previously approved for the requested medication through the plan’s Prior Authorization criteria AND
2. The patient has a diagnosis of Wilson’s disease AND
3. The patient has had clinical benefit with the requested medication as evidenced by ONE of the following:
   1. Improvement and/or stabilization in hepatic abnormality OR 
   2. Reduction in Kayser-Fleischer rings OR 
   3. Improvement and/or stabilization in neurological symptoms (e.g., dystonia, hypertonia, rigidity with tremors, dysarthria, muscle spasms, dysphasia, polyneuropathy, dysautonomia) OR 
   4. Basal urinary copper excretion greater than 200 mcg/24 hours AND 
4. The prescriber is a specialist in the area of the patient’s diagnosis (e.g., gastroenterologist, hepatologist, neurologist) or the prescriber has consulted with a specialist in the area of the patient’s diagnosis AND
5. ONE of the following: 
   1. The requested quantity (dose) does NOT exceed the program quantity limit OR 
   2. BOTH of the following: 
      1. The requested quantity (dose) is greater than the program quantity limit AND 
      2. The prescriber has provided information in support of therapy with a higher dose for the requested indication 

Length of Approval: 12 months