Dosing & Uses
Dosage Forms & Strengths
lyophilized powder for reconstitution
- 250 IU/vial
- 500 IU/vial
- 1000 IU/vial
- 2000 IU/vial
- 3500 IU/vial
Hemophilia B
Indicated for children and adults with hemophilia B (congenital Factor IX deficiency) for
- On-demand control and prevention of bleeding episodes
- Perioperative management of bleeding
- Routine prophylaxis to prevent or reduce the frequency of bleeding episodes
Dose calculation
- Dosage and treatment duration depends on the severity of Factor IX deficiency, the location and extent of bleeding, and the patient’s clinical condition, age, and recovery of Factor IX
- Calculating the required dose is based on the empirical finding that one IU per kg body weight is expected to increase the circulating level of Factor IX by 1.3 IU/dL in patients aged ≥12 yr, and by 1 IU/dL in patients aged <12 yr
- Required units (IU) = Body weight (kg) x desired Factor IX rise (% of normal or IU/dL) x (reciprocal of recovery [IU/dL per IU/kg]), OR
- Increase in Factor IX IU/dL (or % of normal) = Dose (IU) x recovery (IU/dL per IU/kg)/body weight (kg)
- Adjust the dose based on the individual patient’s clinical condition and response
Hemophilia B, Bleeding Episodes
Indicated for on-demand control and prevention of bleeding episodes
Minor or moderate
- Uncomplicated hemarthrosis, muscle bleeding (except iliopsoas), or oral bleeding
- Maintain circulating FIX at 30-60 % or IU/dL; may repeat q48-72hr; duration of at least 1day, until bleeding stops and healing is achieved; single dose should be sufficient for majority of these bleeds
Major
- Life- or limb-threatening hemorrhage; deep muscle bleeding, including iliopsoas, intracranial, and retropharyngeal
- Maintain circulating Factor IX at 60-100 % or IU/dL; may repeat q48-72hr; duration of 7-14 days, until bleeding stops and healing is achieved; consider weekly maintenance dose
Hemophilia B, Perioperative Management
Indicated for perioperative management of bleeding
Minor
- Includes uncomplicated tooth extraction
- Maintain circulating Factor IX at 50-80 % or IU/dL; duration of at least 1day or until healing is achieved; single dose should be sufficient for majority of minor surgeries
Major
- Includes intracranial, pharyngeal, retropharyngeal, or retroperitoneal
- Maintain circulating FactorIX at 60-100 % or IU/dL; repeat q48-72hr for first week or until healing achieved; duration of 7-14 days, until bleeding stops and healing is achieved; maintenance dose 1-2 times per week
Hemophilia B, Routine Prophylaxis
Indicated for routine prophylaxis to prevent or reduce the frequency of bleeding episodes
25-40 IU/kg IV q7days
Patients who are well-controlled on the 7-day regimen may be switched to a 14-day interval regimen of 50-75 IU/kg
Adjust dose based on individual response
Dosing Considerations
Limitation of use: Not indicated for immune tolerance induction in patients with hemophilia B
Dosage Forms & Strengths
lyophilized powder for reconstitution
- 250 IU/vial
- 500 IU/vial
- 1000 IU/vial
- 2000 IU/vial
- 3500 IU/vial
Hemophilia B
Indicated for children and adults with hemophilia B (congenital Factor IX deficiency) for
- On-demand control and prevention of bleeding episodes
- Perioperative management of bleeding
- Routine prophylaxis to prevent or reduce the frequency of bleeding episodes
Dose calculation
- Dosage and treatment duration depends on the severity of Factor IX deficiency, the location and extent of bleeding, and the patient’s clinical condition, age, and recovery of Factor IX
- Calculating the required dose is based on the empirical finding that one IU per kg body weight is expected to increase the circulating level of Factor IX by 1.3 IU/dL in patients aged ≥12 yr, and by 1 IU/dL in patients aged <12 yr
- Required units (IU) = Body weight (kg) x desired Factor IX rise (% of normal or IU/dL) x (reciprocal of recovery [IU/dL per IU/kg]), OR
- Increase in Factor IX IU/dL (or % of normal) = Dose (IU) x recovery (IU/dL per IU/kg)/body weight (kg)
- Adjust the dose based on the individual patient’s clinical condition and response
Hemophilia B, Bleeding Episodes
Indicated for on-demand control and prevention of bleeding episodes
Minor or moderate
- Uncomplicated hemarthrosis, muscle bleeding (except iliopsoas), or oral bleeding
- Maintain circulating Factor IX at 30-60 % or IU/dL; may repeat q48-72hr; duration of at least 1day, until bleeding stops and healing is achieved; single dose should be sufficient for majority of these bleeds
Major
- Life or limb threatening hemorrhage; deep muscle bleeding, including iliopsoas, intracranial, and retropharyngeal
- Maintain circulating Factor IX at 60-100 % or IU/dL; may repeat q48-72hr; duration of 7-14 days, until bleeding stops and healing is achieved; consider weekly maintenance dose
Hemophilia B, Perioperative Management
Indicated for perioperative management of bleeding
Minor
- Includes uncomplicated tooth extraction
- Maintain circulating Factor IX at 50-80 % or IU/dL; duration of at least 1day or until healing is achieved; single dose should be sufficient for majority of minor surgeries
Major
- Includes intracranial, pharyngeal, retropharyngeal, or retroperitoneal
- Maintain circulating Factor IX at 60-100 % or IU/dL; repeat q48-72hr for first week or until healing achieved; duration of 7-14 days, until bleeding stops and healing is achieved; maintenance dose 1-2 times per week
Hemophilia B, Routine Prophylaxis
Indicated for routine prophylaxis to prevent or reduce the frequency of bleeding episodes
<12 years: 40-55 IU/kg IV q7days
≥12 years
- 25-40 IU/kg IV q7days
- Patients who are well-controlled on the 7-day regimen may be switched to a 14-day interval regimen of 50-75 IU/kg
- Adjust dose based on individual response
Dosing Considerations
Limitation of use: Not indicated for immune tolerance induction in patients with hemophilia B
Adverse Effects
1-10%
Headache (1.8%)
<1%
Dizziness
Hypersensitivity
Rash
Eczema
Warnings
Contraindications
Life-threatening hypersensitivity reactions to the drug or its components, including hamster proteins
Cautions
Hypersensitivity reactions, including anaphylaxis, are possible; if hypersensitivity symptoms occur, immediately discontinue administration and initiate appropriate treatment (see Contraindications)
Formation of neutralizing antibodies to Factor IX may occur
Thromboembolism (eg, pulmonary embolism, venous thrombosis, arterial thrombosis) may occur when using Factor IX-containing products
Nephrotic syndrome reported following attempted immune tolerance induction in hemophilia B patients who develop
Patients with Factor IX inhibitors and a history of allergic reactions
Monitor Factor IX plasma levels and development of inhibitors
Pregnancy & Lactation
Pregnancy
No data exist regarding use in pregnant women to inform on drug-associated risk
Animal reproduction studies have not been conducted
Should be given to a pregnant woman only if clearly needed
Lactation
Unknown if distributed in human breast milk
Consider the developmental and health benefits of breastfeeding along with the mother’s clinical need for the drug, and any potential adverse effects on the breastfed infant from the drug or from the underlying maternal condition
Pregnancy Categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk. C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done. D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk. X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist. NA: Information not available.Pharmacology
Mechanism of Action
Recombinant protein that temporarily replaces missing coagulation Factor IX needed for effective hemostasis
Comprised of genetically fused recombinant coagulation Factor IX and recombinant albumin; fusion with recombinant albumin extends the half-life of Factor IX
Absorption
Peak plasma concentration: 41.1-82 IU/dL
AUC: 4658-9345 h·IU/dL
Distribution
Vd: 0.86-1.2 dL/kg
Elimination
Half-life: 104-188 hr
Mean residence time: 143-153 hr
Clearance: 0.57-0.84 mL/hr/kg
Administration
IV Preparation
Each vial contains the recombinant Factor IX potency in international units (IU) that is stated on the carton and vial label
Follow directions within kit by using provided diluent (sterile water for injection) and Mix2Vial provided
IV Administration
For IV use after reconstitution only
Do not mix or administer in the same tubing or container with other medicinal products
Visually inspect the final solution for particulate matter and discoloration; do not use if particulate matter or discoloration observed
Attach the syringe containing the reconstituted solution to a sterile infusion set and administer by IV injection
Adapt the infusion rate to the comfort level of each patient, not exceeding 10 mL/min
Administer at room temperature and within 4 hr of reconstitution
Discard any unused product
Storage
Unreconstituted vial
- Store in original package to protect from light
- May store in refrigeratory or at room temperature (2-25°C [36-77°F])
- Do not freeze
Images
Patient Handout
factor IX recombinant, Fc fusion protein intravenous
FACTOR IX, RECOMBINANT - INJECTION
(FACK-ter nine)
COMMON BRAND NAME(S): Alprolix, Benefix, Ixinity
USES: This medication is used to prevent or control bleeding in people with little or no factor IX (due to hemophilia B, Christmas disease). Factor IX is a protein (clotting factor) in the blood that works with other clotting factors to help the blood clot and stop bleeding. People with little or no factor IX are at risk for bleeding longer after an injury/surgery or bleeding suddenly (often in the joints/muscle) without an obvious cause.This medication should not be used to treat other types of factor deficiencies (such as factors II, VII, VIII, X) or factor problems (such as inhibitor to factor XIII), to reverse the effects of "blood thinners" (such as warfarin), or to treat bleeding from low levels of liver-dependent clotting factors (due to liver problems).
HOW TO USE: Read the Patient Information Leaflet if available from your pharmacist before you start using factor IX and each time you get a refill. If you have any questions, ask your doctor or pharmacist.This medication is given by injection into a vein over several minutes as directed by your doctor. Dosage is based on your medical condition (such as the amount and location of bleeding), weight, and response to treatment.If you are giving this medication to yourself at home, learn all preparation and usage instructions from your health care professional. Before using, check this product visually for particles or discoloration. If either is present, do not use the liquid. Learn how to store and discard medical supplies safely.The medication in the vial and the supplies that come with the medication should be used only once. Do not reuse.For some brands, it is important to limit the amount of blood entering the tubing and to avoid letting any blood enter the syringe. You may need to use a new vial of medication and new set of supplies if this occurs. Consult your pharmacist for details.Tell your doctor if your condition lasts or gets worse.
SIDE EFFECTS: Fever, pain at injection site, chills, headache, flushing, weakness, nausea, or vomiting may occur. If any of these effects last or get worse, tell your doctor or pharmacist promptly.Remember that this medication has been prescribed because your doctor has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.Tell your doctor right away if you have any serious side effects, including: swelling at injection site, shortness of breath, fast heartbeat, signs of kidney problems (such as change in the amount of urine), swelling of the ankles/feet, pain/redness/swelling of arms or legs, new or increased bleeding/bruising.Get medical help right away if you have any very serious side effects, including: bluish fingers, chest pain, trouble breathing.A very serious allergic reaction to this drug is rare. However, get medical help right away if you notice any symptoms of a serious allergic reaction, including: rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, trouble breathing.This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.In the US -Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.
PRECAUTIONS: Before using factor IX, tell your doctor or pharmacist if you are allergic to factor IX products; or if you have any other allergies. This product may contain inactive ingredients (such as latex), which can cause allergic reactions or other problems. Talk to your pharmacist for more details.Before using this medication, tell your doctor or pharmacist your medical history, especially of: other clotting disorders (such as disseminated intravascular coagulation-DIC), recent surgery/procedure, liver disease.Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products).During pregnancy, this medication should be used only when clearly needed. Discuss the risks and benefits with your doctor.It is not known whether this medication passes into breast milk. Consult your doctor before breast-feeding.
DRUG INTERACTIONS: Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.Some products that may interact with this drug include: drugs that help with clotting (such as aminocaproic acid, tranexamic acid).
OVERDOSE: If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call their local poison control center at 1-800-222-1222. Canada residents can call a provincial poison control center.
NOTES: Do not share this medication with others.Lab and/or medical tests (such as factor IX activity) should be done while you are using this medication. Keep all medical and lab appointments. Consult your doctor for more details.
MISSED DOSE: It is important to get each dose of this medication as scheduled. If you miss a dose, ask your doctor or pharmacist right away for a new dosing schedule. Do not double the dose to catch up.
STORAGE: Consult the product instructions and your pharmacist for storage details. Keep all medications away from children and pets.Do not flush medications down the toilet or pour them into a drain unless instructed to do so. Properly discard this product when it is expired or no longer needed. Consult your pharmacist or local waste disposal company.
MEDICAL ALERT: Your condition can cause complications in a medical emergency. For information about enrolling in MedicAlert, call 1-888-633-4298 (US) or 1-800-668-1507 (Canada).
Information last revised December 2022. Copyright(c) 2023 First Databank, Inc.
IMPORTANT: HOW TO USE THIS INFORMATION: This is a summary and does NOT have all possible information about this product. This information does not assure that this product is safe, effective, or appropriate for you. This information is not individual medical advice and does not substitute for the advice of your health care professional. Always ask your health care professional for complete information about this product and your specific health needs.
Formulary
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