Evkeeza

Evkeeza (evinacumab) is a treatment for homozygous familial hypercholesterolaemia (HoFH) in adults and pediatric patients aged 1 year and older, to reduce low-density lipoprotein-cholesterol (LDL-C) levels. Evkeeza is used alongside diet, exercise, and other LDL-C-lowering medicines.

Drug class: Evkeeza is an angiopoietin-like 3 (ANGPTL3) inhibitor.

Administration: Evkeeza is given by intravenous (IV) infusion over approximately 60 minutes, once every four weeks.

What is homozygous familial hypercholesterolaemia (HoFH)? HoFH is a rare genetic condition that causes very high levels of LDL cholesterol and increases the risk of early cardiovascular disease if left untreated.

Key information: This medicine is not a cure for HoFH but helps manage the condition and reduce cholesterol levels. This medicine is not for use in treating high cholesterol that is caused by diet or other factors.

• Evkeeza works by binding and inhibiting a protein called angiopoietin-like 3 (ANGPTL3).
• This protein slows lipid metabolism by blocking the enzymes lipoprotein lipase (LPL) and endothelial lipase (EL).
• By blocking ANGPTL3, Evkeeza restores the activity of LPL and EL enzymes that break down cholesterol and fats. This leads to lower levels of LDL cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C), and triglycerides (TG) in the blood

Evkeeza is a monoclonal antibody that binds to and inhibits the ANGPTL3 protein.

• Evkeeza is given as an infusion, through an intravenous (IV) line, into a vein, usually once per month. A healthcare provider will give you this injection.
• This medicine must be given slowly over 60 minutes.
• If you miss any infusion appointments, call your healthcare provider as soon as possible to reschedule.
• Your healthcare provider may slow down your infusion rate, temporarily stop, or permanently stop treatment with Evkeeza if you have certain side effects.
• Your healthcare provider may prescribe other cholesterol-lowering medicines to use with Evkeeza. Use the other prescribed medicines exactly as your healthcare provider tells you to.

Serious Hypersensitivity Reactions: Tell your doctor and pharmacist if you are allergic to the active ingredient evinacumab, any other medications, or any of the ingredients in this medication. Serious hypersensitivity reactions have occurred with this medicine in clinical trials. If a serious hypersensitivity reaction occurs, discontinue this medicine and treat according to standard-of-care and monitor until signs and symptoms resolve.

Embryo-Fetal Toxicity: This medicine may cause fetal harm based on animal studies. Consider obtaining a pregnancy test before initiating treatment with this medicine in people who can become pregnant. Patients who may become pregnant should use contraception during treatment and for at least 5 months following the last dosage.

You should not be treated with this medicine if you are allergic to the active ingredient evinacumab or any of the inactive ingredients.

Evkeeza may harm an unborn baby. You may need a pregnancy test to make sure you are not pregnant. Use effective birth control while using Evkeeza and for at least 5 months after your last dose.

Tell your doctor if you become pregnant or plan to become pregnant. You may need to take a pregnancy test before beginning treatment with this medicine. You should not become pregnant during your treatment with this injection. You should use effective birth control to prevent pregnancy during your treatment and for 5 months after your final dose. If you become pregnant while receiving this medicine, call your doctor immediately.

Ask a doctor if it is safe to breastfeed while using this medicine.

Common Evkeeza side effects may include:

• cold or flu symptoms such as fever, chills, body aches, stuffy nose, sneezing, sore throat;
• dizziness;
• pain in your arms or legs;
• nausea;
• or lack of energy.

Serious side effects

Get emergency medical help if you have signs of an allergic reaction to Evkeeza: hives, rash, itching; feeling light-headed; wheezing, difficult breathing; swelling of your face, lips, tongue, or throat.

Some side effects may occur during the injection. Tell your medical caregivers if you feel dizzy, nauseated, light-headed, itchy, sweaty, or have a headache, chest tightness, back pain, trouble breathing, or swelling in your face.

Your treatment may be delayed or permanently discontinued if you have certain side effects.

This is not a complete list of side effects, and others may occur. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

Usual 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 diet, exercise, and other LDL-C lowering therapies for the treatment of homozygous familial hypercholesterolemia.

Call your doctor for instructions if you miss an appointment for your Evkeeza injection.

In a medical setting, an overdose would be treated quickly.

Follow your doctor's instructions about any restrictions on food, beverages, or activity.

Other drugs may interact with evinacumab, including prescription and over-the-counter medicines, vitamins, and herbal products. Tell your doctor about all other medicines you use.

Active ingredient: evinacumab-dgnb Inactive ingredients: L-arginine hydrochloride, L-histidine, L-histidine monohydrochloride monohydrate, L-proline, polysorbate 80, and Water for Injection, USP.