483 mg / g granules
sodium phenylbutyrate
What PHEBURANE is and what it is used for
PHEBURANE contains the active substance sodium phenylbutyrate which is used to treat patients of all ages with urea metabolic disorders. These rare diseases are due to a deficiency of certain liver enzymes that are necessary to excrete residual nitrogen in the form of ammonia.
Nitrogen is a building block in protein, which is an important part of the food we eat. When the body breaks down the proteins we eat, residual nitrogen accumulates in the form of ammonia, because the body cannot excrete it. Ammonia is especially toxic to the brain and in severe cases leads to decreased consciousness and coma.
PHEBURANE helps the body excrete residual nitrogen and reduces the amount of ammonia in your body. However, PHEBURANE should be combined with a protein-reduced diet that has been specially developed for you by your doctor or dietitian. This diet must be followed carefully.
What you need to know before taking PHEBURANE
Do not take PHEBURANE:
- if you are allergic to sodium phenylbutyrate or any of the other ingredients of this medicine (listed in section 6);
- if you are pregnant;
- if you are breastfeeding.
Warnings and cautions
Talk to your doctor or pharmacist before taking PHEBURANE if you:
- suffer from chronic heart failure (a type of heart disease where the heart is not able to pump enough blood into the body) or a decrease in kidney function;
- have impaired kidney or liver function, as PHEBURANE is excreted from the body through the kidneys and liver.
PHEBURANE does not prevent the emergence of an acute excess of ammonia in the blood, a condition that usually requires urgent medical attention. If this happens you will have symptoms such as nausea, vomiting, and confusion and will need urgent medical attention.
If you need to do laboratory tests, it is important to remind your doctor that you are taking PHEBURANE, as sodium phenylbutyrate may affect the results of certain laboratory tests (such as electrolytes or protein in the blood or liver function tests).
If you have any further questions, ask your doctor or pharmacist.
Other medicines and PHEBURANE
Tell your doctor or pharmacist if you are taking, have recently taken, or might take any other medicines.
You must tell your doctor if you are taking medicines that contain:
- valproate (a medicine used to treat epilepsy ),
- haloperidol (used in certain psychotic disorders),
- corticosteroids (drugs used to relieve inflamed areas of the body)
- probenecid (for the treatment of hyperuricemia, elevated levels of uric acid in the blood associated with gout ).
These medicines may affect the way PHEBURANE works and you may need to do more frequent blood tests. If you are not sure if your medicines contain these substances, ask your doctor or pharmacist.
Pregnancy and breastfeeding
Do not use PHEBURANE if you are pregnant, as this medicine may harm the fetus.
If you are a woman who may become pregnant, you must use a reliable contraceptive during treatment with PHEBURANE. Talk to a doctor for more information.
Do not use PHEBURANE if you are breastfeeding, as this medicine may pass into breast milk and harm your baby.
Driving and using machines
PHEBURANE is unlikely to affect the ability to drive and use machines.
PHEBURANE contains sodium and sucrose
This medicine contains 124 mg (5.4 mmol) of sodium per gram of sodium phenylbutyrate. Talk to your doctor or pharmacist if you need 3 grams or more daily for an extended period, especially if you have been prescribed a low-salt diet.
This medicine contains 768 mg of sucrose per gram of sodium phenylbutyrate. This should be considered if you have diabetes mellitus. If you have been told by your doctor that you have an intolerance to some sugars, contact your doctor before taking this medicine.
How to take PHEBURANE
Always take this medicine exactly as your doctor has told you. Ask your doctor or pharmacist if you are unsure.
Dosage
The daily dose of PHEBURANE is based on your body weight or body surface area and is adjusted according to your ability to tolerate protein and your diet. You will need regular blood tests to determine the correct daily dosage. Your doctor will tell you how many granules to take.
Method of administration
PHEBURANE should be taken by mouth. Because it dissolves slowly, PHEBURANE should not be given through a gastrostomy (gastric fistula, a tube that passes through the abdominal wall to the stomach) or through a gastric tube (a tube that passes through the nose to the stomach).
PHEBURANE must be taken with a special protein-reduced diet.
You should take PHEBURANE with each meal or feeding. In young children, this can be 4 to 6 times a day.
The dose of PHEBURANE prescribed by your doctor is expressed in grams of sodium phenylbutyrate. A calibrated dosing spoon giving up to 3 g of sodium phenylbutyrate at a time is supplied with this medicine. Use only this dosing spoon to measure the dose of a PHEBURANE. The dosing phase must not be used for any other drug.
Measure dose one as follows:
- The lines on the spoons indicate the amount of PHEBURANE in grams of sodium phenylbutyrate. Take the correct amount according to your doctor’s prescription.
- Pour granules directly into the spoon as shown (on the outer carton and in this leaflet).
- Tap the spoons once against a table to make the granules lie horizontally and continue to refill if necessary.
Granules can be swallowed directly with a drink (water, fruit juice, protein-free infant formula) or sprinkled on a spoon with solid food (mashed potatoes or apple puree). If you mix it with food, you must take it immediately so that the granules do not develop any taste.
You must take this medicine and eat a special diet for life.
If you take more PHEBURANE than you should
Patients who have taken very high doses of sodium phenylbutyrate experienced:
- drowsiness, fatigue, dizziness, and on more rare occasions confusion,
- headache,
- changed taste perception,
- impaired hearing,
- disorientation,
- impaired memory,
- deterioration of existing neurological conditions.
If you experience any of these symptoms, contact your doctor or nearest emergency department immediately for supportive care.
If you forget to take PHEBURANE
Take the next dose as soon as possible at the next meal. Make sure there are at least 3 hours between two doses. Do not take a double dose to make up for a forgotten dose.
If you have any further questions on the use of this product, ask your doctor or pharmacist.
Possible side effects
Like all medicines, this medicine can cause side effects, although not everybody gets them.
If repeated vomiting occurs, contact your doctor immediately.
Very common side effects ( may affect more than 1 in 10 people):
irregular periods or missed periods in fertile women.
If you are sexually active and your period stops completely, do not take it for granted that this has been caused by PHEBURANE. If this happens, you should discuss it with your doctor, as missed periods can also be due to pregnancy (see the section on pregnancy and breastfeeding above) or menopause.
Common side 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, headache, fainting, fluid retention (swelling), taste changes, abdominal pain, vomiting, nausea, constipation, abnormal skin odor, rash, abnormal kidney function, weight gain, altered laboratory test values.
Uncommon side effects ( may affect more than 1 in 1,000 people):
lack of red blood cells due to bone marrow failure, the appearance of bruises, altered heart rhythm, rectal bleeding, gastritis, gastric ulcer, and inflammation of the pancreas.
How to store PHEBURANE
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 carton and bottle label after EXP. The expiration date is the last day of the specified month.
PHEBURANE can be used within 45 days after opening the package.
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 to protect the environment.
Contents of the package and other information
Content declaration
The active substance is sodium phenylbutyrate.
Each gram of granules contains 483 mg of sodium phenylbutyrate.
The other ingredients are sugar balls (sucrose and corn starch, see section 2 PHEBURANE contains sucrose), hypromellose, ethylcellulose N7, macrogol 1500, povidone K25.
What the medicine looks like and the contents of the pack
PHEBURANE granules are white to off-white.
Granules are packaged in a plastic bottle with a child-resistant closure and desiccant.
Each bottle contains 174 g of granules.
Each carton contains a bottle.
A calibrated dosing spoon is included.
Marketing Authorization Holder and Manufacturer
Eurocept International BV
Trapani 5
1244 RL Ankeveen
NETHERLANDS
Contact the representative of the marketing authorization holder to find out more about this medicine:
FrostPharma AB
Tel: +46 775 86 80 02
info@frostpharma.com