ELFABRIO 2 mg /Ml Concentrate For Infusion Solution

Зміст інструкції

1. Elfabrio 2 mg/mL concentrate for solution for infusion
   1. What is Elfabrio and what is it used for
   2. What you need to know before you are given Elfabrio
   3. How Elfabrio is administered
   4. Possible side effects
   5. How to store Elfabrio
   6. Package Contents and Other Information

Elfabrio 2 mg/mL concentrate for solution for infusion

pegunigalsidase alfa
Medicinal product subject to additional monitoring. This will allow the rapid identification of
new safety information. You can contribute by reporting any adverse effects
experienced during the use of this medicine. See the end of section 4 for
information on how to report adverse effects.

Read this leaflet carefully before you start using this medicine as it contains important
information for you.

• Keep this leaflet. You may need to read it again.
• If you have any questions, ask your doctor or pharmacist.
• This medicine has been prescribed for you only. Do not pass it on to others, even if their symptoms are the same as yours, as it could be harmful.
• If you get any side effects, even those not listed in this leaflet, tell your doctor or pharmacist. See section 4.

Contents of this leaflet

• 1. What Elfabrio is and what it is used for
• 2. What you need to know before Elfabrio is administered to you
• 3. How Elfabrio is administered
• 4. Possible side effects
• 5. How to store Elfabrio
• 6. Contents of the pack and other information

1. What is Elfabrio and what is it used for

Elfabrio contains the active substance pegunigalsidase alfa and is used as enzyme replacement therapy
in adult patients with confirmed Fabry disease. Fabry disease is a rare genetic disease
that can affect many parts of the body. In patients with Fabry disease, fatty substances are not
removed from the body's cells and accumulate in the walls of blood vessels, contributing to
causing organ dysfunction. These fats accumulate in the cells of patients
because they do not have sufficient amounts of an enzyme called α-galactosidase-A, which is
responsible for their breakdown. Elfabrio is used long-term to supplement or replace this
enzyme in adult patients with confirmed Fabry disease.

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

Do not use Elfabrio

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

Warnings and precautions
Talk to your doctor before using Elfabrio.
If you are treated with Elfabrio, you may experience an undesirable effect during or immediately after the
infusion used to administer the medicine (see section 4). This effect is known
as an infusion-related reactionand can sometimes be severe.

• Infusion-related reactions include dizziness, headache, nausea, low blood pressure, fatigue and fever. If you experience an infusion-related reaction, contact your doctor immediately.

• If you have an infusion-related reaction, you may be given other medicines to treat or help prevent future reactions. These include medicines used to treat allergies (antihistamines), medicines used to treat fever (antipyretics) and medicines to control inflammation (corticosteroids).
• If the infusion-related reaction is severe, your doctor will immediately stop the infusion and give you appropriate medical treatment or slow down the infusion rate.
• If the infusion-related reactions are severe and/or there is a loss of effect of the medicine, your doctor will perform a blood test to check for antibodies that may affect the outcome of treatment.
• Most of the time, you will still be able to be given Elfabrio even if you experience an infusion-related reaction.

In very rare cases, your immune system may not be able to recognize Elfabrio,
causing an immunological disease of the kidneys (membranoproliferative glomerulonephritis). During
clinical studies, only one case occurred and the only symptom reported was a temporary
reduction in kidney function with an excess of protein in the urine. The symptoms resolved with
discontinuation of treatment.

Children and adolescents
This medicine should not be used in children and adolescents. The safety and efficacy of
Elfabrio in children and adolescents aged 0 to 17 years have not yet been established.

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

Pregnancy and breastfeeding
You must not use Elfabrio if you are pregnant as there is no experience with the use of Elfabrio in pregnant
women. If you are pregnant, suspect you are pregnant or are planning to become pregnant, ask your
doctor for advice before receiving this medicine.
It is not known whether Elfabrio is excreted in breast milk. Tell your doctor if you are breastfeeding or
planning to breastfeed. Your doctor will help you decide whether to stop breastfeeding or stop
therapy with Elfabrio, taking into account the benefit of breastfeeding for the baby and the
benefit of Elfabrio for you.

Driving and using machines
Elfabrio can cause dizziness or vertigo. If you experience dizziness or vertigo on the
day of treatment with Elfabrio, do not drive vehicles and do not use machines until you feel better.

Elfabrio contains sodium
This medicine contains 48 mg of sodium (the main component of table salt) per vial.
This is equivalent to 2% of the maximum recommended daily intake of sodium with the diet of an adult.

3. How Elfabrio is administered

This medicine must be used only under the supervision of a doctor experienced in the treatment
of Fabry disease or other similar diseases and must be administered only by a healthcare
professional.
The recommended dose is 1 mg/kg of body weight, administered once every two weeks.
Your doctor may recommend treatment at home if you meet certain criteria. Contact your
doctor if you would like treatment at home.

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everyone will experience them.
Most side effects occur during or shortly after the infusion
("infusion-related reaction", see section 2 "Warnings and precautions").
During treatment with Elfabrio, you may experience one of the following reactions:

Serious side effects
Common side effects (may affect up to 1 in 10 people)

• hypersensitivity and severe allergic reaction (symptoms include excessive and prolonged contraction of the airway muscles causing difficulty breathing [bronchospasm], swelling of the face, mouth and throat, wheezing, low blood pressure, hives, difficulty swallowing, rash, shortness of breath, redness, chest discomfort, itching, sneezing and nasal congestion)

If these side effects occur, seek immediate medical attention and stop
the infusion. Your doctor will give you pharmacological treatment, if necessary.

Other side effects include
Common (may affect up to 1 in 10 people)

• infusion-related reactions
• weakness
• nausea
• rash
• abdominal pain
• dizziness
• pain
• chest pain
• headache
• muscle and joint pain
• sensations such as numbness, tingling or pinpricks (paresthesia)
• itching
• diarrhea
• vomiting
• chills
• skin redness (erythema)
• feeling of spinning (vertigo), irritability or confusion
• alteration of the normal heart rhythm
• agitation

Uncommon (may affect up to 1 in 100 people)

• tremor
• high blood pressure (hypertension)
• bronchospasm (contraction of the bronchial muscles, which causes obstruction of the airways) and difficulty breathing
• sore throat
• increased body temperature
• difficulty sleeping (insomnia)
• restless legs syndrome
• damage to the nerves of the arms and legs causing pain or numbness, burning and tingling (peripheral neuropathy)
• nerve pain (neuralgia)
• burning sensation
• redness
• gastroesophageal reflux disease (where stomach acid rises into the esophagus)
• inflammation of the stomach lining (dyspepsia)
• indigestion
• gas (flatulence)
• decreased sweating (hypohidrosis)
• immunological kidney disease that causes excess protein in the urine and kidney malfunction (membranoproliferative glomerulonephritis)
• chronic kidney disease
• excess protein in the urine (proteinuria)
• tissue damage when the medicine that is normally infused into a vein leaks out or is accidentally infused into the surrounding tissue (extravasation at the infusion site)
• swelling of the lower legs or hands (edema)
• swelling of the arms or legs
• flu-like illness
• nasal congestion and sneezing
• pain at the infusion site
• increased liver enzymes and uric acid in the blood, increased protein/creatinine ratio in urine, white blood cells in urine, highlighted by laboratory tests
• weight gain
• low blood pressure (hypotension)
• slow heart rate (bradycardia)
• thickening of the wall inside the heart ventricle

Reporting of side effects
If you get any side effects, even those not listed in this leaflet, tell your
doctor. You can also report side effects directly through the national reporting system
described in Annex V. By reporting side effects you can help provide
more information on the safety of this medicine.

5. How to store Elfabrio

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 label and the package after
“EXP” / “Scad.”. The expiry date refers to the last day of that month.
Store in a refrigerator (2 °C - 8 °C).
After dilution, the diluted solution must be used immediately. If not used
immediately, the diluted solution must be stored for no more than 24 hours in a refrigerator
(2 °C - 8 °C) or for no more than 8 hours at room temperature (below 25 °C).
Do not use this medicine if you notice particulate matter or changes in colour.
Do not dispose of any medicine in wastewater or household waste. Ask your pharmacist how to
dispose of medicines you no longer use. This will help protect the environment.

6. Package Contents and Other Information

What Elfabrio contains

• The active substance is pegunigalsidase alfa. Each vial contains 20 mg of pegunigalsidase alfa in 10 mL (2 mg/mL).
• The other ingredients are: tribasic sodium citrate dihydrate, citric acid and sodium chloride (see section 2 "Elfabrio contains sodium").

Description of the appearance of Elfabrio and contents of the pack
Transparent and colourless solution in a transparent glass vial with a rubber stopper and sealed with
a tear-off aluminium closure.
Packs: 1, 5 or 10 vials.
Not all pack sizes may be marketed.

Marketing Authorisation Holder
Chiesi Farmaceutici S.p.A.
Via Palermo 26/A
43122 Parma
Italy

Manufacturer
Chiesi Farmaceutici S.p.A.
Via San Leonardo 96
43122 Parma
Italy
For further information on this medicinal product, contact the local representative of the marketing
authorisation holder:

België/Belgique/Belgien
Chiesi sa/nv
Tél/Tel: + 32 (0)2 788 42 00

Lietuva
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

България
Chiesi Bulgaria EOOD
Тел.: +359 29201205

Luxembourg/Luxemburg
Chiesi sa/nv
Tél/Tel: + 32 (0)2 788 42 00

Česká republika
Chiesi CZ s.r.o.
Tel: + 420 261221745

Magyarország
Chiesi Hungary Kft.
Tel.: + 36-1-429 1060

Danmark
Chiesi Pharma AB
Tlf: + 46 8 753 35 20

Malta
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Deutschland
Chiesi GmbH
Tel: + 49 40 89724-0

Nederland
Chiesi Pharmaceuticals B.V.
Tel: + 31 88 501 64 00

Eesti
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

Norge
Chiesi Pharma AB
Tlf: + 46 8 753 35 20

Ελλάδα
Chiesi Hellas AEBE
Τηλ: + 30 210 6179763

Österreich
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

España
Chiesi España, S.A.U.
Tel: + 34 934948000

Polska
Chiesi Poland Sp. z.o.o.
Tel.: + 48 22 620 1421

France
Chiesi S.A.S.
Tél: + 33 1 47688899

Portugal
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Hrvatska
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

România
Chiesi Romania S.R.L.
Tel: + 40 212023642

Ireland
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Slovenija
CHIESI SLOVENIJA, d.o.o.
Tel: + 386-1-43 00 901

Ísland
Chiesi Pharma AB
Sími: +46 8 753 35 20

Slovenská republika
Chiesi Slovakia s.r.o.
Tel: + 421 259300060

Italia
Chiesi Italia S.p.A.
Tel: + 39 0521 2791

Suomi/Finland
Chiesi Pharma AB
Puh/Tel: +46 8 753 35 20

Κύπρος
Chiesi Farmaceutici S.p.A.
Τηλ: + 39 0521 2791

Sverige
Chiesi Pharma AB
Tel: +46 8 753 35 20

Latvija
Chiesi Pharmaceuticals GmbH
Tel: + 43 1 4073919

United Kingdom (Northern Ireland)
Chiesi Farmaceutici S.p.A.
Tel: + 39 0521 2791

Other sources of information
More detailed information on this medicinal product is available on the website of the European
Medicines Agency, http://www.ema.europa.eu. Links to other websites on rare diseases
and related therapeutic treatments are also provided.

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The following information is intended exclusively for healthcare professionals:
Dilution (use aseptic technique)

• 1) Determine the total number of vials needed for the infusion.

The number of vials needed is based on the total dose required for each individual patient and
requires a dose calculation based on weight.
An example of a total dose calculation for an 80 kg patient prescribed 1 mg/kg
is as follows:

• Patient weight (in kg) ÷ 2 = dose volume (in mL)
• Example: 80 kg patient ÷ 2 = 40 mL (volume to be withdrawn)
• Since 10 mL can be withdrawn from each vial, 4 vials are needed in this example.
• 2) Allow the required number of vials to reach room temperature before dilution (approximately 30 minutes).

Visually inspect the vials. Do not use them if the closure is missing or
damaged. Do not use them if particulate matter is present or if the colour has changed.
Do not shake or agitate the vials.

• 3) Withdraw from the infusion bag and discard a volume of sodium chloride 9 mg/mL (0.9%) infusion solution equal to the volume calculated in step 1.
• 4) Withdraw the required volume of Elfabrio solution from the vials and dilute it with sodium chloride 9 mg/mL (0.9%) infusion solution to a total volume based on the patient's weight, as specified in the following table.

Minimum total infusion volume for patients based on body weight
Inject the Elfabrio solution directly into the infusion bag.
DO NOT inject into the air space within the infusion bag.
Gently invert the infusion bag to mix the solution, avoiding
shaking or vigorous agitation.
The diluted solution must be administered using a low
protein-binding 0.2 μm in-line filter.