ZAVESCA 100 mg Hard Capsule

Зміст інструкції

1. Zavesca 100 mg capsule
   1. What is Zavesca and what is it used for
   2. What you need to know before taking Zavesca
   3. How to take Zavesca
   4. Possible side effects
   5. How to store Zavesca
   6. Contents of the pack and other information

Zavesca 100 mg capsule

miglustat

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

1. What is Zavesca and what is it used for

Zavesca contains the active ingredient miglustat which belongs to a group of medicines that
influence metabolism. It is used to treat two diseases:

• Zavesca is used to treat mild to moderate type 1 Gaucher disease inadults.

Type 1 Gaucher disease is characterized by the accumulation in some cells of the immune system of a substance called glucosylceramide which is not eliminated from the body.
This accumulation can lead to enlargement of the liver and spleen, alterations of the
blood cells and bone diseases.
The treatment usually provided for type 1 Gaucher disease consists of enzyme replacement therapy. Zavesca is used only if a patient is deemed unsuitable for
treatment with enzyme replacement therapy.

• Zavesca is also used to treat progressive neurological symptoms inNiemann-Pick type C disease in adults and children

If you suffer from Niemann-Pick type C disease, fats (such as glycosfingolipids)
increase inside brain cells. This can cause disturbances of neurological functions such as: slowing of eye movements, balance, swallowing, memory and
convulsions.
Zavesca works by inhibiting an enzyme called glucosylceramide synthase which is responsible for
the first stage of the synthesis of most glycosfingolipids.

2. What you need to know before taking Zavesca

Do not take Zavesca:

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

Warnings and precautions
Talk to your doctor or pharmacist before taking Zavesca :

• if you have kidney problems;
• if you have liver problems .

Before and during treatment with Zavesca, your doctor will perform the following checks:

• an examination to check the condition of the nerves in your upper and lower limbs;
• measurement of vitamin B levels.
• monitoring of growth if you are a paediatric or adolescent patient with Niemann-Pick disease type C
• checking the number of platelets in your blood

The reason these tests are performed is that, during the intake of Zavesca, some patients
have experienced tingling or numbness in their hands and feet, or a decrease in body weight. The tests will help
your doctor determine if these effects are due to your illness or other existing conditions or to
undesirable effects of Zavesca (for more information see section 4).
If you have diarrhoea your doctor may: decide to modify your diet to reduce lactose and the consumption of
carbohydrates such as sucrose (cane sugar); decide not to have you take Zavesca with
food; decide to temporarily reduce the dose of Zavesca. In some cases your doctor may
prescribe anti-diarrhoeal medicines such as loperamide. Cases of Crohn's disease (an inflammatory
disease affecting the intestine) have been reported in patients with Niemann-
Pick disease type C treated with Zavesca. If diarrhoea does not respond to the measures
above or if you have any other abdominal discomfort, consult your doctor. In this case, the doctor may
decide to carry out further investigations to determine if your symptoms may be due
to another cause.
Male patients will need to use reliable contraceptive methods during treatment
with Zavesca and for 3 months after its completion.

Children and adolescents
Do not give this medicine to children and adolescents (under 18 years of age) with type 1 Gaucher's disease
because it is not known whether the medicine works in this disease.

Other medicines and Zavesca
Tell your doctor or pharmacist if you are taking, have recently taken or might take
any other medicines.
Tell your doctor if you are taking other medicines containing imiglucerase, which are sometimes used
concurrently with Zavesca. They may decrease your body's exposure to Zavesca.

Pregnancy, breastfeeding and fertility
If you are pregnant, suspect you are pregnant or are planning a pregnancy, you should not take
Zavesca. Your doctor will be able to provide you with further information. While taking Zavesca
you should use an effective method of contraception. Do not breastfeed while
taking Zavesca.
Male patients must continue to use reliable contraceptive methods during the intake
of Zavesca, and for 3 months after the end of treatment.
If you are pregnant, suspect you are pregnant or are planning a pregnancy or if you are breastfeeding
ask your doctor or pharmacist for advice before taking this medicine.

Driving and using machines
Zavesca can cause dizziness. If you experience dizziness, do not drive vehicles or use tools or
machinery.

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

3. How to take Zavesca

Take this medicine following exactly the instructions of your doctor. If you have any doubts, ask your
doctor or pharmacist.

• For Type 1 Gaucher disease: For adults, the usual dose is one capsule (100 mg) three times a day (morning, afternoon and evening), equivalent to a maximum of three capsules a day (300 mg).
• For Type C Niemann-Pick disease: For adults and adolescents (over 12 years of age) the usual dose is 2 capsules (200 mg) 3 times a day (morning, afternoon and evening). This means a maximum daily dose of six capsules (600 mg).

For children under 12 years of age,the doctor will appropriately adjust the dose of medication
to be used for Type C Niemann-Pick disease.
If you have kidney problems you may receive a lower dose at the beginning of treatment. Your doctor
may decide to reduce the dose; for example: one capsule (100 mg) one or two times a day if you
suffer from diarrhoea while taking Zavesca (see section 4). The doctor will decide and communicate
how long the treatment should last.

To remove the capsule:

• 1. Tear along the perforated line
• 2. Remove the paper at the arrows
• 3. Press the product making it come out of the foil

Zavesca can be taken on an empty stomach or with food. The capsule must be swallowed whole with a
glass of water.

If you take more Zavesca than you should
If you take more capsules than you should, consult your doctor immediately. Zavesca has been used in
clinical studies at doses up to 3000 mg: this caused decreases in the amount of white blood cells and
other undesirable effects similar to those described in section 4.

If you forget to take Zavesca
Take the next capsule at the usual time. Do not take a double dose to make up for the
forgotten dose.

If you stop taking Zavesca
Do not stop taking Zavesca without talking to your doctor.
If you have any doubts about the use of this medicine, ask your doctor or pharmacist.

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everyone will experience them.
Serious side effects

Some patients have experienced tingling or numbness in the hands and feet (commonly observed).These could be signs of peripheral neuropathy, due to side effects of Zavesca or they may be due to pre-existing conditions. To evaluate them, the doctor will perform some tests before and during treatment with Zavesca (see section 2).

If you experience any of these effects, please seek medical advice from your doctor as soon as possible.
If you experience a slight tremor, usually tremor in the hands, seek medical advice from your doctoras soon as possible. The tremor often disappears without having to stop treatment. Sometimes your doctor will need to reduce the dose or stop treatment with Zavesca to make the tremor disappear.

Very common:( may affect more than 1 in 10 people)
The most common side effects include: diarrhea, flatulence (excess intestinal gas), abdominal (stomach) pain, weight loss and decreased appetite.

If you lose weightwhen you start treatment with Zavesca, do not worry. Weight loss usually stops as treatment progresses.

Common:( may affect up to 1 in 10 people)
Common side effects of treatment include headache, dizziness, paresthesia (tingling or numbness), abnormal coordination, hypoesthesia (decreased sensitivity to touch), dyspepsia (heartburn), nausea (feeling unwell), constipation and vomiting, abdominal (gastric) swelling or discomfort, thrombocytopenia (reduced levels of platelets in the blood).
Neurological symptoms and thrombocytopenia may be due to the underlying disease.
Other possible side effects are: muscle weakness or spasms, fatigue, chills and malaise, depression, difficulty sleeping, memory loss and decreased libido.
Most patients complain of one or more of these side effects usually at the beginning of treatment or at intervals during treatment. These cases are mostly mild and disappear very quickly. If any of these side effects cause discomfort, consult your doctor. They may reduce the dose of Zavesca or recommend other medicines to counteract the side effects.

Reporting of side effects
If you experience any side effect, including those not listed in this leaflet, please tell your doctor or pharmacist. You can also report side effects directly through the national reporting system detailed in Annex V. By reporting side effects you can help provide more information on the safety of this medicine.

5. How to store Zavesca

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 after the words
“Exp.”. The expiry date refers to the last day of that month.
Do not store at a temperature above 30  C.
Do not dispose of any medicine in waste water 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 Zavesca contains
The active ingredientis miglustat 100 mg.

The other ingredients are:
Sodium starch glycolate,
Povidone (K30),
Magnesium stearate.
Gelatin,
Titanium dioxide (E171).
Black iron oxide (E172),
Lacquer.

Description of the appearance of Zavesca and contents of the pack
Zavesca is a white 100 mg capsule with the inscription in black “OGT 918” on the cap and the inscription “100”
in black on one side.
One pack contains 4 blisters; each blister contains 21 capsules with a total of 84 capsules per
pack.

Marketing Authorisation Holder
Janssen-Cilag International NV
Turnhoutseweg 30
B-2340 Beerse
Belgium

Manufacturer:
Janssen Pharmaceutica NV
Turnhoutseweg 30
B-2340 Beerse
Belgium
For further information on this medicinal product, contact the local representative of the marketing
authorisation holder.

België/Belgique/Belgien
Janssen-Cilag NV
Tel/Tél: +32 14 64 94 11
[email protected]

Lietuva
UAB “JOHNSON & JOHNSON”
Tel: +370 5 278 68 88
[email protected]
l

България
„Джонсън & Джонсън България” ЕООД
Тел.: +359 2 489 94 00

Luxembourg/Luxemburg
Janssen-Cilag NV
Tél/Tel: +32 14 64 94 11
[email protected] [email protected]

Česká republika
Janssen-Cilag s.r.o.
Tel: +420 227 012 227

Magyarország
Janssen-Cilag Kft.
Tel.: +36 1 884 2858
[email protected]

Danmark
Janssen-Cilag A/S
Tlf.: +45 4594 8282
[email protected]

Malta
AM MANGION LTD
Tel: +356 2397 6000

Deutschland
Janssen-Cilag GmbH
Tel: 0800 086 9247 / +49 2137 955 6955
[email protected]

Nederland
Janssen-Cilag B.V.
Tel: +31 76 711 1111
[email protected]

Eesti
UAB “JOHNSON & JOHNSON” Eesti filiaal
Tel: +372 617 7410
[email protected]
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Norge
Janssen-Cilag AS
Tlf: +47 24 12 65 00
[email protected]

Ελλάδα
Janssen-Cilag Φαρμακευτική Μονοπρόσωπη
Α.Ε.Β.Ε.
Tηλ: +30 210 80 90 000
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Österreich
Janssen-Cilag Pharma GmbH
Tel: +43 1 610 300
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España
Janssen-Cilag, S.A.
Tel: +34 91 722 81 00
[email protected]

Polska
Janssen-Cilag Polska Sp. z o.o.
Tel.: +48 22 237 60 00

France
Janssen-Cilag
Tél: 0 800 25 50 75/+33 1 55 00 40 03
[email protected]

Portugal
Janssen-Cilag Farmacêutica, Lda.
Tel: +351 214 368 600

Hrvatska
Johnson & Johnson S.E. d.o.o.
Tel: +385 1 6610 700
[email protected]

România
Johnson & Johnson România SRL
Tel: +40 21 207 1800

Ireland
Janssen Sciences Ireland UC
Tel: 1 800 709 122
[email protected]

Slovenija
Johnson & Johnson d.o.o.
Tel: +386 1 401 18 00
[email protected]

Ísland
Janssen-Cilag AB
c/o Vistor hf.
Sími: +354 535 7000
[email protected]

Slovenská republika
Johnson & Johnson, s.r.o.
Tel: +421 232 408 400

Italia
Janssen-Cilag SpA
Tel: 800.688.777/+39 02 2510 1
[email protected]

Suomi/Finland
Janssen-Cilag Oy
Puh/Tel: +358 207 531 300
[email protected]

Κύπρος
Βαρνάβας Χατζηπαναγής Λτδ
Τηλ: +357 22 207 700

Sverige
Janssen-Cilag AB
Tfn: +46 8 626 50 00
[email protected]

Latvija
UAB “JOHNSON & JOHNSON” filiāle Latvijā
Tel: +371 678 93561
[email protected]

United Kingdom (Northern Ireland)
Janssen Sciences Ireland UC
Tel: +44 1 494 567 444
[email protected]
More detailed information on this medicinal product is available on the website of the European Medicines
Agency: https://www.ema.europa.eu. Additionally, links to other websites on rare diseases and related
therapeutic treatments are provided.