EVKEEZA 150 mg/mL concentrate for infusion solution

2. What you need to know before you are given Evkeeza

You should not be given Evkeeza if:

• if you are allergic to evinacumab or any of the other ingredients of this medicine (listed in section 6).

Warnings and precautions

Talk to your doctor or nurse before you are given Evkeeza.

Be aware of serious side effects

Evkeeza can cause serious allergic reactions.

• Tell your doctor or nurse immediately if you have any symptoms of a serious allergic reaction. The symptoms are listed in ‘Serious side effects’ in section 4.

Children

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

Other medicines and Evkeeza

Tell your doctor if you are taking, have recently taken, or might take any other medicines.

Pregnancy and contraception

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

• Evkeeza may harm your unborn baby.
• Tell your doctor immediately if you become pregnant while being treated with Evkeeza.

If you can become pregnant, you must use effective contraceptive methods to prevent this.

• 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.

Talk to your doctor about the best contraceptive method for you during this time.

Breast-feeding

• If you are breast-feeding or plan to breast-feed, consult your doctor before you are given this medicine.
• It is not known if Evkeeza passes into breast milk.

Driving and using 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 feel affected, do not ride a bicycle, do not drive, or use machines, and tell your doctor (see section 4).

Evkeeza contains proline

This medicine contains 30 mg of proline in each ml. Proline may be harmful for 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 medicine unless your doctor has recommended it.

Evkeeza contains polysorbate 80

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

3. How Evkeeza is given

How much Evkeeza is given

Your doctor will calculate the amount of medicine you should be given. The amount will depend on your body weight.

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

How Evkeeza is given

Evkeeza is usually given by a doctor or nurse. It is given as an infusion into a vein (‘intravenous infusion’) over 60 minutes.

If you miss a dose of Evkeeza

If you have missed an appointment to receive Evkeeza, talk to your doctor or nurse as soon as possible.

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

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody gets them.

Reporting of side effects

Serious allergic reactions(uncommon: may affect up to 1 in 100 people)

Tell your doctor or nurse immediately if you experience any of the following symptoms of a serious allergic reaction (anaphylactic reaction). The infusion will be stopped immediately and you may need to take other medicines to control the reaction:

• swelling, mainly of the lips, tongue, or throat, which may make it difficult to swallow or breathe
• breathing problems or wheezing
• feeling dizzy or fainting
• rash, hives
• itching.

Other side effects

Talk to your doctor or nurse if you experience any of the following side effects:

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

• common cold symptoms, such as runny nose (nasopharyngitis).

Common(may affect up to 1 in 10 people)

• feeling dizzy
• sore throat or infection of the nasal passages (upper respiratory tract infection)
• feeling sick (nausea)
• stomach pain
• constipation
• back pain
• pain in hands or feet (limb pain)
• flu-like symptoms
• feeling tired or weak (asthenia)
• infusion reaction, such as itching at the infusion site.

Other side effects in children from 5 to 11 years of age

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

• feeling tired (fatigue).

Reporting of side effects

If you get any side effects, talk to your doctor or nurse. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the national reporting system listed in Appendix V. By reporting side effects, you can help provide more information on the safety of this medicine.

5. Storage of Evkeeza

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

Do not use this medicine after the expiry date which is stated on the carton and vial after EXP. The expiry date is the last day of the month stated.

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

Do not freeze. Do not shake.

Store in the original package to protect from light.

Do not use this medicine if you notice it is cloudy, has changed colour, or contains visible particles.

Do not keep any unused portion of the infusion solution for reuse. Any unused portion of the infusion solution must not be reused and must be disposed of in accordance with local requirements.

6. Contents of the pack and other information

What Evkeeza contains

• The active substance is evinacumab.

Each ml 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 ingredients are proline, arginine hydrochloride, histidine hydrochloride monohydrate, polysorbate 80, histidine, and water for injections.

Appearance and package contents

Evkeeza concentrate for solution for infusion is a clear to slightly opalescent, colourless to pale yellow solution.

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

Marketing authorisation holder

Ultragenyx Germany GmbH

Rahel-Hirsch-Str. 10

10557 Berlin

Germany

Manufacturer

Ultragenyx Netherlands B.V.

Evert van de Beekstraat 1, Unit 104

1118 CL Schiphol

Netherlands

You can request more information about this medicine from the local representative of the marketing authorisation 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: +49 30 20179810

EL

Medison Pharma Greece Single Member Societe Anonyme, EL

Tel: +30 210 0100 188

FR

Ultragenyx France SAS, FR

Tel: +33 1 85 65 37 61 or 0800 917 924 (free phone number)

Date of last revision of this leaflet:

This medicine has been authorised under ‘exceptional circumstances’. This means that due to the rarity of the disease, it has not been possible to obtain complete information on this medicine. The European Medicines Agency will review any new information on this medicine that becomes available every year, and this leaflet will be updated as necessary.

Other sources of information

Detailed information on this medicine is available on the European Medicines Agency website: http://www.ema.europa.eu

This information is intended only for healthcare professionals:

Traceability

In order to improve the traceability of biological medicinal products, the name and batch number of the product should be clearly recorded.

Instructions for use

Preparation of the solution

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

• Visually inspect the medicine for cloudiness, change in colour, or particles before administration.
• Discard the vial if the solution is cloudy or has changed colour or contains particles.
• Do not shake the vial.
• Withdraw the required volume of evinacumab from the vial or vials based on 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 by gently inverting.
• The final concentration of the diluted solution should be between 0.5 mg/ml and 20 mg/ml.
• Do not freeze or shake the solution.
• Discard any unused portion remaining in the vial.

Disposal of unused medicine and all materials that have come into contact with it should be done in accordance with local regulations.

After dilution

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

• 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 should be administered over 60 minutes through an intravenous line containing a sterile filter, in-line or add-on, of 0.2 microns to 5 microns. Do not administer evinacumab as a bolus or rapid intravenous injection.
• Do not mix other medicines with evinacumab or administer it concomitantly through the same infusion line.

The infusion rate may be slowed, interrupted, or discontinued if the patient exhibits any signs of adverse reactions, including symptoms associated with infusion.