Pheburane Oral Solution 350 mg Oral solution

• Keep this leaflet. You may need to read it again.
• If you have any further questions, ask your doctor or pharmacist.
• 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 or pharmacist. This includes any possible side effects not listed in this leaflet. See section 4.

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

• are allergic to sodium phenylbutyrate or any of the other ingredients of this medicine (listed in section 6).
• are pregnant.
• are breast-feeding.

Talk to your doctor or pharmacist before taking PHEBURANE if you:

• suffer from congestive heart failure (a type of heart disease where the heart cannot pump enough blood around the body) or a decrease in your kidney function.
• have decreased kidney or liver function, since PHEBURANE oral solution is eliminated from the body through the kidney and liver.

PHEBURANE will not prevent the occurrence of an acute excess of ammonia in the blood, a condition which usually constitutes a medical emergency. If this happens you will develop symptoms such as feeling sick (nausea), being sick (vomiting), confusion and will need to get urgent medical help.

If you need laboratory tests, it is important to remind your doctor that you are taking PHEBURANE oral solution, since sodium phenylbutyrate may interfere with certain laboratory test results (such as blood electrolytes or protein, or liver function tests)

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

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

It is especially important to tell your doctor if you are taking medicines containing:

• valproate (an antiepileptic medicine).
• haloperidol (used in certain psychotic disorders).
• corticosteroids (medicines that are used to provide relief for inflamed areas of the body),
• probenecid (for treatment of hyperuricaemia, high levels of uric acid in the blood, associated with gout).

These medicines may change the effect of PHEBURANE and you will need more frequent blood tests. If you are uncertain if your medicines contain these substances, you should check with your doctor or pharmacist.

Do not use PHEBURANE if you are pregnant, because this medicine can harm your unborn baby.

If you are a woman who could get pregnant, you must use reliable contraception, during treatment with PHEBURANE. Talk to your doctor for the details.

Do not use PHEBURANE if you are breast-feeding, because this medicine can pass into the breast milk and may harm your baby.

PHEBURANE oral solution is unlikely to affect your ability to drive and use machines.

This medicine contains 124 mg (5.4 mmol) sodium (main component of cooking/table salt) in each g dose of sodium phenylbutyrate. This is equivalent to 6.2% of the recommended maximum daily dietary intake of sodium for an adult.

The maximum daily dose of this medicine contains 2.5 g sodium This is equivalent to 125% of the recommended maximum daily dietary intake of sodium for an adult.

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 salt (sodium) diet.

This medicine contains 5.7 mg of aspartame per g dose of sodium phenylbutyrate. Aspartame is a source of phenylalanine. It may be harmful if you have phenylketonuria (PKU), a rare genetic disorder in which phenylalanine builds up because the body cannot remove it properly.

This medicine contains 26.55 mg of propylene glycol per drop.

If your baby is less than 4 weeks old, talk to your doctor or pharmacist before giving them this medicine, in particular if the baby is given other medicines that contain propylene glycol or alcohol.

The daily dose of PHEBURANE oral solution will be based on your body weight or body surface and adjusted according to your protein tolerance and diet. You will need regular blood tests to determine the correct daily dose. Your doctor will tell you the amount of liquid you should take.

PHEBURANE oral solution should be taken with meals.

Use only the dosing syringe provided with PHEBURANE oral solution to measure a dose of PHEBURANE oral solution. Do not use other devices/spoons/syringes to administer a dose. The syringe is ranging from 0.5 g to 3 g with 0.25 increments. The graduation of the dosing syringe reflects the grams of sodium phenylbutyrate. Follow the instructions below to administer PHEBURANE oral solution:

Administration for oral use

1. Open the bottle of PHEBURANE oral solution by pushing down on the cap and twisting to the left.

2. Take the CE marked dosing syringe with attached bottle adapter from the sachet.

3. Place (push) the adapter in the neck of the bottle while the syringe is in the adapter.

4. Invert the bottle.

5. Take the required quantity of PHEBURANE oral solution from the bottle (equivalent to the number of grams sodium phenyl butyrate as prescribed by your doctor) with use of the dosing syringe.

6. Take the dosing syringe with PHEBURANE oral solution out of the adapter and pour the quantity of PHEBURANE oral solution in the dosing syringe into a glass with minimum 20 ml water.

7. Close the bottle with PHEBURANE oral solution without removing the bottle adapter inserted in the neck of the bottle.

8. Add one drop of the flavour topping of your like (blackcurrant or lemon-mint) to the content of the glass of water; swirl gently, and then drink (If one drop of flavour topping would not provide the taste intensity of your like, you could use 2 drops).

9. After each administration, wash the syringe with cold to lukewarm water only.

PHEBURANE oral solution must be taken with a special diet reduced in protein.

You should take PHEBURANE oral solution with each meal or feeding. In small children this can be 4 to 6 times per day.

PHEBURANE oral solution can also be administered by nasogastric or gastrostomy tubes.

PHEBURANE oral solution can be administered with tubes of a diameter of 2 mm (7-8 French) and larger. Use the provided oral syringe to measure your dose and follow the instructions below:

Preparation for nasogastric tube or gastrostomy tube administration

1. Follow Step 1 to Step 5 of the Method of administration for oral use;
2. The oral solution of Pheburane is ready-to use and no dilution is needed;
3. When used via nasogastric/gastrostomy the flavour topping should not be added;
4. Insert the tip of the syringe filled with the medicine onto the tip of the nasogastric/gastrostomy tube;
5. Use the plunger of the dosing syringe to administer the prescribed dose of PHEBURANE oral solution into the nasogastric/gastrostomy tube;
6. After each administration, the tube should be flushed once with the adequate volume of lukewarm water and allowed to drain. For adults, 20 ml of lukewarm water should be used. For children weighing less than 20 kg and neonates use 3 mL of water.

You will need to take this medicine and to follow a diet throughout your life.

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

• sleepiness, tiredness, light-headedness and less frequently confusion;
• headache;
• changes in taste (taste disturbances);
• decrease in hearing;
• disorientation;
• impaired memory;
• worsening of existing neurological conditions.

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

You should take a dose as soon as possible with your next meal. Make sure that there are at least 3 hours between two doses. Do not take a double dose to make up for a forgotten dose.

If you get any side effects, talk to your doctor or pharmacist. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the Yellow Card Scheme at www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store. By reporting side effects you can help provide more information on the safety of this medicine.

• The active substance is sodium phenylbutyrate. Each mL of liquid contains 350 mg of sodium phenylbutyrate.
• The other ingredients are: purified water, aspartame (E951), sucralose (E955), glycerol (E422), hydroxyethylcellulose (E1525) (See section 2 “PHEBURANE oral solution contains aspartame”).

Flavour toppings:

• Blackcurrant flavour topping consisting of blackcurrant and mint flavouring, containing propylene glycol (E1520).
• Lemon-mint flavour topping consisting of lemon and mint flavouring.

PHEBURANE oral solution is clear, colourless to pale yellow liquid.

Each pack contains:

• One amber glass bottle containing 100 mL of the oral solution and closed with a plastic child-resistant cap;
• One dosing syringe with ranging from 0.5 g to 3 g with 0.25 increments to measure the dose in grams of sodium phenylbutyrate;
• Bottle Adapter;
• One amber glass bottle containing 3 mL of lemon-mint flavour topping;
• One amber glass bottle containing 3 mL of blackcurrant flavour topping.

For any information about this medicine, please contact the local representative of the Marketing Authorisation Holder: