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Patisiran Dosage

Medically reviewed by Drugs.com. Last updated on July 14, 2020.

Applies to the following strengths: 2 mg/mL

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)

Comments:
-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

Precautions

CONTRAINDICATIONS: None

Safety and efficacy have not been established in patients younger than 18 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
-This drug should be administered by a healthcare professional
-All patients should receive premedication to reduce the risk of infusion-related reactions (IRRs)

IV INFUSION:
-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

Storage requirements:
-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

Reconstitution/preparation techniques:
-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

General:
-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.

Monitoring:
-Monitor for infusion related reactions
-Monitor for vitamin A deficiency

Patient advice:
-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.

Frequently asked questions

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.