Product Information for the Patient

Bylvay 200 micrograms Hard Capsules

Bylvay 400 micrograms Hard Capsules

Bylvay 600 micrograms Hard Capsules

Bylvay 1200 micrograms Hard Capsules

odevixibat

This medicine is subject to additional monitoring, which will facilitate the detection of new information about its safety. You can contribute by reporting any adverse effects you may experience. The final part of section 4 includes information on how to report these adverse effects.

Read this leaflet carefully before starting to take this medicine, as it contains important information for you.

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

Bylvay contains the active ingredient odevixibat. Odevixibat is a medication that increases the elimination of the body of certain substances called bile acids. Bile acids are components of the digestive fluid called 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 done their work. In this way, they can be eliminated from the body through feces.

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

Do not take Bylvay

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

Warnings and precautions

Consult your doctor or pharmacist before starting to take Bylvay:

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

Consult your doctor if you experience diarrhea while taking Bylvay. Patients with diarrhea should drink enough liquid to avoid dehydration.

During treatment with Bylvay, elevated levels of liver enzymes may be observed in liver function tests. Before starting to take Bylvay, your doctor will measure your liver function to check the functioning of your liver. Your doctor will perform regular checks to monitor your liver function.

Your doctor may recommend an evaluation of your blood levels of vitamins A, D, and E and the blood clotting value known as INR before and during treatment with Bylvay.

Children

Bylvay is not recommended for use in babies under 6 months of age, as it is not known if the medicine is safe and effective in this age group.

Use of Bylvay with other medicines

Inform your doctor or pharmacist if you are using, have used recently, or may need to use any other medicine.

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

Pregnancy and breastfeeding

If you are pregnant or breastfeeding, or think you may be 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 if odevixibat can pass into breast milk and affect the baby. Your doctor will help you decide whether to stop breastfeeding or avoid treatment with Bylvay, taking into account the benefits of breastfeeding for the baby and Bylvay for the mother.

Driving and operating machines

The influence of Bylvay on the ability to drive and use machines is negligible or insignificant.

Follow exactly the administration instructions for this medication as indicated by your doctor or pharmacist. In case of doubt, consult your doctor or pharmacist again.

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

The dose of Bylvay depends on your 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 a day
• If the medication does not work sufficiently well 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 a day).

No alternative doses are recommended for adults.

Method of use

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

All capsules can be swallowed whole with a glass of water or opened and sprinkled over some food or a suitable liquid for the age (for example, breast milk, infant formula, or water).

The larger capsules, 200 and 600 micrograms, are designed to be opened and sprinkled over some food or a suitable liquid for the age, but can be swallowed whole. The smaller capsules, 400 and 1,200 micrograms, are designed to be swallowed whole, but can be opened and sprinkled over some food or a suitable liquid for the age.

You will find detailed instructions on how to open the capsules and sprinkle the content over some food or a liquid at the end of this leaflet.

If your condition does not improve 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.

The possible symptoms of an overdose are diarrhea and stomach and intestinal problems.

If you forget to take Bylvay

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

If you interrupt treatment with Bylvay

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

If you have any other questions about the use of this medication, ask your doctor or pharmacist.

Like all medications, this medication may produce adverse effects, although not everyone will experience them.

Adverse effects may appear with the following frequencies:

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

Frequent(may affect up to 1 in 10 people)

•diarrhea, including bloody stools, soft stools

•abdominal pain

•enlargement of the liver

Reporting Adverse Effects

If you experience any type of adverse effect, consult your doctor or pharmacist, even if it is a possible adverse effect not listed in this prospectus. You can also report them directly through theSpanish System for Pharmacovigilance of Medicinal Products for Human Use: https://www.notificaRAM.es.By reporting adverse effects, you can contribute to providing more information about the safety of this medication.

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

Do not use this medication after the expiration date that appears on the box and the bottle after EXP. The expiration date is the last day of the month indicated.

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

Medicines should not be disposed of through drains or in the trash. Ask your pharmacist how to dispose of the containers and medications that you no longer need. This will help protect the environment.

Composition of Bylvay

• The active ingredient is odevixibat.

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

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

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

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

The other components are:

• Hard capsule content

Microcrystalline cellulose Hypromellose

Hard capsule coating

Bylvay 200 micrograms and 600 micrograms hard capsules

Hypromellose

Titanium dioxide (E171)

Yellow iron oxide (E172)

Bylvay 400 micrograms and 1 200 micrograms hard capsules

Hypromellose

Titanium dioxide (E171)

Yellow iron oxide (E172) Red iron oxide (E172)

Printing ink

Lacquer

Propylene glycol

Black iron oxide (E172)

Appearance of the product and contents of the pack

Bylvay 200 micrograms hard capsules:

Size 0 capsules (21.7 mm × 7.64 mm) with a white opaque body and a beige opaque cap; with the inscription "A200" in black ink.

Bylvay 400 micrograms hard capsules:

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

Bylvay 600 micrograms hard capsules:

Size 0 capsules (21.7 mm × 7.64 mm) with a white opaque body and a beige opaque cap; with the inscription "A600" in black ink.

Bylvay 1 200 micrograms hard capsules:

Size 3 capsules (15.9 mm × 5.82 mm) with a white opaque body and an orange opaque cap; with the inscription "A1200" in black ink.

The hard capsules of Bylvay are packed in a plastic bottle with a polypropylene closure, with a safety seal and child-resistant. Pack size: 30 hard capsules.

Marketing authorisation holder

Ipsen Pharma

65 quai Georges Gorse

92100 Boulogne-Billancourt

France

Responsible person for manufacturing

Almac Pharma Services Limited

Seagoe Industrial EstatePortadown, Craigavon

County Armagh

BT63 5UA

United Kingdom (Northern Ireland)

For further information about this medicinal product, please contact the representative in your country of the marketing authorisation holder:

Fecha of the last revision of this leaflet:

This medicinal product has been authorised under «exceptional circumstances». This type of authorisation means that due to the rarity of this disease, it has not been possible to obtain complete information about this medicinal product.

The European Medicines Agency will review annually any new information about this medicinal product that may become available and this leaflet will be updated as necessary.

Otras fuentes de información

The detailed information about this medicinal product 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.

Instrucciones

Instrucciones para abrir las cápsulas y espolvorear el contenido sobre algún alimento:

Step1. Put a small amount of a soft food in a bowl (2cucharadas/30ml of yoghurt, apple sauce, mashed banana, chocolate pudding, rice pudding or oatmeal). The food should be at room temperature or cooler.

Instrucciones para abrir las cápsulas y espolvorear el contenido en un líquido adecuado para la edad:

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

Consult your pharmacist if you do not have an oral syringe suitable for administering the medicinal product at home.