Dosage:
Your doctor will decide how much Nonafact you require. The exact dose will depend on the seriousness of the clinical situation, your body weight and the amount of factor IX in your blood. If you have to undergo surgery or have a tooth extracted, inform your doctor or dentist that you have factor IX deficiency. They will then ensure that factor IX is administered to you, should this prove necessary.
Nonafact is given to you by your doctor or nurse. Nonafact may be self administered if it is an approved practice in your country and when you have been trained sufficiently. Always use Nonafact exactly as your doctor has told you. You should check with your doctor or pharmacist if you are not sure.
It is important that the concentration of factor IX in your blood is checked regularly. If you are administered 1 IU per kilogram of body weight, the concentration of factor IX in your blood will increase by 1.1 % of normal activity.
The required dosage is determined using the following formula:
Required units = body weight (kg) x desired factor IX rise (%) (IU/dl) x 0.9
Your doctor will determine the dose of Nonafact you need and how frequently you should use it depending on the situation. The following table can be used to guide dosing in bleeding episodes and surgery:
Degree of bleeding Type of surgical procedure Factor IX level Frequency of doses hoursDuration required of therapy days IUdl
Bleeding 20-40 Early signs of bleeding in joints, muscle bleeding or oral bleeding Repeat every 24 hours. At least 1 day, until the bleeding episode as indicated by pain is resolved or healing is achieved. 30-60 More extensive bleeding in joints, muscle bleeding or haematoma Repeat infusion every 24 hours for 3-4 days or more until pain and acute disability are resolved.
Life threatening bleeding 60-100 Repeat infusion every 8 to 24 hours until threat is resolved.
Surgery 30-60 Minor including tooth extraction Every 24 hours, at least 1 day, until healing is achieved.
80-100 Major Repeat infusion every 8-24 hours until adequate wound healing, then therapy for at least another 7 days. pre- and postoperative
During the course of treatment it is advised that the concentrations of factor IX in your blood are checked regularly. Particularly with major surgery, it is important that the concentrations of factor IX in your blood are checked carefully before and after the operation.
For long-term prevention of bleeding in individuals with severe haemophilia B, doses of 20 IU to 40 IU of factor IX per kilogram body weight should be given at intervals of 3 to 4 days. In some cases, especially in younger patients, shorter dosage intervals or higher doses may be necessary.
If you have the impression that the effect of Nonafact is too strong or too weak, talk to your doctor. Individuals with haemophilia B may develop antibodies (inhibitors) against factor IX. As a result, the factor IX product used becomes inactivated (see Possible side effects). It is advised that the treatmentof this serious condition be taken care of in a haemophilia treating centre where an appropriate dose will be determined. The administration of factor IX can repress these inhibitors.
Instructions for use:
Dissolving the powder
The powder should be dissolved in the supplied amount of water for injections. The solution should not be too cold when it is administered. In addition, the powder will dissolve more readily if both vials are first allowed to reach room temperature (15°C ? 25°C).
1. Remove from the refrigerator and allow the two vials to reach room temperature (15°C ? 25°C).
2. Remove the plastic cap from the vials.
3. Disinfect the surface of the stoppers of both vials with a gauze soaked in 70 % alcohol.
4. Remove the protective sheath from one end of a transfer needle and pierce the stopper of the vial containing water for injections. Remove the protective sheath from the other end of the transfer needle. Invert the solvent vial and pierce the stopper of the vial containing the powder.
5. Tilt the product vial when transferring the solvent to allow the solvent to flow down the side of the vial.
6. Remove the empty vial and the transfer needle.
7. Swirl the vial gently to completely dissolve the powder. Do not shake! The powder dissolves within 5 minutes to produce a clear, colourless to light-yellow solution.
Immediately before administration, the solution should be inspected: the solution should be clear and must be free of lumps. Once dissolved, the product should be used immediately (at least within 3 hours).
Nonafact should never be mixed with other medicinal products.
Administration
1. Using a hypodermic needle and syringe, draw the dissolved product out of the vial.
2. Nonafact should be injected into a vein (intravenous administration).
3. Inject the dissolved product very gradually (approx. 2 ml per minute).
Disposal
Any unused product or waste material must be discarded as instructed by your doctor or pharmacist.
Duration of treatment:
Haemophilia B is a chronic disease, therefore lifelong treatment with a factor IX product may be necessary.
If you use more Nonafact than you should:
No symptoms of overdose with human coagulation factor IX have been reported.