What is it and how is it used?
BeneFIX is a coagulation factor IX product that is produced by recombinant technology. Patients with haemophilia B (Christmas disease) are deficient in coagulation factor IX. BeneFIX works by replacing factor IX in haemophilia B patients to enable their blood to clot.
BeneFIX is used for the treatment and prevention of bleeding (prophylaxis) in patients with haemophilia B (congenital factor IX deficiency).
What do you have to consider before using it?
Do not take BeneFIX
Take special care with BeneFIX
carefully monitored for the development of factor IX inhibitors. Sufficient data have not been obtained from ongoing clinical studies on the treatment of previously untreated patients (PUPs), with BeneFIX.
Taking other medicines
Please tell your doctor or pharmacist if you are taking or have recently taken any other medicines, including medicines obtained without a prescription.
Pregnancy and breast-feeding
If you are pregnant or breast-feeding you should only take BeneFIX upon specific instructions from your physician. It is not known whether BeneFIX can affect reproductive capacity or cause foetal harm when given to pregnant women. Your doctor may advise you to stop treatment with BeneFIX if you are breast-feeding or become pregnant.
Ask your doctor or pharmacist for advice before taking any medicine.
Driving and using machines
No studies on the effects on the ability to drive and use machines have been performed.
How is it used?
Always take BeneFIX exactly as your doctor has instructed you. You should check with your doctor or pharmacist if you are not sure.
Your doctor will decide the dose of BeneFIX you will receive. This dose and duration will depend upon your individual needs for replacement factor IX therapy and upon pharmacokinetics (recovery and half-life) that have to be checked regularly. You may notice a difference in the dose you receive if you are changing from a plasma-derived factor IX product to BeneFIX.
Your doctor may decide to change the dose of BeneFIX you receive during your treatment.
Reconstitution and administration
The procedures below are provided as guidelines for the reconstitution and administration of BeneFIX. Patients should follow the specific venipuncture procedures provided by their physicians.
BeneFIX is administered by intravenous (IV) injection after reconstitution of the powder for injection with the supplied solvent (0.234% w/v sodium chloride solution) in the pre-filled syringe.
Always wash your hands prior to performing the following procedures. Aseptic technique (meaning clean and germ free) should be used during the reconstitution procedure.
Reconstitution:
BeneFIX will be administered by intravenous infusion (IV) after reconstitution with sterile solvent for injection.
1. Allow the vial of lyophilised BeneFIX and the pre-filled syringe to reach room temperature.
2. Remove the plastic flip-top cap from the BeneFIX vial to expose the central portion of the rubber stopper.
3. Wipe the top of the vial with an alcohol swab provided, or use another antiseptic solution and allow to dry. After cleaning do not touch the rubber stopper with your hand or allow it to touch any surface.
4. Peel back the lid from the clear plastic vial adapter package. Do not remove the adapter from the package.
5. Place the vial on a flat surface. While holding the adapter in the package, place the vial adapter over the vial. Press down firmly on the package until the adapter snaps into place on top of the vial, with the adapter spike penetrating the vial stopper.
6. Lift the package away from the adapter and discard the package.
7. Attach the plunger rod to the solvent syringe by pushing and turning firmly.
8. Break off the tamper-resistant plastic tip cap from the solvent syringe by snapping the perforation of the cap. This is done by bending the cap up and down until the perforation is broken. Do not touch the inside of the cap or the syringe tip. The cap may need to be replaced (if not administering reconstituted BeneFIX immediately), so set it aside by placing it on its top.
9. Place the vial on a flat surface. Connect the solvent syringe to the vial adapter by inserting the tip of the syringe into the adapter opening while firmly pushing and turning the syringe clockwise until the connection is secured.
10. Slowly depress the plunger rod to inject all the solvent into the BeneFIX vial
11. With the syringe still connected to the adapter, gently rotate the vial until the powder is dissolved.
12. The final solution should be inspected visually for particulate matter before administration. The solution should appear clear and colourless.
Note: If you use more than one vial of BeneFIX per infusion, each vial should be reconstituted as per the previous instructions. The solvent syringe should be removed, leaving the vial adapter in place, and a separate large luer lock syringe may be used to draw back the reconstituted contents of each individual vial.
13. Ensuring that the syringe plunger rod is still fully depressed, invert the vial. Slowly draw back all the solution into the syringe.
14. Detach the syringe from the vial adapter by gently pulling and turning the syringe counter-clockwise. Discard the vial with the adapter attached.
Note: If the solution is not to be used immediately, the syringe cap should be carefully replaced. Do not touch the syringe tip or the inside of the cap.
BeneFIX should be administered immediately or within 3 hours after reconstitution. The reconstituted solution may be stored at room temperature prior to administration.
Administration (Intravenous Injection):
BeneFIX should be administered using the pre-filled solvent syringe provided or a single sterile disposable plastic luer lock syringe. In addition, the solution should be withdrawn from the vial using the vial adapter.
BeneFIX should be injected intravenously over several minutes. Your doctor may change your recommended infusion rate to make the infusion more comfortable.
Because the use of BeneFIX by continuous infusion (drip) has not been eva luated, BeneFIX should not be mixed with infusion solutions or be given in a drip.
Please dispose of all unused solution, empty vials and used needles and syringes in an appropriate container for throwing away waste as it may hurt others if not handled properly.
If you take more BeneFIX than you should
Please contact your doctor immediately if you inject more BeneFIX than your doctor recommends.
If you stop taking BeneFIX
If you have any further questions on the use of this product, ask your physician or pharmacist.
What are possible side effects?
Like all medicines, BeneFIX can cause side effects, although not everybody gets them.
Should you experience a significant increase in your usage of BeneFIX in order to control a bleed, please contact your doctor immediately; your blood should be checked for inhibitors to factor IX activity.
Possible side effects are listed according to the following categories:
Uncommon: between 1 in 100 and 1 in 1,000 patients
Rare: between 1 in 1,000 and 1 in 10,000 patients
Immune system disorders
Uncommon Rare Nausea Vomiting
General disorders and administration site conditions
* See additional information below.
Hypersensitivity/allergic reactions
Hypersensitivity or allergic reactions have been infrequently observed in patients treated with factor IX containing products, including BeneFIX. In some cases, these reactions have progressed to severe anaphylaxis. Allergic reactions have occurred in close temporal association with development of factor IX inhibitor (see also “Take special care with BeneFIX”).
The aetiology of the allergic reactions to BeneFIX has not yet been elucidated. These reactions are potentially life-threatening. If allergic/anaphylactic reactions occur, stop the infusion immediately and contact your doctor or seek emergency medical care immediately. In case of severe allergic reactions, alternative therapy should be considered. The treatment required depends on the nature and severity of side-effects (see also “Take special care with BeneFIX”).
Inhibitor development
Patients with haemophilia B may develop neutralising antibodies (inhibitors) to factor IX. If such inhibitors occur, the condition may manifest itself as an insufficient clinical response. In such cases, it is recommended that a specialised haemophilia centre be contacted.
A clinically relevant, low responding inhibitor was detected in 1 out of 65 BeneFIX patients who had previously received plasma-derived products. This patient was able to continue treatment with BeneFIX with no rise in inhibitor or anaphylaxis. Patients treated with BeneFIX should be monitored for inhibitor development.
There are insufficient data to provide information on inhibitor incidence in previously untreated patients (PUPs).
Due to the production process BeneFIX may contain trace amounts of hamster cell proteins. Hypersensitivity responses can occur.
A kidney disorder has been reported following high doses of plasma-derived Factor IX to induce immune tolerance in haemophilia B patients with factor IX inhibitors and a history of allergic reactions (see also “Take special care with BeneFIX”).
Renal
One patient developed severe abdominal pain which was caused by an area of the kidney that lacked the necessary blood supply (referred to as a renal infarct). The relationship of the infarct to prior administration of BeneFIX is uncertain.
Thrombotic events
BeneFIX may increase the risk of thrombosis (abnormal blood clots) in your body if you have risk factors for developing blood clots, including an indwelling venous catheter, through which BeneFIX is given by continuous infusion. There have been reports of severe blood clotting events, including life-threatening blood clots in critically ill neonates, while receiving continuous-infusion BeneFIX through a central venous catheter. Cases of peripheral thrombophlebitis and deep venous thrombosis have also been reported; in most of these cases, BeneFIX was administered via continuous infusion, which is not an approved method of administration.
Inadequate therapeutic response and inadequate factor IX recovery
Inadequate therapeutic response and inadequate factor IX recovery have been reported during the post-marketing use of BeneFIX.
If any of the side effects get serious, or if you notice any side effect not listed in this leaflet, please tell your doctor or pharmacist.
How should it be stored?
Keep out of the reach and sight of children.
Do not use after the expiry date stated on the outer box and vial label. The expiry date refers to the last day of that month.
Do not use BeneFIX if the solution is not clear or colourless.
BeneFIX must be stored continuously in a refrigerator at a temperature of 2°C to 8°C and must be used by the expiry date on the label. For the purpose of ambulatory use the product may be removed from such storage for one single period of maximum 6 months at room temperature (up to 30°C).At the end of this period the product should not be put back in the refrigerator, but should be used or discarded. The date the product is removed from the refrigerator and set at room temperature (not exceeding 30 oC) and the date the vial should be withdrawn should be noted on the outer carton.
Do not freeze in order to prevent damage to the pre-filled syringe.
Use the reconstituted solution immediately or within 3 hours.
Use only the pre-filled syringe provided in the box for reconstitution. Other sterile disposable syringes may be used for administration.
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.
