Leaflet: information for the user

AMMONAPS 500 mg tablets

Sodium phenylbutyrate

Read this leaflet carefully before you start taking this medicine, as 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 must not give it to others even if they have the same symptoms as you, as it may harm them.
• If you experience any side effects, consult your doctor or pharmacist, even if they are not listed in this leaflet. See section 4.

1. Possible side effects
2. Storage of AMMONAPS

1. Contents of the pack and additional information

AMMONAPS is prescribed to patients with urea cycle disorders. Patients with these rare disorders have a deficiency of certain liver enzymes, making it impossible for them to eliminate nitrogen waste products. Since nitrogen is a constituent element of proteins, it accumulates in the body after protein consumption. Nitrogen waste products, in the form of ammonia, are especially toxic to the brain and, in severe cases, can cause loss of consciousness and coma.

AMMONAPS helps the body to eliminate excess nitrogen and reduces the amount of ammonia in the body.

Do not take AMMONAPS:

• If you are pregnant.
• If you are breastfeeding.
• If you are allergic (hypersensitive) to sodium phenylbutyrate or to any of the other components of this medication (listed in section 6).

Warnings and precautions

Consult your doctor or pharmacist before starting to take AMMONAPS.

• If you have difficulty swallowing. AMMONAPS tablets may get stuck in the esophagus and cause ulcers. If you have difficulty swallowing them, it is recommended that you use AMMONAPS granules as an alternative.
• If you have heart failure, kidney failure, or any other condition that may worsen due to the sodium content of this medication.
• If you have liver or kidney failure, as AMMONAPS is eliminated from the body through the kidneys and liver.
• When administering it to small children, as they may be unable to swallow the tablets and choke. As an alternative, it is recommended to administer AMMONAPS granules.

AMMONAPS must be combined with a low-protein diet that your doctor or dietitian has specifically developed for your case. You must follow that diet carefully.

AMMONAPS does not completely prevent the production of excess ammonia in the blood and is not suitable for treating this condition, which is considered a medical emergency.

If you are to undergo testing, it is essential that you remind your doctor that you are taking AMMONAPS, as sodium phenylbutyrate may alter the results of some tests.

Use of AMMONAPS with other medications

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

It is especially important to inform your doctor if you are taking medications that contain:

• Valproic acid (an antiepileptic medication)
• Haloperidol (used to treat certain psychotic disorders)
• Corticosteroids (medications similar to cortisone used to alleviate inflamed areas of the body)
• Probencid (for the treatment of hyperuricemia associated with gout)

These medications may modify the effect of AMMONAPS, and more frequent blood tests will be required. If you are unsure whether the medications you are taking contain these active principles, consult your doctor or pharmacist.

Pregnancy and breastfeeding

Do not take AMMONAPS if you are pregnant, as this medication may harm the fetus. If you may become pregnant, you must use an effective contraceptive method during treatment with AMMONAPS.

Do not use AMMONAPS if you are breastfeeding, as this medication may pass into breast milk and harm your baby.

Driving and operating machinery

No studies have been conducted on the effects on the ability to drive and operate machinery.

AMMONAPS contains sodium

Each AMMONAPS tablet contains 62 mg of sodium.

Consult your doctor or pharmacist if you need 6 or more tablets daily for a prolonged period, especially if you have been recommended a low-sodium diet.

Follow exactly the administration instructions for this medication as indicated by your doctor.

If in doubt, consult your doctor or pharmacist again.

Dosage

The daily dose of AMMONAPS is calculated based on your tolerance to proteins, diet, and body weight or surface area. Regular blood tests will be necessary to adjust the correct daily dose. Your doctor will tell you how many tablets to take.

Administration Form

AMMONAPS must be taken orally in equal doses with each meal (for example, three times a day). Take AMMONAPS with plenty of water.

AMMONAPS must be taken while following a special low-protein diet.

AMMONAPS tablets should not be administered to children who are unable to swallow the tablets. As an alternative, it is recommended to administer AMMONAPS granules.

You will need to follow a diet and treatment for life, unless you undergo a successful liver transplant.

If you take more AMMONAPS than you should

Patients who have taken very high doses of AMMONAPS experience:

• drowsiness, fatigue, dizziness, and, less frequently, confusion
• headache
• changes in taste (gustatory disorders)
• decreased auditory capacity
• disorientation
• memory problems,
• worsening of existing neurological conditions

In case you experience any of these symptoms, contact your doctor or the nearest hospital emergency service immediately to receive symptomatic treatment.

If you forgot to take AMMONAPS

Take a dose as soon as possible with the next meal. Make sure at least 3 hours pass between two doses. Do not take a double dose to compensate for the missed doses.

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 all people will experience them.

Below are the frequencies of possible adverse effects.

Very frequent:Affect more than 1 in 10 people

Frequent:Affect 1 to 10 in 100 people

Infrequent:Affect 1 to 10 in 1,000 people

Rare:Affect 1 to 10 in 10,000 people

Very rare:Affect less than 1 in 10,000 people

Unknown:The frequency cannot be calculated from the available data

Very frequent adverse effects: irregular menstruation and cessation of menstruation.

It is possible that the absence of menstruation in sexually active women is not caused by the use of AMMONAPS. If this occurs, consult your doctor, as you may be pregnant (see "Pregnancy and breastfeeding" in the previous section).

Frequent adverse effects: changes in blood cell count (red blood cells, white blood cells, and platelets), reduced appetite, depression, irritability, headaches, dizziness, fluid retention (swelling), changes in taste (gustatory disorders), abdominal pain, vomiting, nausea, constipation, body odor, rash, abnormal kidney function, weight gain, altered laboratory results.

Infrequent adverse effects: low red blood cell count (anemia) due to bone marrow depression, cardenals, abnormal heart rhythm, rectal hemorrhage, stomach irritation, gastric ulcer, pancreatitis.

If you have persistent vomiting, you must consult your doctor immediately.

Reporting Adverse Effects

If you experience any type of adverse effect, consult your doctor or pharmacist, even if it is a possible adverse effect that does not appear in this prospectus. You can also report themdirectly through the national notification system included in theAnnex V. 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 packaging and the label of the bottle after “CAD”. The expiration date is the last day of the month indicated.

Do not store at a temperature above 30 °C.

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

Composition of AMMONAPS

• The active ingredient is sodium phenylbutyrate

Each AMMONAPS tablet contains 500 mg of sodium phenylbutyrate.

• The other components are microcrystalline cellulose, magnesium stearate, and anhydrous colloidal silica.

Appearance of the product and contents of the packaging

AMMONAPS tablets are white, oval-shaped, and embossed with “UCY 500”.

The tablets are packaged in plastic bottles with child-resistant caps. Each bottle contains 250 or 500 tablets.

Holder of the marketing authorization

Immedica Pharma AB

SE-113 63 Stockholm

Sweden

Responsible for manufacturing

PATHEON France – BOURGOIN JALLIEU

40 boulevard de Champaret

BOURGOIN JALLIEU

38300

France

Date of the last review of this leaflet:

The detailed information on this medicine is available on the website of the European Medicines Agencyhttp://www.ema.europa.eu/