Amifix (Aimafix)

The preparation should be reconstituted in accordance with the instructions given in the section "Preparation and administration of reconstituted drug solution". The reconstituted drug solution is administered intravenously by injection or slow infusion. It is recommended to inject no more than 100 IU / kg of body weight per day. The rate of drug administration is set individually for each patient.

Preparation and administration of the reconstituted drug solution

Restoration of the lyophilisate with a solvent

1. Heat the lyophilizate and the solvent to room temperature.

2. Remove protective covers from vials of lyophilizate and solvent.

3. Wipe the surfaces of the lids of both vials with alcohol.

4. To open the package with a needleless transfer device, carefully remove the top part without touching the inside (figure 1).

5. Do not remove the needleless transfer device from the package.

6. Turn the box over and insert the plastic tip through the lid of the solvent vial (Figure 2).

7. Take the edge of the package and remove it, releasing the needleless transfer device (Figure 3).

8. Without removing the solvent bottle, turn the needleless transfer device so that the solvent bottle is above the device.

9. Insert the second plastic tip of the device through the bottle stopper with lyophilizate. Since the powder in the vial is under vacuum, the solvent will penetrate into the vial with lyophilizate (Figure 4).

10. Shake the system until the powder is completely dissolved (Figure 5), otherwise the undissolved drug will not pass through the device filter.

The average time of complete recovery of the drug is less than 3 minutes.

Solution administration

The solution should be clear or slightly opalescent. Before using the drug, check that the appearance of the solution corresponds to the description, does not contain visible mechanical inclusions, and does not change the color.

Do not use if the solution is cloudy or contains sediment.

1. Turn the needleless gear valve down towards the vial of the lyophilizate and remove the lid covering it (Fig. 6).

2. Fill the syringe with air, pulling the piston back, attach it to the device valve and insert air into the vial of the lyophilizate (Fig. 7).

3. Without moving the piston, turn the system so that the vial of the lyophilizate is above the device, and slowly draw the solution into the syringe, pulling the piston back (Figure 8).

4. Carefully turn the valve to its original position and pull the syringe out of the device.

5. Attach the butterfly needle to the syringe and inject the drug intravenously with infusion or slow injection.

Remains of the drug and used materials must be destroyed in the prescribed manner.

Treatment with the drug is carried out under the supervision of a doctor. The dose and duration of replacement therapy depend on the severity of hemostatic function, localization and intensity of bleeding and clinical manifestations of the disease.

The activity of the coagulation factor IX is expressed either as a percentage of normal human plasma, or in International Units (ME) in relation to the international standard of coagulation factor IX, adopted by the World Health Organization (WHO). One unit of coagulation factor IX activity, expressed in ME, is equivalent to the amount of coagulation factor IX in 1 ml of normal human plasma.The calculation of the required dose of coagulation factor IX is based on the empirically established fact that 1 ME coagulation factor IX per 1 kg of body weight increases the activity of coagulation factor IX in plasma by 0.8% of normal activity.

The required dose of the drug (ME) is calculated by the following formula:

dose (ME) = body weight (kg) x required increase in coagulation factor activity IX (%) x inverse of the observed increase in coagulation factor IX activity

The amount of the drug administered and the frequency of application should be established, based on the clinical effectiveness of the drug in each individual case. Drugs of coagulation factor IX in rare cases, inject more than one injection per day.

In the case of hemorrhagic hemorrhages given below, the activity of the blood coagulation factor IX should not be lower than the indicated activity in blood plasma, expressed in% of the norm, in the corresponding period. In this table, recommended doses of the drug in case of bleeding and surgery:

During the course of therapy, it is recommended to monitor the blood coagulation factor IX activity in blood plasma to establish the recommended dose and frequency of administration of the drug.Particularly when extensive surgery necessarily is conducted careful monitoring substitution therapy by examining blood coagulation (clotting activity of factor IX in the blood plasma of the patient). For some patients can show a different susceptibility to the IX blood clotting factor, can achieve different levels of recovery activity in vivo and the half-life of the coagulation factor IX in plasma.

In cases of long-term prevention of bleeding in patients with severe haemophilia B drug is administered at a dose of 20-40 ME coagulation factor IX for 1 kg of body weight of the patient at intervals of 3-4 days.

In some cases, especially in young patients, it is possible to administer the drug at shorter time intervals or at a higher dose. When the drug is administered, it is necessary to control the appearance in the blood of inhibitors of the coagulation factor IX. If the expected level of activity of the clotting factor IX in the blood plasma has not been reached, or if the effective dose has not been established for the bleeding, you need to maintain control of blood coagulation factor IX inhibitor in a patient's blood plasma.

For patients with a high content of a coagulation factor inhibitor IX, coagulation factor IX therapy may be ineffective, in which case alternative therapy is possible. The management of such patients should be conducted by a specialist with experience in the treatment of patients with hemophilia.