Dose

Each vial of Profilnine is labeled with total units expressed as International Units (IU). According to the WHO International Standard, one unit approximates the activity in one mL of normal plasma.

A 1% increase in factor IX (0.01 units) per unit administered per kg body weight can be expected1. The amount of Profilnine required to establish hemostasis will vary with each patient and circumstance. Use the following formula and example as guides in determining the number of units to be administered:

Due to variability among patients and their clinical condition, monitor the factor IX level of each patient frequently during replacement therapy.

Table 2 below provides treatment guidelines for hemorrhagic events and surgery in patients with factor IX deficiency.

Dosing requirements and frequency of dosing are calculated on the basis of an initial response of 1% FIX increase achieved per IU of FIX infused per kg body weight and an average half-life for FIX of 24 hours. If dosing studies reveal that a particular patient exhibits a lower response, monitor blood levels and adjust the dose accordingly.

Reconstitution

Use Aseptic Technique

1. Ensure that concentrate (Profilnine) and diluent (Sterile Water for Injection, USP) are at room temperature (but not above 37° C) before reconstitution.
2. Remove the plastic flip off cap from the diluent vial.
3. Gently swab the exposed stopper surface with a cleansing agent such as alcohol. Avoid leaving any excess cleansing agent on the stopper.
4. Open the Mix2Vial® package by peeling away the lid (Figure 1). Leave the Mix2Vial in the clear outer packaging.
5. Place the diluent vial upright on an even surface, hold the vial tightly, and pick up the Mix2Vial in its clear outer packaging. While holding the diluent vial securely, push the blue end of the Mix2Vial vertically down through the diluent vial stopper (Figure 2).
6. While holding onto the diluent vial, carefully remove the clear outer packaging from the Mix2Vial set, ensuring the Mix2Vial remains attached to the diluent vial (Figure 3).
7. Place the product vial upright on an even surface, invert the diluent vial with the Mix2Vial attached.
8. While holding the product vial securely on a flat surface, push the clear end of the Mix2Vial set vertically down through the product vial stopper (Figure 4). The diluent will automatically transfer out of its vial into the product vial.
   NOTE: If the Mix2Vial is connected at an angle, the vacuum may be released from the product vial and the diluent will not transfer into the product vial.
9. With the diluent and product vials still attached to the Mix2Vial, gently swirl the product vial to ensure the product is fully dissolved (Figure 5). Reconstitution requires less than 10 minutes. Do not shake the vial.
10. Disconnect the Mix2Vial into two separate pieces (Figure 6) by holding each vial adapter and twisting counterclockwise. After separating, discard the diluent vial with the blue end of the Mix2Vial.
11. Draw air into an empty, sterile syringe. Keeping the product vial upright with the clear end of the Mix2Vial attached, screw the disposable syringe onto the luer lock portion of the Mix2Vial device by pressing and twisting clockwise. Inject air into the product vial.
12. While keeping the syringe plunger depressed, invert the system upside down and draw the reconstituted product into the syringe by pulling the plunger back slowly (Figure 7).
13. When the reconstituted product has been transferred into the syringe, firmly hold the barrel of the syringe and the clear vial adapter (keeping the syringe plunger facing down) and unscrew the syringe from the Mix2Vial (Figure 8). Hold the syringe upright and push the plunger until no air is left in the syringe. Attach the syringe to a venipuncture set.
    NOTE: If the same patient is to receive more than one vial of concentrate, the contents of two vials may be drawn into the same syringe through a separate unused Mix2Vial set before attaching to the venipuncture set.
14. After reconstitution, inspect parenteral drug products visually for particulate matter and discoloration prior to administration, whenever solution and container permit. When reconstitution procedure is strictly followed, a few small particles may occasionally remain. The Mix2Vial set will remove particles and the labeled potency will not be reduced.
15. Do not refrigerate after reconstitution. The reconstituted product is stable for 3 hours at room temperature; use as soon as possible within 3 hours after reconstitution.