Bylvay 400 micrograms hard capsules

Package leaflet: Information for the user

Introduction

Package leaflet: information for the patient

Bylvay 200 microgram hard capsules

Bylvay 400 microgram hard capsules

Bylvay 600 microgram hard capsules

Bylvay 1200 microgram hard capsules

odevixibat

This medicinal product is subject to additional monitoring, which will allow for faster identification of new safety information. You can help by reporting any side effects you may experience. Information on how to report side effects is included at the end of section 4.

Read the entire leaflet carefully before you start taking this medicine, because it contains important information for you.

• Keep this leaflet, as you may need to read it again.
• If you have any questions, consult your doctor or pharmacist.
• This medicine has been prescribed for you only, and you should not give it to others, even if they have the same symptoms as you, because it may harm them.
• If you experience any side effects, consult your doctor or pharmacist, even if they are side effects not listed in this leaflet (see section 4).

Leaflet contents

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

1. What Bylvay is and what it is used for

Bylvay contains the active substance odevixibat. Odevixibat is a medicine that increases the elimination from the body of substances called bile acids. Bile acids are components of the digestive fluid known as bile, which is produced by the liver and secreted into the intestine. Odevixibat blocks the mechanism that normally reabsorbs them from the intestine after they have performed their function. This allows them to be removed from the body through the faeces.

Bylvay is used to treat progressive familial intrahepatic cholestasis (PFIC) in patients aged 6 months and older. PFIC is a liver disease caused by the accumulation of bile acids (cholestasis) that worsens over time and is often associated with severe itching.

2. What you need to know before taking Bylvay

Do not take Bylvay

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

Warnings and precautions

Talk to your doctor or pharmacist before taking Bylvay:

• if you have been diagnosed with complete absence or lack of function of the bile acid export pump protein;
• if you have severe liver dysfunction;
• if you have reduced gastric or intestinal motility or reduced circulation of bile acids between the liver, bile, and small intestine due to medications, surgical procedures, or diseases other than PFIC, as the effect of odevixibat may be reduced in these cases.

Talk to your doctor if you experience diarrhea while taking Bylvay. It is recommended that patients with diarrhea drink enough fluids to prevent dehydration.

Elevated liver enzyme levels may be observed during treatment with Bylvay in liver function tests. Before starting Bylvay, your doctor will measure your liver function to assess how well your liver is working. Your doctor will perform regular monitoring of your liver function.

Before and during treatment, your doctor may also monitor your blood levels of vitamin A, D, and E, as well as your INR (International Normalized Ratio, which measures your risk of bleeding).

Children

The use of Bylvay is not recommended in infants under 6 months of age, as it is not known whether the medicine is safe and effective in this age group.

Taking Bylvay with other medicines

Tell your doctor or pharmacist if you are taking, have recently taken, or might need to take any other medicines.

Treatment with odevixibat may affect the absorption of fat-soluble vitamins, such as vitamins A, D, and E, and of certain medications.

Pregnancy and breastfeeding

If you are pregnant or breastfeeding, think you may be pregnant, or are planning to become pregnant, consult your doctor before using this medicine.

Bylvay is not recommended during pregnancy or in women of childbearing potential who are not using contraceptive methods.

It is not known whether odevixibat passes into breast milk and could affect the baby. Your doctor will help you decide whether it is better to stop breastfeeding or to avoid treatment with Bylvay, taking into account the benefits of breastfeeding for the baby and the benefits of Bylvay for the mother.

Driving and using machines

The effect of Bylvay on the ability to drive and use machines is negligible or none.

3. How to take Bylvay

Follow exactly the instructions for administering this medicine as given by your doctor or pharmacist. If in doubt, consult your doctor or pharmacist again.

Treatment should be initiated and supervised by a physician experienced in the treatment of progressive liver disease with reduced bile flow.

The dose of Bylvay depends on your body weight. Your doctor will calculate the correct number of capsules you should take and their concentration.

The recommended dose is

• 40 micrograms of odevixibat per kilogram of body weight once daily.
• If the medicine is not working well enough after 3 months, your doctor may increase the dose to 120 micrograms of odevixibat per kilogram of body weight (up to a maximum of 7,200 micrograms once daily).

Different doses are not recommended in adults.

Method of administration

Take the capsules once daily in the morning, with or without food.

All capsules can be swallowed whole with a glass of water, or they can be opened and the contents sprinkled onto food or into a liquid suitable for the patient's age (e.g., breast milk, infant formula, or water).

The larger capsules (200 and 600 micrograms) are designed to be opened and their contents sprinkled onto food or into a liquid, but they may also be swallowed whole. The smaller capsules (400 and 1,200 micrograms) are designed to be swallowed whole, but they may also be opened and their contents sprinkled onto food or into a liquid suitable for the patient's age.

Detailed instructions on how to open the capsules and sprinkle the contents onto food or into a liquid can be found at the end of this leaflet.

If the medicine does not improve your condition after 6 months of continuous daily treatment, your doctor will recommend an alternative treatment.

If you take more Bylvay than you should

Inform your doctor if you think you have taken too much Bylvay.

Possible symptoms of overdose include diarrhea and stomach and intestinal problems.

If you forget to take Bylvay

Do not take a double dose to make up for a missed dose. Take the next dose at the usual time.

If you stop taking Bylvay

Do not stop taking Bylvay without first talking to your doctor.

If you have any further questions 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 gets them.

Side effects may occur with the following frequencies:

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

• diarrhoea, including diarrhoea with bloody stools, soft stools
• vomiting
• abdominal pain

Common (may affect up to 1 in 10 people)

• enlargement of the liver

Reporting of side effects

If you experience any type of side effect, talk to your doctor or pharmacist, even if it is a possible side effect not listed in this leaflet. You can also report them directly through the national reporting system included in Appendix V. By reporting side effects, you can help provide more information on the safety of this medicine.

5. Storage of Bylvay

Keep this medicine out of the sight and reach of children.

Do not use this medicine after the expiry date stated on the carton and bottle after EXP. The expiry date refers to the last day of the month indicated.

Store in the original packaging to protect from light. Do not store above 25 °C.

Medicines must not be disposed of via wastewater or household waste. Ask your pharmacist how to dispose of any unused medicines and their containers. This helps protect the environment.

6. Contents of the pack and other information

Composition of Bylvay

• The active substance is odevixibat.

Each Bylvay 200 microgram hard capsule contains 200 micrograms of odevixibat (as sesquihydrate).

Each Bylvay 400 microgram hard capsule contains 400 micrograms of odevixibat (as sesquihydrate).

Each Bylvay 600 microgram hard capsule contains 600 micrograms of odevixibat (as sesquihydrate).

Each Bylvay 1 200 microgram hard capsule contains 1 200 micrograms of odevixibat (as sesquihydrate).

The other components are:

• Capsule contents

Microcrystalline cellulose

Hypromellose

Capsule coating

Bylvay 200 microgram and 600 microgram hard capsules

Hypromellose

Titanium dioxide (E171)

Yellow iron oxide (E172)

Bylvay 400 microgram and 1 200 microgram hard capsules

Hypromellose

Titanium dioxide (E171)

Yellow iron oxide (E172)

Red iron oxide (E172)

Printing ink

Shellac

Propylene glycol

Black iron oxide (E172)

Nature of the product and pack contents

Bylvay 200 microgram hard capsules:

Size 0 (21.7 mm × 7.64 mm) capsules with an opaque ivory cap and an opaque white body; printed with "A200" in black ink.

Bylvay 400 microgram hard capsules:

Size 3 (15.9 mm × 5.82 mm) capsules with an opaque orange cap and an opaque white body; printed with "A400" in black ink.

Bylvay 600 microgram hard capsules:

Size 0 (21.7 mm × 7.64 mm) capsules with opaque ivory cap and body; printed with "A600" in black ink.

Bylvay 1 200 microgram hard capsules:

Size 3 (15.9 mm × 5.82 mm) capsules with opaque orange cap and body; printed with "A1200" in black ink.

Bylvay hard capsules are supplied in a plastic bottle with a polypropylene closure, featuring a security seal and child-resistant cap. Pack size: 30 hard capsules.

Marketing Authorisation Holder

Ipsen Pharma

70 rue Balard

75015 Paris

France

Manufacturer

Almac Pharma Services Limited

Seagoe Industrial Estate

Portadown, Craigavon

County Armagh

BT63 5UA

United Kingdom (Northern Ireland)

For further information on this medicinal product, please contact the local representative of the Marketing Authorisation Holder:

Date of the most recent review of this leaflet:

This medicine has been authorised under "exceptional circumstances". This type of approval means that, due to the rarity of the disease, it has not been possible to obtain complete information about this medicine.

The European Medicines Agency will review any new information about this medicine annually, and this leaflet will be updated as necessary.

Other sources of information

Detailed information on this medicine is available on the website of the European Medicines Agency: http://www.ema.europa.eu.

There are also links to other websites about rare diseases and orphan medicinal products.

Instructions

Instructions for opening the capsules and sprinkling the contents onto food:

Step 1. Place a small amount of soft food in a bowl (2 tablespoons/30 ml of yoghurt, apple sauce, banana or carrot purée, chocolate pudding, rice pudding, or oatmeal). The food should be at room temperature or colder.

Instructions for opening the capsules and sprinkling the contents into a suitable liquid for the patient's age:

Do not administer the medicine through a baby bottle or a sippy cup, as the granules will not pass through the opening. The granules will not dissolve in liquids.

Contact your pharmacy if you do not have an appropriate oral syringe to administer the medicine at home.