USUAL DOSE (International units [units])
Dose must be individualized based on body weight, laboratory values, and patient’s clinical condition.
Initial dose in adult and pediatric patients:
40 units/kg.
Subsequent doses in adult and pediatric patients:
Should be guided by the most recent trough factor XIII (coagulation factor XIII) activity level. Dose every 28 days (4 weeks) to maintain a trough factor XIII activity level of approximately 5% to 20%. Recommended dosing adjustments of ±5 units/kg should be based on trough factor XIII activity levels as shown in the chart in Dose Adjustments and on the patient’s clinical condition.
Guide dose adjustments based on a specific assay used to determine factor XIII levels (e.g., Berichrom Activity Assay).
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Available as a single-use vial containing 1,000 to 1,600 units of factor XIII as a lyophilized concentrate. Actual units of potency stated on vial label. Sterile diluent and Mix2Vial filter transfer set provided. Sterile technique imperative. Confirm expiration date. Record the batch number of the product in the patient’s medical record with each infusion. Bring factor XIII concentrate and diluent to RT. Place vial, diluent, and Mix2Vial transfer set on a flat surface. Remove flip caps on vial and diluent, and wipe stoppers with provided alcohol swab; allow to dry. Peel away the lid on the Mix2Vial transfer set, but leave it in the clear package. Hold the diluent vial tightly on a flat surface and pick up the Mix2Vial transfer set by its clear package. Push the plastic spike at the blue end of the Mix2Vial transfer set through the center of the diluent vial stopper. Carefully remove only the clear packaging from the transfer set. Invert the diluent vial with the Mix2Vial transfer set attached, and push the plastic spike of the transparent adapter firmly through the center of the factor XIII concentrate. Diluent will automatically transfer into the vial. With all parts still attached, gently swirl to fully dissolve. Do not shake. Solution should be colorless to slightly yellowish and slightly opalescent. Grasp the factor XIII side with one hand and the diluent side with the other and unscrew the set into 2 pieces. Draw air into an empty 20-mL sterile syringe. With the factor XIII vial upright, screw the syringe to the Mix2Vial transfer set. Inject air into the factor XIII vial. Keep the syringe plunger pressed, and invert the system upside down to draw the concentrate into the syringe by pulling the plunger back slowly. Keep the plunger facing down and remove syringe from transfer set. Attach the syringe to a suitable IV administration set. If the same patient is to receive more than one vial, contents of multiple vials may be pooled. Use a separate, unused Mix2Vial transfer set for each vial. Must be used within 4 hours of reconstitution.
Mix2Vial filter transfer set provided.
Protect from light; refrigerate vials and diluent in carton at 2° to 8° C (36° to 46° F). Do not freeze. Stable for 24 months up to the expiration date on the carton under refrigeration. May be stored at RT not to exceed 25° C (77° F) for up to 6 months; however, it cannot be returned to refrigeration. Mark date removed from refrigeration. Do not use beyond expiration date on carton and vial labels or beyond end of RT storage, whichever comes first. Must be used within 4 hours of reconstitution. Do not refrigerate or freeze the reconstituted solution. Contains no preservatives; a single-use vial; discard partially used vials.
Manufacturer states, “Do not mix with other medicinal products, and administer through a separate infusion line.”
A heat-treated, lyophilized factor XIII (coagulation factor XIII) concentrate made from pooled human plasma. Several manufacturing steps are used to inactivate or remove both enveloped and nonenveloped viruses. Factor XIII circulates in blood and is present in platelets, monocytes, and macrophages. Activated factor XIII (factor XIIIa) promotes cross-linking of fibrin during coagulation and is essential to the physiologic protection of the clot against fibrinolysis. Cross-linked fibrin is the end result of the coagulation cascade and provides tensile strength to a primary hemostatic platelet plug. Half-life is approximately 6.6 ± 2.29 days. The increase in plasma levels of factor XIII after administration lasts approximately 28 days.
Routine prophylactic treatment of congenital factor XIII deficiency in adult and pediatric patients. To be effective, a trough factor XIII activity level of approximately 5% to 20% should be maintained.
There are no controlled trials demonstrating a direct benefit on treatment of bleeding episodes.
Known anaphylactic or severe hypersensitivity reactions to human plasma–derived products or to any components of the product.
For IV use only.
■ Hypersensitivity reactions have occurred; emergency equipment, medications, and supplies must be available.
■ Neutralizing inhibitory antibodies against factor XIII have been detected in patients receiving factor XIII.
■ Thromboembolic complications have been reported. Assess benefit versus risk in pregnant women because of their hypercoagulable state and potential for increased risk of thromboembolic events.
■ Made from human plasma and may contain infectious agents (e.g., HIV, Creutzfeldt-Jakob disease, hepatitis B, or hepatitis C). Numerous steps in the manufacturing process are used to reduce the potential for infection.
■ Consider appropriate vaccination against hepatitis A and B.
Monitor trough factor XIII activity levels as outlined in Usual Dose and Dose Adjustments.
■ Monitor for S/S of hypersensitivity reactions (e.g., chest pain, dizziness, dyspnea, fever, flushing, hypotension, nausea, pruritus, rash, rigors, urticaria).
■ Monitor for possible development of inhibitory antibodies (e.g., response to treatment is inadequate, expected plasma factor XIII activity levels are not attained, or breakthrough bleeding occurs).
■ Monitor for thromboembolic complications (e.g., chest pain, dyspnea, edema, hemoptysis, leg pain, or positive Homans’ sign).
■ Monitor for S/S of viral infection (e.g., chills, drowsiness, fever, runny nose followed by joint pain and rash or abdominal pain, dark urine, jaundice, nausea, vomiting).
Manufactured from pooled human plasma. Possibility of viral transmission exists. Promptly report S/S of viral infections (e.g., chills, drowsiness, fever, runny nose followed by joint pain and rash or abdominal pain, dark urine, jaundice, nausea, vomiting).
■ Promptly report difficulty breathing, pruritus, or rash.
■ Promptly report breakthrough bleeding.
Category C: use only if clearly needed; see Precautions.
■ Use only if clearly needed in breast-feeding women.
■ No apparent differences in the safety profile in pediatric patients compared with adults. Pediatric patients under 16 years of age had a shorter half-life and faster clearance compared with adults.
Numbers in clinical studies insufficient to determine whether elderly patients respond differently than younger subjects.
The most commonly reported side effects included arthralgia, chills, elevated thrombin-antithrombin levels, fever, headache, hypersensitivity reactions (including allergy, erythema, pruritus, and rash), and increased hepatic enzymes. Other reported side effects included abdominal pain, diarrhea, epistaxis, flu-like syndrome, hematoma, URT infection, and vomiting.
Hypersensitivity reactions (including anaphylaxis), factor XIII inhibition (neutralizing antibodies), thrombotic events (e.g., embolism, thrombosis), viral infection (possible).