Drug Detail:Givosiran (Givosiran [ gi-voe-si-ran ])
Drug Class: Miscellaneous metabolic agents
Usual Adult Dose for Porphyria
2.5 mg/kg subcutaneously once a month
Comments:
- Dosing is based on actual body weight.
- Administration should be performed by a healthcare professional.
Use: For the treatment of acute hepatic porphyria (AHP).
Renal Dose Adjustments
No adjustment recommended; monitoring recommended
Liver Dose Adjustments
Interrupt or discontinue treatment for severe or clinically significant transaminase elevations
Reinstituting Therapy:
- In patient who have subsequent improvement of transaminase: Reduce dose to 1.25 mg/kg once a month
- For patients who resume dosing at 1.25 mg/kg once monthly without recurrence of severe or clinically significant transaminase elevations: Dose may be increased to 2.5 mg/kg once a month
Precautions
CONTRAINDICATIONS:
- Known severe hypersensitivity to the active substance; reactions have included anaphylaxis
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:
- Healthcare professional should administer via subcutaneous injection once a month
- During administration, adequate medical support to manage an anaphylactic reaction should be readily available
- Administer subcutaneously into abdomen, back or side of the upper arms, or thighs
- Rotate injection sites
- Do not inject into scar tissue or areas that are reddened, inflamed, or swollen
Missed dose: Administer as soon as possible after the missed dose; resume dosing at monthly intervals following administration of the missed dose
Storage requirements:
- Store at 2C to 25C (35F to 77F); store in original container until ready to use
Preparation techniques:
- Available as a ready-to-use single-dose vial that does not require additional reconstitution or dilution
- Withdraw calculated injection volume using a 21-gauge or larger needle
- Divide doses requiring volumes greater than 1.5 mL equally into multiple syringes
- For injection, use a 25-gauge or 27-gauge needle with 1/2-inch or 5/8-inch needle length
General:
- On average, patients with acute hepatic porphyria (AHP) experienced 70% fewer porphyria attacks compared to placebo patients in clinical trials.
Monitoring:
- Monitor for signs and symptoms of anaphylaxis
- Monitor renal function as clinically indicated
- Obtain baseline liver function tests and repeat monthly for the first 6 months, then as clinically indicated
Patient advice:
- Patients should understand the risk and symptoms of anaphylaxis that may occur with treatment.
- Patients should be informed about the potential for injection site reactions.
