AGAMREE 40 mg /Ml Oral Suspension

Зміст інструкції

1. AGAMREE 40 mg/mL oral suspension
   1. What is AGAMREE and what is it used for
   2. What you need to know before taking AGAMREE
   3. How to take AGAMREE
   4. Possible side effects
   5. How to store AGAMREE
   6. Contents of the pack and other information

AGAMREE 40 mg/mL oral suspension

vamorolone
▼ Medicine subject to additional monitoring. This will allow the rapid identification of
new safety information. You can contribute by reporting any adverse effects
experienced during the use of this medicine. See the end of section 4 for
information on how to report adverse effects.

Read this leaflet carefully before taking this medicine as it contains
important information for you.

• Keep this leaflet. You may need to read it again.
• If you have any questions, ask your doctor or pharmacist.
• This medicine has been prescribed for you only. Do not pass it on to others, even if their symptoms are the same as yours, as it may be harmful.
• If any side effects occur, including those not listed in this leaflet, tell your doctor or pharmacist. See section 4.

Contents of this leaflet

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

1. What is AGAMREE and what is it used for

AGAMREE is a steroid anti-inflammatory medicine that contains the active ingredient vamorolone.
AGAMREE is indicated for the treatment of patients aged 4 years and older with Duchenne muscular dystrophy (DMD). DMD is a genetic disease caused by defects in the dystrophin gene, which normally produces a protein that keeps muscles healthy and strong. In patients with DMD, this protein is not produced and the body is unable to grow new muscle cells or replace damaged muscle. This causes the muscles of the body to become weaker over time.
AGAMREE is used to stabilize or improve muscle strength in patients with DMD.

2. What you need to know before taking AGAMREE

Do not take AGAMREE

• if you are allergic to vamorolone or any of the other ingredients of this medicine (listed in section 6);
• if you have a severe liver problem;
• if you are planning to have or have had a vaccination with live or live-attenuated vaccines (for example against measles, mumps, rubella or chickenpox) in the last 6 weeks. Talk to your doctor if you are already being treated with AGAMREE and are planning to have such a vaccination.

Warnings and precautions
Talk to your doctor before taking AGAMREE
Endocrine function changes: adrenal insufficiency
AGAMREE reduces the amount that the body can produce of a hormone called cortisol. This is called
adrenal insufficiency.

• Do not reduce the amount or stop taking AGAMREE without talking to your doctor; if you suddenly reduce or stop taking AGAMREE for a few days, you may develop symptoms of acute adrenal insufficiency, such as excessive fatigue, dizziness or confusion, which could be life-threatening. Your doctor may need to monitor your treatment more closely if the dose is changed.
• If you are in a state of unusual stress (due to acute infection, traumatic injuries or major surgery), you may need to take an additional steroid medicine to prevent acute adrenal insufficiency. Talk to your doctor about what to do in case of unusual stress before starting treatment with AGAMREE.
• If you are being treated with another corticosteroid such as prednisone, you may be able to switch to AGAMREE directly, but your doctor will prescribe the dose of AGAMREE that you should take.
• If you have a type of tumor in the adrenal glands called pheochromocytoma, your doctor may need to monitor your treatment more closely.

IMPORTANT WARNING: the AGAMREE pack contains an alert card for the
patient that provides important information on safety in case of adrenal crisis. Keep this
card with you at all times.
Weight gain

• AGAMREE may increase your appetite and therefore your weight, especially in the first few months of treatment; your doctor or nurse will give you advice on the diet to follow before and during treatment.

Patients with altered thyroid function

• If you suffer from hypothyroidism (reduced thyroid activity) or hyperthyroidism (overactivity of the thyroid) your doctor may need to monitor your treatment more closely or adjust the dose.

Ophthalmic effects

• If you or someone in your family suffers from glaucoma (increased eye pressure), your doctor may need to check your treatment more closely.

Increased risk of infections
AGAMREE can reduce your natural resistance to infections.

• If you suffer from a reduced immune response (due to an immunodeficiency syndrome, illness or other medicines that suppress the immune system), your doctor may need to monitor your treatment more closely.
• If you develop an infection during treatment with AGAMREE, your doctor may need to monitor you more closely and you may need to follow treatment with an additional steroid medicine.

Diabetes mellitus

• The use of AGAMREE over the years may increase the likelihood of developing diabetes mellitus (a disease related to sugar); your doctor may need to check your sugar levels regularly.

Vaccination

• If you plan to be vaccinated with live or live-attenuated vaccines, vaccination should occur at least 6 weeks before starting treatment with AGAMREE.
• If you have never had chickenpox or have not been vaccinated against this disease, you may consider vaccination with your doctor before starting therapy with AGAMREE.

Thromboembolic events

• If you have had thromboembolic events (a blood clot inside the body) or a disease that increases the risk of blood clotting, your doctor may need to check your treatment more closely.

Hepatic impairment

• If you have a liver disease, your doctor may need to adjust the dose.

Children
Do not give AGAMREE to children under 4 years of age as it has not been tested on
this age group.

Other medicines and AGAMREE
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 of the following medicines:

• medicines for the treatment of seizures and neuropathic pain, such as carbamazepine or phenytoin, as they may affect the effect of the medicine;
• medicines for the treatment of fungal infections (including candidiasis and aspergillosis) known as triazoles, such as itraconazole and voriconazole, as they may affect the effect of the medicine;
• antibiotics known as macrolides (such as clarithromycin) or “ketolides” (such as telithromycin), as they may affect the effect of the medicine;
• antibiotics known as rifamycins, such as rifampicin, as they may affect the effect of the medicine;
• spironolactone or eplerenone, known as potassium-sparing diuretic treatments (treatments that increase urine production), which may be prescribed to lower blood pressure and protect cardiovascular function, as they may have effects similar to those of AGAMREE. Your doctor may need to monitor potassium levels and adjust the dose of these medicines;
• St. John's wort (Hypericum perforatum), a herbal medicine for the treatment of depression and emotional disorders as it may affect the effect of the medicine.

If you need to be vaccinated, ask your doctor for advice first (see section 2: “Do not take
AGAMREE”). You should not be given certain types of vaccine (live or live-attenuated
vaccines) for a period of up to 6 weeks before starting treatment with
AGAMREE, as in such an association the aforementioned vaccines could cause the infection that
they should prevent.

AGAMREE with food and drink
Avoid grapefruit and grapefruit juice during treatment with AGAMREE as they can
affect the effect of the medicine.

Pregnancy, breastfeeding and fertility
If you are pregnant, suspect you are pregnant or are planning a pregnancy, or if you are breastfeeding, ask your doctor for advice before taking this medicine.
If you are pregnant, you should not take AGAMREE unless clearly indicated by
your doctor.
If you are a woman of childbearing potential, you should use effective contraception during treatment with
AGAMREE.
Studies in animals have shown that long-term treatment with AGAMREE can
harm male and female fertility.

Driving and operating machinery
Ask your doctor if your illness allows you to drive vehicles, including a bicycle, and operate
machinery safely. AGAMREE should not alter your ability to drive vehicles, ride a bicycle or operate machinery.

AGAMREE contains sodium benzoate and sodium
AGAMREE contains 1 mg of sodium benzoate (E 211) per mL.
AGAMREE contains less than 23 mg of sodium per 7.5 mL, i.e. it is essentially “sodium-free”.

3. How to take AGAMREE

Take this medicine following exactly the instructions of your doctor or pharmacist. If you
have any doubts, ask your doctor or pharmacist.
The recommended dose of AGAMREE depends on your body weight and your age.
If you are 4 years or older and your weight is less than 40 kg, the dose is generally 6 mg per kg of weight
body weight, to be taken once a day.
If you are 4 years or older and your weight is equal to or greater than 40 kg, the dose is generally 240 mg, taken
once a day.
If you experience certain undesirable effects during the intake of AGAMREE (see section 4),
your doctor may reduce the dose or interrupt the treatment temporarily or permanently. If
you suffer from a liver disease, your doctor may reduce the dose.
This medicine is taken orally. AGAMREE can be taken with or without food (see
section 2 “AGAMREE with food and beverages”).
To take the medicine, use one of the oral administration syringes contained in the
package. Use only these oral administration syringes to measure the dose. The
doctor will tell you how much medicine to take with the syringe for your daily dose.
Caregivers must provide assistance in administering AGAMREE, especially regarding
the use of oral administration syringes in order to measure and administer the prescribed
dose.
Shake the bottle well before taking the medicine with the syringe. Take the dose with the
oral administration syringe, then empty the syringe immediately and slowly directly into your mouth.
Read the instructions below for more information on how to measure and take the dose
correctly. If you have any doubts about how to use the oral administration syringe, ask your doctor
or pharmacist.
After taking the prescribed dose, disassemble the oral administration syringe, rinse the syringe and
the plunger with cold tap water and let it air dry. Store the
oral administration syringe, thus washed, in the package until the next use. One oral administration syringe must be used for a maximum of 45 days. After this time, discard it and use the second oral administration syringe provided in the package. If you have any
doubts about the use of this medicine, ask your doctor or pharmacist.

HOW TO PREPARE THE DOSE OF AGAMREE ORAL SUSPENSION
Before taking/administering
AGAMREE
Step 1Make sure the child-resistant cap is securely fastened
and shake the bottle well.

Step 2Remove the child-resistant cap by pushing it firmly
down and turning it counterclockwise.

Step 3Firmly insert the adapter into the
bottle.
This operation must be done at the
first opening of the bottle. The adapter
must then remain in the bottle.
If the bottle adapter falls to the ground,
clean it with cold running water and let it
air dry for at least 2 hours.

Preparing the dose of
AGAMREE
Step 4Hold the bottle in an upright position.
Before inserting the tip of the oral administration syringe
into the bottle adapter, push the
plunger completely towards the tip
of the oral administration syringe. Firmly insert the tip
into the opening of the bottle adapter.

Step 5	Hold the oral administration syringe firmly and carefully invert the bottle. Pull the plunger slowly outwards until the desired amount of medicine is drawn into the oral administration syringe. If there are large air bubbles in the oral administration syringe (see figure on the left) or if the dose of AGAMREE drawn is incorrect, firmly insert the tip of the syringe into the adapter while the bottle is in an upright position. Push the plunger down so that AGAMREE flows back into the bottle and repeat steps 4 to 6.	and F a r m a c o
Step 6	Check that the dose corresponds to the milliliters (mL) prescribed by the doctor. To find the graduation indicating the dose in milliliters (mL) on the plunger, proceed as shown in the figure on the right. On the graduation shown on the syringe, each line corresponds to 0.1 mL. In the example, a dose of 1 mL is shown. Do not take a dose greater than the daily dose prescribed. a i l a t I a i z	a d
Step 7 A	n Put the bottle upright and carefully remove the oral administration syringe from the bottle. e Do not hold the oral administration syringe by the plunger g because the latter may come out.

Administering AGAMREE
Step 8Do not mix the medicine with liquids
of any kind before
administering it.
The patient should be sitting in
an upright position while taking
the medicine.
Empty the syringe directly into
your mouth.
Gently press the plunger to
empty the syringe. Do not push
hard on the plunger.
To avoid the risk of choking,
do not spray the medicine on the back
of the mouth or throat.

After administering
AGAMREE
Step 9Close the bottle with the child-resistant cap after each use.

Step 10
Disassemble the oral administration syringe, rinse with
cold running water and let it air dry before next use.
The oral administration syringes supplied with AGAMREE can
be used for a maximum of 45 days
each.

Enteral feeding tube
AGAMREE can be administered via an enteral feeding tube, following the instructions
provided in the tube kit. Administer the prescribed dose of AGAMREE, without the need for
dilution. Do not mix with nutritional formulas or other products. The tube must be rinsed
before and after administering AGAMREE using the syringe provided in the enteral feeding tube kit. Rinse the tube with at least 20 mL of water.

If you take more AGAMREE than you should
If you take more AGAMREE than you should, contact your doctor or hospital for advice. Show
doctor.

If you forget to take AGAMREE
Do not take a larger dose of AGAMREE and do not repeat the dose.
Take the next dose as usual.
If you are worried, contact a healthcare professional.

If you stop taking AGAMREE
Take AGAMREE for the entire period prescribed by your doctor. Contact your doctor before
stopping treatment with AGAMREE because the dose must be gradually reduced to
avoid undesirable effects.
If you have any doubts about 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.
Treatment with AGAMREE causes adrenal insufficiency. Consult your doctor before starting
therapy with AGAMREE (see section 2 for further information).
The following side effects have been reported with AGAMREE at a very common frequency
(may affect more than 1 in 10 people):

• more rounded and swollen appearance of the face (Cushingoid)
• weight gain
• increased appetite
• irritability
• vomiting

The following side effects have been reported at a common frequency (may affect up to
1 in 10 people):

• abdominal pain (stomach ache)
• upper abdominal pain (pain in the upper part of the abdomen)
• diarrhea
• headache

Reporting of side effects
If you get any side effects, talk to your doctor or pharmacist. You can also report side effects directly
through the national reporting system listed in Annex V.
By reporting side effects you can help provide more information on the safety of
this medicine.

5. How to store AGAMREE

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 package and on the label of the
vial after “Exp.”. The expiry date refers to the last day of that month.
This medicine does not require any particular storage temperature.
After the first opening of the AGAMREE vial, store it in a refrigerator (2 °C – 8 °C) in
an upright position. The medicine may be stored in a refrigerator for up to 3 months.
Dispose of any unused medicine within 3 months of the first opening of the vial.
Do not dispose of any medicine in wastewater or household waste. Ask your pharmacist how to
dispose of medicines you no longer use. This will help protect the environment.

6. Contents of the pack and other information

What AGAMREE contains
The active ingredient is vamorolone. Each mL of suspension contains 40 mg of vamorolone.
The other ingredients are: citric acid (monohydrate) (E 330), disodium phosphate (E 339), glycerol
(E 422), orange flavour, purified water, sodium benzoate (E 211) (see section 2, «AGAMREE
contains sodium benzoate»), sucralose (E 955), xanthan gum (E 415) and hydrochloric acid (for the
pH adjustment). See section 2 «AGAMREE contains sodium benzoate and sodium».

Description of the appearance of AGAMREE and contents of the pack
AGAMREE is an oral suspension of colour from white to whitish. The medicinal product is presented in a
amber glass bottle with a child-resistant tamper-evident closure made of polypropylene and
with a low-density polyethylene lining. The bottle contains 100 mL of oral suspension.
Each pack contains one bottle, a bottle adapter and two identical oral administration syringes for dosage.
The oral administration syringes are graduated from 0 to 8 mL in increments of 0.1 mL.

Marketing authorisation holder and manufacturer
Santhera Pharmaceuticals (Deutschland) GmbH
Marie-Curie-Straße 8
D-79539 Lörrach
Germany
More detailed information on this medicinal product is available on the website of the European
Medicines Agency: http://www.ema.europa.eu