Introduction

Prospect: Information for the User

Firazyr 30 mg injectable solution in pre-filled syringe

Icatibant

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

Keep this prospect, as you may need to read it again.
If you have any doubts, consult your doctor or pharmacist.
This medication has been prescribed only to you and should not be given to other people even if they have the same symptoms as you, as it may harm them.
If you experience any adverse effects, consult your doctor or pharmacist, even if they do not appear in this prospect. See section 4.

1. What is Firazyr and what is it used for

Firazyr contains the active ingredient icatibant.

Firazyr is used for the treatment of symptoms of hereditary angioedema (HAE) in adults, adolescents, and children over 2 years old.

In HAE, blood concentrations of a substance called bradykinin increase, producing symptoms such as swelling, pain, nausea, and diarrhea.

Firazyr blocks the activity of bradykinin and, therefore, slows the progression of symptoms of a HAE attack.

2. What you need to know before using Firazyr

No use Firazyr

If you are allergic to icatibant or any of the components of this medication (listed in section 6).

Warnings and precautions

Consult your doctor before starting to use Firazyr:

If you suffer from angina pectoris (decreased blood flow to the heart).
If you have recently had a stroke.

The side effects related to Firazyr are similar to the symptoms of your own disease.

Consult your doctor immediately if you notice that the symptoms of the crisis worsen after Firazyr is administered.

Additionally:

You or your caregiver must learn the technique of subcutaneous injection (under the skin) before self-administering or having Firazyr administered by your caregiver.
Immediately after self-administering Firazyr or having it administered by your caregiver while experiencing a laryngeal crisis (upper airway obstruction), you must seek medical attention at a medical institution.
If your symptoms do not resolve after an injection of Firazyr self-administered or administered by your caregiver, you must consult your doctor about the administration of additional Firazyr injections. In adult patients, up to 2 additional injections may be administered within 24 hours.

Children and adolescents

Firazyr is not recommended for use in children under 2 years of age or weighing less than 12 kg, as it has not been studied in these patients.

Other medications and Firazyr

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

No interactions of Firazyr with other medications are known. If you are taking an angiotensin-converting enzyme inhibitor (ACE inhibitor) (e.g., captopril, enalapril, ramipril, quinapril, lisinopril) to reduce blood pressure or for any other reason, inform your doctor before using Firazyr.

Pregnancy and breastfeeding

If you are pregnant or breastfeeding, or think you may be pregnant, consult your doctor before starting to use Firazyr.

If you are breastfeeding, do not breastfeed your child for 12 hours after the last administration of Firazyr.

Driving and operating machinery

Do not drive or operate machinery if you feel tired or dizzy as a result of the AEH crisis or after using Firazyr.

Firazyr contains a small amount of sodium

This medication contains less than 23 milligrams (1 mmol) of sodium per syringe, making it essentially "sodium-free".

3. How to use Firazyr

Follow exactly the administration instructions of this medication as indicated by your doctor. In case of doubt, consult your doctor again.

If you have never received Firazyr before, the first dose must always be injected by medical or nursing staff. Your doctor will discharge you when they consider it safe for you to go home. After analyzing it with your doctor or nurse, and after learning the technique for subcutaneous injections (under the skin), you or the person who cares for you can administer Firazyr if you have an acute angioedema attack. It is essential to inject Firazyr subcutaneously (under the skin) as soon as you notice an acute angioedema attack. Healthcare personnel will teach you and the person who cares for you how to inject Firazyr safely, following the package insert instructions.

When and how often should you use Firazyr?

Your doctor has determined the exact dose of Firazyr and will tell you how often to use it.

Adults

The recommended dose of Firazyr is one injection (3 ml, 30 mg) administered subcutaneously (under the skin) as soon as you notice the acute angioedema attack (for example, with increased skin swelling, especially on the face and neck, or increased abdominal pain).

If you do not notice an improvement in symptoms, after six hours, seek medical advice on the administration of additional Firazyr injections. In adults, up to two additional injections can be administered within 24 hours.

You should not receive more than three injections in a 24-hour period, and if you need more than eight injections in a month, seek medical advice.

Children and adolescents aged 2 to 17 years

The recommended dose of Firazyr is one injection of 1 ml up to a maximum of 3 ml based on body weight subcutaneously (under the skin) as soon as you notice symptoms of an acute angioedema attack (for example, increased skin swelling, especially on the face and neck, or increased abdominal pain).

See the usage instructions section to see the dose you should inject.

If you are unsure about the dose you should inject, consult your doctor, pharmacist, or nurse.

If your symptoms worsen or do not improve, seek medical advice immediately.

How should Firazyr be administered?

Firazyr is administered by subcutaneous injection (under the skin). Each syringe should only be used once.

Firazyr is injected with a short needle into the fatty tissue located under the skin of the abdomen (stomach).

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

The following step-by-step instructions are intended for:

Self-administration (adults)
Administration by a caregiver or healthcare professional for adults, adolescents, or children over 2 years old (who weigh at least 12 kg).

The instructions include the following main steps:

General information

2a) Preparation of the syringe for children and adolescents (2-17 years) who weigh 65 kg or less

2b) Preparation of the syringe and needle for injection (all patients)

Preparation of the injection site
Injection of the solution
Disposal of injection materials

Step-by-step instructions for injection

General information

Clean the work surface area to be used before starting the process.

Wash your hands with water and soap

Open the tray by removing the seal

Remove the pre-loaded syringe from the tray

Loosen and remove the cap from the end of the pre-loaded syringe

Leave the pre-loaded syringe once the cap has been loosened

2a) Preparation of the syringe for children and adolescents (2-17 years) who weigh 65 kg or less:

Important information for healthcare professionals and caregivers:

When the dose is less than 30 mg (3 ml), the following equipment is needed to extract the correct dose (see information below):

Pre-loaded syringe of Firazyr (with icatibant solution)
Adapter (connector)
3 ml graduated syringe

The required injection volume in ml must be prepared in a 3 ml graduated syringe (see table below).

Table 1: Dosage regimen for children and adolescents

Body weight	Injection volume
12 kg to 25 kg	1.0 ml
26 kg to 40 kg	1.5 ml
41 kg to 50 kg	2.0 ml
51 kg to 65 kg	2.5 ml

Patients who weigh more than 65 kg will use the entire contents of the pre-loaded syringe (3 ml).

If you are unsure about the volume of solution you should extract, consult your doctor, pharmacist, or nurse.

Remove the caps from both ends of the adapter.

Avoid touching the adapter ends and syringe tips to prevent contamination.

Attach the adapter to the pre-loaded syringe.

Connect the graduated syringe to the other end of the adapter, ensuring both connections fit securely.

Transfer the icatibant solution to the graduated syringe:

To initiate the transfer of the icatibant solution, push the plunger of the pre-loaded syringe (on the left side of the image below).

If the icatibant solution does not start to transfer to the graduated syringe, pull the plunger of the graduated syringe until the icatibant solution starts to flow into the graduated syringe (see the image below).

Continue pushing the plunger of the pre-loaded syringe until the required injection volume (dose) is transferred to the graduated syringe. See Table 1 for dosage information.

If there is air in the graduated syringe:

Turn the connected syringes so that the pre-loaded syringe is on top (see the image below).

Pull the plunger of the graduated syringe to transfer the air back into the pre-loaded syringe (this step may need to be repeated several times).

Extract the required volume of icatibant solution.

Remove the pre-loaded syringe and adapter from the graduated syringe.

Dispose of the pre-loaded syringe and adapter in the sharps container for disposal of materials that can harm others if not handled correctly.

2b) Preparation of the syringe and needle for injection::
All patients (adults, adolescents, and children)

Remove the needle cap from the blister

Remove the seal from the needle cap (the needle should remain inside the cap)

Hold the syringe firmly. Carefully connect the needle to the pre-loaded syringe with the colorless solution.

Secure the syringe to the needle, still attached to the cap

Pull the syringe to remove the needle from the cap. Do not pull the plunger.

The syringe is now ready for injection

3) Preparation of the injection site

Select the injection site. The injection site should be a skin fold on the abdomen, approximately 5-10 cm (2-4 inches) below the navel, to one side or the other. The area should be at least 5 cm (2 inches) away from any scars. Do not choose an area with hematomas, inflammation, or pain.

Clean the injection site by rubbing with an alcohol wipe, and let it dry.

4) Injection of the solution

Hold the syringe between two fingers, with your thumb on the end of the plunger

Verify that there is no air in the syringe, by pushing the plunger until the first drop appears at the tip of the needle

Hold the syringe with the needle pointing at the skin at an angle of 45 to 90 degrees

Hold the syringe in one hand, and with the other, take a skin fold between your thumb and fingers, in the previously cleaned area

Hold the skin fold, bring the syringe close, and quickly insert the needle into the skin fold

Slowly push the plunger with firm pressure, until all the fluid has been injected into the skin and no liquid remains in the syringe

Push slowly, so that the process takes approximately 30 seconds

Release the skin fold and remove the needle gently

5) Disposal of injection materials

Dispose of the syringe, needle, and cap in the sharps container for disposal of materials that can harm others if not handled correctly.

4. Possible Adverse Effects

Like all medications, Firazyr may produce adverse effects, although not everyone will experience them. Almost all patients who receive Firazyr notice a reaction at the site of injection (such as skin irritation, inflammation, pain, itching, redness of the skin, and burning). These effects are usually mild and improve without the need for any additional treatment.

Very common (may affect up to 1 in 10 people) are:

Additional reactions at the site of injection (sensation of pressure, bruising, decreased sensitivity, and/or numbness, increased skin eruption with itching and heat).

Common (may affect up to 1 in 10 people) are:

Nausea

Headache

Dizziness

Fever

Itching

Rash

Redness of the skin

Abnormal liver function tests

Unknown frequency (cannot be estimated from available data):

Hives (urticaria)

Inform your doctor immediately if you notice that your symptoms worsen after receiving Firazyr.

If you experience adverse effects, consult your doctor, even if they are adverse effects that do not appear in this prospectus.

Reporting Adverse Effects

If you experience any type of adverse effect, consult your doctor or pharmacist, even if it is a possible adverse effect that does not appear 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.

5. Conservation of Firazyr

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

Do not use this medication after the expiration date that appears on the packaging after“CAD”. The expiration date is the last day of the month indicated.

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

Do not use this medication if you observe that the syringe or needle container is damaged or if you observe visible signs of deterioration; for example, if the solution is turbid, if it contains floating particles, or if the solution color has changed.

Medicines should not be thrown into the drains or trash. Ask your pharmacist how to dispose of the packaging and medicines that you no longer need. In this way, you will help protect the environment.

6. Content of the container and additional information

Composition of Firazyr

The active ingredient is icatibant. Each pre-filled syringe contains 30 milligrams of icatibant (in the form of acetate). The other components are sodium chloride, glacial acetic acid, sodium hydroxide, and water for injection.

Appearance of Firazyr and content of the container

Firazyr is presented as a transparent and colourless injectable solution in a pre-filled glass syringe of 3 ml.

The container contains a hypodermic needle.

Firazyr is available in a single pre-filled syringe with a needle or in a multiple pack of three pre-filled syringes with three needles.

Only some sizes of containers may be commercially available.

Holder of the marketing authorisation

Shire Pharmaceutical Ireland Limited

Block 2 & 3 Miesian Plaza

50 – 58 Baggot Street Lower

Dublin 2

D02 Y754, Ireland

Phone:+44(0)1256 894 959
Email:medinfoEMEA@shire.com

Last update of the leaflet:

Other sources of information

Detailed information on this medicine is available on the European Medicines Agency website:http://www.ema.europa.eu. It also provides links to other websites on rare diseases and orphan medicines.