Evkeeza 150 mg/ml concentrado para solucion para perfusion

Prospect: information for the patient

Evkeeza 150 mg/ml concentrate for solution for infusion

evinacumab

This medicine is subject to additional monitoring, which will expedite the detection of new information about its safety. You may contribute by reporting any adverse effects you experience. The final part of section 4 includes information on how to report these adverse effects.

Read this prospect carefully before starting to use this medicine, because it contains important information for you.

• Keep this prospect, as you may need to read it again.
• If you have any questions, consult your doctor or nurse.
• If you experience adverse effects, consult your doctor or nurse, even if they are possible adverse effects not listed in this prospect. See section 4.

What is Evkeeza

Evkeeza contains the active ingredient evinacumab. It is a type of medication called a «monoclonal antibody». Monoclonal antibodies are proteins that bind to other substances in the body.

How is Evkeeza used

Evkeeza is used to treat adults and children aged 6 months and older with extremely high cholesterol caused by a condition called homozygous familial hypercholesterolemia. Evkeeza is used with a low-fat diet and other medications to reduce cholesterol levels.

Homozygous familial hypercholesterolemia is hereditary and is usually passed down from both parents.

People with this condition have extremely high levels of low-density lipoprotein (LDL) cholesterol («bad cholesterol») from birth. Such high levels can cause heart attacks, heart valve diseases, or other problems at a young age.

How Evkeeza works

Evinacumab, the active ingredient in Evkeeza, binds to a protein in the body called ANGPTL3 and blocks its effects. ANGPTL3 is involved in the control of cholesterol production, and blocking its effect reduces cholesterol production. In this way, Evkeeza can reduce LDL cholesterol levels in the blood and thus prevent problems caused by high levels of LDL cholesterol.

Do not administer Evkeeza if:

• you are allergic to evinacumab or any of the other components of this medication (listed in section 6).

Warnings and precautions

Consult with your doctor or nurse before Evkeeza is administered to you.

Be aware of severe side effects

Evkeeza may cause severe allergic reactions.

• Inform your doctor or nurse immediately if you experience any symptoms of a severe allergic reaction. Symptoms are listed in «Severe side effects» in section 4.

Children

Evkeeza is not recommended for children under 6 months of age because there is not enough information about its use in this patient group.

Other medications and Evkeeza

Inform your doctor if you are taking, have taken recently, or may need to take any other medication.

Pregnancy and contraception

If you are pregnant, think you may be pregnant, or plan to become pregnant, consult your doctor before using this medication.

• Evkeeza may harm the fetus.
• Inform your doctor immediately if you become pregnant while being treated with Evkeeza.

Use effective contraceptive methods to avoid pregnancy.

• use effective contraceptive methods while being treated with Evkeeza and
• use effective contraceptive methods for at least 5 months after the last administration of Evkeeza.

Consult with your doctor about the best contraceptive method for you during this time.

Breastfeeding

• If you are breastfeeding or plan to breastfeed, consult your doctor before Evkeeza is administered to you.
• The safety of Evkeeza in breastfeeding women is unknown.

Driving and operating machines

Evkeeza may make you feel dizzy and tired and may affect your ability to ride a bicycle, drive, or use tools or machines. If you think it affects you, do not ride a bicycle, do not drive, or use machines and inform your doctor (see section 4).

Evkeeza contains proline

This medication contains 30 mg of proline in each ml. Proline may be harmful to patients with hyperprolinemia, a rare genetic disorder in which proline accumulates in the body. If you (or your child) have hyperprolinemia, do not use this medication unless your doctor has recommended it.

Evkeeza contains polysorbate 80

This medication contains 1 mg of polysorbate 80 in each ml. Polysorbates may cause allergic reactions. Inform your doctor if you (or your child) have any known allergies.

How much Evkeeza is administered

Your doctor will calculate the amount of medication that you should receive. The amount will depend on your body weight.

• The recommended dose is 15 milligrams per kilogram of body weight.
• You will receive the medication approximately once a month.

How Evkeeza is administered

Evkeeza is usually administered by a doctor or nurse. It is administered as an intravenous infusion over 60 minutes.

What to do if a dose of Evkeeza is missed

If a scheduled appointment for Evkeeza has been missed, consult with your doctor or nurse as soon as possible.

If you have any other questions about the use of this medication, ask your doctor or nurse.

Like all medications, this medication may produce adverse effects, although not everyone will experience them.

Reporting Adverse Effects

Severe Allergic Reactions(rare: may affect up to 1 in 100 people)

Inform your doctor or nurse if you experience any of the following severe allergic reaction symptoms (anaphylaxis). The drip will stop immediately and you may need to take other medications to control the reaction:

• Swelling, mainly of the lips, tongue, or throat, making it difficult to swallow or breathe
• Respiratory problems or wheezing
• Feeling dizzy or fainting
• Hives, urticaria
• Itching.

Other Adverse Effects

Consult your doctor or nurse if you experience any of the following adverse effects:

Very Common(may affect more than 1 in 10 people)

• Common cold symptoms, such as nasal discharge (nasopharyngitis).

Common(may affect up to 1 in 10 people)

• Feeling dizzy
• Sore throat or upper respiratory tract infection (sinusitis)
• Feeling unwell (nausea)
• Abdominal pain
• Constipation
• Back pain
• Pain in the hands or feet (extremity pain)
• Flu-like symptoms
• Feeling tired or exhausted (asthenia)
• Reaction to infusion, such as itching at the site where the drip is administered.

Other Adverse Effects in Children Aged 5 to 11 Years

Very Common(may affect more than 1 in 10 people)

• Feeling tired (fatigue).

Reporting Adverse Effects

If you experience any type of adverse effect, consult your doctor or nurse, even if it is a possible adverse effect not listed in this prospectus. You can also report them directly through the national notification system included in theAppendix V. By reporting adverse effects, you can contribute to providing more information about the safety of this medication.

Keep this medication out of the sight and reach of children.

Do not use this medication after the expiration date that appears on the box and vial after EXP. The expiration date is the last day of the month indicated.

Store in refrigerator (between 2 °C and 8 °C).

Do not freeze. Do not shake.

Store in the original packaging to protect it from light.

Do not use this medication if you observe that it is cloudy, presents color changes or contains visible particles.

Do not conserve any unused portion of the infusion solution for reutilization. Any unused portion of the infusion solution should not be reutilized and should be disposed of according to local requirements.

Evkeeza Composition

• The active ingredient is evinacumab.

Each milliliter of concentrate for solution for infusion contains 150 mg of evinacumab.

Each vial contains 345 mg of evinacumab in 2.3 ml of concentrate or 1,200 mg of evinacumab in 8 ml of concentrate.

• The other components are proline, arginine hydrochloride, histidine hydrochloride monohydrate, polisorbate 80, histidine, and water for injection.

Appearance of the product and contents of the pack

Evkeeza concentrate for solution for infusion is a transparent to slightly opalescent, colorless to pale yellow solution.

It is available in glass vials of 1 vial of 2.3 ml of concentrate or 1 vial of 8 ml of concentrate.

Marketing Authorization Holder

Ultragenyx Germany GmbH

Rahel-Hirsch-Str. 10

10557 Berlin

Germany

Responsible Person

Ultragenyx Netherlands B. V.

Evert van de Beekstraat 1, Unit 104

1118 CL Schiphol

Netherlands

You can request more information about this medication by contacting the local representative of the marketing authorization holder:

BE, BG, CZ, DK, DE, EE, ES, HR, IE, IS, IT, CY, LI, LV, LT, LU, HU, MT, NL, NO, AT, PL, PT, RO, SI, SK, FI, SE

Ultragenyx Germany GmbH, DE

Tel/Tél /Teπ./Tlf/Τηλ/Puh/Sími: + 49 30 20179810

EL

Medison Pharma Greece Single Member Societe Anonyme, EL

Tel: +30 210 0100 188

FR

Ultragenyx France SAS, FR

Tél: + 33 1 85 65 37 61 ou 0800 917 924 (numéro vert)

Last update date of this leaflet:

Thismedicament has been authorized in «exceptional circumstances». This authorization modality means that due to the rarity of the disease, it has not been possible to obtain complete information about this medicament. The European Medicines Agency will review annually the new information available about this medicament and this leaflet will be updated as necessary.

Other sources of information

The detailed information about this medicament is available on the European Medicines Agency website:http://www.ema.europa.eu

This information is intended solely for healthcare professionals:

Traceability

In order to improve the traceability of biological medicines, the name and batch number of the medicament administered must be clearly recorded.

Instructions for use

Preparation of the solution

Evkeeza is supplied exclusively as a single-use vial. During preparation and reconstitution, a strictly aseptic technique must be used.

• Visually inspect the medicament to detect turbidity, color changes, or particles before administration.
• Discard the vial if the solution is turbid or has color changes or contains particles.
• Do not agitate the vial.
• Withdraw the required volume of evinacumab from the vial or vials according to the patient's body weight and transfer it to an intravenous infusion bag containing sodium chloride 9 mg/ml (0.9%) or dextrose 50 mg/ml (5%) for infusion. Mix the diluted solution gently.
• The final concentration of the diluted solution must be between 0.5 mg/ml and 20 mg/ml.
• Do not freeze or agitate the solution.
• Discard any unused portion remaining in the vial.

The disposal of unused medicament and all materials that have come into contact with it will be carried out in accordance with local regulations.

After dilution

Once prepared, administer the diluted solution immediately. If the diluted solution is not administered immediately, it can be temporarily stored:

• Refrigerated between 2 °C and 8 °C for no more than 24 hours from the time of preparation of the infusion to the end of the infusion

or

• At room temperature up to 25 °C for no more than 6 hours from the time of preparation of the infusion to the end of the infusion.

Administration

• If refrigerated, allow the solution to reach room temperature (up to 25 °C) before administration.
• Evinacumab must be administered over 60 minutes by intravenous infusion through a sterile filter, in-line or additional, of 0.2 micrometers to 5 micrometers. Do not administer evinacumab as a bolus or rapid intravenous injection.
• Do not mix other medicaments with evinacumab or administer it concomitantly through the same infusion line.

The infusion rate can be slowed, interrupted, or suspended if the patient shows any signs of adverse reactions, including symptoms associated with infusion.