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Treatment of Hereditary Transthyretin-Mediated Amyloidosis in Adult Patients

Patisiran Only
It is given as intravenous infusion based on body weight.

For individuals less than 100 kg: 0.3 mg/kg once every 3 weeks

For individuals weighing 100 kg or more: 30 mg once every 3 weeks.

Treatment requires premedication with intravenous corticosteroid, oral acetaminophen, intravenous H1 blocker, and intravenous H2 blocker prior to its administration to reduce the risk of infusion-related reactions. For premedications not available or not tolerated intravenously, equivalents may be administered orally.

Inotersen Only
It is given as subcutaneous injection 284 mg once weekly.

Contraindicated if platelet count is less than 100 x 109 /L or if the individual has a history of acute glomerulonephritis caused by inotersen or if the individual has a history of a hypersensitivity reaction to inotersen.

Assess platelet count prior to treatment and monitored during treatment as inotersen causes reductions in platelet count that may result in sudden and unpredictable thrombocytopenia, which can be life-threatening.

Monitor alanine amino transferase, aspartate aminotransferase, and total bilirubin every 4 months during treatment and in case of symptoms of hepatic dysfunction.

Assess serum creatinine, estimated glomerular filtration rate (eGFR), urine protein to creatinine ratio (UPCR), and a urinalysis prior to starting treatment as inotersen can cause glomerulonephritis that may require immunosuppressive treatment and may result in dialysis dependent renal failure. During treatment, monitor serum creatinine, eGFR urinalysis, and UPCR every 2 weeks. Inotersen should not be given to patients who develop a UPCR of 1000 mg/g or higher, or eGFR below 45 mL/minute/1.73 m 2, pending further evaluation of the cause.

Inotersen is available only through a restricted program under a Risk Evaluation and Mitigation Strategy (REMS) called the TEGSEDI REMS Program, because of risks of serious bleeding caused by severe thrombocytopenia and because of glomerulonephritis. Important requirements of REMS program include:

Prescribers must be certified within the program by enrolling and completing training.

Individuals must enroll in the program and comply with ongoing monitoring requirements.

Pharmacies must be certified with the program and must only dispense to patients who are authorized to receive inotersen.

Vutrisiran Only
It is given as subcutaneous injection.

25 mg once every 3 months (Quarterly)

Injection should be administered by a healthcare professional.

Patisiran, Inotersen, and Vutrisiran
Treatment leads to a decrease in serum vitamin A levels and therefore vitamin A supplementation at the recommended daily allowance is advised. Individuals should be referred to an ophthalmologist if they develop ocular symptoms suggestive of vitamin A deficiency (e.g., night blindness).

Tafamidis and Tafamidis Meglumine
The recommended dose for tafamidis meglumine (brand name Vyndaqel) is 80 mg orally once daily as four 20 mg capsules. The recommended dose tafamidis (brand name Vyndamax) is 61 mg orally once daily as a single 61 mg capsule. Tafamidis and tafamidis meglumine are not substitutable on a per milligram basis.

787-277-6653 787-474-6326