100 U / ml concentrate for solution for infusion. 
Leonidas

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 or pharmacist.
  • 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 or pharmacist. This also applies to any side effects not mentioned in this information. See section 4.

In this leaflet you will find information about: 
1. What Aldurazyme is and what it is used for 
2. What you need to know before you are given Aldurazyme 
3. How Aldurazyme is given 
4. Possible side effects 
5. How Aldurazyme should be stored 
6. Contents of the packaging and other information 

1. What Aldurazyme is and what it is used for

Aldurazyme is used to treat patients with MPS I (mucopolysaccharidosis I). It is given to treat the non-neurological symptoms of the disease.

People with MPS I either have no or too low a level of an enzyme called α-L-iduronidase, which breaks down certain substances (glycosaminoglycans) in the body. Therefore, these substances are not broken down and processed by the body as they should. They accumulate in many of the body’s tissues, giving symptoms of MPS I.

Aldurazyme is an artificial enzyme called laronidase. This can replace the natural enzyme that is missing in the disease MPS I.

2. What you need to know before you are given Aldurazyme

Aldurazyme should not be given

If you are allergic (hypersensitive) to laronidase or any of the other ingredients of this medicine (listed in section 6).

Warnings and cautions

Talk to your doctor before using Aldurazyme. If you are being treated with Aldurazyme, you may develop infusion-associated reactions. An infusion-associated reaction is a side effect that occurs during infusion one or until the end of the infusion day (see section 4 “Possible side effects”). Some of these side effects can be serious. When you get such a reaction after administering Aldurazyme, you should contact your doctor immediately.

If these reactions occur, the infusion of Aldurazyme should be stopped immediately and your doctor will start appropriate treatment. 
These reactions can be particularly severe if you already have a narrowing of the upper respiratory tract associated with MPS I. 
You may be given other medicines such as antihistamines and paracetamol to prevent allergic reactions.

Other medicines and Aldurazyme

Tell your doctor if you are taking medicines containing chloroquine or procaine as there is a potential risk that the effect of Aldurazyme will be reduced.

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

Pregnancy, breastfeeding, and fertility

There is insufficient experience with Aldurazyme in pregnant women. Aldurazyme should not be given during pregnancy unless clearly necessary.

It is unknown if Aldurazyme passes into breast milk. You should not breast-feed during treatment with Aldurazyme.

There is no information available regarding the effects of Aldurazyme on fertility.

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

The effects on driving ability and the use of machines have not been studied.

Aldurazyme contains sodium

This medicine contains 30 mg sodium (the main ingredient in cooking/table salt) per vial. This corresponds to 1.5% of the recommended maximum daily intake of sodium for an adult.

3. How Aldurazyme is given

Instructions for use – dilution, and administration The 
concentrate for solution for infusion must be diluted before administration and is for intravenous use (see information for doctors and healthcare professionals). Aldurazyme should be administered in a suitable clinical setting where resuscitation equipment to deal with acute risk situations is readily available.

Posology The 
the recommended dose for Aldurazyme is 100 U / kg body weight given once a week as an intravenous infusion. The initial infusion rate of 2 U / kg/hour can be increased gradually every fifteen minutes, if the patient tolerates it, to a maximum of 43 U / kg/hour. The total administration volume should be added over approximately 3-4 hours.

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

If you take more Aldurazyme than you should:

No case of an overdose of Aldurazyme has been reported.

If you miss an infusion of Aldurazyme:

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

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

4. Possible side effects

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

Side effects were mainly observed during or shortly after receiving the drug (infusion-associated reactions). If you experience any such reaction, tell your doctor immediately. The number of side effects decreased the longer the patients received Aldurazyme. Most side effects were mild or moderate in severity. However, severe systemic allergic reaction ( anaphylactic reaction ) has been observed in patients during or up to three hours after infusion of Aldurazyme. Some of the symptoms of these severe allergic reactions were life-threatening and included significant difficulty breathing, swelling of the throat, low blood pressure, and low oxygen content in the body. Some patients with a previous history of severe upper respiratory tract and lung disease caused by MPS I suffered severe reactions, including bronchospasm(narrowing of the airways), respiratory arrest, and swelling of the face. The frequency of bronchospasm and respiratory arrest is unknown. Severe allergic reactions (anaphylactic reactions) and swelling of the face are considered to be common side effects and may occur in up to 1 in 10 users.

Very common symptoms (may affect more than 1 in 10 people) that are not serious include headache, nausea, abdominal pain, rash, joint disease, joint pain, back pain, pain in arms or legs, redness, fever, chills, increased heart rate, increased blood pressure and reaction at the infusion site.

Other side effects include the following:

Common (may affect up to 1 in 10 people)

  • elevated body temperature
  • knitting
  • dizziness
  • cough
  • difficulty breathing
  • vomiting
  • diarrhea
  • swelling of the throat
  • hives
  • itching
  • hair loss
  • cold sweats, heavy sweating
  • muscle pain
  • pallor
  • cold hands or feet
  • the feeling of heat, feeling of cold
  • fatigue
  • flu-like illness
  • restlessness

Has been reported (occurs in an unknown number of users)

  • bluish skin (due to low oxygen content in the blood)
  • faster breathing
  • reddening of the skin
  • Leakage of the drug into tissue one at the infusion site may cause swelling or redness
  • swelling in arms and/or legs

Reporting of side effects

If you get any side effects, talk to your doctor, pharmacist, or nurse. This includes any possible side effects not listed in this leaflet. You can also report side effects directly to the Medical Products Agency, www.lakemedelsverket.se. By reporting side effects, you can help increase drug safety information. 

5. How to store Aldurazyme

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

Unopened vials: 
Store in a refrigerator (2 ° C – 8 ° C).

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 laronidase. One ml of the solution in the vial contains 100 U of laronidase. Each vial of 5 ml contains 500 U laronidase.
  • The other ingredients are: sodium chloride, monobasic sodium phosphate monohydrate, dibasic sodium phosphate heptahydrate, polysorbate 80, water for injections

What the medicine looks like and the contents of the pack

Aldurazyme is supplied as a concentrate for solution for infusion. It is a solution that is transparent to slightly opalescent, and colorless to pale yellow.

Pack size: 1, 10, and 25 vials per carton. Not all pack sizes may be marketed.

Marketing Authorization Holder and Manufacturer

Marketing Authorization Holder 
Genzyme Europe BV, Paasheuvelweg 25, 1105 BC Amsterdam, The Netherlands.

Manufacturer 
Genzyme Ltd., 37 Hollands Road, Haverhill, Suffolk CB9 8PU, UK.

Genzyme Ireland Ltd., IDA Industrial Park, Old Kilmeaden Road, Waterford, Ireland

For any information about this medicine, please contact the local representative of the Marketing Authorization Holder:

Sanofi AB

Tel: +46 (0) 8 634 50 00

Other sources of information 
Further information on this medicine is available on the European Medicines Agency There are also links to other websites regarding rare diseases and treatments.

This leaflet is available in all EU / EEA languages ​​on the European Medicines Agency’s website.

This leaflet was last modified on 11/12/2020.

The following information is intended for healthcare professionals only:

Each vial of Aldurazyme is intended for single use only. The concentrate for solution for infusion must be diluted with sodium chloride 9 mg/ml (0.9%) solution for infusion using an aseptic technique. It is recommended that the diluted Aldurazyme solution be administered to patients using an infusion set equipped with a 0.2 µm in-line filter.

From a microbiological safety point of view , the drug should be used immediately. If not used immediately, the in-use solution should not be stored for more than 24 hours at 2 ° C-8 ° C, provided that dilution has taken place under controlled and validated aseptic conditions.

Aldurazyme must not be mixed with other medicines in the same infusion.

Preparation of Aldurazyme infusion (using the aseptic technique)

  • Determine the number of vials to be diluted based on the weight of the individual patient. Remove the required vials from the refrigerator approximately 20 minutes in advance to allow them to reach room temperature (below 30 ° C).
  • Before dilution, visually inspect each vial for particulate matter and discoloration. The clear to slightly opalescent and colorless to the pale yellow solution must be free of visible particles. Do not use vials with particles or discoloration.
  • Determine the total infusion volume based on the weight of the individual patient, either 100 ml (if the bodyweight is less than or equal to 20 kg) or 250 ml (if the body weight is more than 20 kg) sodium chloride 9 mg/ml (0.9%) solution for infusion, solution.
  • Withdraw and discard the same volume of sodium chloride 9 mg/ml (0.9%) solution for infusion from the infusion bag as the total volume of Aldurazyme to be added.
  • Withdraw the required volume from the Aldurazyme vials and combine the drawn volumes.
  • Add the combined volumes of Aldurazyme to sodium chloride 9 mg/ml (0.9%) solution for infusion.
  • Gently mix the infusion solution.
  • Before use, visually inspect the solution for particles. Only transparent and colorless solutions without visible particles may be used.

Any unused product or waste material should be disposed of in accordance with local requirements.

Muhammad Nadeem

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