VPRIV 200 U Proszek do sporządzania roztworu do infuzji dożylnej

Treść ulotki

1. VPRIV 400 Units powder for solution for infusion
   1. What is VPRIV and what is it used for
   2. What you need to know before using VPRIV
   3. How to use VPRIV
   4. Possible side effects
   5. How to store VPRIV
   6. Contents of the pack and other information

VPRIV 400 Units powder for solution for infusion

velaglucerase alfa

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.
• 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 may be harmful.
• If you get any side effects, even those not listed in this leaflet, tell your doctor (see section 4).

Contents of this leaflet

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

1. What is VPRIV and what is it used for

VPRIV is a long-term enzyme replacement therapy (ERT) in patients affected by type 1 Gaucher disease.
Gaucher disease is a genetic disorder caused by a deficiency or malfunction of an enzyme called glucocerebrosidase. In cases of deficiency or malfunction of this enzyme,
a substance called glucocerebroside accumulates inside the body's cells, causing
the signs and symptoms of Gaucher disease.
VPRIV contains a substance called velaglucerase alfa, which is used to replace the deficient or defective enzyme, glucocerebrosidase, in patients with Gaucher disease.

2. What you need to know before using VPRIV

Do not use VPRIV

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

Warnings and precautions
Talk to your doctor before receiving VPRIV

• If you are treated with VPRIV, you may develop undesirable effects during or after the infusion (see section 4, Possible side effects). These effects are called infusion-related reactions and can manifest as a hypersensitivity reaction, with symptoms such as nausea, rash, difficulty breathing, back pain, chest discomfort (chest tightness), hives, joint pain or headache.
• In addition to hypersensitivity symptoms, infusion-related reactions may present with dizziness, high blood pressure, fatigue, fever, itching, blurred vision or vomiting. If you develop any of these symptoms, you must inform your doctor immediately.
• You may be prescribed other medicines to treat or prevent future reactions. These medicines may include: antihistamines, antipyretics or corticosteroids.

Tell your doctor if you have previously developed an infusion reaction to other ERTs for
Gaucher's disease.

Children
Do not use in children under 4 years of age as there is no experience with the use of the medicine in
this age group.

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

Pregnancy
Gaucher's disease may worsen during pregnancy and for several weeks after delivery. Women with
Gaucher's disease who are pregnant or planning to become pregnant should consult their doctor before
receiving this medicine.

Breastfeeding
It is not known whether VPRIV can pass into breast milk. If you are breastfeeding or planning to breastfeed
with breast milk, consult your doctor before receiving this medicine. Your doctor will help you decide whether
to stop breastfeeding or to stop using VPRIV, considering the benefit of breastfeeding for the baby and the
benefit of VPRIV for the mother.

Driving and using machines
VPRIV does not alter or negligibly alters the ability to drive vehicles or use machines.

VPRIV contains sodium
This medicine contains 12.15 mg of sodium (the main component of table salt) per
vial. This is equivalent to 0.6% of the maximum daily recommended intake with the diet
of an adult.

3. How to use VPRIV

This medicine must be used only under the appropriate medical supervision of a physician
competent in the therapy of Gaucher’s disease, who will also determine the dose you should take.
This medicine is administered by intravenous infusion by a doctor or nurse.
Remaining under the doctor’s supervision, VPRIV can be self-administered (by you or by the
person assisting you) after adequate training provided by the doctor and/or nurse.
Self-administration must take place in the presence of a responsible adult.

Dose
The recommended dose is 60 Units/kg to be administered every other week.
If you are currently being treated with another ERT for Gaucher’s disease and your doctor wishes to
prescribe VPRIV, you can start therapy with VPRIV at the same dose and frequency of
administration as the other ERT therapy.

Use in children and adolescents
VPRIV can be administered to children and adolescents (aged 4 to 17 years) at the same dose and
frequency as adults.

Use in elderly patients
VPRIV can be administered to elderly patients (over 65 years of age) at the same dose and
frequency as adults.

Response to treatment
The doctor will monitor the response to treatment and may modify the dose (increasing or
decreasing it) over time.
If you tolerate the infusions well that are administered in a hospital setting, the doctor or
nurse may administer VPRIV in home treatment.

Administration
VPRIV is supplied in a vial as a compact powder to be mixed with sterile water and
further diluted with sodium chloride 9 mg/ml (0.9%) infusion solution before intravenous
infusion.
Once prepared, the medicine will be administered to you by the doctor or nurse by intravenous
infusion (via an intravenous line into a vein) over 60 minutes.
For self-administration, the prescribed dose and infusion rate must not be changed
without the agreement of the treating physician.

If you use more VPRIV than you should
If you think you have used more VPRIV than you should have, contact your doctor.

If you use less VPRIV than you should
If you think you have used less VPRIV than you should have, contact your doctor.

If you forget to use VPRIV
If you have missed an infusion of VPRIV, contact your doctor.

If you stop using VPRIV
If you stop using VPRIV, contact your doctor. If you stop treatment, symptoms
may recur. If you have any doubts about the use of this medicine, contact your doctor.

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody gets them.
With a common frequency (may affect up to 1 in 10 people), patients have developed
a severe allergic reaction, with difficulty breathing, chest discomfort (chest tightness),
feeling unwell (nausea), swelling of the face, lips, tongue or throat (anaphylactic/anaphylactoid
reactions); a skin allergic reaction such as hives, severe rash or itching is also common.
If you experience such side effects, inform your doctor immediately.
Most side effects, including allergic reactions, have been observed during
the infusion or shortly after. These effects are known as infusion-related reactions.

• If a severe reaction occurs at the clinical center, the doctor will immediately stop the intravenous infusion and begin to provide you with appropriate medical treatment.
• In case of administration at home, if a severe infusion-related reaction occurs, including anaphylaxis, immediately stop the infusion, seekimmediate emergency careand contact your doctor.
• In case of severe reactions and/or loss of efficacy of the medicine, the doctor will prescribe you a blood test to check if you have developed antibodies that may affect the outcome of treatment.
• The doctor or nurse may decide to continue administering VPRIV even if you experience an infusion-related reaction. Your condition will be carefully monitored.

Other infusion-related reactions that have occurred very commonly (may affect
more than 1 in 10 people) are headache, dizziness, fever/increased body temperature, back pain,
joint pain and fatigue, as well as high blood pressure (reported with common
frequency), blurred vision and vomiting (reported with uncommon frequency). If you experience such
side effects, inform your doctor immediately.
Other side effects include:

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

• bone pain
• weakness/loss of strength
• abdominal pain

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

• increased time it takes for bleeding to stop from a cut which may lead to ease/spontaneity of bleeding/ease of bruising
• skin redness
• fast heartbeat
• development of antibodies to VPRIV (see section 2)
• decreased blood pressure

Reporting of side effects
If you get any side effects, talk to your doctor or pharmacist. You can also report side effects directly
through the national reporting system listed in Annex V. By reporting side effects you can
help provide more information on the safety of this medicine.

5. How to store VPRIV

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 package and label after
Exp. The expiry date refers to the last day of that month.
Store in a refrigerator (2°C – 8°C).
Do not freeze.
Keep the vial in the outer packaging to protect the medicine from light.
Reconstituted and diluted infusion solution:
Use immediately. Do not exceed 24 hours at 2°C – 8°C.
Do not use this medicine if the solution changes color or contains foreign
particles.
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. Contents of the pack and other information

What VPRIV contains

• The active substance is velaglucerase alfa. Each vial contains 400 Units of velaglucerase alfa. After reconstitution, each ml of solution contains 100 Units of velaglucerase alfa.
• The other ingredients are sucrose, sodium citrate dihydrate, citric acid monohydrate and polysorbate 20 (see section 2 “VPRIV contains sodium”).

Description of the appearance of VPRIV and contents of the pack
Glass vial of 20 ml containing a white to off-white powder for solution for
infusion.
Packs of 1, 5 or 25 vials.
Not all pack sizes may be marketed.

Marketing authorisation holder and manufacturer
Marketing authorisation holder
Takeda Pharmaceuticals International AG Ireland Branch
Block 2 Miesian Plaza
50-58 Baggot Street Lower
Dublin 2
D02 HW68
Ireland

Manufacturer
Takeda Pharmaceuticals International AG Ireland Branch
Block 2 Miesian Plaza
50-58 Baggot Street Lower
Dublin 2
D02 HW68
Ireland
Shire Pharmaceuticals Ireland Limited
Block 2 & 3 Miesian Plaza

• 50 – 58 Baggot Street Lower Dublin 2 Ireland

For further information on this medicinal product, contact the local representative of the marketing
authorisation holder:

België/Belgique/Belgien
Takeda Belgium NV
Tél/Tel: +32 2 464 06 11
[email protected]

Lietuva
Takeda, UAB
Tel: +370 521 09 070
[email protected]

България
Такеда България ЕООД
Тел.: +359 2 958 27 36
[email protected]

Luxembourg/Luxemburg
Takeda Belgium NV
Tél/Tel: +32 2 464 06 11
[email protected]

Česká republika
Takeda Pharmaceuticals Czech Republic s.r.o.
Tel: +420 234 722 722
[email protected]

Magyarország
Takeda Pharma Kft.
Tel.: +36 1 270 7030
[email protected]

Danmark
Takeda Pharma A/S
Tlf.: +45 46 77 10 10
[email protected]

Malta
Drugsales Ltd
Tel: +356 21419070
[email protected]

Deutschland
Takeda GmbH
Tel: +49 (0)800 825 3325
[email protected]

Nederland
Takeda Nederland B.V.
Tel: +31 20 203 5492
[email protected]

Eesti
Takeda Pharma OÜ
Tel: +372 6177 669
[email protected]

Norge
Takeda AS
Tlf: +47 800 800 30
[email protected]

Ελλάδα
Τakeda ΕΛΛΑΣ Α.Ε.
Tηλ: +30 210 6387800
[email protected]

Österreich
Takeda Pharma Ges.m.b.H.
Tel: +43 (0) 800-20 80 50
[email protected]

España
Takeda Farmacéutica España, S.A
Tel: +34 917 90 42 22
[email protected]

Polska
Takeda Pharma Sp. z o.o.
Tel.: +48223062447
[email protected]

France
Takeda France SAS
Tél: + 33 1 40 67 33 00
[email protected]

Portugal
Takeda Farmacêuticos Portugal, Lda.
Tel: + 351 21 120 1457
[email protected]

Hrvatska
Takeda Pharmaceuticals Croatia d.o.o.
Tel: +385 1 377 88 96
[email protected]

România
Takeda Pharmaceuticals SRL
Tel: +40 21 335 03 91
[email protected]

Ireland
Takeda Products Ireland Ltd
Tel: 1800 937 970
[email protected]

Slovenija
Takeda Pharmaceuticals farmacevtska družba d.o.o.
Tel: + 386 (0) 59 082 480
[email protected]

Ísland
Vistor hf.
Sími: +354 535 7000
[email protected]

Slovenská republika
Takeda Pharmaceuticals Slovakia s.r.o.
Tel: +421 (2) 20 602 600
[email protected]

Italia
Takeda Italia S.p.A.
Tel: +39 06 502601
[email protected]

Suomi/Finland
Takeda Oy
Puh/Tel: 0800 774 051
[email protected]

Κύπρος
A.POTAMITIS MEDICARE LTD
Τηλ: +357 22583333
[email protected]

Sverige
Takeda Pharma AB
Tel: 020 795 079
[email protected]

Latvija
Takeda Latvia SIA
Tel: +371 67840082
[email protected]

United Kingdom (Northern Ireland)
Takeda UK Ltd
Tel: +44 (0) 3333 000 181
[email protected]

Other sources of information
More detailed information on this medicinal product is available on the website of the European
Medicines Agency, https://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:
VPRIV is a powder for solution for infusion. It must be diluted and reconstituted and is only to be
used for intravenous infusion. VPRIV is for single use and is administered with a 0.2 or 0.22 µm filter.
Dispose of any unused solution. VPRIV must not be administered with other
medicinal products in the same infusion as compatibility in solution with other
medicinal products has not been evaluated. The total volume of the infusion must be administered over 60 minutes.
Use aseptic technique.
Prepare VPRIV as illustrated below:

• 1. Determine the number of vials to reconstitute based on the individual patient's weight and the prescribed dose.
• 2. Remove the necessary number of vials from the refrigerator. Reconstitute each vial using sterile water for injections: Vial capacity Sterile water for injections 400 Units 4.3 ml
• 3. After reconstitution, gently swirl the contents of the vials. Do not shake.
• 4. Before further diluting the reconstituted medicinal product, visually inspect the solution contained in the vials: it should appear clear or slightly opalescent and colourless. Do not use if there is a change in colour or if foreign particulate matter is present.
• 5. Withdraw the calculated volume of medicinal product from the appropriate number of vials. Some solution will remain in the vial: Vial capacity Withdrawable volume 400 Units 4.0 ml
• 6. Dilute the total required volume in 100 ml of sodium chloride 9 mg/ml (0.9%) solution for infusion. Gently swirl. Do not shake. Start the infusion within 24 hours of reconstitution.

From a microbiological point of view, use the medicinal product immediately. If not used
immediately, the storage times and conditions prior to use are the responsibility of
the user. Do not exceed 24 hours at a temperature of 2  C - 8  C.
Do not dispose of the medicinal product in the wastewater or household waste. Dispose of unused medicinal
product and waste derived from this medicinal product in accordance with local regulations.

Traceability
In order to improve the traceability of biological medicinal products, clearly record the name and
batch number of the medicinal product administered.