Treatment should be started under the supervision of a doctor who has experience in the treatment of hemophilia.
Doses
Doses and duration of substitution therapy depend on the extent of factor IX deficiency, localization and intensity of bleeding, as well as on the clinical condition of the patient.
The amount of introduced factor IX is expressed in the International Units (ME) of activity established in relation to the international WHO standard for factor IX preparations currently in force.
The activity of factor IX in plasma is expressed either as a percentage (relative to normal human plasma) or in International Units (relative to the international standard for factor IX concentrates).
One International Unit (ME) of factor IX activity is equivalent to the activity of factor IX contained in 1 ml of normal human plasma.
The calculation of the required dose of factor IX is based on empirical observation that the introduction of one International UnitME) factor IX per kilogram of body weight increases the activity of factor IX in plasma by 0.8% from the normal level. The required dose is calculated using the following formula:
| Necessary dose = body weight (kg) x desired increase in factor IX (%) x 1.2 |
When calculating the amount of the drug administered and determining the frequency of the introduction, it is always necessary to focus on clinical effectiveness in each specific case. Drugs of factor IX rarely require administration more than once a day.
In the cases of bleeding mentioned below, the activity of factor IX should not fall below this level (in % or IU / dl) in the relevant period.
When calculating the doses for episodes of bleeding and surgical interventions, the following table can be used:
| The degree of bleeding or type of surgery | Required level of factor IX in plasma (% from the norm or ME / dl) | Multiplicity of administration / duration of therapy |
| Bleeding |
| Beginning hemarthrosis, muscle hemorrhage or bleeding in the oral cavity | 20-40 | Repeat every 24 hours (at least 1 day) until complete relief of bleeding, which is assessed by pain syndrome, or until the wound is healed. |
| Extensive hemarthroses, muscle hemorrhage or hematoma | 30-60 | Repeat infusion every 24 hours for 3-4 days or more until the disappearance of pain and recovery of motor activity. |
| Life-threatening bleeding, such as surgical interventions on the head, bleeding from the larynx, massive abdominal bleeding | 60-100 | Repeat infusion every 8-24 hours until life threatens. |
| Surgical interventions |
| Small surgical interventions, including tooth extraction | 30-60 | Every 24 hours (at least 1 day) until the wound is completely healed. |
| Extensive surgery | 80-100 (before and after operation) | Repeat infusion every 8-24 hours before wound healing, after that the therapy should be continued for a minimum of 7 days, maintaining the activity of factor IX at the level of 30-60%. |
In the course of treatment, it is recommended to determine the level of factor IX to check the correctness of dose selection and the frequency of repeated infusions. In particular, careful monitoring of substitution therapy through a clotting assay (activity of factor IX in plasma) is necessary in the case of extensive surgical interventions. Individual patients may react differently to factor IX, while they have different levels of recovery of factor IX and different half-lives.
For long-term prophylaxis of bleeding in patients with severe hemophilia B, doses of 20 to 40 IU / kg body weight are usually given with an interval of administration from 3 to 4 days.
In some cases, especially in young patients, shorter intervals between administrations or higher doses may be necessary.
Development of inhibitors
Patients should be monitored for the possible development of inhibitors to factor IX. If the expected level of activity of factor IX in the plasma can not be achieved, or bleeding is not stopped by administering the appropriate dose of the drug, it is necessary to conduct a test for the presence of inhibitors to factor IX. In patients with high levels of inhibitors, factor IX therapy may not be effective and other therapeutic options should be considered. The management of such patients should be conducted by physicians with experience in the treatment of patients with hemophilia.
See also "Special warnings and warnings".
Children
There are limited data on use in children younger than 6 years of age.
Mode of application
The drug must be administered intravenously.
It is not recommended to administer the drug at a rate of more than 2 ml / min.
To restore and administer the drug, you must use the kit for dissolution and administration contained in the package with the drug.
IMMUNIN is to be dissolved strictly immediately before administration. The solution should be clear or slightly opalescent. A cloudy solution or solution with inclusions should not be used. The prepared solution should be quickly used (the preparation does not contain preservatives). The reconstituted product must be checked for foreign particles and discoloration before injection.
Before the introduction of the drug IMMUNIN recommended to wash the port of venous access with an isotonic solution.
Breeding
Observe the rules of asepsis!
1. Heat the sealed bottle with a solvent (water for injections) to room temperature (maximum up to 37 ° C).
2. Remove protective caps from vials with lyophilizate and solvent (Fig. A) and disinfect the rubber stoppers of both vials.
3. Remove the protective packaging from one end of the supplied adapter needle by turning and sipping it (Fig. B). The free end of the needle is inserted into the rubber stopper of the vial with a solvent (Fig. B).
4. Carefully remove the protective packaging from the other end of the adapter needle, without touching the open end.
5. Flip the vial with the solvent, place it over the vial with lyophilizate and insert the free end of the needle into this vial through the rubber stopper (Fig. D). The solvent will flow into the vial with the lyophilizate under the action of a vacuum.
6. Disconnect the vials by removing the needle-adapter from the vial with lyophilizate (Fig. D). Slightly shake or rotate the vial with lyophilizate to accelerate dissolution.
7. After completely dissolving the lyophilizate, insert the supplied airway needle into the vial (Figure E), and the entire foam will settle. Remove the airway needle.
Introduction
Observe the rules of asepsis!
1. Remove the protective packaging from the supplied filter needle by turning and sipping it, and insert the needle onto a sterile disposable syringe. Draw the solution into the syringe (Figure F).
2. Remove the needle filter from the syringe and slowly inject the solution intravenously (maximum injection rate 2 ml / min) with the attached transfusion butterfly needle (or the supplied disposable needle).
When carrying out the infusion, use a one-time infusion system with the appropriate filter.
Any unused medication or residue must be disposed of in accordance with the established requirements.