GIVLAARI 189 mg /Ml Инъекционный Раствор

Содержание инструкции

1. Givlaari 189 mg/mL injectable solution
   1. What is Givlaari and what is it used for
   2. What you need to know before you receive Givlaari
   3. How Givlaari is administered
   4. Possible side effects
   5. How to store Givlaari
   6. Contents of the pack and other information

Givlaari 189 mg/mL injectable solution

givosiran

Read this leaflet carefully before you receive 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 nurse.
• If any side effects occur, including those not listed in this leaflet, ask your doctor or nurse. See section 4.

Contents of this leaflet

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

1. What is Givlaari and what is it used for

What is Givlaari
Givlaari contains the active substance givosiran.

What is Givlaari used for
Givlaari is used for the treatment of acute hepatic porphyria in adults and adolescents aged 12 years and above.

What is acute hepatic porphyria
Acute hepatic porphyria is a rare disease that is passed down within families. It is caused by a
defect in one of the proteins that form a molecule in the liver, called heme. Because there is a
problem in one of the proteins needed to form heme, there is a build-up of certain substances
used to produce heme, namely aminolevulinic acid (ALA) and porphobilinogen (PBG). An
excess amount of ALA and PBG can damage the nerves and cause severe painful attacks, nausea,
muscle weakness and changes in mental activity. Some people affected by acute hepatic porphyria
may also experience symptoms such as pain and nausea between attacks. Long-term complications
that can be observed in people with acute hepatic porphyria include high blood pressure, chronic
kidney and liver disease.

How Givlaari works
This medicine works by decreasing the amount of an enzyme called ALAS1 which controls the
amounts of ALA and PBG produced by the liver. By reducing the level of ALAS1, the liver produces less
ALA and PBG. This mechanism can help to reduce the effects of this disease.

2. What you need to know before you receive Givlaari

Do not use Givlaari

• if you have had a severe allergic reaction to givosiran or to any of the other ingredients of this medicine (listed in section 6).

Warnings and precautions
Talk to your doctor or nurse before you are given Givlaari.
Severe allergic reaction

• Immediately tell your doctor or nurse if you experience signs of a severe allergic reaction. The signs are listed in section 4 “Serious side effects”.
• If you should experience a severe allergic reaction, your doctor or nurse will immediately stop using the medicine and you may need to take other medicines to control the symptoms.

Liver problems
The use of this medicine may affect the liver. You will undergo blood tests to
check your liver function before starting treatment with Givlaari and periodically
during treatment. If your blood tests show abnormal results, your doctor or
nurse will decide whether to suspend or permanently discontinue treatment. In some
patients treated with this medicine, abnormal results have been observed, mainly after 3–
5 months from the start of treatment.
Kidney problems
The use of this medicine may affect the kidneys, especially if you have already been diagnosed with kidney
problems. During the use of this medicine, your doctor will check how your kidneys are functioning,
especially if you already have kidney problems.
Tests for homocysteine levels
During the intake of this medicine, blood tests may show an increase
in homocysteine, a type of amino acid, compared to levels before the start of treatment. The
doctor will check homocysteine levels in the blood before and during treatment. If your homocysteine levels are high, your doctor may prescribe therapy to reduce homocysteine levels.

Children
This medicine should not be used in children under 12 years of age, because there is no
experience with the use of the medicine in this age group.

Other medicines and Givlaari
Tell your doctor or pharmacist if you are taking, have recently taken or might take any other
medicines.
When some medicines are used, this drug may prolong or enhance their effects or
change their side effects.

Pregnancy
If you are pregnant, suspect you are pregnant or are planning to become pregnant, ask your doctor
or nurse for advice before using this medicine.

Breast-feeding
Studies in animals suggest that this medicine may pass into breast milk. If you are breast-feeding
with breast milk, ask your doctor for advice before using this medicine. The doctor will help you
decide whether to stop breast-feeding or therapy with Givlaari, taking into account the benefit
of breast-feeding for your baby and the benefit of therapy for you.

Driving and operating machinery
It is unlikely that this medicine will affect your ability to drive vehicles and operate
machinery.

Givlaari contains sodium
This medicine contains less than 1 mmol (23 mg) of sodium per mL, i.e. essentially ‘sodium-free’.

3. How Givlaari is administered

How much Givlaari is administered
Your doctor will determine the amount of medicine to be administered to you. The amount will depend on your body weight.

• The recommended dose is 2.5 milligrams per kilogram of weight.
• This medicine will be administered once a month (every 4 weeks).
• If blood tests show liver problems, your doctor may suspend or permanently discontinue treatment with Givlaari. Your doctor may consider the possibility of resuming treatment at a lower dose.

How Givlaari is administered
This medicine will be administered to you once a month by your doctor or nurse. It is
administered as an injection under the skin (subcutaneous) in the area of the stomach (abdomen) or, in some
cases, in the upper arm or thigh. Injection sites will be alternated. If the dose is
greater than 1 mL, more than one vial will be needed and multiple
subcutaneous injections may be necessary.

If you are given too much Givlaari
In the unlikely event that your doctor or nurse administers a quantity greater than
necessary (overdose), they will monitor you to check for any adverse effects.

If you do not attend your doctor for the administration of Givlaari
If you do not attend the appointment for the injection, contact your doctor or nurse as soon as
possible.
If you have any doubts about the use of this medicine, consult your doctor or nurse.

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody gets them.

Serious side effects
Serious allergic reactions (uncommon: may affect up to 1 in 100 people)
Tell your doctor or nurse immediately if you experience any of the following signs of a
serious allergic reaction (anaphylactic reaction), because the injection will need to be stopped and you
may need to take other medicines to treat the reaction:

• swelling, mainly of the lips, tongue or throat, which may make it difficult to swallow or breathe;
• difficulty breathing or wheezing;
• feeling dizzy or fainting;
• skin rash, hives;
• itching.

Other side effects
Tell your doctor or nurse if you experience any of the following side effects:

Very common:may affect more than 1 in 10 people

• nausea
• blood tests showing an increase in transaminase levels, which are liver enzymes (a sign of possible liver inflammation)
• skin rashes, including red, itchy or dry skin, eczema or hives
• blood tests showing an increase in creatinine, a substance eliminated from the body through the kidneys, or a decrease in glomerular filtration rate (signs of possible kidney disorders)
• redness, pain, itching or swelling at the injection site (injection site reaction);
• feeling tired

Common:may affect up to 1 in 10 people

• a type of allergic reaction (hypersensitivity), with symptoms such as hives, skin rash, swelling of the eyes, mouth or face, difficulty breathing, itching
• inflammation of the pancreas (pancreatitis)
• blood test showing an increase in homocysteine (a type of amino acid)

Reporting of side effects
If you get any side effects, talk to your doctor or nurse. This includes any possible side effects not listed
in this leaflet. You can also report side effects directly through the national reporting system listed in
Appendix V. By reporting side effects you can help provide more information on the safety of this medicine.

5. How to store Givlaari

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 pack and vial after
Exp. The expiry date refers to the last day of that month.
This medicine is for single use only. Once the product is opened, it must be
used immediately.
Do not store at a temperature above 25°C.
Keep the vial in the outer packaging to protect the medicine from light.
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 Givlaari contains

• The active substance is givosiran.
• Each mL of solution contains givosiran sodium equivalent to 189 mg of givosiran.
• The other components are sodium hydroxide, phosphoric acid and water for injections. See section 2 “Givlaari contains sodium”.

Description of the appearance of Givlaari and contents of the pack
This medicine is a transparent injectable solution, from colourless to yellow.
Each pack contains one vial of 1 mL injectable solution.

Marketing authorisation holder and manufacturer
Alnylam Netherlands B.V.
Antonio Vivaldistraat 150
1083 HP Amsterdam
Netherlands
For further information on this medicine, contact the local representative of the marketing
authorisation holder:

België/Belgique/Belgien
Alnylam Netherlands B.V.
Tél/Tel: 0800 81 443 (+32 234 208 71)
[email protected]

Luxembourg/Luxemburg
Alnylam Netherlands B.V.
Tél/Tel: 80085235 (+352 203 014 48)
[email protected]

България
Genesis Pharma Bulgaria EOOD
Teл.: +359 2 969 3227
[email protected]

Malta
Genesis Pharma (Cyprus) Ltd
Tel: +357 22765715
[email protected]

Česká republika
Alnylam Czech s.r.o.
Tel: 800 050 450 (+420 234 092 195)
[email protected]

Nederland
Alnylam Netherlands B.V.
Tel: 08002820025 (+31 203697861)
[email protected]

Danmark
Alnylam Sweden AB
Tlf.: 433 105 15 (+45 787 453 01)
[email protected]

Norge
Alnylam Sweden AB
Tlf: 800 544 00 (+472 1405 657)
[email protected]

Deutschland
Alnylam Germany GmbH
Tel: 08002569526 (+49 8920190112)
[email protected]

Österreich
Alnylam Austria GmbH
Tel: 0800070339 (+43 720 778 072)
[email protected]

Ελλάδα
ΓΕΝΕΣΙΣ ΦΑΡΜΑ Α.Ε
Τηλ: +30 210 87 71 500
[email protected]

Portugal
Alnylam Portugal
Tel: 707201512 (+351 707502642)
[email protected]

España
Alnylam Pharmaceuticals Spain SL
Tel: 900810212 (+34 910603753)
[email protected]

România
Genesis Biopharma Romania SRL
Tel: +40 21 403 4074
[email protected]

France
Alnylam France SAS
Tél: 0805542656 (+33 187650921)
[email protected]

Slovenija
Genesis Biopharma SL d.o.o.
Tel: +386 1 292 70 90
[email protected]

Hrvatska
Genesis Pharma Adriatic d.o.o
Tel: +385 1 5530 011
[email protected]

Suomi/Finland
Alnylam Sweden AB
Puh/Tel: 0800 417 452 (+358 942 727 020)
[email protected]

Ireland
Alnylam Netherlands B.V.
Tel: 1800 924260 (+353 818 882213)
[email protected]

Sverige
Alnylam Sweden AB
Tel: 020109162 (+46 842002641)
[email protected]

Italia
Alnylam Italy S.r.l.
Tel: 800 90 25 37 (+39 02 89 73 22 91)
[email protected]

Eesti, Ísland, Latvija, Lietuva, Magyarország,
Polska, Slovenská republika
Alnylam Netherlands B.V.
Tel/Sími: +31 20 369 7861
[email protected]

Κύπρος
Genesis Pharma (Cyprus) Ltd
Τηλ: +357 22765715
[email protected]

Other sources of information
More detailed information on this medicine is available on the website of the European
Medicines Agency, https://www.ema.europa.eu /.
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The following information is intended exclusively for healthcare professionals:

Instructions for use
For subcutaneous use only.

• Prepare the materials not included in the pack necessary for administration, namely a sterile syringe (1 mL or 3 mL), a 21 gauge (G) or larger needle, a 25 G or 27 G needle and a sharps container.
• Calculate the required volume of Givlaari based on the recommended dose in relation to body weight. If the dose is greater than 1 mL, more than one vial will be needed and multiple subcutaneous injections may be necessary. The maximum acceptable volume for a single injection is 1.5 mL.
• To withdraw Givlaari, hold the vial upright or slightly tilted and ensure that the flat end of the needle is pointing downwards.
• Withdraw the indicated volume for injection using a 21 G or larger needle.
• Divide doses requiring a volume greater than 1.5 mL equally into multiple syringes, so that each injection is approximately the same volume.
• Point the syringe and needle upwards and tap the syringe to allow any air bubbles to rise to the top. Then, gently push the plunger to expel the air bubbles from the syringe. Check that you still have the correct volume of medicine in the syringe.
• Once the dose has been prepared in the syringe for administration, replace the 21 G or larger needle with a 25 G or 27 G needle.
• Note: do not push the medicine into the 25 G or 27 G needle.
• Administer the injection into the abdomen or, if necessary, into the posterior or upper lateral aspect of the arm or thigh. Alternate injection sites. Do not administer the medicine into scar tissue or areas that are red, inflamed or swollen.
• Note: when performing a subcutaneous injection into the abdomen, avoid an area of 5.0 cm in diameter around the navel.
• Clean the area where you want to perform the injection with an alcohol swab and wait for it to dry completely.
• Be careful to use a correct injection technique. Do not inject into a vein or muscle.
• Pinch and lift the skin at the chosen injection site. Insert the needle at a right angle (90 degrees) to deliver the solution just below the skin. In patients with poor subcutaneous tissue or if the needle is longer than 2.5 cm, insert the needle at a 45-degree angle.
• Do not push the plunger while entering the skin. Once the needle is inserted into the skin, release the pinched skin and administer the dose slowly and steadily. After administering this medicine, wait at least 5 seconds before removing the needle from the skin. Gently press gauze or cotton wool on the injection site if necessary. Do not recap the needle.
• Note: do not aspirate after inserting the needle to avoid tissue damage, hematoma and bruising.
• If more than one injection is needed for a single dose of Givlaari, the injection sites must be at least 2 cm apart.
• Use the vial only once. Once the dose has been injected, dispose of any unused medicine in the vial according to local regulations.
• Use syringes, transfer needles and injection needles only once. Dispose of used syringes and needles according to local regulations.