Evinacumab for Homozygous Familial Hypercholesterolemia

Homozygous familial hypercholesterolemia is a rare disorder in which individuals are genetically unable to remove LDL cholesterol (bad cholesterol) from their blood. This disease The disease approximately affects 1 in 250,000 people in the United States and many are not diagnosed (people with homozygous FH are not diagnosed until adulthood, delaying proper management and treatment).

Evinacumab (EVKEEZA, an injectable prescription medicine) is a fully human monoclonal antibody that inhibits ANGPTL3 and is used along with other low-density lipoproteins (LDL) lowering medicines in people 12 years of age and older to reduce the bad cholesterol or HoFH from the blood.

You shouldn’t use evinacumab if you are allergic to evinacumab-dgnb or to any of the ingredients in evinacumab.

Before receiving EVKEEZA, tell your healthcare provider about all of your medical conditions, including if you:

  • Are pregnant or planning to become pregnant. Tell your healthcare provider if you become pregnant while using evinacumab. You should do a pregnancy test to see if you are pregnant before starting evinacumab and you should use effective birth control during treatment with evinacumab and also for 5 months straight after the last dose of evinacumab. You can talk to your healthcare provider about birth control. Tell your healthcare provider right away if you become pregnant or think that you are pregnant during treatment with rituximab.
  • If you are breastfeeding or planning to breastfeed, consult your healthcare provider. It is not known if evinacumab passes into your breast milk. You should consult your healthcare provider about the best way to feed your baby while you receive evinacumab.
  • You should also tell your healthcare provider about all the prescriptions, over-the-counter medicines, vitamins, and herbal supplements you’re planning to take.

How will I receive Evinacumab?

  • You will receive evinacumab into your veins through an intravenous (IV) line over 60 minutes.
  • It will be given once every month (4 weeks).
  • If you miss any appointments, call your healthcare provider to reschedule.
  • Your healthcare provider may temporarily stop, slow down infusion rate, or permanently stop treatment with evinacumab if you have any side effects.
  • Your healthcare provider may prescribe other cholesterol-lowering medicines, to use with evinacumab.

What are the side effects of Einacumab?

Evinacumab can cause serious side effects, including:

  • Allergic reactions include a severe reaction known as anaphylaxis. Tell your healthcare provider right away if you get any of the following symptoms: hives or rash, itching, swelling of the face (especially the lips, tongue, and throat), feeling dizzy, difficulty swallowing, shortness of breath, difficulty breathing, or wheezing.
  • Common side effects of evinacumab include common cold, flu, dizziness, pain in legs or arms, nausea, and feeling weak or decreased energy.

Tell your healthcare provider if you have any of the above side effects.

What are the ingredients in Evinacumab?

Active ingredients are evinacumab-dgnb and inactive ingredients are L-histidine monohydrochloride monohydrate L-arginine hydrochloride, L-histidine, L-proline, polysorbate 80, and Water for injection.