Orfadin Oral Suspension

Nitisinone

Consumer Medicine Information

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.

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 can form and accumulate 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 that is low in 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.

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.

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 on how to maintain 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.

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.

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, ask your doctor or pharmacist for help.

Your doctor or pharmacist can advise you how to use the oral syringe correctly.

The recommended starting dose is 1 mg/kg body weight /day divided in 2 doses administered orally. Your doctor will prescribe the correct dose for you.

Your doctor may change your total dose from twice a day to once a day if you weigh more than 20 kg and you have been taking ORFADIN twice daily for at least 4 weeks.

Your doctor will adjust your ORFADIN dose based on your response to ORFADIN and your test results.

Take ORFADIN with food.

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 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.

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.

1.Remove the bottle from the refrigerator. Write the date when the bottle should be discarded in the space provided on the bottle label, which is 2 months after it is first opened

Figure 1A

2.Before measuring the first dose, shake the unopened 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 adaptor firmly into the neck of the bottle as far as you can (Figure 1C). Leave the plastic adaptor in place for the duration of the use of this bottle of medicine. Once the plastic adaptor is firmly in place, if you do not intend to administer the first dose now, close the bottle by screwing the child resistant cap back onto the bottle. If you intend to administer the first dose now, do not close the bottle but instead follow the instructions below from Step 3 (Figure 2C) onwards.

Follow the instructions below for all subsequent dosing.

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 syringe plunger down completely

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

Figure 2D

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

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

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.Immediately screw the child resistant cap onto the bottle. The bottle adaptor should not be removed.

10.The opened bottle of medicine may be stored at a temperature below 25°C or in the refrigerator (2-8°C) and must be discarded 2 months after opening. The date it should be discarded should be noted on the bottle label.

Clean the oral syringe IMMEDIATELY after use with water. Separate barrel and plunger and rinse both with water. Shake off excess water and leave the disassembled oral syringe to dry until it is required for the next dose.

If you are taking ORFADIN twice daily, take your first dose in the morning and 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.

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.

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.

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.

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 do regular blood and urine checks to ensure that you are on the right dose of ORFADIN 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.

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.

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.

Do not breast-feed while taking ORFADIN.

ORFADIN is not known to cause dizziness or light headedness, but ORFADIN can affect your vision if you develop certain side effects. Be careful driving or operating machinery or doing other jobs until you know how ORFADIN affects you.

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 some unwanted side effects. 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.

If you experience red eyes, or any of the following eye problems, tell your doctor immediately.

Your doctor will immediately check your eyes.

Eye problems could be a sign of inadequate dietary control.

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

Tel your doctor or pharmacist if you notice any of the following symptoms and they worry you:

rash

itching or irritating sensation of skin

redness and peeling of skin

headache

stomach pain

constipation

The above list includes the more common side effects of your medicine.

There may be other side effects affecting your liver and certain blood cell counts that your doctor will carefully monitor by doing regular blood and urine checks.

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.

Keep your oral suspension in the original bottle until it is time to take it.

If you take the oral suspension out of the bottle, it may not keep well.

Keep your unopened oral suspension bottle in its box, in a refrigerator between 2°C – 8°C. Do not freeze. Store the bottle upright.

After opening, keep the bottle in its box in a cool place where the temperature stays below 25°C, for a single period of 2 months.

Oral suspension must be discarded 2 months after opening.

To help you remember, write the discard date in the space provided on the ORFADIN bottle when you first open the bottle. 

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.

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.

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

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.

Each mL contains 4 mg nitisinone.

It also contains the following inactive ingredients:

Glycerol

Citric acid monohydrate

Hypromellose

Sodium citrate

Sodium benzoate

Flavour: Strawberry 501440 T (Proprietary Ingredient Number 11565)

Polysorbate 80

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 adaptor and three oral syringes.

ORFADIN is supplied in by:

A. Menarini Australia Pty. Ltd.

Level 8, 67 Albert Avenue

CHATSWOOD NSW 2067

Medical Information: 1800 644 542

Australian Registration Number:

ORFADIN Oral Suspension 4 mg/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 September 2020.

For the most up to date version of this leaflet, please go to www.menarini.com.au/cmi

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