Orfadin 2 mg hard capsules

The active ingredient of Orfadin is nitisinone. Orfadin is used for treatment of a rare disease called hereditary tyrosinemia type 1.

In this disease your body is unable to completely break down the amino acid tyrosine (amino acids are building blocks of our proteins), forming harmful substances. These substances are accumulated in your body.
Orfadinblocks the breakdown of tyrosine and the harmful substances are not formed.

You must follow a special diet while you are taking Orfadin, because tyrosine will remain in your body. This special diet is based on low tyrosine and phenylalanine content.

Do not take Orfadin

• if you are allergic (hypersensitive) to nitisinone or any of the other ingredients of Orfadin (see section 6).

Take special care with Orfadin

• 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.

During the treatment, blood samples will be drawn in order for your doctor to check whether the treatment is adequate and to make sure that there are no possible side effects causing blood disorders.

Your liver will be checked at regular intervals because the disease affects the liver.

Follow-up by your doctor should be performed every 6 months. If you experience any side effects, shorter intervals are recommended.

Taking other medicines
Please tell your doctor or pharmacist if you are taking or have recently taken any other medicines, including medicines obtained without a prescription.

Taking Orfadin with food and drink

If you start Orfadin treatment by taking it with food, it is recommended that you carry on taking it with food throughout your course of treatment.

Pregnancy and Breast-feeding

The safety of Orfadin has not been studied in pregnant and breast-feeding women. Please contact your doctor if you plan to become pregnant. If you become pregnant you should contact your doctor immediately.
Do not breast-feed while taking this medicine..

Driving and using machines
No studies on the effect on the ability to drive and use machines have been performed. If you experience side effects affecting the vision you should consider your ability to drive or use machines.

Nitisinone treatment should be initiated and supervised by a physician experienced in the treatment of hereditary tyrosinemia type 1 patients.

Always take Orfadin exactly as your doctor has told you. You should check with your doctor or pharmacist if you are not sure.

The usual total daily dose is 1 mg/kg body weight/day divided in 2 doses. Your doctor will adjust the dose individually.
If you have problems with swallowing the capsules, they may be opened and the powder mixed with a small amount of water or formula diet just before you take it..

If you take more Orfadinthan you should
If you have taken more of this medicine than you should contact your doctor or pharmacist, as soon as possible.

If you forget to take Orfadin

Do not take a double dose to make up for a forgotten dose. If you forget to take a dose, contact your doctor or pharmacist.

If you stop taking Orfadin
If you have the impression that Orfadin is not working properly, talk to your doctor. Do not change the dose or stop the treatment without talking to your doctor.

If you have any further questions on the use of this product, ask your doctor or pharmacist.

Like all medicines, Orfadin can cause side effects, although not everybody gets them.

If you notice any side effects relating to the eyes, talk to your doctor immediately for an eye examination.

The frequency of possible side effects listed below is defined using the following convention Very common Common Uncommon Rare Very rare Not known Affects more than 1 user in 10 Affects 1 to 10 users in 100 Affects 1 to 10 users in 1,000 Affects 1 to 10 users in 10,000 Affects less than 1 user in 10,000 Frequency cannot be estimated from the available data

Common side effects

• Various eye symptoms; conjunctivitis (inflammation in the eye), opacity and inflammation in the cornea, sensitivity to light, eye pain,
• reduced number of platelets and white blood cells, shortage of certain white blood cells (granulocytopenia).

Uncommon side effects

• Inflammation of the eyelid,
• increased number of white blood cells,
• itching, skin inflammation (exfoliative dermatitis), rash.

If any of the side effects gets serious, or if you notice any side effects not listed in this leaflet, please tell your doctor or pharmacist.

Keep out of the reach and sight of children.

Do not use Orfadin after the expiry date which is stated on the bottle and the carton after ?EXP?. The expiry date refers to the last day of that month.

Store in a refrigerator (2°C ? 8°C).

Orfadincapsules can be removed from the refrigerator for one single period of maximum 3 months and stored at a temperature not above 25°C, after which the product must be discarded.Do not forget to mark the date on the bottle, when removed from the refrigerator.

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.

What Orfadin contains
The active substance is:

• nitisinone. Each capsule contains 2 mg, 5 mg or 10 mg nitisinone.

The other ingredients are
Capsule content:
pregelatinised starch (from maize).
Capsule shell:
gelatine
titanium dioxide (E 171).
Printing ink:
iron oxide (E 172)

shellac
propylene glycol

What Orfadin looks like and contents of the pack

Orfadincapsules are white, opaque, hard gelatin capsules, imprinted with ?NTBC? and the strength ?2 mg?, ?5 mg? or ?10 mg?, in black. The capsule contains a white to off-white powder.

The capsules are packaged in plastic bottles with child resistant closures. Each bottle contains 60 capsules.

Marketing Authorisation Holder

Swedish Orphan Biovitrum International AB
SE-112 76 Stockholm
Sweden

Manufacturer

Apotek Produktion & Laboratorier AB
Prismavägen 2
SE-141 75 Kungens Kurva
Sweden

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