WILATE 500 UI FVIII/500 UI VWF Powder and solvent for injectable solution

Зміст інструкції

1. Wilate “500 IU VWF/ 500 IU FVIII - powder and solvent for injectable solution”
   1. What is Wilate and what is it used for
   2. What you need to know before using Wilate
   3. How to use WILATE
   4. Possible side effects
   5. How to store WILATE
   6. Content of the pack and other information

Wilate “500 IU VWF/ 500 IU FVIII - powder and solvent for injectable solution”

Wilate “1000 IU VWF / 1000 IU FVIII - powder and solvent for injectable solution”
Human von Willebrand factor / human coagulation factor VIII

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

1. What is Wilate and what is it used for

Wilate belongs to the pharmacotherapeutic category of medicines called Coagulation Factors
and contains von Willebrand Factor (VWF) and human blood coagulation Factor VIII.
These proteins are involved together in blood coagulation.
Von Willebrand Disease
Wilate is used to treat and prevent bleeding in patients with von Willebrand disease
(VWD), which is actually a family of related diseases. Von Willebrand disease is a disorder
of blood coagulation in which bleeding may continue for longer than expected. This
is due to a lack of VWF in the blood or VWF that does not function as it should.
Hemophilia A
Wilate is used to treat and prevent bleeding in patients with Hemophilia A. This is a
condition in which bleeding may continue for longer than expected. The cause is a congenital deficiency
of factor VIII in the blood.

2. What you need to know before using Wilate

Do not use Wilate
If you are allergic (hypersensitive) to von Willebrand factor or coagulation factor VIII of human blood or to any of the other ingredients of this medicine (listed in section 6).

Warnings and precautions
Tell your doctor or pharmacist before using Wilate

• Any medicine, such as Wilate, that is prepared from human blood (containing proteins) and is injected into a vein (administered intravenously) can cause allergic reactions. Please pay attention to the first signs of allergic reactions (hypersensitivity), such as hives, skin rashes, tightness in the chest, wheezing, low blood pressure, or anaphylaxis (when some or all of the above symptoms develop rapidly and are severe). If these symptoms occur, stop administration immediately and contact your doctor.
• When medicines are made from human blood or plasma, certain standard measures are taken to prevent infections from being transmitted to patients. These measures include careful selection of blood and plasma donors to ensure that those at risk of transmitting infections are excluded, testing of each donation and plasma pools to detect the presence of viruses/infections, and inclusion of procedures in the processing of blood or plasma that can inactivate or remove viruses. Despite these measures, when medicines made from human blood or plasma are administered, the possibility of transmitting infectious agents cannot be completely excluded. This is also true for unknown or emerging viruses and other types of infections. The measures taken are considered effective against enveloped viruses such as the human immunodeficiency virus (HIV), hepatitis B virus and hepatitis C virus, and against the non-enveloped hepatitis A virus. These measures may be of limited effectiveness against non-enveloped viruses such as parvovirus B19. Infection with parvovirus B19 can be severe in pregnant women (fetal infection) and in individuals with a compromised immune system or who have certain forms of anemia (such as sickle cell anemia or abnormal red blood cell breakdown). It is strongly recommended that each time a dose of Wilate is received, the name and lot number of the product are recorded in order to maintain a record of the lots used. Your doctor may recommend considering vaccination against hepatitis A and B if you receive regular/repeated treatments with plasma-derived products containing VWF/factor VIII.

Von Willebrand Disease (VWD)

• Please see Section 4. (Von Willebrand Disease (VWD)) for adverse reactions related to the treatment of VWD.

Hemophilia A
The formation of inhibitors (antibodies) is a known complication that can occur during treatment with all factor VIII-based medicines. Inhibitors, especially at high levels, prevent the treatment from working properly, and you or your child will be carefully monitored to check for the development of these inhibitors. If Wilate does not control bleeding in you or your child, tell your doctor immediately.

• Please see Section 4. (Hemophilia A) for adverse reactions related to the treatment of Hemophilia A.

Other medicines and Wilate
Tell your doctor or pharmacist if you are taking, have recently taken or might take any other medicines.
Although no interactions with Wilate from other medicines are known, tell your doctor or pharmacist if you are taking or have recently taken any other medicines (including those available without a prescription).
It is recommended not to mix Wilate with any other medicine during injection.

Pregnancy, breastfeeding and fertility
If you are pregnant, suspect you may be pregnant, are planning to become pregnant, or are breastfeeding, ask your doctor or pharmacist for advice before using this medicine.

Wilate contains sodium
This medicine contains up to 58.7 mg of sodium (the main component of table salt) per vial in the 500 IU vial of VWF and FVIII, and up to 117.3 mg of sodium per vial in the 1000 IU vial of VWF and FVIII. This is equivalent to 2.94% and 5.87% respectively of the maximum recommended daily intake of sodium with the diet for an adult.

3. How to use WILATE

WILATE must be injected into a vein (intravenous administration), after reconstitution with
the provided solvent. Treatment must be initiated under medical supervision.

Dosage
Your doctor will inform you about your personal dosage and how often you should use Wilate.
Always use Wilate exactly as your doctor has instructed. If you have any doubts, consult your doctor or
pharmacist.

If you use more WILATE than you should
No symptoms of overdose with VWF or human factor VIII have been reported. However, the recommended
dose should not be exceeded.

If you forget to use WILATE
Do not take a double dose to make up for a missed dose.
If you have any doubts about the use of this medicine, consult your doctor or pharmacist.

4. Possible side effects

Like all medicines, Wilate can cause side effects, although not everybody gets them.
manifest.

• Even if uncommon, hypersensitivity or allergic reactions have been observed. These may include: burning and stinging pain at the infusion site, chills, flushing, headache, tingling (urticaria), low blood pressure (hypotension), fatigue (lethargy), feeling unwell (nausea), restlessness, increased heart rate (tachycardia), chest tightness, pins and needles sensation (formication), vomiting, wheezing, sudden swelling in various parts of the body (angioedema). If you experience any of the above symptoms, tell your doctor.

You must immediately stop administering Wilate and inform your doctor immediately if
you experience symptoms of angioedema, such as:
> swelling of the face, tongue or throat (pharynx)
> difficulty swallowing
> hives and difficulty breathing

• Also uncommonis the occurrence of fever.
• Abdominal pain, back pain, chest pain, cough and dizziness may also occur, but the frequency of these side effects is unknown.
• In very rare cases, hypersensitivity can lead to a severe allergic reaction called anaphylaxis (when one or all of the above symptoms develop rapidly and are intense), which can lead to shock. In case of anaphylactic shock, specific treatment is essential, according to current medical recommendations for the treatment of shock. Von Willebrand Disease (VWD)
• When using a VWF-containing product containing factor VIII for the treatment of VWD, continued treatment may cause an excessive increase in factor VIII in the blood. This may increase the risk of disturbances in your blood flow (thrombosis). If you are a patient with known clinical or laboratory risk factors, you should be monitored for the earliest signs of thrombosis. Prevention (prophylaxis) of thrombotic events must be decided by your doctor, according to current recommendations.
• Patients with VW disease (especially type 3) may develop inhibitors (neutralizing antibodies) to VWF during treatment with VWF. In these cases, very rare, inhibitors may prevent Wilate from acting appropriately. If bleeding continues, your blood should be tested for these inhibitors.

Inhibitors may increase the risk of developing severe allergic reactions (anaphylactic shock). If an allergic reaction occurs, you should undergo testing to determine
the presence of inhibitors.
If inhibitors are found in your blood, it is recommended to consult a
doctor experienced in the care of patients with coagulation disorders. In
patients with high inhibitor levels, another type of
treatment may be helpful and should be considered.

Hemophilia A

• In children not previously treated with factor VIII-based medicines, the formation of inhibitor antibodies (see section 2) may be very common (more than 1 in 10 patients); however, in patients who have received previous treatment with factor VIII (more than 150 days of treatment) the risk is uncommon (less than 1 in 100 patients). If this happens, the medicine for you or your child may stop working properly and you or your child may experience persistent bleeding. If this happens, you should contact your doctor immediately. Inhibitors may increase the risk of developing severe allergic reactions (anaphylactic shock). If you experience allergic reactions, you should undergo testing to check for the presence of inhibitors. Uncommon: affects up to 1 in 100 people Rare:affects up to 1 in 1000 people Very rare:affects up to 1 in 10,000 people. There is not enough data to recommend the use of Wilate in previously untreated patients. Experience with Wilate treatment in children under 6 years of age is limited. For information on viral safety see section 2. (Warnings and precautions).

If you get any side effects, even if not listed in this leaflet, tell your
doctor or pharmacist.
You can also report side effects directly through the national system of
https://www.aifa.gov.it/content/segnalazioni-reazioni-avverse .
By reporting side effects you can help provide more information on the safety of
this medicine.

5. How to store WILATE

Keep this medicine out of the sight and reach of children.
Store the powder and solvent vials in a refrigerator (2°C–8°C).
Do not freeze.
Keep the vials in the outer packaging to protect them from light.
Do not use Wilate after the expiry date which is stated on the label.
Wilate can be stored at room temperature (not more than 25°C) for 2 months. In this case the
product’s expiry period expires 2 months after it has been removed from the refrigerator for the first
time. Note the new expiry period on the outer packaging.
The powder must only be dissolved immediately before injection. The stability of the solution has been
demonstrated for 4 hours at room temperature. However, to avoid contamination, the solution must
be used immediately and at one time.
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. Content of the pack and other information

What Wilate contains

• The active ingredients are human von Willebrand factor and human coagulation factor VIII.
• The excipients are: sodium chloride, glycine, sucrose, sodium citrate and calcium chloride. Solvent: water for injections with 0.1% polysorbate 80.

Description of the appearance of Wilate and content of the pack
Lyophilised powder: white or pale yellow powder or friable solid.
Reconstituted solution: must be clear or slightly opalescent
Wilate is supplied as powder and solvent for injection solution.
It is presented in two packs:

• Wilate 500, 500 IU of VWF and 500 IU of FVIII, powder and solvent for injection solution, each vial contains nominally 500 IU of human vWF and 500 IU of human coagulation factor VIII. The product contains approximately 100 IU/ml of human vWF and 100 IU/ml of human coagulation factor VIII, after reconstitution with 5 ml of water for injections with 0.1% polysorbate 80 (Solvent).
• Wilate 1000, 1000 IU of VWF and 1000 IU of FVIII, powder and solvent for injection solution, each vial contains nominally 1000 IU of human vWF and 1000 IU of human coagulation factor VIII. The product contains approximately 100 IU/ml of human vWF and 100 IU/ml of human coagulation factor VIII, after reconstitution with 10 ml of water for injections with 0.1% polysorbate 80 (Solvent).

Content of the pack
1 vial of lyophilised powder
1 vial of solvent
1 pack containing the following medical devices:
1 single-use syringe
1 transfer device (Mix2Vial)
1 infusion device
2 alcohol swabs
Marketing authorisation holder and manufacturer
OCTAPHARMA ITALY S.p.A.
Via Cisanello 145 - 56124 PISA - Italy
For any further information on this medicinal product, please contact the local Representative
of the Marketing Authorisation Holder.

Manufacturers responsible for batch release
Octapharma Pharmazeutika
Produktionsges.m.b.H.
Oberlaaerstr. 235
A-1100 Vienna
Austria

This medicinal product is authorised in the Member States of the European Economic Area with the
following denominations:
Austria, Belgium, Bulgaria, Croatia, Cyprus, Czech Republic, Estonia, Germany, Hungary, Ireland,
Italy, Latvia, Lithuania, Luxembourg, Malta, Netherlands, Poland, Portugal, Romania, Slovenia,
Slovakia, Spain, United Kingdom: Wilate 500/ Wilate 1000
Finland, Norway, Sweden: Wilate
Denmark: Wilnativ
France: Eqwilate 500/ Eqwilate 1000
Instructions for home treatment

• Please read all instructions and follow them carefully!
• Do not use Wilate after the expiry date indicated on the label.
• Sterility must be maintained during the procedure described below!
• The reconstituted medicinal product must be visually inspected for the presence of particles or colour changes before administration.
• The solution in the syringe must be clear or slightly opalescent. Do not use solutions that are cloudy or with deposits.
• Use the prepared solution immediately to avoid microbial contamination.
• Use only the supplied injection set. The use of other injection/infusion devices may cause additional risks and treatment failure.

Instructions for preparing the solution:

• 1. Do not use the product immediately after removing it from the refrigerator. Allow the solvent and powder in the closed vials to reach room temperature.
• 2. Remove the tear-off caps from both vials and clean the rubber stoppers with one of the supplied alcohol swabs.
• 3. The Mix2Vial is shown in Fig. 1:

Place the solvent vial on a flat surface and hold it firmly.
Take the Mix2Vial and turn it upside down.
Place the blue part of the Mix2Vial on top of the solvent vial and press down firmly until it locks (Fig. 2 + 3).

• 4. Place the powder vial on a flat surface and hold it firmly.

Take the solvent vial attached to the Mix2Vial and turn it upside down.
Place the transparent part on top of the powder vial
and press down firmly until it locks (Fig. 4). The solvent
will automatically pass into the powder vial.

• 5. With both vials still attached, gently turn the powder vial upside down until the product is dissolved. Dissolution occurs in less than 10 minutes at room temperature. Slight foaming may occur during preparation.

Unscrew the Mix2Vial into two parts (Fig. 5).
The foam will disappear.

Dispose of the empty solvent vial with the blue part of the Mix2Vial

Instructions for injection:
As a precaution, the heart rate should be monitored before and during the injection. If a
marked increase in heart rate occurs, reduce the injection rate or interrupt the
administration for a short period.

• 1. Connect the syringe to the transparent part of the Mix2Vial. Turn the vial upside down and draw the solution into the syringe (Fig. 6).

The solution in the syringe must be clear or with slightly pearly reflections.
Once the solution has been transferred, hold the syringe plunger firmly (pointing
downwards) and remove the syringe from the Mix2Vial (Fig. 7).
Dispose of the Mix2Vial and the empty vial.

• 2. Clean the chosen injection site with one of the supplied alcohol swabs.

• 3. Attach the supplied injection needle to the syringe.
• 4. Insert the injection needle into the chosen vein. If you have used a tourniquet to make the vein more visible, the tourniquet must be loosened before starting the injection of Wilate. Blood must not flow back into the syringe due to the risk of fibrin clot formation.
• 5. Inject the solution into the vein at a slow rate, no faster than 2-3 ml per minute. If you are using more than one vial of Wilate powder for a single treatment, you can reuse the same needle and syringe. The Mix2Vial is for single use only.

Unused medicinal product and waste materials derived from such medicinal product should be disposed of in accordance
with local regulations.
Wilate must not be mixed or injected (with the same infusion set) with other medicinal products.
Use only the specially supplied infusion set.
The use of another injection/infusion device may lead to an additional risk of
treatment failure (consequence of the adsorption of VWF/factor VIII to the internal surfaces
of some infusion devices).