Medically reviewed by Drugs.com. Last updated on Jul 14, 2020.
Applies to the following strengths: 2 mg/mL
Usual Adult Dose for:
Additional dosage information:
Usual Adult Dose for Amyloidogenic Transthyretin Amyloidosis
Dosing is based on actual body weight; premedication should be given at least 60 minutes prior to the start of the infusion
Weight less than 100 kg: 0.3 mg/kg via IV infusion every 3 weeks
Weight 100 kg or greater: 30 mg via IV infusion every 3 weeks
Initial infusion rate should be approximately 1 mL/min for the first 15 minutes, then increase to approximately 3 mL/min for the remainder of the infusion; in the event of an infusion related reaction, the duration of infusion may be extended; see administration advice for infusion instructions
PREMEDICATION: Administer on day of infusion at least 60 minutes prior to start of infusion:
-Acetaminophen 500 mg orally
-IV corticosteroid (e.g., dexamethasone 10 mg or equivalent)
-IV H1 blocker (e.g. diphenhydramine 50 mg or equivalent)
-IV H2 blocker (e.g., ranitidine 50 mg or equivalent)
-Premedications are required to reduce the risk of infusion related reactions (IRR).
-If IV premedications are not available or not tolerated, oral equivalents may be used
-If patients are tolerating the infusion, but experiencing adverse reactions to corticosteroid premedication, reduce corticosteroid in 2.5 mg increments to a minimum IV dose of dexamethasone 5 mg or equivalent
-Some patients may require additional or higher doses of one or more premedications to reduce the risk of IRRs.
Use: For the treatment of polyneuropathy of hereditary transthyretin-mediated amyloidosis.
Renal Dose Adjustments
Mild or moderate renal impairment: No adjustment recommended
Severe renal impairment (CrCl less than 30 mL/min): Data not available
Liver Dose Adjustments
Mild hepatic impairment (bilirubin 1 times upper limit of normal [1xULN] and AST less than 1 x ULN; or bilirubin greater than 1 and less than 1.5 x ULN): No adjustment recommended
Moderate to severe hepatic impairment: Data not available
Safety and efficacy have not been established in patients younger than 18 years.
Consult WARNINGS section for additional precautions.
Data not available
-This drug should be administered by a healthcare professional
-All patients should receive premedication to reduce the risk of infusion-related reactions (IRRs)
-Use a dedicated line with an infusion set containing 1.2 micron polyethersulfone in-line infusion filter and infusion sets that are di(2-ethylhexyl)phthalate-free (DHEP-free)
-Administer through a free-flowing venous access line; monitor for possible infiltration; suspected extravasation should be managed according to local standard of practice for non-vesicants
-Initial infusion rate should be approximately 1 mL/min for the first 15 minutes, then increase to approximately 3 mL/min for the remainder of the infusion
-In the event of an IRR, the duration of infusion may be extended
-Flush line upon completion of infusion
Missed dose: If a dose is missed, it should be administered as soon as possible
-If administration is within 3 days of the missed dose, dosing may be resumed on original schedule
-If administered more than 3 days after the missed dose, continue dosing every 3 weeks, thereafter
-Vials should be stored refrigerated at 36F to 46F (2C to 8C); do not freeze; discard if frozen
-May store at room temperature (up to 77F [25C]) for up to 14 days
-This drug should be filtered (0.45 micron polyethersulfone syringe) and diluted in an infusion bag of 0.9% Sodium Chloride (total volume 200 mL) prior to administration
-Infusion bags should be di(2-ethylhexyl)phthalate-free (DHEP-free)
-Gently invert bag to mix solution; do not shake; do not mix or dilute with other drugs
-Diluted solution should be administered immediately after preparation; however, may be stored at room temperature (up to 86F [30C]) for up to 16 hours (including infusion time); do not freeze
-Serum TTR is a carrier of retinol binding protein (which is involved in the transport of vitamin A in blood); a 45% mean reduction in serum retinol binding protein and 62% reduction in serum vitamin A have been observed with treatment of over 18 months.
-Monitor for infusion related reactions
-Monitor for vitamin A deficiency
-Patients should understand that infusion-related reactions may occur and that premedications will be provided to lessen the chance of a reaction.
-Patients should understand that treatment causes a decrease in serum vitamin A levels patients will need to supplement with the recommended daily allowance of vitamin A.
-Patients developing ocular symptoms suggestive of vitamin A deficiency (e.g. reduced night vision or night blindness, persistent dry eyes, eye inflammation, corneal inflammation or ulceration, corneal thickening or corneal perforation) should notify their health care professional.
-Patients who are, or intend to become pregnant should speak with their healthcare provide.
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