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Idelvion

Generic Name: Coagulation Factor IX (Recombinant, Human), Albumin Fusion Protein (rIX-FP)
Class: Hemostatics

Introduction

Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) is a hemostatic.

Uses for Idelvion

Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) has the following uses:

Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP), a recombinant human blood coagulation factor, is indicated in children and adults with hemophilia B (congenital factor IX deficiency) for on-demand control and prevention of bleeding episodes, perioperative management of bleeding, and routine prophylaxis to prevent or reduce the frequency of bleeding episodes. 1

Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) has the following limitations of use:

Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) is not indicated for immune tolerance induction in patients with hemophilia B.1

Idelvion Dosage and Administration

General

Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) is available in the following dosage form(s) and strength(s):

Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) is available as a lyophilized powder in single-use vials containing nominally 250, 500, 1000 or 2000 international units (IU, units).1

Dosage

It is essential that the manufacturer's labeling be consulted for more detailed information on dosage and administration of this drug. Dosage summary:

For intravenous use after reconstitution only. 1

  • Each vial of coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) is labeled with the actual factor IX potency in units.1

  • One unit of coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) per kg body weight is expected to increase the circulating activity of factor IX as follows:

    Adolescents and adults: 1.3 units/dL per unit/kg

    Pediatrics (<12 years): 1 unit/dL per unit/kg1

  • Administer intravenously. Do not exceed infusion rate of 10 mL per minute.1

Control and Prevention of Bleeding Episodes and Perioperative Management

  • Dosage and duration of treatment with coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) depends on the severity of the factor IX deficiency, the location and extent of bleeding, and the patient's clinical condition, age, and recovery of factor IX.1

  • Determine the initial dose using the following formula: 1

Required dose (units) = body weight (kg) × desired factor IX rise (% of normal or units/dL) × (reciprocal of recovery (units/kg per units/dL))

  • Adjust dose based on the patient's clinical condition and response.1

Routine Prophylaxis

  • Patients ≥12 years of age: 25-40 units/kg body weight every 7 days. Patients who are well-controlled on this regimen may be switched to a 14-day interval at 50-75 units/kg body weight.1

  • Patients <12 years of age: 40-55 units/kg body weight every 7 days.1

Cautions for Idelvion

Contraindications

Do not use in patients who have had life-threatening hypersensitivity reactions to coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) or its components, including hamster proteins.1

Warnings/Precautions

Hypersensitivity Reactions

Hypersensitivity reactions, including anaphylaxis, are possible. Early signs of hypersensitivity reactions, which can progress to anaphylaxis include angioedema, chest tightness, hypotension, generalized urticaria, wheezing, and dyspnea. If hypersensitivity symptoms occur, immediately discontinue administration and initiate appropriate treatment.1

Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) contains trace amounts of Chinese hamster ovary (CHO) proteins. Patients treated with this product may develop hypersensitivity to these non-human mammalian proteins.1

Neutralizing Antibodies

The formation of neutralizing antibodies (inhibitors) to factor IX may occur. Monitor all patients treated with coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) for the development of neutralizing antibodies (inhibitors) by appropriate clinical observations or laboratory tests. Perform an assay that measures factor IX inhibitor concentration if expected plasma factor IX activity levels are not attained, or if the bleeding is not controlled with an appropriate dose.1

Patients with factor IX inhibitors are at an increased risk of severe hypersensitivity reactions or anaphylaxis if re-exposed to factor IX. Evaluate patients experiencing allergic reactions for the presence of an inhibitor and closely monitor patients with inhibitors for signs and symptoms of acute hypersensitivity reactions, particularly during early phases of exposure to the product.1

Thromboembolic Complications

Thromboembolism (e.g., pulmonary embolism, venous thrombosis, and arterial thrombosis) may occur when using factor IX-containing products. Because of the potential risk for thromboembolism with the use of factor IX products, monitor for early signs of thromboembolism and consumptive coagulopathy when administering coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) to patients with liver disease, fibrinolysis, perioperative status, or risk factors for thromboembolic events or disseminated intravascular coagulation.1

Nephrotic Syndrome

Nephrotic syndrome has been reported following attempted immune tolerance induction in hemophilia B patients with factor IX inhibitors and a history of allergic reactions. The safety and efficacy of using coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) for immune tolerance induction have not been established.1

Monitoring Laboratory Tests

  • Monitor factor IX plasma levels by a one-stage clotting assay to confirm that adequate factor IX levels have been achieved and maintained. Factor IX activity assay results may vary with the type of activated partial thromboplastin time (aPTT) reagent used in the assay system. For example, kaolin-based aPTT reagents along with other reagents designed to exhibit low responsiveness to lupus anticoagulant have been shown to result in approximately 50% lower than expected recovery based on labeled potency.1

  • Consistent with similar findings for other recombinant factor IX products, overestimation of factor IX activity in spiked samples of coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) (mean overestimation 32%) occurred at low factor IX levels with commonly used aPTT reagents.1

  • Monitor patients for the development of inhibitors if expected factor IX activity plasma levels are not attained, or if bleeding is not controlled with the recommended dose of coagulation factor IX (recombinant), albumin fusion protein (rIX-FP). Assays used to determine if a factor IX inhibitor is present should be titered in Bethesda Units (BUs).1

Specific Populations

Pregnancy

There are no data with coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) use in pregnant women to inform on drug-associated risk. Animal reproduction studies have not been conducted using coagulation factor IX (recombinant), albumin fusion protein (rIX-FP). It is not known whether coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) can cause fetal harm or affect reproduction capacity when administered to a pregnant woman. Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) should be given to a pregnant woman only if clearly needed. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2-4% and 15-20%, respectively.1

Lactation

There is no information regarding the excretion of coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) in human milk, the effect on the breastfed infant, or the effects on milk production. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) and any potential adverse effects on the breastfed infant from coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) or from the underlying maternal condition.1

Pediatric Use

In clinical studies that included 34 subjects <18 years old, the prophylactic administration with coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) every 7 days was successful in prevention of spontaneous bleeding episodes requiring treatment. Of these, 7 subjects were ≥12 and <18 years old; 5 of these subjects switched to a 10- or 14-day prophylactic administration with coagulation factor IX (recombinant), albumin fusion protein (rIX-FP). There were no apparent differences in the safety profile in subjects <18 years as compared to adults. 1

Compared to adults, incremental rIX-FP recovery appeared to be slightly lower and body weight-adjusted clearance appeared to be higher. Children may have higher factor IX body weight-adjusted clearance, shorter half-life, and lower recovery. Higher dose per kilogram body weight or more frequent dosing may be needed in these patients.1

Geriatric Use

Clinical studies of coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) did not include subjects over 65 years to determine whether or not they respond differently from younger subjects.1

Common Adverse Effects

The most common adverse reaction (incidence ≥1%) reported in clinical trials was headache.1

Drug Interactions

Specific Drugs

It is essential that the manufacturer's labeling be consulted for more detailed information on interactions with this drug, including possible dosage adjustments. Interaction highlights:

Please see product labeling for drug interaction information.

Actions

Mechanism Of Action

Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) is a recombinant protein that temporarily replaces the missing coagulation factor IX needed for effective hemostasis. Coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) is comprised of genetically fused recombinant coagulation factor IX and recombinant albumin. Fusion with recombinant albumin extends the half-life of factor IX.1

Advice to Patients

Patient Counseling Information

Advise patients to read the FDA-approved patient labeling (Patient Information and Instructions for Use).1

Advise patients to report any adverse reactions or problems following coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) administration to their healthcare provider.1

Inform patients of the early signs and symptoms of hypersensitivity or allergic reactions (including hives, generalized urticaria, chest tightness, wheezing, and hypotension). Instruct patients to discontinue use of coagulation factor IX (recombinant), albumin fusion protein (rIX-FP) and contact their healthcare provider and/or seek immediate emergency care if these symptoms occur.1

Advise patients to contact their healthcare provider or hemophilia treatment center for further treatment and/or assessment if they experience a lack of clinical response to factor IX replacement therapy, as in some cases this may be a manifestation of an inhibitor.1

Additional Information

AHFS First Release. For additional information until a more detailed monograph is developed and published, the manufacturer's labeling should be consulted. It is essential that the manufacturer's labeling be consulted for more detailed information on usual uses, dosage and administration, cautions, precautions, contraindications, potential drug interactions, laboratory test interferences, and acute toxicity.

Preparations

Excipients in commercially available drug preparations may have clinically important effects in some individuals; consult specific product labeling for details.

Please refer to the ASHP Drug Shortages Resource Center for information on shortages of one or more of these preparations.

Coagulation Factor IX (Recombinant), Albumin Fusion Protein (rIX-FP)

Routes

Dosage Forms

Strengths

Brand Names

Manufacturer

Parenteral

For injection, for IV use only

number of units indicated on label (nominally 250, 500, 1000, or 2000 units)

Idelvion (with sterile water for injection diluent and filter transfer set)

CSL Behring

AHFS DI Essentials. © Copyright, 2016, American Society of Health-System Pharmacists, Inc., 7272 Wisconsin Avenue, Bethesda, Maryland 20814.

Date modified: October 12, 2016
Last reviewed: September 13, 2016
Date published: September 13, 2016

References

1. CSL Behring Recombinant Facility AG. IDELVION (Coagulation Factor IX (Recombinant), Albumin Fusion Protein (rIX-FP)) prescribing information. 2016 Mar.

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