Prospecto: information for the user

Berinert2000UI

Powder and solvent for subcutaneous injectable solution

Human C1 esterase inhibitor

1.What is Berinert and how it is used

2.What you need to know before starting to use Berinert

3.How to use Berinert

4.Possible adverse effects

5.Storage of Berinert

6. Contents of the package and additional information

What is Berinert?

Berinert is presented as powder and solvent.The prepared solution must be administered by injection under the skin.

Berinert is prepared from human plasma (the liquid part of the blood). The active ingredient is human C1 esterase inhibitor protein, obtained from plasma.

What is Berinert used for?

Berinert is used for the prevention of recurrent attacks of hereditary angioedema (HAE) in adolescent and adult patients.Hereditary angioedema is a congenital vascular system disease. It is not an allergic disease.HAEis caused by insufficient, absent, or defective synthesis of C1 esterase inhibitor, an important protein.The disease is characterized by the following symptoms:

• Sudden swelling of hands and feet,
• Sudden swelling of the face with a feeling of tightness,
• Swelling of the eyelids, lips, possible swelling of the larynx (voice organ) with respiratory difficulties,
• Swelling of the tongue,
• Colicky pain in the abdominal region.

Generally, all parts of the body can be affected.

The following sections contain information that your doctor should consider before administering Berinert to you.

Do not use Berinert:

• If you have experienced a life-threatening immediate hypersensitivity reaction, including anaphylaxis, to the C1 esterase inhibitor protein or to any of the other components of this medication (including those listed in section6).

Inform your doctor or pharmacist if you are allergic to any medication or food.

Warnings and precautions

Consult your doctor or pharmacist before starting to use Berinert,

• ifyou experience severe allergic or anaphylactic reactions(severe allergic reaction that causes severe breathing difficulties or dizziness).The administration of Berinert should be stopped immediately (for example, by interrupting the injection).
• If you have a history of blood clotting problems. Blood clots have appeared in patients treated with Berinert intravenously. The use of Berinert at very high doses in diseases other than HAE may increase the risk of blood clots. However, in the case of subcutaneous Berinert, no established relationship exists with blood clots at the dose recommended by your doctor. Consult your doctor if you have a history of heart disease or blood vessel disease, myocardial infarction, blood clots, or thick blood, a permanent catheter/device in one of your veins, or have been immobile for some time. These situations may increase the risk of developing a clot after using Berinert. Also inform your doctor about the medications you are using, as some medications, such as contraceptives or certain androgens, may increase the risk of developing a blood clot.

Your doctor will carefully weigh the benefits of treatment with Berinert against the risk of developing these complications.

Viral safety

When administering blood-derived or plasma-derived medications, certain measures must be taken to prevent the transmission of infections to patients. Such measures include:

• Careful selection of blood and plasma donors to exclude those at risk of being carriers of infectious diseases, and
• Analysis of specific virus and infection markers in individual donations and plasma mixtures.

The manufacturers of these products also include stages in the processing of blood or plasma to eliminate/inactivate viruses. Despite this, when administering blood-derived or plasma-derived medications, the possibility of transmission of infectious agents cannot be completely ruled out. This also applies to emerging or unknown viruses or other types of infections.

The measures applied are considered effective for enveloped viruses such as human immunodeficiency virus (HIV, the AIDS virus), hepatitis B virus, hepatitis C virus (liver inflammation), and non-enveloped viruses such as hepatitis A virus (liver inflammation) and parvovirus B19.

Your doctor may recommend hepatitis A and B vaccination if you are treated periodically/repeatedly with plasma-derived medications.

It is strongly recommended that each time Berinert is administered, the date of administration, the batch number, and the injected volume be recorded.

Use of Berinert with other medications

• Inform your doctor or pharmacist if you are using, have used recently, or may need to use any other medication, including over-the-counter medications.
• Berinert should not be mixed with other medications and diluents in the same syringe.

Pregnancy and breastfeeding

• If you are pregnant or breastfeeding, or if you think you may be pregnant, consult your doctor or pharmacist before using this medication.

Driving and operating machinery

Berinert does not affect your ability to drive and operate machinery.

Berinert contains sodium

This medication contains less than 23 mg of sodium (1 mmol) per vial; that is, it is essentially “sodium-free”.

Berinert is indicated for self-administration by subcutaneous injection. You or your caregiver should be trained, as necessary, on how to administer Berinert.

Dosage

The recommended dose of Berinert is 60 IU/kg of body weight.

Pediatric population

The recommended dose is the same as in adults.

Using more Berinert than you should

No cases of overdose have been described.

Reconstitution and administration

If your doctor decides that you can treat yourself at home, they will give you detailed instructions. You will be provided with a logbook where you will record each injection administered at home, which you will bring with you each time you visit your doctor. You will be regularly reviewed by your doctor or caregiver on how you administer the injections to ensure that you do it correctly over time.

General instructions

• The powder must be dissolved and extracted from the vial under aseptic conditions. Use the syringe provided with the product.
• The prepared solution must be transparent and clear to slightly opalescent.After filtering or transferring the solution (see below), it must be visually checked that the solution does not contain particles or present discoloration before administering it.
• Do not use the solution if it is visibly turbid or contains particles or residues.
• Any unused amount of the medication or any residual material must be disposed of in accordance with local regulations and following the instructions of your doctor.

Reconstitution

Before opening any vial, temper the Berinert powder and the solvent until they are at room temperature.To achieve this, you can leave the vials at room temperature for about an hour or you can hold them in your closed hands for a few minutes.Do not expose the vials to direct heat.The vials should not be heated to a temperature above the body temperature (37 °C).

Remove the protective capsules from the solvent vial and the powder vial with care.Clean the exposed rubber stoppers of both vials with an alcohol-soaked swab and let them dry.You can now transfer the solvent to the powder vial with the administration system included (Mix2Vial).Please follow the instructions below:

Transfer and administration

Administration

Self-administration (subcutaneous administration)

Your doctor will teach you how to administer Berinert safely. Once you know how to self-administer the medication, follow the instructions provided below.

Table 2. Instructions for self-administration of Berinert

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

Seek immediate medical attention

• if any adverse effect occurs or
• if you notice any adverse effect not mentioned in this prospectus.

Adverse effects with Berinert are rare.

The following adverse effects have been observed very frequently (may affect more than 1 in 10 people):

• Reactions at the injection site (redness, cold sensation, supuration, erythema, hematoma, hemorrhage, hardening, edema, pain, pruritus, urticaria, warmth).
• Nasopharyngitis (stuffy nose, sneezing, watery eyes).

The following adverse effects have been observed frequently (may affect up to 1 in 10 people):

• Reactions of hypersensitivity or allergic reactions(such as hypersensitivity, pruritus, urticaria, and rash)
• Dizziness

Reporting Adverse Effects

If you experience any type of adverse effect, consult your doctor or pharmacist, even if it is a possible adverse effect not listed in this prospectus. You can also report them directly through the Spanish System for Pharmacovigilance of Medicinal Products for Human Use:www.notificaRAM.es. By reporting adverse effects, you can contribute to providing more information on 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 label and on the carton packaging after EXP.
• Do not store at a temperature above 86 °F (30 °C).
• Do not freeze.
• Store the vial in the outer packaging to protect it from light.
• Berinert does not contain preservatives, so it is preferable to use the prepared solution immediately.
• If the prepared solution is not administered immediately, it should be used within 8 hours and should only be stored in the vial.

Composition of Berinert

The active ingredient is:

Human C1 esterase inhibitor (2,000 UI/vial; after reconstitution with 4 ml of water for injectable preparations 500 UI/ml).

For more information see the section“This information is intended solely for healthcare professionals”.

The other components are:

Glycine, sodium chloride, sodium citrate.

Solvent:water for injectable preparations.

Appearance of the product and contents of the package

Berinert is presented as a white powder and is supplied with water for injectable preparations as a solvent.

The prepared solution must be transparent and clear to slightly opalescent.

Presentation

One package contains:

1 vial with powder

1 vial with 4 ml of water for injectable preparations

1 transfer device with filter 20/20

Administration equipment (inner box):

1 disposable 5 ml syringe

1 hypodermic needle

1 subcutaneous injection equipment (butterfly)

2 alcohol wipes

1 dressing

Multiple package of 5 x 2,000 UI, including a box with 5 administration equipment sets.

Multiple package of 20 x 2,000 UI, including 4 boxes with 5 administration equipment sets.

Only some package sizes may be commercially available.

Marketing authorization holder and manufacturer

CSL Behring GmbH

Emil-von-Behring-Strasse 76

35041 Marburg

Germany

For further information about this medicinal product, please contact the local representative of the marketing authorization holder:

CSL Behring S.A.

c/ Tarragona 157, planta 18

08014 Barcelona

Spain

This medicinal product is authorized in the member states of the European Economic Areaand in the United Kingdom (Northern Ireland)under the following names:

Berinert 2000 I.E. Powder and

Solvent for the preparation of an

Injection solutionAustria

Berinert 2000 IE, powder and solvent

for solution for injectionBelgium

Berinert 2000Cyprus, Germany, Greece, Poland, Portugal

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C1-??????????????????,???????Bulgaria

Berinert 2000 IUCzech Republic, Slovakia

BerinertDenmark, Italy

Berinert 2000 IU, dry substance and solvent

for solution for injectionFinland

Berinert2000 UI, powder and solvent for

solution for injectionFrance, Luxembourg

Berinert 2000 NE per oldószer

oldatos injekcióhozHungary

Berinert 2000 IU powder and solvent

for solution for injection, preparationNorway

Berinert 2000 2000 UI, powder and solvent

for solution for injectionRomania

Berinert 2000 i.e. prašek in vehikel za

raztopino za injiciranjeSlovenia

Berinert 2000 UI powder and solvent

for subcutaneous injection solutionSpain

Berinert 2000 IE, powder and solvent

for solution for injection, preparationSweden

Berinert 2000 IU Powder and solvent

for solution for injection _____________________ United Kingdom, Malta

Last update of this leaflet:October 2021

The detailed and updated information about this medicinal product is available on the website of the Spanish Agency for Medicines and Medical Devices (AEMPS)http://www.aemps.gob.es

This information is intended solely for healthcare professionals

QUALITATIVE AND QUANTITATIVE COMPOSITION

The potency of the human C1 esterase inhibitor is expressed in International Units (UI), which is related to the current WHO standard for C1 esterase inhibitor products.