1 mg / ml concentrate for solution for infusion .
agalsidas alfa
What Replagal is and what it is used for
The active substance in Replagal is agalsidase alfa (1 mg/ml). An agalsidase alfa is a form of the enzyme α-galactosidase found in humans. It has been made by activating the α-galactosidase A gene in cells. The enzyme is then removed from the cells and converted to a sterile concentrate for solution for infusion.
Replagal is used to treat both adult patients and adolescents and children from the age of 7 with a confirmed diagnosis of Fabry’s disease. It is used as a long-term enzyme replacement therapy when the enzyme is missing in the body or its level is lower than normal, which is the case with Fabry’s disease.
After 6 months of treatment, Replagal significantly reduced patients’ pain compared to placebo-treated patients. Replagal decreased left ventricular mass in treated patients compared with placebo-treated patients. These results indicate that the disease symptoms improve or that the disease becomes stable.
What you need to know before you get Replagal
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 cautions
Talk to your doctor or pharmacist before using Replagal.
If you notice any of these effects during or after an infusion, tell your doctor immediately:
- high fever, chills, sweating, fast heart rate
- vomiting
- feeling drunk
- hives
- swollen hands, feet, ankles, face, lips, mouth, or throat that may make it difficult to swallow or breathe
Your doctor may temporarily stop the infusion (5-10 minutes) until the symptoms go away and then start the infusion again.
Your doctor may also treat the symptoms with other medicines ( antihistamines or corticosteroids ). For the most part, you can continue with Replagal even if these symptoms occur.
If you get severe allergic reactions (anaphylactic reactions), the administration of one of Replagal must be stopped immediately and appropriate treatment initiated by your doctor.
If treatment with Replagal causes your body to make antibodies, this will not stop the action of Replagal and the antibodies may disappear over time.
If you have advanced kidney disease, you may notice that Replagal treatment may affect your kidneys to a limited extent. Talk to your doctor or pharmacist before using Replagal.
Children
The experience for children aged 0-6 years is limited, and therefore no dosage 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 are taking any medicines that contain chloroquine, amiodarone, booking, or gentamicin. There is a theoretical risk of decreased alpha activity of agalsida.
Pregnancy and breastfeeding
Very limited clinical data on pregnancies in which Replagal has been exposed have not shown any adverse effects on the mother or the newborn.
If you are pregnant or breastfeeding, 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 can drive and use machines during treatment with Replagal.
Replagal contains sodium
This medicine contains 14.2 mg sodium (the main ingredient in table salt/table salt) per vial. This corresponds to 0.7% of the highest recommended daily intake of sodium for adults.
Documentation
To facilitate the traceability of biological drugs, the name and manufacturing batch number of the drug must be documented by your healthcare provider. Talk to your healthcare provider if you are unsure.
How to get Replagal
This medicine should be administered and supervised by staff with adequate training, who will also calculate the dose you should receive.
The recommended dose for infusion is 0.2 mg/kg body weight. This means about 14 mg or 4 vials of Replagal for a person with average body weight (70 kg).
Use for children and adolescents
For children and adolescents aged 7-18 years, a dose of 0.2 mg/kg every two weeks can be used.
Children and adolescents may be more likely to have an infusion-related reaction than adults. Tell your doctor if you get any side effects while you are receiving an infusion.
Method of administration
Replagal must be diluted with sterile 9 mg/ml (0.9%) sodium chloride solution before use. After dilution, Replagal is given into a vein. This happens mostly in the arm.
The infusions are given every two weeks.
At each treatment, one infusion of Replagal takes at least 40 minutes. Your treatment is monitored by a doctor who specializes in treating Fabry’s disease.
If you have any further questions on the use of this product, ask your doctor or pharmacist.
Possible side effects
Like all medicines, this medicine can cause side effects, although not everybody gets them.
If you get a severe allergic reaction (of the anaphylactic type), the administration of Replagal will be stopped immediately, and adequate treatment must be instituted by your doctor.
Most side effects are mild to moderate. More than 1 in 10 people (frequency “very common”) may experience a reaction during or after a Replagal infusion (infusion-related reaction). These reactions include chills, headache, nausea, fever, fatigue, instability, difficulty breathing, shaking, coughing, and vomiting. However, some reactions can be severe and require treatment. Infusion-related reactions involving the heart, such as myocardial ischemia (lack of blood supply to the heart muscle) and heart failure, may occur in patients with Fabry disease affecting the structures of the heart (“no known frequency” (can not be calculated from available data)). Your doctor may stop the infusion one temporarily (5 10 min) until the symptoms have disappeared and then start infusion one again. Your doctor may also treat the symptoms with other medicines ( antihistamines or corticosteroids ). In most cases, you can still get Replagal even if these symptoms occur.
List of other side effects:
Very common: may affect more than 1 in 10 people
- swelling in tissue one (eg bone, arm)
- tingling or numbness or pain in fingers or toes
- ringing in the ears
- palpitation
- sore throat
- abdominal pain, diarrhea
- rash at the infusion site
- back or extremity pain, muscle aches, joint pain
- chest pain, cold symptoms, fever, nausea
Common: may affect up to 1 in 10 people:
- changes in how the food tastes, prolonged sleep
- the increased amount of tear fluid
- increased ringing in the ears
- increased heart rate, problems with heart rhythm
- high blood pressure, low blood pressure, flushing
- hoarseness or congestion in the throat, runny nose
- the feeling of discomfort in the abdomen
- acne, red, itchy or flammable skin, heavy sweating
- feeling discomfort in muscles or bones, swelling in extremities or joints
- hypersensitivity
- the feeling of tightness in the chest, increased lack of energy, feeling of heat or cold, flu-like symptoms, feeling of discomfort
Uncommon: may affect up to 1 in 100 people:
- severe allergic reaction (of anaphylactic type)
- abnormal blink reflex
- increased heart rate
- low oxygen level in the blood and tough mucus in the throat
- altered sense of smell
- accumulation of fluid under the skin which can lead to swelling of body parts, net-like discoloration of the skin e.g. benet
- feeling of heaviness
- rash at the injection site
Children and young people
The adverse reactions reported in children were generally similar to those reported in adults. However, infusion-related reactions (fever, difficulty breathing, chest pain) and aggravated pain were more common.
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 expiration date is the last day of the specified month.
Store in a refrigerator (2 ° C-8 ° C).
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.
Contents of the pack and other information
Content declaration
– The active substance is alpha agalsidase. Each ml of Replagal contains 1 mg of agalsidase alfa.
| – The other ingredients are: | monobasic sodium phosphate, monohydrate |
| polysorbate 20 | |
| sodium chloride | |
| sodium hydroxide | |
| water for injections |
Replagal contains sodium. See section 2.
What the medicine looks like and the contents of the pack
Replagal is a concentrated solution for infusion. Your medicine is available in vials containing 3.5 mg / 3.5 ml agalsidase alfa. Pack sizes of 1, 4, or 10 vials are available.
Not all pack sizes may be marketed.
Marketing Authorisation Holder
Takeda Pharmaceuticals International AG Ireland Branch
Block 3 Miesian Plaza
50 – 58 Baggot Street Lower
Dublin 2
Ireland
Tel: +800 66838470
Email: medinfoEMEA@takeda.com
Manufacturer
Shire Pharmaceuticals Ireland Limited
Block 2 & 3 Miesian Plaza
50 – 58 Baggot Street Lower
Dublin 2
Ireland