Prospecto:information for the user

PHEBURANE 483mg tablets

Sodium phenylbutyrate

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

• Keep this prospectus, as you may need to read it again.
• If you have any doubts, consult your doctor or pharmacist.
• This medication has been prescribed only to you, and you should not give it to other people even if they have the same symptoms as you, as it may harm them.
• If you experience adverse effects, consult your doctor or pharmacist,even if they are not listed in this prospectus.See section 4.

1.What is PHEBURANE and for what it is used

2.What you need to know before starting to take PHEBURANE

3.How to take PHEBURANE

4.Possible adverse effects

5.Storage of PHEBURANE

6.Contents of the package and additional information

PHEBURANE contains the active ingredient sodium phenylbutyrate, which is used to treat patients of all ages with disorders of the urea cycle. These rare disorders are caused by a deficiency of certain liver enzymes that are necessary for eliminating residual nitrogen in the form of ammonia.

Nitrogen is a constituent element of proteins, which are an essential part of the foods we eat. As the body breaks down proteins after eating, residual nitrogen, in the form of ammonia, accumulates because the body cannot eliminate it. Ammonia is especially toxic to the brain and, in severe cases, can cause loss of consciousness and coma.

PHEBURANE helps the body to eliminate residual nitrogen and reduces the amount of ammonia in the body.

However, PHEBURANE must be used with a low-protein diet, which has been specifically developed for your case by your doctor or dietitian. You must follow that diet conscientiously.

Do not take PHEBURANE:

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

Warnings and precautions

Consult your doctor or pharmacist before starting to take PHEBURANE if you:

• have heart failure (a type of heart disease in which the heart cannot pump enough blood for the body) or a decrease in renal function.
• have a decrease in liver function, as PHEBURANE is eliminated from the body by these organs.

PHEBURANE does not completely prevent the occurrence of an excess of ammonia in the blood, a condition that usually constitutes a medical emergency. If this occurs, you will present symptoms such as nausea, vomiting, confusion, and you will need to receive medical attention urgently.

If you are to undergo testing, it is essential that you remind your doctor that you are taking PHEBURANE, as sodium phenylbutyrate may interfere with the results of some tests (such as electrolytes or proteins in the blood, or liver function tests).

In case of any doubt, consult your doctor or pharmacist.

Other medications and PHEBURANE

Inform your doctor or pharmacist if you are taking, have taken recently, or may need to take any other medication.

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

• valproate (an antiepileptic medication),
• haloperidol (used to treat some psychotic disorders),
• corticosteroids (medications used to alleviate inflamed areas of the body),
• probenecid (for the treatment of hyperuricemia, high concentration of uric acid in the blood, associated with gout)

These medications may modify the effect of PHEBURANE, and you will need to have blood tests more frequently. If you are unsure whether the medications you are using contain these active principles, consult your doctor or pharmacist.

Pregnancy and lactation

Do not take PHEBURANE if you are pregnant, as this medication may harm the fetus.

In case you may become pregnant, you must use, during treatment with PHEBURANE, an effective contraceptive method.Consult your doctor for details.

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

Driving and operating machinery

It is unlikely that PHEBURANE will affect your ability to drive and operate machinery.

PHEBURANE contains sodium and saccharose.

This medication contains 124 mg (5.4 mmol) of sodium per 1 g of sodium phenylbutyrate. Talk to your doctor or pharmacist if you need 3 or more grams daily for a prolonged period, especially if you have been advised to follow a low-sodium diet.

This medication contains 768 mg of saccharose per 1 g of sodium phenylbutyrate. This should be taken into account if you have diabetes mellitus. If your doctor has told you that you have an intolerance to certain sugars, consult your doctor before taking this medication.

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

Dosage

The daily dose of PHEBURANE will be based on your body weight or body surface area, and will be adjusted according to your tolerance to proteins and diet. Regular blood tests will be required to adjust the correct daily dose. Your doctor will indicate the amount you should take.

Administration form

PHEBURANE should be taken orally. Due to its slow dissolution, PHEBURANE should not be administered through a gastrostomy (a tube that goes into the stomach through the abdomen) or a nasogastric tube (a tube that goes into the stomach through the nose).

PHEBURANE should be taken while following a special low-protein diet.

You should take PHEBURANE with each meal. In small children, this may be 4 to 6 times a day.

The doses of PHEBURANE prescribed by your doctor are expressed in grams of sodium phenylbutyrate. This medication includes a calibrated spoon that dispenses up to 3 g of sodium phenylbutyrate at a time. Use this spoon only to measure the dose of PHEBURANE. The spoon should not be used for any other medication.

To measure the dose:

• The lines on the spoon indicate the amount of PHEBURANE in grams of sodium phenylbutyrate. Take the correct amount as prescribed by your doctor.
• Empty the granules directly into the spoon as shown in the photo (in the outer box and in this leaflet).

• Gently tap the spoon once to obtain a horizontal level of granules and continue filling if necessary.

The granules can be swallowed directly with a drink (water, fruit juice, protein-free formulas for infants), or added to a spoonful of solid food (mashed potatoes or apple sauce). If you mix it with food, it is essential to take it immediately. This will allow you to maintain the flavor masking.

You should take this medication and follow a lifelong diet.

If you take more PHEBURANE than you should

Patients who have taken very high doses of sodium phenylbutyrate have experienced:

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

If you experience any of these symptoms, you should immediately contact your doctor or the emergency department of the nearest hospital to receive symptomatic treatment.

If you forgot to take PHEBURANE

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 doubts 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.

If persistent vomiting occurs, you should call your doctor immediately.

Very common adverse effects (may affect more than 1 in 10 people): irregular menstrual cycles and amenorrhea in fertile women.

The absence of menstruation in sexually active women may not be caused by the use of PHEBURANE. If this occurs, consult your doctor, as it could be caused by pregnancy (see "Pregnancy and breastfeeding" in the previous section) or menopause.

Common adverse effects (may affect more than 1 in 100 people): changes in the number of blood cells (red blood cells, white blood cells, and platelets), changes in the amount of bicarbonate in the blood, decreased appetite, depression, irritability, headaches, dizziness, fluid retention (swelling), changes in taste (gustatory disorders), abdominal pain, vomiting, nausea, constipation, body odor, hives, abnormal kidney function, weight gain, abnormal test results.

Rare adverse effects (may affect more than 1 in 10,000 people): low red blood cell count (anemia) due to bone marrow failure, petechiae, arrhythmia, rectal bleeding, stomach inflammation, gastric ulcers, pancreatitis.

Reporting Adverse Effects

If you experience adverse effects, consult your doctor or pharmacist,even if they do not appear in this prospectus.You can also report them directly through thenational notification system included in theAppendix 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 PHEBURANE after the expiration date that appears on the box and the bottle label after "CAD". The expiration date is the last day of the month indicated.

After opening it for the first time, PHEBURANE can be used within a period of 45 days.

Medicines should not be thrown down the drain or in the trash. Ask your pharmacist how to dispose of the containers and the medications that you no longer need. In this way, you will help to protect the environment.

Composition of PHEBURANE

The active ingredient is sodium phenylbutyrate

Each gram of granule contains 483 mg of sodium phenylbutyrate.

The other components are: sugar spheres (sucrose and cornstarch, see section 2 "PHEBURANE contains sucrose"), hypromellose, ethylcellulose N7, macrogol 1500, povidone K25.

Appearance of the product and contents of the pack

PHEBURANE granule is white to off-white.

The granule is packed in a plastic bottle, with a child-resistant closure and a desiccant. Each bottle contains 174g of granule. Each box contains one bottle.

Includes a calibrated measuring spoon.

Marketing Authorization Holder and Responsible Person for Manufacturing

Eurocept International BV

Trapgans 5

1244 RL Ankeveen

The Netherlands

For more information about this medicine, please contact the local representative of the marketing authorization holder:

Last update of this leaflet:

For detailed information about this medicine, please visit the website of the European Medicines Agencyhttp://www.ema.europa.eu.

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