Usual Adult Dose for Homozygous Familial Hypercholesterolemia

15 mg/kg via IV infusion once a month (every 4 weeks)

Comments:

• Assess LDL-C (low density lipoprotein cholesterol) when clinically appropriate; LDL-C may be measured as soon as 2 weeks after initiating therapy.
• The effects on cardiovascular morbidity and mortality have not been determined.

Use: As an adjunct to other LDL-C lowering therapies for the treatment of homozygous familial hypercholesteremia.

Usual Pediatric Dose for Homozygous Familial Hypercholesterolemia

12 years or older: 15 mg/kg via IV infusion once a month (every 4 weeks)

Comments:

• Assess LDL-C (low density lipoprotein cholesterol) when clinically appropriate; LDL-C may be measured as soon as 2 weeks after initiating therapy.
• The effects on cardiovascular morbidity and mortality have not been determined.

Use: As an adjunct to other LDL-C lowering therapies for the treatment of homozygous familial hypercholesteremia.

Renal Dose Adjustments

No adjustment recommended

Liver Dose Adjustments

No adjustment recommended

Precautions

CONTRAINDICATIONS:

• Serious hypersensitivity reaction to the active substance or any product excipients

Safety and efficacy have not been established in patients younger than 12 years.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:

• Administer by IV infusion over 60 minutes once monthly (every 4 weeks)
• Administer through an IV line with 0.2 to 5-micron filter
• Rate of infusion may be slowed, interrupted, or discontinued if signs of adverse reactions, including infusion or hypersensitivity reactions develop
• May be administered without regard to the timing of lipoprotein apheresis

MISSED DOSE: If a dose is missed, administer as soon as possible; thereafter, dose should be scheduled monthly from the date of the last dose

Storage requirements:

• Store vials in refrigerator (2C to 8C [36F to 46F]) in the original carton to protect from light; do not freeze

Reconstitution/preparation techniques:

• Do not shake vial
• Dilute in an IV infusion bag of 0.9% sodium chloride or 5% dextrose; maximum volume of infusion bag should be 250 mL; final concentration should be 0.5 mg/mL to 20 mg/mL
• Administer immediately; if not used immediately, store refrigerated (2C to 8C [36F to 46F]) for up to 24 hours OR at room temperature (up to 25C [77F]) for up to 6 hours from time of infusion to end of infusion; do not freeze diluted solution

IV compatibility:

• Compatible with 0.9% sodium chloride or 5% dextrose
• Should not be mixed or administered via the same infusion line with other medications

General:

• The safety and effectiveness of this drug have not been established in patients with other causes of hypercholesterolemia, including those with heterozygous familial hypercholesterolemia.

Monitoring:

• Assess LDL-C when clinically appropriate; may be measured as early as 2 weeks after initiation
• Monitor for hypersensitivity reactions

Patient advice:

• Patients should be instructed to read the US FDA-approved patient labeling (Patient Information).
• Females of childbearing potential should be instructed to speak to their healthcare provider if they plan on becoming pregnant; effective contraception should be used during therapy and for 5 months after the last dose.

Frequently asked questions

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