ORFADIN ® Oral Suspension
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Nitisinone
Consumer Medicine Information

What is in this leaflet

This leaflet answers some common questions about ORFADIN Oral Suspension. It does not contain all the available information. It does not take the place of talking to your doctor or pharmacist.
All medicines have risks and benefits. Your doctor has weighed the risks of you taking ORFADIN against the benefits they expect it will have for you.
If you have any concerns about taking this medicine, ask your doctor or pharmacist.
Keep this leaflet with the medicine.
You may need to read it again.

What ORFADIN is used for

ORFADIN contains a medicine called nitisinone which is used for the treatment of a disease called hereditary tyrosinaemia type 1 (HT-1). In this disease the body is unable to completely break down the amino acid tyrosine. Harmful substances will be formed and accumulated in the body.
ORFADIN blocks the breakdown of tyrosine and by doing so the harmful substances are not formed. However, tyrosine will remain in the body and therefore a special diet (with low tyrosine and phenylalanine content) must be followed when taking ORFADIN.
Nitisinone belongs to a group of medicines called 'other alimentary and metabolism products'.
Ask your doctor if you have any questions about why this medicine has been prescribed for you.
Your doctor may have prescribed it for another reason.
ORFADIN is not addictive.
ORFADIN is available only with a doctor's prescription.

Before you take ORFADIN

When you must not take it

Do not take ORFADIN if you have an allergy to:
any medicine containing nitisinone
any of the ingredients listed at the end of this leaflet.
Some of the symptoms of an allergic reaction may include:
shortness of breath
wheezing or difficulty breathing
swelling of the face, lips, tongue or other parts of the body
rash, itching or hives on the skin
Do not breast-feed if you are taking this medicine.
Do not take this medicine after the expiry date printed on the pack or if the packaging is torn or shows signs of tampering.
If it has expired or is damaged, return it to your pharmacist for disposal.
If you are not sure whether you should start taking this medicine, talk to your doctor.

Before you start to take it

Tell your doctor if you have allergies to any other medicines, foods, preservatives or dyes.
Tell your doctor about your diet regimen.
Your doctor will advise you regarding a diet restricted in tyrosine and phenylalanine content.
Tell your doctor if you are pregnant or plan to become pregnant or are breastfeeding.
Your doctor can discuss with you the risks and benefits involved.
If you have not told your doctor about any of the above, tell him/her before you start taking ORFADIN.

Taking other medicines

Tell your doctor or pharmacist if you are taking any other medicines, including any that you get without a prescription from your pharmacy, supermarket or health food shop.
Some medicines and ORFADIN may interfere with each other. These include:
warfarin, a medicine used to thin your blood
phenytoin, a medicine used to treat epilepsy
furosemide (frusemide), a diuretic
These medicines may be affected by ORFADIN or may affect how well it works. You may need different amounts of your medicines, or you may need to take different medicines.
Your doctor and pharmacist have more information on medicines to be careful with or avoid while taking ORFADIN.

How to take ORFADIN

Treatment with ORFADIN should be initiated and supervised by a physician experienced in the treatment of HT-1 patients.
Follow all directions given to you by your doctor or pharmacist carefully.
They may differ from the information contained in this leaflet.
The oral suspension is taken with an oral syringe directly in the mouth without dilution.
ORFADIN must not be injected.
Do not attach a needle to the syringe.
If you do not understand the instructions on the box/bottle, ask your doctor or pharmacist for help.

How much to take

The recommended initial dose is 1 mg/kg body weight /day divided in 2 doses administered orally.
Your doctor may change your total dose from twice a day to once a day. You will need to have taken ORFADIN twice daily for at least 4 weeks before your doctor may consider changing your dose. You will need to be at least 20 kg for the ORFADIN to be used once a day.
The dose of ORFADIN will be adjusted individually for you by your doctor.

How to take it

Take ORFADIN with food.

How to prepare the dose to be administered

The dose that your doctor prescribes you should be given in mL of suspension and not in mg. This is because the oral syringe which is used to withdraw the correct dose from the bottle is marked in mL.
If your prescription is in mg, contact your pharmacist or doctor for advice.
The pack contains a bottle of medicine with a cap, a bottle adaptor and three oral syringes (1 mL, 3 mL and 5 mL). Always use one of the oral syringes provided to take the medicine.
The 1-mL oral syringe (the smallest oral syringe) is marked from 0.1 mL to 1 mL with minor 0.01 mL graduations. It is used for measuring doses of less than or up to 1 mL.
The 3 mL oral syringe (the middle sized oral syringe), is marked from 1 mL to 3 mL with minor 0.1 mL graduations. It is used for measuring doses of more than 1 mL and up to 3 mL.
The 5 mL oral syringe (the largest oral syringe), is marked from 1 mL to 5 mL with minor 0.2 mL graduations. It is used for measuring doses of more than 3 mL.
It is important that you use the correct oral syringe when taking the medicine. Your doctor, pharmacist or nurse will advise which oral syringe to use depending on the dose that has been prescribed.
Follow the instructions given below for dose preparation and administration carefully, in order to ensure that the correct dose is administered.

How to prepare a new bottle of medicine for first time use

Before taking the first dose, shake the bottle vigorously since during long-term storage the particles will form a solid cake at the bottom of the bottle. Follow the instructions below:

1. Remove the bottle from the refrigerator. Note the date when the bottle is removed from the refrigerator on the bottle label.

Figure 1A

2. Shake the bottle vigorously for AT LEAST 20 SECONDS until the solid cake at the bottom of the bottle is completely dispersed (Figure 1A).

Figure 1B

3. Remove the child resistant screw cap by pushing it down firmly and turning it anti-clockwise (Figure 1B).

Figure 1C

4. Place the open bottle upright on a table. Push the plastic adapter firmly into the neck of the bottle as far as you can (Figure 1C). Once the plastic adapter is firmly in place, close the bottle by screwing the child resistant cap back onto the bottle.

For subsequent dosing see the instructions below on 'How to prepare a dose of medicine'.

How to prepare a dose of medicine

Figure 2A

1. Shake the bottle vigorously for AT LEAST 5 SECONDS (Figure 2A).

Figure 2B

2. Immediately thereafter, open the bottle by removing the child resistant screw cap (Figure 2B).

Figure 2C

3. Push the plunger inside the oral syringe fully down.

4. Keep the bottle in an upright position and insert the oral syringe firmly into the hole at the top of the bottle (Figure 2C).

Figure 2D

5. Carefully turn the bottle upside down with the oral syringe in place (Figure 2D).

6. In order to withdraw the prescribed dose (mL), pull the plunger SLOWLY down until the top edge of the black ring is exactly level with the line marking the dose (Figure 2D). If any air bubbles are observed inside the filled oral syringe, push the plunger back up until the air bubbles are expelled. Then pull the plunger down again until the top edge of the black ring is exactly level with the line marking the dose.

7. Turn the bottle to an upright position again. Disconnect the oral syringe by gently twisting it out of the bottle.

8. The dose should be administered in the mouth immediately (without dilution) in order to avoid caking in the oral syringe. The oral syringe must be emptied SLOWLY to allow swallowing; rapid squirting of the medicine may cause choking.

9. Replace the child resistant screw cap directly after use. The bottle adapter should not be removed.

10. The bottle may be stored at a temperature below 25°C or in the refrigerator.

Cleaning

Clean the oral syringe IMMEDIATELY with water. Separate barrel and plunger and rinse both with water. Shake off excess water and leave the disassembled oral syringe to dry until reassemble for next dosing occasion.

When to take it

The usual daily dose is 1 mg/kg body weight divided in morning and evening administrations.
Take your first dose in the morning. Take your second dose in the evening.
Your doctor will advise you on the correct time to take ORFADIN when moving you to once daily dose.
Take ORFADIN at about the same time each day.
Taking it at the same time each day will have the best effect. It will also help you remember when to take it.

How long to take it

Continue taking ORFADIN for as long as your doctor tells you.
This medicine helps to control your condition, but does not cure it. It is important to keep taking your medicine even if you feel well.

If you forget to take it

Do not take a double dose to make up for the dose that you missed.
This may increase the chance of you getting an unwanted side effect.
If you are not sure what to do, ask your doctor or pharmacist.
If you have trouble remembering to take your medicine, ask your pharmacist for some hints.

If you take too much (overdose)

Immediately telephone your doctor or the Poisons Information Centre (telephone 13 11 26) for advice, or go to Accident and Emergency at the nearest hospital, if you think that you or anyone else may have taken too much ORFADIN. Do this even if there are no signs of discomfort or poisoning.
You may need urgent medical attention.

While you are using ORFADIN

Things you must do

It is very important that you stay on the special diet, with low tyrosine and phenylalanine content that your doctor recommended. If you do not understand the instructions on your diet, ask your doctor for help.
If you are about to be started on any new medicine, remind your doctor and pharmacist that you are taking ORFADIN.
Tell any other doctors, dentists, and pharmacists who treat you that you are taking ORFADIN. Remind them if any new medicines are about to be started.
If you are going to have surgery, tell the surgeon or anaesthetist that you are taking ORFADIN.
It may affect other medicines used during surgery.
If you become pregnant while taking this medicine, tell your doctor immediately.
If you are about to have any blood tests, tell your doctor that you are taking this medicine.
It may interfere with the results of some tests.
Keep all of your doctor's appointments so that your progress can be checked.
Your Doctor will test your blood to check if the amounts of ORFADIN given are enough and to make sure that there are no possible side effects causing blood disorders.
Your doctor will also check your liver regularly as the disease can affect the liver.
Your doctor will also check on your general development.

Things you must not do

ORFADIN must not be injected.
Do not attach a needle to the syringe.
Do not take ORFADIN to treat any other complaints unless your doctor tells you to.
It may not be safe to use ORFADIN for another complaint.
Do not give ORFADIN to anyone else, even if they have the same condition as you.
Do not stop taking ORFADIN or lower the dosage without checking with your doctor.
If you stop taking it suddenly, your condition may worsen or you may have unwanted side effects.

Things to be careful of

Be careful driving or operating machinery or doing other jobs that require you to be alert until you know how ORFADIN affects you.
If you feel light-headed, dizzy or faint when getting out of bed or standing up, get up slowly.
Standing up slowly, especially when you get up from bed or chairs, will help your body get used to the change in position and blood pressure. If this problem continues or gets worse, talk to your doctor.

Side effects

Tell your doctor or pharmacist as soon as possible if you do not feel well while you are taking ORFADIN.
This medicine helps most people with HT-1, but it may have unwanted side effects in a few people. All medicines can have side effects. Sometimes they are serious, most of the time they are not. You may need medical attention if you get some of the side effects.
Do not be alarmed by the following lists of side effects. You may not experience any of them.
Ask your doctor or pharmacist to answer any questions you may have.
Tell your doctor or pharmacist if you notice any of the following and they worry you:
different eye symptoms as listed below:
scarring or clouding of the cornea
inflammation in the cornea
inflammation in the eye
inflammation in the eyelid
sensitivity to light
eye pain
rash
itching or irritating sensation of skin
redness and peeling of skin
headache
abdominal pain
constipation
If you get red eyes or any other signs of effects on the eyes, contact your doctor immediately for an eye examination. Eye problems could be a sign of inadequate dietary control.
The above list includes the more common side effects of your medicine.
There may be other side effects that your doctor will carefully monitor by taking blood samples. These are:
reduced number of platelets and white blood cells
shortage of certain white blood cells (granulocytopenia)
Tell your doctor or pharmacist if you notice anything that is making you feel unwell.
Other side effects not listed above may also occur in some people.
Some of these side effects can only be found when your doctor does tests from time to time to check your progress.

After using ORFADIN

Storage

Keep your oral suspension in the original bottle until it is time to take them.
If you take the oral suspension out of the bottle, it may not keep well.
Keep your unopened oral suspension in a refrigerator (2°C - 8°C). Do not freeze. Store the bottle upright.
After first opening, the in-use stability of ORFADIN is a single period of 2 months stored at a temperature below 25°C, after which it must be discarded.
Do not forget to mark the date on the bottle, when removed from the refrigerator.
Do not store ORFADIN or any other medicine in the bathroom or near a sink. Do not leave it on a window sill or in the car, especially on hot or cold days.
Heat and dampness can destroy some medicines.
Keep it where children cannot reach it.
A locked cupboard at least one-and-a-half metres above the ground is a good place to store medicines.

Disposal

If your doctor tells you to stop taking this medicine or the expiry date has passed, ask your pharmacist what to do with any medicine that is left over.

Product description

What it looks like

ORFADIN oral suspension is a white, slightly thicker opaque suspension.
Before shaking the bottle, it may look like a solid cake in the bottom and a slightly opalescent liquid.

Ingredients

Each mL contains 4 mg nitisinone.
It also contains the following inactive ingredients:
Hypromellose
Glycerol
Polysorbate 80
Sodium benzoate
Citric acid monohydrate
Sodium citrate
Strawberry 501440 T
Purified water
This medicine does not contain lactose, sucrose, gluten, tartrazine or any other azo dyes.
ORFADIN oral suspension is provided in a 100 mL brown glass bottle with a white, child resistant screw cap. Each bottle contains 90 mL suspension.
Each pack contains one bottle, one bottle adapter and three oral syringes.

Supplier

ORFADIN is supplied in Australia by:
A. Menarini Australia Pty. Ltd.
Level 8, 67 Albert Avenue
CHATSWOOD NSW 2067
Medical Information: 1800 644 542
 
Australian Registration Number:
ORFADIN 4mg/mL: AUST R 297736
 
ORFADIN ® is a registered trademark of Swedish Orphan Biovitrum International AB, used under licence by A. Menarini Australia Pty. Ltd.
 
This leaflet was revised in February 2019.
For the most up to date version of this leaflet, please go to www.menarini.com.au/cmi
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