Respreeza 5.000 mg polvo y disolvente para solucion para perfusion

Label: Information for the User

Respreeza 1.000 mg powder and solvent for solution for infusion

Respreeza 4.000 mg powder and solvent for solution for infusion

Respreeza 5.000 mg powder and solvent for solution for infusion

Alpha 1 human proteinase inhibitor

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

• Keep this label, as you may need to read it again.
• If you have any questions, consult your doctor or healthcare professional.

• This medicine has been prescribed only for you, and you must not give it to other people even if they have the same symptoms as you, as it may harm them.
• If you experience any side effects, consult your doctor or healthcare professional, even if they are not listed in this label. See section 4.

What is Respreeza

This medication contains the active ingredient human alpha1 proteinase inhibitor, which is a normal component of blood and is found in the lungs, where its primary function is to protect lung tissue by limiting the action of a certain enzyme called neutrophil elastase. This can cause damage if its action is not controlled (for example, if you suffer from a deficiency of alpha1 proteinase inhibitor).

What is Respreeza used for

This medication is used in adults with a known severe deficiency of alpha1 proteinase inhibitor (a hereditary condition also known as alpha1 antitrypsin deficiency) who have developed a lung condition called emphysema.

Emphysema develops when the lack of alpha1 proteinase inhibitor affects the proper control of neutrophil elastase, which damages the tiny air sacs in the lungs through which oxygen passes into the body. As a result of this damage, the lungs do not function properly.

The regular use of this medication increases blood and lung concentrations of alpha1 proteinase inhibitor, thereby reducing the progression of emphysema.

Do not use Respreeza

Warnings and precautions

• Consult your doctor or healthcare professional before using Respreeza.

Information about allergic reactions: when to stop or slow down the infusion?

You may be allergic to human alpha1 proteinase inhibitor even if you have previously received alpha1 proteinase inhibitors and tolerated them well. In some cases, severe allergic reactions can occur. Your doctor will inform you about the signs of allergic reactions (for example, chills, redness, faster heart rate, drop in blood pressure, dizziness, rash, itching, difficulty breathing or swallowing, as well as swelling of your hands, face, or mouth) (see also section 4).

• If you notice these reactions during the infusion of your medicine, inform your doctor or healthcare professional immediately. Depending on the type and intensity of the reaction, your doctor may decide to slow down or stop the infusion and start the appropriate treatment.
• In the case of self-administration/home treatment, stop the infusion immediatelyand contact your doctor or healthcare professional.

Information about safety with respect to infections

Respreeza is made from human blood plasma (this is the liquid part of blood from which the blood cells have been removed).

Since infections can be transmitted through blood, when medicines are made from human blood or plasma, certain measures are taken to prevent these from being present in the medicine and transmitted to patients. These measures include:

The measures taken are considered effective for viruses such as human immunodeficiency virus (HIV), hepatitis A virus, hepatitis B virus, hepatitis C virus, and parvovirus B19.

However, despite these measures, when administering medicines prepared from human blood or plasma, it is not possible to completely rule out the possibility of transmitting an infection. This also applies to any unknown or emerging viruses or other types of infections.

Your doctor may recommend that you consider vaccination against hepatitis A and B if you receive regular/repeated treatment with human plasma-derived proteinase inhibitors.

• It is strongly recommended that each time you receive a dose of Respreeza, you record the name and batch number of the product to keep a record of the batches used.

Safety information regarding smoking

Since cigarette smoke is a major risk factor for the development and progression of emphysema, you are strongly advised to quit smoking and avoid passive exposure to cigarette smoke.

Children and adolescents

This medicine is not for use in children or adolescents under 18 years of age.

Use of Respreeza with other medicines

• Inform your doctor or healthcare professional if you are taking, have taken recently, or may need to take any other medicine.

Pregnancy, breastfeeding, and fertility

• If you are pregnant or breastfeeding, or think you may be pregnant or planning to become pregnant, consult your doctor or healthcare professional before using this medicine.

Since alpha1 proteinase inhibitor is a normal component of human blood, the recommended dose of this medicine is not expected to cause any harm to the developing fetus. However, since there is no information available on the safety of using Respreeza during pregnancy, if you are pregnant, this medicine should only be administered with caution.

It is not known whether Respreeza passes into breast milk. If you are breastfeeding your baby, your doctor will explain the risks and benefits of using this medicine.

There is no information available on the effects on fertility, although since alpha1 proteinase inhibitor is a normal component of human blood, it is not expected to cause adverse effects on fertility if you use Respreeza at the recommended dose.

Driving and operating machinery

You may experience dizziness after receiving this medicine. If you feel dizzy, you should not drive or operate machinery until the dizziness has passed (see section 4).

Respreeza contains sodium

Respreeza 1,000 mg powder and diluent for solution for infusion:

This medicine contains approximately 37 mg of sodium (main component of table salt/for cooking) in each vial of Respreeza 1,000 mg. This is equivalent to 1.9% of the maximum daily sodium intake recommended for an adult.

Respreeza 4,000 mg powder and diluent for solution for infusion:

This medicine contains approximately 149 mg of sodium (main component of table salt/for cooking) in each vial of Respreeza 4,000 mg. This is equivalent to 7.4% of the maximum daily sodium intake recommended for an adult.

Respreeza 5,000 mg powder and diluent for solution for infusion:

This medicine contains approximately 186 mg of sodium (main component of table salt/for cooking) in each vial of Respreeza 5,000 mg. This is equivalent to 9.3% of the maximum daily sodium intake recommended for an adult.

Your doctor or healthcare professional will take this into account if you are following a low-sodium diet.

After reconstitution, Respreeza is administered by infusion into a vein. A healthcare professional experienced in the treatment of alpha1 antitrypsin deficiency will supervise the initial infusions.

Home treatment/Self-administration

After the initial infusions, you or a person responsible for your care may also administer Respreeza, but only after receiving proper training. If your doctor decides that you are suitable for home treatment/self-administration, they will teach you about:

• • how to prepare and administer this medication (see the illustrated instructions at the end of this leaflet in “Information for healthcare professionals and patients suitable for home treatment/self-administration”)
• how to maintain the product sterile (aseptic infusion techniques)
• how to keep a daily record of treatment
• how to identify adverse effects, including signs of allergic reactions, and the measures to be taken in case they occur (see also sections 2 and 4)

Your doctor or healthcare professional will regularly review your infusion technique or that of the person responsible for your care to ensure that you are acting properly.

Dose

The amount of Respreeza you receive is based on your body weight. The recommended dose is 60 mg per kg of body weight and should be administered once a week. The infusion solution is usually administered over 15 minutes (approximately 0.08 ml of solution per kg of body weight per minute). Depending on your weight and tolerance to infusion, your doctor will determine the appropriate infusion rate for you.

If you use more Respreeza than you should

The consequences of an overdose are unknown.

• Inform your doctor or healthcare professional if you think you have used more Respreeza than you should so that appropriate measures can be taken.

If you forget to use Respreeza

• Administer the next dose immediately and continue at regular intervals following the instructions of your doctor or healthcare professional.
• Do not take a double dose to make up for the missed dose.

If you interrupt treatment with Respreeza

• Do not stop using this medication without consulting your doctor or healthcare professional first. If treatment with Respreeza is suspended, your condition may worsen.

Like all medicines, this medicine can cause side effects, although not everyone will experience them. These adverse reactions can occur even if you have previously received human alpha1 proteinase inhibitors and have tolerated them well.

Some side effects can be severe:

Rare allergic reactions (affecting up to 1 in 100 people) have been observed.

In some very rare cases (affecting up to 1 in 10,000 people), they can become severe allergic reactions even if you have not shown signs of allergy with previous infusions.

• Inform your doctor or healthcare professionalimmediatelyif you perceive any signs of allergic reactions (such as chills, redness, faster heart rate, drop in blood pressure, dizziness, rash, urticarial hives, itching, difficulty breathing or swallowing, as well as swelling of your hands, face, or mouth) during the administration of Respreeza.

Depending on the type and intensity of the reaction, your doctor or healthcare professional may decide to reduce the infusion rate or stop it completely and institute appropriate treatment for the reaction.

In the case of self-administration/home treatment, stop the infusionimmediatelyand contact your doctor or healthcare professional.

Other side effectsmay include:

Common(affecting up to 1 in 10 people)

Dizziness, headache, difficulty breathing (dyspnea), nausea.

Rare(affecting up to 1 in 100 people)

Altered sensation of touch such as burning, tingling, or numbness in your hands, arms, legs, or feet (paresthesia), redness, rashes (urticaria), scaly rash, and rash all over the body, physical weakness (asthenia), reactions at the infusion site (such as burning, stinging, pain, swelling, or redness at the infusion site (hematoma)).

Very rare(affecting up to 1 in 10,000 people)

Decreased sensation of touch such as burning, tingling, or numbness in your hands, arms, legs, or feet (hypoesthesia), excessive sweating (hyperhidrosis), itching, chest pain, chills, fever (pyrexia).

Unknown frequency(cannot be estimated from available data)

Swollen lymph nodes (masses of tissue in the shape of an oval that are located throughout the body and can be palpated, for example, in the armpits, groin, or neck), swelling of the face, eyes, and lips.

Reporting of side effects

• If you experience any type of side effect, consult your doctor or healthcare professional, even if it is a side effect that does not appear in this leaflet. You can also report them directly through the Spanish System for Pharmacovigilance of Medicines for Human Use:www.notificaRAM.es. By reporting side effects, you can contribute to providing more information on the safety of this medicine.

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 on the vial labels after EXP. The expiration date is the last day of the month indicated.

Do not store at a temperature above 25 °C. Do not freeze.

After reconstitution, the solution must be used immediately. If this is not possible, the solutions can be stored for up to 3 hours at room temperature (up to 25 °C). Do not freeze the reconstituted solution.

Composition of Respreeza

The active principle is the human alpha 1 proteinase inhibitor. A vial contains approximately 1,000 mg, 4,000 mg or 5,000 mg of the human alpha 1 proteinase inhibitor.

The other components are sodium chloride, sodium dihydrogen phosphate monohydrate and mannitol (see section 2).

Vehicle: water for injection.

Appearance of the product and contents of the pack

This medicinal product is a white to off-white powder.

After reconstitution with water for injection, the solution should be transparent, colourless or slightly yellowish and free of visible particles.

Presentations:

Contents of the pack:

Respreeza 1,000 mg powder and diluent for solution for infusion

• 1 vial for single use
• 1 vial of diluent containing 20 ml of water for injection
• 1 20/20 transfer set (Mix2Vial) for reconstitution

Respreeza 4,000 mg powder and diluent for solution for infusion

• 1 vial for single use
• 1 vial of diluent containing 76 ml of water for injection
• 1 20/20 transfer set (Mix2Vial) for reconstitution

Administration equipment (inner box):

• 1 IV infusion set
• 1 type Y infusion catheter
• 3 pre-sterilised alcohol wipes

Respreeza 5,000 mg powder and diluent for solution for infusion

• 1 vial for single use
• 1 vial of diluent containing 95 ml of water for injection
• 1 20/20 transfer set (Mix2Vial) for reconstitution

Administration equipment (inner box):

• 1 IV infusion set
• 1 type Y infusion catheter
• 3 pre-sterilised alcohol wipes

Only some pack sizes may be marketed.

Marketing authorisation holder and responsible manufacturer

CSL Behring GmbH

Emil-von-Behring-Strasse 76

D-35041 Marburg

Germany

Further information on this medicinal product can be obtained from the local representative of the marketing authorisation holder:

Last update of this leaflet: 12/2024

Further detailed information on this medicinal product is available on the website of the European Medicines Agency:http://www.ema.europa.eu.

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The following information is intended for healthcare professionals and patients who are eligible for home treatment/self-administration

General instructions

• The reconstitution must be carried out in accordance with the instructions provided below.
• The product must be reconstituted, administered and handled with caution using aseptic techniques to maintain the sterility of the product.
• Do not use the provided sterile accessories for reconstitution and administration if the packaging is open or damaged.
• The powder must be reconstituted with the diluent (water for injection).
• The total reconstitution of the powder must be completed within 5 minutes (presentation of 1,000 mg) or within 10 minutes (presentations of 4,000 mg and 5,000 mg).
• Inspect the reconstituted solution for particles and discoloration before administration.
• The reconstituted solution must be transparent, colourless or slightly yellowish and free of visible particles.

Follow the steps below for the preparation and reconstitution of Respreeza:

Administration

The reconstituted solution must be administered using an IV infusion set (supplied with the 4,000 mg and 5,000 mg presentations).

Each vial of Respreeza is for single use only.

Any unused product or residual material must be disposed of in accordance with the instructions of your doctor or healthcare professional.