Kayfanda

Package leaflet: Information for the patient

KAYFANDA 200 microgram capsules, 400 microgram capsules, 600 microgram capsules, 1 200 microgram capsules

odevixibat
This medicinal product is under additional monitoring. This will allow for rapid identification of new safety information. You can help by reporting any side effects you experience while taking this medicine (see the end of section 4 for information on how to report side effects).
Please read all of this leaflet carefully before 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 give it to other people, even if their symptoms are the same as yours, as it may be harmful.
• If you experience any side effect, including those not listed in this leaflet, talk to your doctor or pharmacist (see section 4).

Contents of this leaflet

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

1. What KAYFANDA is and what it is used for

KAYFANDA contains the active substance odevixibat. This medicine helps remove bile acids present in the digestive fluid called bile, a fluid produced by the liver that helps digest fats in the intestine. After aiding digestion, bile acids are reabsorbed by the intestine and return to the liver.

What KAYFANDA is used for
KAYFANDA is used to treat pruritus caused by accumulation of bile acids in patients aged 6 months and older with Alagille syndrome (ALGS).
Alagille syndrome is a rare genetic disease that can affect several parts of the body, including the liver, heart, eyes, face, skeleton, blood vessels, and kidneys. Patients with this syndrome have reduced bile flow, leading to accumulation of bile acids in the blood and liver (cholestasis), which worsens over time and is often accompanied by severe pruritus.

How KAYFANDA (odevixibat) works
The active substance in KAYFANDA, odevixibat, reduces the intestinal absorption of bile acids. This allows bile acids to be excreted from the body in the faeces, preventing their accumulation in the liver. In this way, odevixibat reduces the amount of bile acids present in the blood.

2. What you need to know before taking KAYFANDA

Do not take KAYFANDA

• 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 KAYFANDA if:

• you have severely reduced liver function;
• you have reduced stomach or intestinal function, or reduced bile acid flow between the liver, gallbladder, and small intestine due to taking medicines, surgery, or diseases other than ALGS. These conditions may reduce the effect of odevixibat.

Contact your doctor if you develop diarrhea while taking KAYFANDA. If diarrhea occurs, drink plenty of fluids to prevent dehydration.
During treatment with KAYFANDA, an increase in liver enzyme levels may be observed in liver function tests. Your doctor will check your liver function before and during treatment with KAYFANDA. Your doctor should recommend more frequent monitoring if elevated liver function test results occur.
Before and during treatment with KAYFANDA, your doctor should also monitor your blood levels of vitamin A, D, and E, as well as your INR (International Normalized Ratio, which measures bleeding risk).

Children
KAYFANDA is not recommended for children under 6 months of age, as it is unknown whether the medicine is safe and effective in this age group.

Other medicines and KAYFANDA
Tell your doctor or pharmacist if you are taking, have recently taken, or might take any other medicines.
Treatment with odevixibat may affect the absorption of fat-soluble vitamins, such as vitamins A, D, and E.

Pregnancy and breastfeeding
If you are pregnant, think you might be pregnant, planning to become pregnant, or are breastfeeding, consult your doctor before taking this medicine.
The use of KAYFANDA is not recommended during pregnancy and in women of childbearing potential who are not using contraceptive measures.
It is unknown whether odevixibat passes into breast milk and could affect the breastfed infant. Your doctor will help you decide whether to discontinue breastfeeding or avoid treatment with odevixibat, taking into account the benefits of breastfeeding for the infant and of odevixibat treatment for the mother.

Driving and using machines
KAYFANDA does not alter or alters negligibly the ability to drive and use machines.

3. How to take KAYFANDA

Take this medicine exactly as instructed by your doctor or pharmacist. If
you have any doubts, consult your doctor or pharmacist.
Treatment must be initiated and supervised by a physician experienced in managing progressive liver disease
with reduced biliary flow.
The dose of KAYFANDA to take

• The dose of odevixibat depends on body weight. Your doctor will determine the correct amount and dosage of capsules to take.
• The recommended dose is 120 micrograms of odevixibat per kilogram of body weight once daily (up to a maximum of 7,200 micrograms once daily). Your doctor may recommend reducing the dose to 40 micrograms of odevixibat per kilogram of body weight once daily if you experience diarrhoea lasting ≥ 3 days, if the diarrhoea is considered severe, or if it requires intravenous hydration.

If this medicine does not improve your condition after 6 months of continuous daily treatment,
your doctor will advise you on an alternative treatment.
How to take KAYFANDA
Take the capsules once daily in the morning, with or without food.
All capsules may be swallowed whole with a glass of water or may be opened and sprinkled onto food or dispersed in a liquid appropriate for age (breast milk, infant formula, or water).
The larger capsules (200 and 600 micrograms) are intended to be opened and sprinkled onto food or dispersed in a liquid appropriate for age, but may also be swallowed whole.
The smaller capsules (400 and 1,200 micrograms) are intended to be swallowed whole, but may also be opened and sprinkled onto food or dispersed in a liquid appropriate for age.
Instructions for opening the capsules and sprinkling the contents onto food or into a liquid are provided at the end
of this patient information leaflet.
If you take more KAYFANDA than you should
Inform your doctor if you think you have taken an excessive amount of KAYFANDA.
Possible symptoms of overdose include diarrhoea, stomach problems, and intestinal problems.
If you forget to take KAYFANDA
If you miss a dose of KAYFANDA, take the missed dose as soon as possible, but do not take more than one dose per day.
If you stop taking KAYFANDA
Do not stop treatment with KAYFANDA without first consulting your doctor.
If you have any questions about how to use this medicine, consult your doctor or pharmacist.

4. Possible side effects

Like all medicines, this medicine can cause side effects, although not everybody will experience them.
The following side effects may occur with the listed frequency:
very common (may affect more than 1 in 10 people)

• diarrhoea
• abdominal pain (stomach ache)

common (may affect up to 1 in 10 people)

• vomiting
• enlarged liver

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

5. How to store KAYFANDA

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 container after
Exp. The expiry date refers to the last day of that month.
Store in the original packaging to protect the medicine from light. Do not store above 25 °C.
Do not dispose of any medicine via wastewater or household waste. Ask your pharmacist how to dispose of medicines you no longer use. This will help protect the environment.

6. Package contents and other information

What KAYFANDA contains

• The active substance is odevixibat. Each KAYFANDA 200 microgram hard capsule contains 200 micrograms of odevixibat (as sesquihydrate). Each KAYFANDA 400 microgram hard capsule contains 400 micrograms of odevixibat (as sesquihydrate). Each KAYFANDA 600 microgram hard capsule contains 600 micrograms of odevixibat (as sesquihydrate). Each KAYFANDA 1 200 microgram hard capsule contains 1 200 micrograms of odevixibat (as sesquihydrate).
• The other components are:

Contents of the capsule
Microcrystalline cellulose, hypromellose
Capsule shell
KAYFANDA 200 micrograms and 600 micrograms hard capsules
Hypromellose, titanium dioxide (E171), yellow iron oxide (E172)
KAYFANDA 400 micrograms and 1 200 micrograms hard capsules
Hypromellose, titanium dioxide (E171), yellow iron oxide (E172), red iron oxide (E172)
Printing ink
Shellac, propylene glycol, black iron oxide (E172)

Description of the appearance of KAYFANDA and contents of the pack
KAYFANDA 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.
KAYFANDA 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.
KAYFANDA 600 microgram hard capsules:
Size 0 (21.7 mm × 7.64 mm) capsules with an opaque ivory cap and body, printed with “A600” in black ink.
KAYFANDA 1 200 microgram hard capsules:
Size 3 (15.9 mm × 5.82 mm) capsules with an opaque orange cap and body, printed with “A1200” in black ink.

KAYFANDA hard capsules are packaged in a plastic bottle (high-density polyethylene (HDPE)) with a tamper-evident, child-resistant closure.
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 about this medicinal product, please contact the local representative of the Marketing Authorisation Holder:

Belgium/Belgium/Belgium/Luxembourg/Luxembourg
Ipsen NV
België/Belgique/Belgien
Tél/Tel: +32 9 243 96 00

Italy
Ipsen SpA
Tel: +39 02 39 22 41

Bulgaria
Swixx Biopharma EOOD
Tel.: +359 (0)2 4942 480

Latvia
Ipsen Pharma representative office
Tel: +371 67622233

Czech Republic
Ipsen Pharma s.r.o
Tel: +420 242 481 821

Lithuania
Ipsen Pharma SAS Lietuvos filialas
Tel: +370 700 33305

Denmark, Norway, Finland, Sweden, Iceland
Institut Produits Synthèse (IPSEN) AB
Sweden/Ruotsi/Svíþjóð
Tlf/Puh/Tel/Sími: +46 8 451 60 00

Hungary
IPSEN Pharma Hungary Kft.
Tel.: +36 1 555 5930

Germany, Austria
Ipsen Pharma GmbH
Germany
Tel: +49 89 2620 432 89

Netherlands
Ipsen Farmaceutica B.V.
Tel: +31 (0) 23 554 1600

Estonia
Centralpharma Communications OÜ
Tel: +372 60 15 540

Poland
Ipsen Poland Sp. z o.o.
Tel.: +48 22 653 68 00

Greece, Cyprus, Malta
Ipsen Monoprosope EPE
Greece
Tel: +30 210 984 3324

Portugal
Ipsen Portugal - Produtos Farmacêuticos S.A.
Tel: +351 21 412 3550

Spain
Ipsen Pharma, S.A.U.
Tel: +34 936 858 100

Romania
Ipsen Pharma România SRL
Tel: +40 21 231 27 20

France
Ipsen Pharma
Tél: +33 1 58 33 50 00

Slovenia
Swixx Biopharma d.o.o.
Tel: +386 1 2355 100

Croatia
Swixx Biopharma d.o.o.
Tel: +385 1 2078 500

Slovakia
Ipsen Pharma, organizačná zložka
Tel: +420 242 481 821

Ireland, United Kingdom (Northern Ireland)
Ipsen Pharmaceuticals Limited
Tel: +44 (0)1753 62 77 77

This medicinal product has been authorised under “exceptional circumstances”. This means that, due to the rarity of the disease, complete information about this medicinal product could not be obtained.
The European Medicines Agency will review any new information on this medicinal product annually, and this package leaflet will be updated if necessary.

Other sources of information
More detailed information on this medicinal product is available on the website of the European Medicines Agency, http://www.ema.europa.eu. In addition, links to other websites on rare diseases and related therapeutic treatments are provided.

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, applesauce, banana or carrot purée, chocolate pudding, rice pudding, or oatmeal). The food should be at room temperature or cooler.

	Step 2: • Hold the capsule horizontally at both ends and twist them in opposite directions.
	Step 3: • Separate them to empty the contents into the bowl with soft food. • Gently tap the capsule to ensure all granules come out. • Repeat the previous step if the prescribed dose requires more than one capsule.
	Step 4: • Gently mix the capsule contents into the soft food;
Take the entire dose immediately after mixing. Do not store the mixture for later use. • Drink a glass of water after taking the dose. • Dispose of the empty capsule shells.

Instructions for opening the capsules and pouring the contents into a liquid suitable for the patient's age:
Contact the pharmacy if an appropriate oral administration syringe for home use is not available.

	Step 1: • Hold the capsule horizontally at both ends and twist them in opposite directions. • Separate the two parts and empty the contents into a dosing cup or a small cup. The granules will not pass through the opening of a baby bottle or sippy cup. • Gently tap the capsule to ensure all granules are released. Repeat this step if the prescribed dose requires more than one capsule.
	Add 1 teaspoon (5 mL) of a suitable liquid for the child's age (e.g., breast milk, infant formula, or water). • Allow the granules to remain in the liquid for approximately 5 minutes to ensure they are fully wetted (the granules do not dissolve).
	Step 2: • After 5 minutes, insert the tip of the oral syringe completely into the dosing cup. • Slowly pull back the plunger of the syringe to draw the liquid/granule mixture into the syringe. Gently push the plunger and expel the liquid/granule mixture back into the dosing cup. Repeat this process 2 or 3 times to ensure complete mixing of the granules with the liquid.
	Step 3: • Draw the entire contents into the oral syringe by pulling back the plunger to the end of the syringe.
	Step 4: • Place the tip of the oral syringe in the front of the child’s mouth, between the tongue and the inner cheek. Gently push the plunger to release the liquid/granule mixture between the tongue and the inner cheek. Do not administer the liquid/granule mixture at the back of the child’s throat, as this may cause gagging or choking.
If any of the liquid/granule mixture remains in the dosing cup, repeat steps 3 and 4 until the entire dose has been administered. • Administer the full dose immediately after mixing. Do not store the liquid/granule mixture for later use. • After administering the dose, give breast milk, infant formula, or another age-appropriate liquid. • Dispose of all empty capsule shells.