Elaprase ® 6 mg/3 ml Solution

ELAPRASE is used as enzyme replacement therapy to treat children and adults with Hunter syndrome (Mucopolysaccharidosis II). If you suffer from Hunter syndrome, a carbohydrate called glycosaminoglycan which is normally broken down by your body, is not broken down and slowly accumulates in various body organs (spleen, liver, lungs, heart, connective tissues and brain). This causes cells to function abnormally, thereby causing problems for various organs in your body which can lead to tissue destruction and organ malfunction and failure. ELAPRASE contains an active substance called idursulfase which works by acting as a replacement for the enzyme that is at a low level, thereby breaking down this carbohydrate in affected cells. Enzyme replacement therapy is usually administered as a long-term treatment.

You should not be given ELAPRASE: If you have experienced severe or potentially life-threatening allergic-type reactions to idursulfase or to any of the ingredients of ELAPRASE (see section 6).

Warnings and precautions:

Take special care with ELAPRASE: Inform your health care provider: If you have severe underlying airway disease. If you develop rash, itching, hives, fever, headache, high blood pressure and flushing. In the majority of cases, slowing of the infusion-rate has improved these reactions. If you develop an anaphylactoid/anaphylactic reaction, urgently inform your health care provider, as an anaphylactoid/anaphylactic reaction may be life-threatening. The nature of your genotype (a genetic make-up of all active genes in human cells, which determines one’s specific, individual characteristics) may influence your therapeutic response to this medicine, as well as your risk of developing antibodies and infusion-related side effects. In individual cases, so-called ‘neutralising antibodies’ may develop, which may diminish activity of ELAPRASE and your response to treatment. The longer-term effects of antibody development on response to treatment have not been established. Please consult your health care provider for additional information.

Other medicines and ELAPRASE:

Always tell your health care provider if you are taking any other medicine, including complementary or traditional medicines. No formal interaction studies have been conducted with ELAPRASE.

Pregnancy, breastfeeding and fertility:

The use of ELAPRASE during pregnancy is not recommended. ELAPRASE may be excreted into breast milk, therefore you should not receive ELAPRASE if you are breastfeeding your baby. Safety during pregnancy and breastfeeding has not been established. If you are pregnant or breastfeeding your baby, think you may be pregnant or are planning to have a baby, please consult your doctor, pharmacist or other health care provider for advice before receiving ELAPRASE.

Driving and using machines:

ELAPRASE may affect your ability to drive and use machines. Take care until you know how ELAPRASE affects your ability to drive or use machines.

ELAPRASE contains sodium: ELAPRASE contains 11,1 mg sodium (main component of cooking/table salt) in each vial. This is equivalent to 0,6 % of the recommended maximum daily dietary intake of sodium for an adult.

Your health care provider will administer ELAPRASE to you. ELAPRASE will be given to you under the supervision of a doctor or nurse who is knowledgeable in the treatment of Hunter syndrome or other inherited metabolic disorders. The recommended dose is an infusion of 0,5 mg for every kg you weigh. ELAPRASE has to be diluted in sodium chloride 9 mg/mL (0,9 %) solution for infusion before use. After dilution your medicine is given through a vein (drip feed). The infusion will normally last for 1 – 3 hours and be given every week. Infusion of ELAPRASE at home may be considered for patients who are tolerating their infusions well.

Use in children and adolescents

The recommended dose in children and adolescents is the same as in adults.

If you receive more ELAPRASE than you should Your doctor will supervise the administration of ELAPRASE and will closely monitor your response and condition and control the dosage. In the unlikely event of overdosage, your doctor will treat the side effects symptomatically. Consult your doctor if you think you have had an overdose of this medicine.

ELAPRASE can have side effects. Not all side effects reported for ELAPRASE are included in this leaflet. Should your general health worsen or if you experience any untoward effects while receiving ELAPRASE, please consult your health care provider for advice. If any of the following happens, stop receiving ELAPRASE and tell your doctor immediately or go to the casualty department at your nearest hospital:

• Swelling of the hands, feet, ankles, face, lips, mouth or throat, which may cause difficulty in swallowing or breathing.
• Rash or itching.
• Fainting.
• Yellowing of the skin and eyes, also called jaundice.

These are all very serious side effects. If you have them, you may have had a serious reaction to ELAPRASE. You may need urgent medical attention or hospitalisation. Tell your doctor immediately or go to the casualty department at your nearest hospital if you notice any of the following side effects:

Frequent:

• Chest pain.
• Wheezing, difficulty breathing, airway spasms.
• A decrease in oxygen levels in the body (including feeling tired, breathless, light-headed, confused and disorientated).
• Blue or purple discolouration of the skin due to low oxygen levels, also called cyanosis.
• An increase or decrease in blood pressure.
• Changes in the way your heart beats, for example, if you notice it beating faster or abnormally.

Less frequent:

• Rapid breathing.

These are all serious side effects. You may need urgent medical attention. Tell your doctor if you notice any of the following side effects:

Frequent:

• Headache.
• Dizziness, uncontrolled shaking (tremor).
• Flushing (redness of the skin, typically over the cheeks or neck).
• Abdominal pain, nausea, vomiting, diarrhoea, indigestion.
• Swollen tongue.
• Coughing.
• Skin reactions including redness of the skin, rash, hives and itching.
• Joint pain.
• Pain and swelling at injection site.
• Fever.
• Swelling of the face or limbs.

If you notice any side effects not mentioned in this leaflet, please inform your doctor or pharmacist.

Reporting of side effects: If you get side effects, talk to your doctor or pharmacist. You can also report side effects to:

• The Pharmacovigilance Unit at Sanofi: [email protected] (email) or 011 256 3700 (tel), or
• SAHPRA via the “6.04 Adverse Drug Reaction Reporting Form”, found online under SAHPRA’s publications: https://www.sahpra.org.za/Publications/Index/8

By reporting side effects, you can help provide more information on the safety of ELAPRASE.

Store in a refrigerator (2 – 8 °C). Do not freeze. For single use only. Discard any unused portion. Chemical and physical in-use stability has been demonstrated for 8 hours at 25 °C. After dilution, from a microbiological safety point of view, the diluted product should be used immediately. If not used immediately, in-use storage times and conditions prior to use are the responsibility of the user and should not be longer than 24 hours at 2 – 8 °C. STORE ALL MEDICINES OUT OF REACH OF CHILDREN.