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Replagal 1 mg/ml concentrate for solution for infusion

Last Updated on eMC 15-Oct-2014 View changes  | Shire Human Genetic Therapies Contact details

Black Triangle icon This medicinal product is subject to additional monitoring. This will allow quick identification of new safety information.

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Below is a text only representation of the Patient Information leaflet. The original may contain images or tables and can be viewed in PDF format using the link to the left. This PIL may be available from the RNIB in large print, Braille or audio CD. For further information please call the RNIB Medicine Leaflet line on 0800 198 5000. The product code(s) for this leaflet is/are: EU/1/01/189/001, EU/1/01/189/002, EU/1/01/189/003.



Replagal 1 mg/ml concentrate for solution for infusion

Package leaflet: Information for the user

Replagal 1 mg/ml concentrate for solution for infusion

Agalsidase alfa

▼ This medicine is subject to additional monitoring. This will allow quick identification of new safety information. You can help by reporting any side effects you may get. See the end of section 4 for how to report side effects.

Read all of this leaflet carefully before you start taking this medicine because it contains important information for you.

  • Keep this leaflet. 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 pass it on to others. It may harm them, even if their signs of illness are the same as yours.
  • If you get any side effects, talk to your doctor or pharmacist. This includes any possible side effects not listed in this leaflet. See section 4.

What is in this leaflet:

1. What Replagal is and what it is used for
2. What you need to know before you are given Replagal
3. How Replagal is given
4. Possible side effects
5. How to store Replagal
6. Contents of the pack and other information

1. What Replagal is and what it is used for

The active substance in Replagal is agalsidase alfa (1mg/ml). Agalsidase alfa is a form of the human enzyme α-galactosidase. It is produced by switching on the gene for α-galactosidase A in cells. The enzyme is then removed from the cells and made into a sterile concentrate for solution for infusion.

Replagal is used to treat adult patients, as well as adolescents and children from the age of 7, with confirmed diagnosis of Fabry Disease. It is used as long-term enzyme replacement therapy when the level of enzyme in the body is absent or lower than normal as in Fabry Disease.

After 6 months of therapy Replagal significantly reduced pain in patients when compared to placebo (dummy) treated patients. Replagal reduced left ventricle mass in treated patients compared to placebo treated patients. These results suggest the symptoms of the disease are improving or the disease is becoming stable.

2. What you need to know before Replagal is given

You must not be given Replagal

  • if you are allergic to agalsidase alfa or any of the other ingredients of this medicine (listed in section 6).

Warnings and precautions

Talk to your doctor or pharmacist before Replagal is used.

If you notice any of these effects during or after an infusion you should tell your doctor immediately:

  • high fever, chills, sweating, fast heart rate;
  • vomiting;
  • light-headedness;
  • hives;
  • swelling in your hands, feet, ankles, face, lips, mouth or throat which may cause difficulty in swallowing or breathing.

Your doctor may stop the infusion temporarily (5 –10 min) until the symptoms go away and then begin the infusion again.

Your doctor may also treat the symptoms with other medicines (antihistamines or corticosteroids).

Most of the time you can still be given Replagal even if these symptoms occur.

If you experience a severe allergic (anaphylactic-type) reaction, the administration of Replagal will be immediately discontinued and an appropriate treatment will have to be initiated by your doctor.

If treatment with Replagal makes your body produce antibodies this will not stop Replagal from working and the antibodies may disappear with time.

If you have advanced renal disease, you may find that your Replagal treatment has a limited effect on your kidneys. Talk to your doctor or pharmacist before using Replagal.

Children

The experience in children 0-6 years old is limited and therefore no dose can be recommended for this age group.

Other medicines and Replagal

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

Tell your doctor if you use any medicines containing chloroquine, amiodarone, benoquin or gentamicin. There is a theoretical risk of decreased agalsidase alfa activity.

Pregnancy and breast feeding

Very limited clinical data on pregnancies exposed to Replagal have shown no adverse effects on the mother and newborn child.

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

You may drive and use machines whilst on Replagal.

3. How Replagal is given

This medicine should be applied and supervised by appropriately trained personnel, who will also calculate the dose that you will be given.

The recommended dose is an infusion of 0.2 mg for every kg you weigh. This would be about 14 mg or 4 vials (glass bottles) of Replagal for an average size (70 kg) individual.

Use in children and adolescents

For children and adolescents 7-18 years old a dose of 0.2 mg/kg every other week may be used.

Children and adolescents may be more likely than adults to experience an infusion related reaction. Tell your doctor if you experience any side effects whilst having the infusion.

Method of administration

Replagal has to be diluted in 9 mg/ml (0.9%) sodium chloride solution before use. After dilution Replagal is given in a vein. This will usually be in your arm.

The infusion will be given every two weeks.

Each time you are treated it will take 40 minutes for Replagal to be given to you in a vein.

Your treatment will be supervised by a doctor who specialises in the treatment of Fabry Disease.

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

4. Possible side effects

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

If you experience a severe allergic (anaphylactic-type) reaction, the administration of Replagal will be immediately discontinued and an appropriate treatment will have to be initiated by your doctor.

Most side effects are mild to moderate. About 1 out of 7 patients (frequency “very common”) may have a reaction during or following an infusion of Replagal (infusion related reaction).

These effects include chills, headache, nausea, fever, facial flushing (redness), tiredness, low blood pressure, unsteadiness, sweating, difficulty breathing, itching, shaking, cough and vomiting. However some effects may be serious and may need treatment. Infusion related reactions involving the heart including heart rhythm problems, heart muscle ischemia and heart failure, may occur in patients with Fabry disease involving the heart structures (frequency “not known” (cannot be estimated from the available data)). Your doctor may stop the infusion temporarily (5 - 10 min) until the symptoms go away and then begin the infusion again. Your doctor may also treat the symptoms with other medicines (antihistamines or corticosteroids). Most of the time you can still be given Replagal even if these symptoms occur.

List of other side effects:

Very common: may affect more than 1 in 10 people

  • general pain or discomfort.

Common: may affect up to 1 in 10 people:

  • tingling or numbness or pain in fingers or toes, change in the taste of food, eyes tearing, blink reflex abnormal, ears ringing, shakes, prolonged sleep
  • palpitations, increased heart rate, increased blood pressure
  • cough, chest pain or tightness, hoarseness, sore or tight throat, sticky throat secretions, runny nose, cold symptoms
  • vomiting, abdominal pain or discomfort, diarrhoea
  • acne, red or itchy or mottled skin, rash at the infusion site
  • back or limb pain, muscle pain, joint pain, muscle and bone discomfort, swelling of the extremities or joints
  • feeling cold or hot, flu-like symptoms, feeling sick, feeling lack of energy

Uncommon: may affect up to 1 in 100 people:

  • severe allergic (anaphylactic-type) reaction

Children and adolescents

Side effects reported in children were, in general, similar to those reported in adults.

However, infusion related reactions (fever, difficulty breathing, chest pain) and pain aggravated occurred more frequently.

Reporting of side effects

If you get any side effects, talk to your doctor or pharmacist. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the national reporting system listed in Appendix V. By reporting side effects you can help provide more information on the safety of this medicine.

5. How to store Replagal

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 expiry date refers to the last day of that month.

Store in a refrigerator (2°C – 8°C).

Do not use Replagal if you notice that there is discolouration or other foreign particles present.

Do not throw away any medicines via wastewater or household waste. Ask your pharmacist how to throw away medicines you no longer use. These measures will help to protect the environment.

6. Contents of the pack and other information

What Replagal contains

  • The active substance is agalsidase alfa. Each ml of Replagal contains 1 mg of agalsidase alfa.
  • The other ingredients are: Sodium phosphate monobasic, monohydrate
    Polysorbate 20
    Sodium chloride
    Sodium hydroxide
    Water for injections

What Replagal looks like and contents of the pack

Replagal is a concentrate for solution for infusion. Your medicine is available in vials containing 3.5 mg/ 3.5 ml of agalsidase alfa. Pack sizes of 1, 4 or 10 vials are available.

Not all pack sizes may be marketed.

Marketing Authorisation Holder

Shire Human Genetic Therapies AB
Svärdvägen 11 D
182 33 Danderyd
Sweden
Tel: +46 8 5449 6400
Fax: +46 8 5449 6429

Manufacturer

Shire Human Genetic Therapies AB
Åldermansgatan 13
227 64 Lund
Sweden

This leaflet was last revised in 07/2014

This medicine has been authorised under “exceptional circumstances”.

This means that because of the rarity of this disease it has been impossible to get complete information on this medicine.

The European Medicines Agency will review any new information on the medicine every year and this leaflet will be updated as necessary.

Other sources of information

Detailed information on this medicine is available on the European Medicines Agency web site: http://www.ema.europa.eu There are also links to other websites about rare diseases and treatments.

Company contact details

Shire Human Genetic Therapies

Company image
Address

Hampshire International Business Park, Crockford Lane, Basingstoke, Hampshire, RG24 8EP, UK

Medical Information Direct Line

0800 055 6614

Telephone

+44 (0)1256 894000

Medical Information e-mail

Before you contact this company: often several companies will market medicines with the same active ingredient. Please check that this is the correct company before contacting them. Why?

Active ingredients

agalsidase alfa

Legal categories

POM - Prescription Only Medicine

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