agalsidase alfa ghu 3.5 mg / 3.5 mL concentrated injection vial
Consumer Medicine Information
What is in this leaflet
This leaflet answers some common questions about REPLAGAL. It does not contain all
of the available information. It does not take the place of talking to your doctor
Please read this leaflet before you start using REPLAGAL.
All medicines have risks and benefits. Your doctor has weighed the possible risks
of using REPLAGAL against the expected benefits.
If you have any concerns about using REPLAGAL, ask your doctor or pharmacist.
Keep this leaflet. You may want to read it again.
What is REPLAGAL used for
REPLAGAL is used to treat Fabry Disease. REPLAGAL is given as enzyme replacement therapy
when the level of enzyme in the body is lower than normal as in Fabry Disease.
Agalsidase alfa is a form of the human enzyme α-galactosidase A. 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.
Before REPLAGAL is given to you, it is mixed with 0.9% sodium chloride intravenous
solution (saline). The prepared solution will be infused into a vein in your arm over
a 40 minute period.
Your doctor may have prescribed REPLAGAL for another use. Ask your doctor if you have
any questions about why REPLAGAL has been prescribed for you.
REPLAGAL is not addictive.
REPLAGAL is not expected to affect your ability to drive a car or operate machinery.
REPLAGAL is only available on a doctor's prescription.
Before you use REPLAGAL
When you must not use it
Do not use REPLAGAL if:
You are allergic (hypersensitive) to agalsidase alfa or any of the other ingredients
The package is torn or shows signs of tampering
The expiry date (EXP) printed on the pack has passed. If you use this medication after
the expiry date has passed, it may not work as well
Before you start to use it
Tell your doctor if:
1. You think you are allergic to any of the ingredients contained in REPLAGAL.
2. You have previously used REPLAGAL and have had any unusual reactions such as skin
rash or "flu-like symptoms" to any injections of REPLAGAL in the past
3. You are pregnant or intend to become pregnant, or are breastfeeding or wish to breastfeed
Your doctor will discuss the risks and benefits of using REPLAGAL if you are pregnant
4. You are taking other medicines, including any that you buy without a prescription
from your pharmacy, supermarket or health food shop
If you have not told your doctor about any of the above, tell him/her before you use
REPLAGAL has not been studied in children less than 6 years old, and only limited
information is available in children 7-18 years of age.
How is REPLAGAL given
REPLAGAL has to be diluted in 0.9% sodium chloride intravenous solution (saline) before
use. After dilution REPLAGAL is given in a vein. This will usually be in your arm.
The usual 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.
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 your vein. Your treatment will be supervised by a doctor who specialises in the
treatment of Fabry Disease. You may need to be treated with REPLAGAL long term.
If your conditions have been stabilised in a controlled hospital setting and you are
tolerating your infusion well, a doctor or nurse may administer REPLAGAL infusion
to you at your home.
If you forget to use it
If you miss an infusion, the enzyme levels which REPLAGAL is intended to replace
will remain low. Consult your doctor and he/she will decide when you need your next
If you stop using REPLAGAL, the level of the enzyme which is responsible for Fabry
Disease will remain low and the symptoms of the disease will not be treated.
If you use too much (overdose)
There is no experience with overdose of REPLAGAL. In the unlikely event that this
may occur, your doctor will arrange the appropriate care.
Telephone your doctor or the Poisons Information Centre (Australia: 13 11 26; New
Zealand: 0800 POISON or 0800 764766) for advice if you think that you or anyone else
may have used too much REPLAGAL. Do this even if there are no signs of discomfort
While you are using REPLAGAL
Things you must do
Make sure that all of your doctors and pharmacists know you are using REPLAGAL. Remind
them if any new medicines are about to be started.
Things that you must not do
Do not use any other medications while using REPLAGAL unless you have discussed this
with your doctor or pharmacist. This includes medicines you can buy without a prescription
from a pharmacy, supermarket or health food shop.
Do not use REPLAGAL to treat any complaint other than that directed by your doctor.
It may not be safe to use REPLAGAL for another complaint.
REPLAGAL should only be used by the person for whom it was prescribed. Do not give
REPLAGAL to someone else even if his/her symptoms are the same. It may not be safe
for another person to use REPLAGAL.
Tell your doctor or pharmacist as soon as possible if you do not feel well while you
are using REPLAGAL.
Like all medicines, REPLAGAL can have side effects. The most commonly reported side
effects reported in clinical trials of REPLAGAL were allergic reaction, dizziness,
fever, flushing and nausea occurring in approximately 10% of patients. Most side effects
are mild to moderate and many were associated with infusion reactions which are explained
in the following paragraph. However some may be serious and may require treatment.
13.7% of patients treated with REPLAGAL in clinical studies have experienced reactions
during or following infusion of REPLAGAL. Most reactions were mild. The most common
symptoms were chills, headache, nausea, fever, facial flushing (redness) and tiredness.
These reactions have generally occurred 2-4 months after the start of treatment and
then decreased over time. However, they may begin more than 1 year after the start
of treatment. More serious reactions with fever, chills, fast heart rate, hives, vomiting,
swelling of the throat and tongue causing difficulty swallowing and breathing, have
been reported uncommonly.
Most of the time you can still be given REPLAGAL even if these symptoms occur. If
you experience an allergic side effect following the administration of REPLAGAL, you
should immediately contact your doctor.
If symptoms occur during your infusion:
Your doctor or nurse may stop the infusion temporarily (5-10 min) until the symptoms
go away and then begin the infusion again
Your doctor or nurse may also treat the symptoms with other medicines (antihistamines
Tell your doctor immediately if you notice any of the following:
swelling of the hands, feet, ankles, face, lips, mouth or throat which may cause difficulty
in swallowing or breathing
Tell your doctor as soon as possible if you notice any of the following:
signs of infection
shortness of breath
changes in the way your heart beats (for example, if you notice it beating faster)
pain or tenderness in chest, muscles or joints
itching or rash
Other common side effects (occurring in less than 1 in 10 treated patients) include
tingling or numbness or pain in fingers or toes, change in the taste of food, eyes
tearing, ears ringing, shakes, prolonged sleep
palpitations, increased blood pressure
cough, chest tightness, hoarseness, sore or tight throat, sticky throat secretions,
vomiting, abdominal pain/discomfort, diarrhoea
acne, red or itchy or mottled skin, rash at the infusion site
back or limb pain, swelling of the extremities or joints
feeling cold or hot, general pain/discomfort, flu-like symptoms, generally feeling
If you notice any side effects not mentioned in this leaflet, please inform your doctor.
After using REPLAGAL
Keep REPLAGAL out of the reach and sight of children.
Store at 2-8°C (in a refrigerator). Do not freeze. REPLAGAL will usually be kept in
the pharmacy department of the hospital where you are receiving the treatment and
the infusion prepared there for you individually. Any unused solution from the preparation
would be discarded.
The infusion should be given immediately after preparation, unless otherwise instructed
by your physician. REPLAGAL does not contain any preservatives to prevent bacterial
Do not use after the expiry date stated on the label and carton. REPLAGAL will not
be given to you if there is discolouration or other foreign particles present. A slight
haziness is normal.
What REPLAGAL looks like
REPLAGAL is a sterile, clear and colourless solution intended for intravenous administration.
A minute amount of fine particulate matter, causing the solution to appear slightly
hazy, may be present.
REPLAGAL is supplied as a single vial in a carton.
REPLAGAL contains 3.5 mg agalsidase alfa ghu, in 3.5 mL.
The other ingredients are:
sodium phosphate - monobasic monohydrate
Shire Australia Pty Limited
Level 39, 225 George Street
Sydney, NSW 2000
Telephone: 1800 012 612
Shire New Zealand Limited
C/o Crowe Horwath
Level 29, 188 Quay Street
Telephone: 0508 169 077
This leaflet was prepared in January 2018.
Australian Registration Number:
AUST R 82818
REPLAGAL is a trademark of Shire Human Genetic Therapies, Inc., USA.