Ruconest 2100 U powder for solution for injection

Ruconest contains conestat alfa as the active substance. Conestat alfa is a recombinant form of human C1inhibitor ( rhC1INH) and is produced using recombinant DNA technology from the milk of rabbits.

Ruconest is to be used by adults with a rare inherited blood disorder, called Hereditary Angioedema(HAE). These patients have a shortage of the C1 inhibitor protein in their blood. This can lead to repeated attacks of swelling, pain in the abdomen, difficulty breathing and other symptoms.

The administration of conestat alfa (Ruconest) is to resolve the shortage of C1 inhibitor and will lead to reduction of symptoms of an acute attack of HAE.

Do not use Ruconest
- If you are or think you are allergic (hypersensitive) to rabbits
- If you are allergic (hypersensitive) to conestat alfa or any of the other ingredients of the medicinal product (see section 6).

Only use Ruconest if you have a negative test for rabbit allergy (IgE). Repeat this test every year or after every 10 treatments with Ruconest, whichever occurs first.

Take special care with Ruconest

If you experience allergic reactions e.g. hives, rash, itching, dizziness, wheezing, difficulty breathing or your tongue swells up following the administration of Ruconest, you should seek emergency medical assistanceso that symptoms of your allergic reaction can be treated urgently.

Children and adolescents

Ruconest is not indicated for use in children and adolescents under 18 years old.

Using other medicines
Please inform your doctor if you are taking or have recently taken any other medicines, including medicines obtained without a prescription.
If you are receiving acute treatment for blood clots, you should not be treated with Ruconest at the same time.

Pregnancy and breast-feeding

It is not recommended to use Ruconest during pregnancy or breast-feeding.
If you plan becoming pregnant, discuss with your doctor before starting to use Ruconest.

Driving and using machines

Do not drive or use machinery if you feel dizzy or suffer from headache after using Ruconest.

Ruconest will be given to you directly into a vein over a period of approximately 5 minutes by your doctor or by a nurse. Your dose will be worked out based on your weight.
Most of the time a single dose is sufficient, but a second dose may be needed. No more than 2 doses should be given within 24 hours.

The instructions for use are clearly described in the doctor?s information leaflet and are attached.

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

If your symptoms get worse and/or you develop a rash, tingling, difficulty breathing or your face or tongue swells up, get medical attention immediately.
This may indicate that you have developed an allergy to Ruconest.

Some side effects may occur during treatment with Ruconest:
Common (affect 1 to 10 users in 100):
headache

Uncommon (affect 1 to 10 users in 1,000):
sensation of tingling, prickling or numbness in the skin or limb (paraesthesia), dizziness, throat irritation, abdominal pain, diarrhoea, nausea, hives and swelling of the skin.

If any of the side effects gets serious, or if you notice any side effects not listed in the leaflet, please inform your doctor or pharmacist.

Keep out of the reach and sight of children.
Do not use this medicine after the expiry date stated on the carton and on the label of the vial after EXP.
The expiry date refers to the last day of that month

Do not store above 25°C.
Store in the original package in order to protect from light.

Before Ruconest can be administered, it needs to be dissolved in water for injections, by a healthcare professional.
Once reconstituted, the product should be used immediately.

What Ruconest contains

Conestat alfa 2100 units per vial, corresponding to 2100 units per 14 ml after reconstitution, or a concentration of 150 units/ml.

Conestat alfa is the recombinant analogue of the human C1 esterase inhibitor (rhC1INH)

The other ingredients are sucrose, sodium citrate (E331) and citric acid (E330)

What Ruconest looks like and contents of the pack

Ruconest is presented as a single glass vial containing a white to off-white powder for solution for injection. After dissolving in water for injections, the solution is clear and colourless. Ruconest is supplied in a carton box containing one vial powder.

Marketing Authorisation Holder and Manufacturer

Pharming Group N.V.
Darwinweg 24
NL-2333 CR Leiden
The Netherlands

This leaflet was last approved in

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The following information is intended for medical or healthcare professionals only:

POSOLOGY AND METHOD OF ADMINISTRATION

• Adults up to 84 kg body weightOne intravenous injection of 50 U/kg body weight.
• Adults of 84 kg body weight or greaterOne intravenous injection of 4200 U (two vials).

In the majority of cases a single dose of Ruconest is sufficient to treat an acute angioedema attack. In case of an insufficient clinical response, an additional dose (50 U/kg body weight up to 4200 U) can be administered (see section 5.1).
Not more than two doses should be administered within 24 hours.

Dose calculation
Determine the patient?s body weight.

• Adults up to 84 kg body weight For patients up to 84 kg calculate the volume required to be administered according to the formula below:

body weight kg times 50 Ukg body weight kg Volume to be administered ml 150 Uml 3

• Adults of 84 kg body weight or greater For patients of 84 kg or above the volume required to be administered is 28 ml corresponding to 4200 U (2 vials).

Reconstitute each vial with 14 ml water for injections (see section on Reconstitution below). The reconstituted solution in each vial contains 2100 U conestat alfa at 150 U/ml.

The required volume of the reconstituted solution should be administered as a slow intravenous injection over approximately 5 minutes.

SPECIAL PRECAUTIONS FOR DISPOSAL AND OTHER HANDLING

Each vial of Ruconest is for single use only.
An aseptic technique should be used for reconstitution, combining and mixing the solutions.

Reconstitution

Each vial of Ruconest (2100 U) should be reconstituted with 14 ml water for injections. Water for injections should be added slowly to avoid forceful impact on the powder and mixed gently to avoid foaming of the solution. The reconstituted solution in each vial contains 2100 U conestat alfa at 150 U/ml and appears as a clear colourless solution.

The reconstituted solution in each vial should be inspected for particulate matter and discoloration. A solution exhibiting particulates or discoloration should not be used. The medicinal product should be used immediately.

No special requirements for disposal.