NITISINONA DIPHARMA 10 mg HARD CAPSULES

Introduction

Package Leaflet: Information for the User

Nitisinona Dipharma 5 mg hard capsules EFG

Nitisinona Dipharma 10 mg hard capsules EFG

nitisinona

Read all of 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 further questions, ask your doctor, pharmacist, or nurse.
• This medicine has been prescribed for you only. Do not pass it on to others. It may harm them, even if their signs of illness are the same as yours.
• If you get any side effects, talk to your doctor, pharmacist, or nurse. This includes any possible side effects not listed in this leaflet. See section 4.

Contents of the pack and other information:

1. What is Nitisinona Dipharma and what is it used for
2. What you need to know before you take Nitisinona Dipharma
3. How to take Nitisinona Dipharma
4. Possible side effects
5. Storage of Nitisinona Dipharma
6. Contents of the pack and further information

1. What is Nitisinona Dipharma and what is it used for

Nitisinona Dipharma contains the active substance nitisinone. Nitisinone is used to treat:

• a rare disease called hereditary tyrosinemia type 1 in adults, adolescents, and children (of any age range).
• a rare disease called alkaptonuria (AKU) in adults.

In these diseases, your body cannot fully break down the amino acid tyrosine (amino acids are the basic elements of proteins), forming toxic substances. These substances accumulate in your body. Nitisinone blocks the breakdown of tyrosine, and the toxic substances are not formed.

For the treatment of hereditary tyrosinemia type I, you must follow a special diet while taking this medicine, because tyrosine will still be in your body. This diet is based on a low content of tyrosine and phenylalanine (another amino acid).

For the treatment of AKU, your doctor may advise you to follow a special diet.

2. What you need to know before you take Nitisinona Dipharma

Do not take Nitisinona Dipharma

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

Do not breastfeed while taking this medicine (see section “Pregnancy and breastfeeding”).

Warnings and precautions

Consult your doctor or pharmacist before starting to take Nitisinona Dipharma.

• An ophthalmologist will examine your eyes before treatment and regularly during treatment with nitisinone. If you notice redness of the eyes or any other effect on the eyes, contact your doctor immediately for an ophthalmological examination. Eye problems (see section 4) may be a sign of inadequate dietary control.

During treatment, blood samples will be taken to check if the treatment is adequate and to ensure that there are no side effects causing blood alterations.

If you receive Nitisinona Dipharma for the treatment of hereditary tyrosinemia type I, you will have regular liver checks because the disease affects the liver.

Your doctor should perform a follow-up every 6 months. If you experience any side effects, it is recommended to use shorter time intervals.

Other medicines and Nitisinona Dipharma

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

Nitisinona Dipharma may interfere with the effect of other medicines, such as:

• medicines for epilepsy (such as phenytoin)
• medicines against blood clot formation (such as warfarin)

Taking Nitisinona Dipharma with food

If you start treatment taking it with food, it is recommended to follow this regimen throughout the treatment.

Pregnancy and breastfeeding

The safety of this medicine in pregnant women and breastfeeding women has not been studied.

If you are pregnant or breastfeeding, think you may be pregnant, or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.

Do not breastfeed while taking this medicine (see section “Do not take Nitisinona Dipharma”).

Driving and using machines

The influence of this medicine on the ability to drive and use machines is small. However, if you experience side effects that affect your vision, do not drive or use machines until your vision has returned to normal (see section 4 “Possible side effects”).

3. How to take Nitisinona Dipharma

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

For hereditary tyrosinemia type I, treatment with this medicine must be initiated and supervised by a doctor with experience in the treatment of the disease.

For hereditary tyrosinemia type I, the recommended daily dose is 1 mg/kg body weight administered orally. Your doctor will adjust the dose individually.

It is recommended to administer the dose once a day.

However, due to limited data in patients with a body weight <20 kg, in this patient population, it is recommended to divide the total daily dose into two doses per day.< p>

For AKU, the recommended dose is 10 mg once a day.

If you have problems swallowing the capsules, you can open the capsules and mix the powder with a small amount of water or dietary supplement before taking it.

If you take more Nitisinona Dipharma than you should

If you have taken more of this medicine than you should, tell your doctor or pharmacist immediately.

In case of overdose or accidental ingestion, consult your doctor or pharmacist immediately or call the Toxicology Information Service, phone: 91 562 04 20, indicating the medicine and the amount ingested.

If you forget to take Nitisinona Dipharma

Do not take a double dose to make up for forgotten doses. If you have forgotten to take a dose, tell your doctor or pharmacist.

If you stop taking Nitisinona Dipharma

If you think the action of the medicine is not adequate, tell your doctor. Do not change the dose or stop treatment without talking to your doctor first.

If you have any further questions on the use of this product, ask your doctor or pharmacist.

4. Possible side effects

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

If you notice any side effect related to the eyes, tell your doctor immediately for an ophthalmological examination. Treatment with nitisinone increases tyrosine levels in the blood, which can cause eye-related symptoms. In patients with hereditary tyrosinemia type 1, frequently reported eye side effects (may affect more than 1 in 100 people) due to higher tyrosine levels are eye inflammation (conjunctivitis), corneal opacity and inflammation (keratitis), sensitivity to light (photophobia), and eye pain. Eyelid inflammation (blepharitis) is an uncommon side effect (may affect up to 1 in 100 people).

In patients with AKU, eye irritation (keratopathy) and eye pain are very frequently reported side effects (may affect more than 1 in 10 people).

The following are other side effects reported in patients with hereditary tyrosinemia type 1:

Other frequent side effects

• Decrease in platelet count (thrombocytopenia) and white blood cells (leucopenia), reduction of certain types of white blood cells (granulocytopenia).

Other uncommon side effects

• • increase in white blood cells (leucocytosis),
  • itching (pruritus), skin inflammation (exfoliative dermatitis), rash.
• The following are other side effects reported in patients with AKU:
• Other frequent side effects
• • bronchitis,
  • pneumonia,
  • itching (pruritus), rash.

Reporting of side effects

If you experience any side effects, talk to your doctor, pharmacist, or nurse, even if it is possible that they are not listed in this leaflet. You can also report side effects directly through the Spanish Medicines Surveillance System for Human Use (www.notificaRAM.es). By reporting side effects, you can help provide more information on the safety of this medicine.

5. Storage of Nitisinona Dipharma

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 blister after “EXP” and on the bottle and carton after “CAD”. The expiry date is the last day of the month stated.

Store in a temperature below 30°C.

Medicines should not be disposed of via wastewater or household waste. Ask your pharmacist how to dispose of medicines no longer required. These measures will help protect the environment.

6. Contents of the pack and further information

Composition of Nitisinona Dipharma

• The active substance is nitisinone.

Nitisinona Dipharma 5 mg: each capsule contains 5 mg of nitisinone.

Nitisinona Dipharma 10 mg: each capsule contains 10 mg of nitisinone.

• The other ingredients (excipients) are:

Contents of the capsule:

Pregelatinized starch (from corn)

Stearic acid

Capsule shell:

Gelatin

Titanium dioxide (E171)

Printing ink:

Shellac

Propylene glycol

Aluminum lacquer indigo carmine (E132)

Appearance of the product and contents of the pack

The capsules are hard, white, opaque, made of gelatin, and have the company logo and dose “5” or “10” printed in dark blue. The capsule contains a powder that may be white or off-white.

Nitisinona Dipharma is available in plastic bottles with a tamper-evident closure of 60 capsules and OPA/Alu/PVC–Alu blister precut unit dose of 60 capsules.

Not all pack sizes may be marketed.

Marketing authorisation holder

Dipharma Arzneimittel GmbH

Offheimer Weg 33

65549 Limburg a.d. Lahn

Germany

Manufacturer

Doppel Farmaceutici srl

Via Volturno 48

20089 Quinto de Stampi – Rozzano (MI), Italy

You can obtain further information on this medicine from the local representative of the marketing authorisation holder:

Aurovitas Spain, S.A.U.

Avda. de Burgos, 16-D

28036 Madrid

Spain

Phone: 91 630 86 45

This medicine is authorised in the Member States of the European Economic Area under the following names:

Germany Nitisinone Dipharma

Austria Nitisinon Dipharma

Belgium Nitisinone Dipharma

Denmark Nitisinone Dipharma

Spain Nitisinona Dipharma

France Nitisinone Dipharma

Ireland Nitisinone Dipharma

Italy Nitisinone Dipharma

Norway Nitisinone Dipharma

Netherlands Nitisinone Dipharma

Portugal Nitisinona Dipharma

United Kingdom Nitisinone Dipharma

Sweden Nitisinone Dipharma

Date of last revision of this leaflet:February 2022

Detailed information on this medicine is available on the website of the Spanish Agency for Medicines and Health Products (AEMPS) (http://www.aemps.gob.es/).