2 mg / ml concentrate for solution for infusion solution 
idursulfase

Read all of this leaflet carefully before you start using this medicine. It contains information that is important to you.

  • Save this information, you may need to read it again.
  • If you have any further questions, ask your doctor, pharmacist, or nurse.
  • This medicine has been prescribed for you only. Do not give it to others. It can harm them, even if they show signs of illness similar to yours.
  • If you get any side effects, talk to your doctor, pharmacist, or nurse. This also applies to any side effects not mentioned in this information. See section 4.

In this leaflet: 
1. What Elaprase is and what it is used for 
2. What you need to know before using Elaprase 
3. How to use Elaprase 
4. Possible side effects 
5. How to store Elaprase 
6. Contents of the pack and other ingredients information

1. What Elaprase is and what it is used for

Elaprase is used as an enzyme replacement therapy to treat children and adults with Hunter’s syndrome (mucopolysaccharidosis II) when the level of enzyme et iduronate-2-sulphatase in the body is lower than normal and helps to improve the symptoms of the disease. If you suffer from Hunter’s syndrome, your body does not break down a carbohydrate called glycosaminoglycan, which is normally broken down in the body, but it accumulates slowly in various cells in your body. This causes these cells to function abnormally and thereby cause problems in various organs in your body, which can lead to tissue destruction, organs not functioning properly, and organ failure. Glycosaminoglycan usually accumulates in the spleen, liver, lungs, heart, and connective tissue. In some patients, glucosaminoglycan also accumulates in the brain. Elaprase contains an active substance called idursulfase which acts by acting as a replacement for the enzyme which is present at a low level, thereby breaking down this carbohydrate in the affected cells.

Enzyme replacement therapy is usually given as long-term treatment.

2. What you need to know before using Elaprase

Do not use Elaprase

  • if you have had severe or potentially life-threatening allergic reactions to idursulfase or any of the other ingredients of this medicine (listed in section 6) and these cannot be controlled with appropriate medical treatment.

Warnings and cautions

Talk to your doctor or nurse before using this medicine.

If you are being treated with Elaprase, you may experience side effects during and after an infusion (see section 4, Possible side effects ). The most common symptoms are itching, rash, hives, fever, headache, high blood pressure, and swelling ( redness). For the most part, you can continue with this medicine even if these symptoms occur. If you have any allergic side effects after taking this medicine, contact your doctor immediately. You can get other medicines such as antihistamines and corticosteroids to treat or prevent allergic reactions.

If severe allergic reactions occur, your doctor will immediately discontinue the infusion and start giving you appropriate treatment. You may need to be hospitalized.

Your response to treatment with this drug, as well as the risk of developing antibodies and infusion-related side effects, may be affected by the type of genetic predisposition (a genetic makeup of all active genes in human cells, which determines a person’s specific, individual characteristics). . In some cases, so-called neutralizing antibodies may be formed, which may reduce the activity of Elaprase and your response to treatment. The long-term effects of antibody development on treatment response have not been established. Contact your doctor for more information.

Record keeping

To facilitate the traceability of biological drugs, the name and manufacturing batch number of the drug must be documented. Talk to your healthcare provider if you are unsure.

Other medicines and Elaprase

There is no known interaction between this drug and other drugs.

Tell your doctor, pharmacist, or nurse if you are taking, have recently taken, or might take any other medicines.

Pregnancy and breastfeeding

If you are pregnant or breast-feeding, think you may be pregnant, or are planning to have a baby, ask your doctor or pharmacist for advice before taking this medicine.

Driving and using machines

This medicine has no or negligible effect on the ability to drive and use machines.

Elaprase contains sodium

This medicine contains 11.1 mg of sodium (the main ingredient in common salt/table salt) per vial. This corresponds to 0.6% of the maximum recommended daily intake of sodium for adults.

3. How to use Elaprase

Always use this medicine exactly as your doctor has told you. Consult a doctor if you are unsure.

You will receive this medicine under the supervision of a doctor or nurse with knowledge in the treatment of Hunter’s syndrome or other hereditary metabolic disorders.

The recommended dose for infusion is 0.5 mg (half a milligram) for each kg you weigh.

Elaprase must be diluted with sodium chloride 9 mg / ml (0.9%) solution for infusion before use. After dilution, this drug is given into a vein (infused). Infusion one normally lasts for 1 to 3 hours and is given weekly.

Use for children and adolescents

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

If you use more Elaprase then you should

Contact your doctor if you have received an overdose of this medicine.

If you forget to use Elaprase

If you have missed an Elaprase infusion, consult your doctor.

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

4. Possible side effects

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

Most side effects are mild to moderate and are associated with infusion one, but some side effects can be serious. Over time, the number of reactions associated with infusion decreases.

If you have difficulty breathing, with or without bluish skin, contact a doctor immediately and seek medical attention.

Very common side effects (may affect more than 1 in 10 people) are:

  • Headache
  • Hemorrhoids ( redness )
  • Shortness of breath, wheezing
  • Abdominal pain, nausea, vomiting, frequent and/or loose stools
  • Chest pain
  • Hives, rash, itching, reddening of the skin
  • Fever
  • Infusion-related reaction (see section “Warnings and precautions”)

Common side effects (may affect up to 1 in 10 people) are:

  • Dizziness, tremors
  • Fast heartbeat, irregular heart rate, bluish skin
  • Elevated blood pressure, decreased blood pressure
  • Difficulty breathing, coughing, low blood oxygen levels
  • Swollen tongue, indigestion
  • Joint pain
  • Swelling at the infusion site, swollen extremities, facial swelling

Uncommon side effects (may affect up to 1 in 100 people) are:

  • Faster breathing

Side effects with no known frequency (cannot be calculated from the available data) are:

  • Severe allergic reactions

5. How to store Elaprase

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 label and carton after EXP. The expiration date is the last day of the specified month.

Store in a refrigerator (2 -C-8 ºC)

Do not freeze

Do not use this medicine if the liquid is discolored or contains foreign particles.

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.

6. Contents of the packaging and other information

Content declaration

  • The active substance is idursulfase, which is a form of the human enzymeiduronate-2-sulfatase. Idursulfase is produced in a human cell line using genetic engineering (genetic information is introduced into human cells in the laboratory and these then produce the desired product).

Each vial of Elaprase contains 6 mg of idursulfase. Each ml contains 2 mg of idursulfase.

  • The other ingredients are polysorbate 20, sodium chloride, disodium phosphate heptahydrate, sodium dihydrogen phosphate monohydrate, and water for injections.

What the medicine looks like and the contents of the pack

This medicine is a concentrate for solution for infusion. It comes in a glass vial as a clear to slightly opalescent, colorless solution.

Each vial contains 3 ml of concentrate for solution for infusion.

Elaprase is delivered in packs of 1, 4, and 10 vials per carton. Not all pack sizes may be marketed.

Manufacturer

Shire Pharmaceuticals Ireland Limited

Block 2 & 3 Miesian Plaza

50 – 58 Baggot Street Lower

Dublin 2

Ireland

This leaflet was last modified 05/2020

This medicinal product has been authorized under “Exceptional Authorization” rules. This means that it has not been possible to obtain complete information about this medicine as the disease is rare. 
Every year, the European Medicines Agency reviews all new information that may have come to light about this medicine and updates this leaflet when necessary.

Muhammad Nadeem

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