Alprolix 4000unit Inj 1 Vial
It is used to treat or prevent bleeding in people with hemophilia.
Drug Class
Antihemophilic agent
Pharmacologic Class
Coagulation Factor IX
Pregnancy Category
Not available
FDA Approved
Mar 2014
DEA Schedule
Not Controlled
Overview
What is this medicine?
Alprolix is a medicine used to treat and prevent bleeding in people with hemophilia B. Hemophilia B is a bleeding disorder where the body doesn't make enough of a protein called Factor IX, which is needed for blood clotting. Alprolix works by replacing this missing Factor IX, helping your blood to clot properly and reduce bleeding episodes. It's given as an injection into a vein.
How to Use This Medicine
Proper Use of This Medication
To use this medication correctly, follow your doctor's instructions and read all accompanying information carefully. This medication is administered as an intravenous injection. If you are self-administering, your doctor or nurse will provide guidance on the proper injection technique. Before and after handling the medication, wash your hands thoroughly.
Preparing the Medication
Before use, this medication must be mixed according to the instructions provided by your doctor. If the medication has been stored in the refrigerator, allow it to reach room temperature before mixing. Do not heat the medication, and avoid shaking the vial. The mixed solution should be used within 3 hours of preparation. After mixing, do not refrigerate the solution. Protect it from direct sunlight.
Inspecting the Solution
Before administering the medication, inspect the solution for any signs of cloudiness, leakage, or particulate matter. Do not use the medication if the solution appears cloudy, is leaking, or contains particles. Additionally, do not use the medication if the solution has changed color.
Disposing of Needles and Supplies
Dispose of used needles and other sharp objects in a designated needle/sharp disposal box. Do not reuse needles or other items. When the disposal box is full, follow local regulations for proper disposal. If you have any questions or concerns, consult your doctor or pharmacist.
Storage and Disposal
Store this medication in the refrigerator, but do not freeze it. If you miss a dose, contact your doctor to determine the best course of action.
To use this medication correctly, follow your doctor's instructions and read all accompanying information carefully. This medication is administered as an intravenous injection. If you are self-administering, your doctor or nurse will provide guidance on the proper injection technique. Before and after handling the medication, wash your hands thoroughly.
Preparing the Medication
Before use, this medication must be mixed according to the instructions provided by your doctor. If the medication has been stored in the refrigerator, allow it to reach room temperature before mixing. Do not heat the medication, and avoid shaking the vial. The mixed solution should be used within 3 hours of preparation. After mixing, do not refrigerate the solution. Protect it from direct sunlight.
Inspecting the Solution
Before administering the medication, inspect the solution for any signs of cloudiness, leakage, or particulate matter. Do not use the medication if the solution appears cloudy, is leaking, or contains particles. Additionally, do not use the medication if the solution has changed color.
Disposing of Needles and Supplies
Dispose of used needles and other sharp objects in a designated needle/sharp disposal box. Do not reuse needles or other items. When the disposal box is full, follow local regulations for proper disposal. If you have any questions or concerns, consult your doctor or pharmacist.
Storage and Disposal
Store this medication in the refrigerator, but do not freeze it. If you miss a dose, contact your doctor to determine the best course of action.
Lifestyle & Tips
- Maintain regular physical activity as advised by your doctor, avoiding activities that pose a high risk of injury.
- Wear medical alert identification (e.g., bracelet or necklace) indicating you have hemophilia B.
- Keep a detailed record of all infusions, including date, time, dose, and reason for infusion.
- Avoid aspirin and NSAIDs (non-steroidal anti-inflammatory drugs) unless specifically approved by your doctor, as they can increase bleeding risk.
- Practice good dental hygiene to prevent gum bleeding.
Available Forms & Alternatives
Available Strengths:
- Alprolix 500unit Inj 1 Vial
- Alprolix 1000 Unit Inj 1 Vial
- Alprolix 2000 Unit Inj 1 Vial
- Alprolix 3000unit Inj 1 Vial
- Alprolix 4000unit Inj 1 Vial
- Alprolix 250unit Inj 1 Vial
- Alprolix 4000unit Inj 1 Vial
- Alprolix 250unit Inj, 1 Vial
- Alprolix 500unit Inj 1 Vial
- Alprolix 1000unit Inj 1 Vial
- Alprolix 2000unit Inj 1 Vial
- Alprolix 3000unit Inj 1 Vial
Dosing & Administration
Adult Dosing
Standard Dose:
Individualized based on patient's Factor IX deficiency, location and extent of bleeding, and clinical condition. Prophylaxis: Initial dose 50 IU/kg once weekly. On-demand treatment: 20-100 IU/kg depending on severity of bleed.
Dose Range:
20 - 100 mg
Condition-Specific Dosing:
prophylaxis:
50 IU/kg once weekly, or 100 IU/kg every 10 days, or 60 IU/kg every 7 days (for some patients)
on_demand_minor_hemorrhage:
20-40 IU/kg to achieve 20-40% Factor IX activity
on_demand_moderate_hemorrhage:
40-60 IU/kg to achieve 40-60% Factor IX activity
on_demand_major_hemorrhage:
75-100 IU/kg to achieve 75-100% Factor IX activity
Pediatric Dosing
Neonatal:
Not established
Infant:
Dosing individualized based on Factor IX deficiency and clinical condition. Similar to adult dosing on a per kg basis.
Child:
Dosing individualized based on Factor IX deficiency and clinical condition. Similar to adult dosing on a per kg basis.
Adolescent:
Dosing individualized based on Factor IX deficiency and clinical condition. Similar to adult dosing on a per kg basis.
Dose Adjustments
Renal Impairment:
Mild:
No specific dose adjustment recommended.
Moderate:
No specific dose adjustment recommended.
Severe:
No specific dose adjustment recommended.
Dialysis:
No specific dose adjustment recommended. Factor IX is a large protein and not significantly removed by dialysis.
Hepatic Impairment:
Mild:
No specific dose adjustment recommended.
Moderate:
No specific dose adjustment recommended.
Severe:
No specific dose adjustment recommended.
Pharmacology
Mechanism of Action
Eftrenonacog alfa is a recombinant coagulation Factor IX Fc fusion protein. It replaces the deficient Factor IX in patients with hemophilia B. Factor IX is a vitamin K-dependent coagulation factor that is synthesized in the liver and circulates in an inactive form. It is activated by Factor XIa in the intrinsic coagulation pathway and by Factor VIIa in the extrinsic coagulation pathway. Activated Factor IX (FIXa), in conjunction with Factor VIIIa, activates Factor X, which then converts prothrombin to thrombin. Thrombin then converts fibrinogen to fibrin, which forms a clot. The Fc fusion protein extends the half-life of Factor IX by utilizing the neonatal Fc receptor (FcRn) pathway, which recycles IgG antibodies and prolongs their circulation.
Pharmacokinetics
Absorption:
Bioavailability:
100% (intravenous administration)
Tmax:
Not applicable (IV administration, peak activity is at end of infusion)
FoodEffect:
Not applicable (IV administration)
Distribution:
Vd:
Approximately 100-120 mL/kg
ProteinBinding:
Not applicable (circulates as a protein)
CnssPenetration:
Limited (large protein)
Elimination:
HalfLife:
Approximately 82.1 hours (terminal half-life, significantly longer than conventional Factor IX products)
Clearance:
Approximately 1.3 mL/hr/kg
ExcretionRoute:
Proteolytic degradation products are excreted renally.
Unchanged:
Not applicable (protein degradation)
Pharmacodynamics
OnsetOfAction:
Immediate (upon completion of infusion)
PeakEffect:
Immediately after infusion
DurationOfAction:
Extended due to prolonged half-life, allowing for less frequent dosing (e.g., once weekly or every 10-14 days for prophylaxis).
Safety & Warnings
Side Effects
Urgent Side Effects: Seek Medical Help Right Away
Although rare, some people may experience severe and potentially life-threatening side effects while taking this medication. If you notice any of the following symptoms, contact your doctor or seek immediate medical attention:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
Signs of kidney problems, including:
+ Inability to pass urine
+ Changes in urine output
+ Blood in the urine
+ Sudden weight gain
Abnormal sensations, such as:
+ Burning
+ Numbness
+ Tingling
Gastrointestinal issues, including:
+ Upset stomach
+ Vomiting
Dizziness or fainting
Restlessness
Weakness on one side of the body
Difficulty speaking or thinking
Changes in balance
Drooping on one side of the face
Blurred vision
Confusion
Signs of a blood clot, such as:
+ Chest pain or pressure
+ Coughing up blood
+ Shortness of breath
+ Swelling, warmth, numbness, color changes, or pain in a leg or arm
+ Difficulty speaking or swallowing
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only minor ones, it's essential to discuss any concerns with your doctor. If you experience any of the following side effects or any other unusual symptoms, contact your doctor for advice:
Headache
Numbness or tingling in the mouth
Redness of the skin at the injection site
Reporting Side Effects
This list is not exhaustive, and you may experience other side effects. If you have questions or concerns, contact your doctor. You can also report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
Although rare, some people may experience severe and potentially life-threatening side effects while taking this medication. If you notice any of the following symptoms, contact your doctor or seek immediate medical attention:
Signs of an allergic reaction, such as:
+ Rash
+ Hives
+ Itching
+ Red, swollen, blistered, or peeling skin with or without fever
+ Wheezing
+ Tightness in the chest or throat
+ Trouble breathing, swallowing, or talking
+ Unusual hoarseness
+ Swelling of the mouth, face, lips, tongue, or throat
Signs of kidney problems, including:
+ Inability to pass urine
+ Changes in urine output
+ Blood in the urine
+ Sudden weight gain
Abnormal sensations, such as:
+ Burning
+ Numbness
+ Tingling
Gastrointestinal issues, including:
+ Upset stomach
+ Vomiting
Dizziness or fainting
Restlessness
Weakness on one side of the body
Difficulty speaking or thinking
Changes in balance
Drooping on one side of the face
Blurred vision
Confusion
Signs of a blood clot, such as:
+ Chest pain or pressure
+ Coughing up blood
+ Shortness of breath
+ Swelling, warmth, numbness, color changes, or pain in a leg or arm
+ Difficulty speaking or swallowing
Other Possible Side Effects
Like all medications, this drug can cause side effects. While many people may not experience any side effects or only minor ones, it's essential to discuss any concerns with your doctor. If you experience any of the following side effects or any other unusual symptoms, contact your doctor for advice:
Headache
Numbness or tingling in the mouth
Redness of the skin at the injection site
Reporting Side Effects
This list is not exhaustive, and you may experience other side effects. If you have questions or concerns, contact your doctor. You can also report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
Seek Immediate Medical Attention If You Experience:
- Signs of allergic reaction: hives, rash, itching, swelling of the face, lips, tongue, or throat, chest tightness, wheezing, difficulty breathing, dizziness, fainting.
- Signs of a blood clot: pain, swelling, warmth, or redness in an arm or leg; sudden shortness of breath, chest pain, rapid heart rate.
- Signs of bleeding that is not controlled by treatment: persistent or severe bleeding, unusual bruising, blood in urine or stool, severe headache, sudden vision changes.
Before Using This Medicine
Before Taking This Medication: Important Information to Share with Your Doctor
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, any of its components, or other substances, such as foods or drugs. Be sure to describe the symptoms you experienced as a result of the allergy.
Potential interactions with other medications or health conditions. This medication may interact with other drugs or exacerbate certain health problems.
To ensure safe use, provide your doctor and pharmacist with a comprehensive list of:
All prescription and over-the-counter (OTC) medications you are currently taking
Any natural products or vitamins you are using
* Existing health problems or conditions
Before making any changes to your medication regimen, consult with your doctor to confirm that it is safe to take this medication in conjunction with your other drugs and health conditions. Do not start, stop, or adjust the dosage of any medication without first consulting your doctor.
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, any of its components, or other substances, such as foods or drugs. Be sure to describe the symptoms you experienced as a result of the allergy.
Potential interactions with other medications or health conditions. This medication may interact with other drugs or exacerbate certain health problems.
To ensure safe use, provide your doctor and pharmacist with a comprehensive list of:
All prescription and over-the-counter (OTC) medications you are currently taking
Any natural products or vitamins you are using
* Existing health problems or conditions
Before making any changes to your medication regimen, consult with your doctor to confirm that it is safe to take this medication in conjunction with your other drugs and health conditions. Do not start, stop, or adjust the dosage of any medication without first consulting your doctor.
Precautions & Cautions
It is essential to inform all your healthcare providers, including doctors, nurses, pharmacists, and dentists, that you are taking this medication.
This drug may increase the risk of blood clots. If you have a history of blood clots, notify your doctor promptly. Discuss your individual situation with your doctor to assess the potential risks.
Regular blood work and laboratory tests are crucial while taking this medication. Adhere to your doctor's instructions for scheduling these tests to ensure your safety.
If you find that the usual dose is not effective, contact your doctor immediately to discuss alternative options. Before traveling, consult with your doctor to determine the necessary amount of medication to bring along to maintain uninterrupted treatment.
If you are pregnant, planning to become pregnant, or breastfeeding, inform your doctor. It is crucial to discuss the potential benefits and risks of this medication to both you and your baby to make an informed decision about treatment.
This drug may increase the risk of blood clots. If you have a history of blood clots, notify your doctor promptly. Discuss your individual situation with your doctor to assess the potential risks.
Regular blood work and laboratory tests are crucial while taking this medication. Adhere to your doctor's instructions for scheduling these tests to ensure your safety.
If you find that the usual dose is not effective, contact your doctor immediately to discuss alternative options. Before traveling, consult with your doctor to determine the necessary amount of medication to bring along to maintain uninterrupted treatment.
If you are pregnant, planning to become pregnant, or breastfeeding, inform your doctor. It is crucial to discuss the potential benefits and risks of this medication to both you and your baby to make an informed decision about treatment.
Overdose Information
Overdose Symptoms:
- Risk of thrombosis (blood clot formation) if very high doses are administered, especially in patients with risk factors for thrombosis.
- Disseminated intravascular coagulation (DIC) in rare, extreme cases.
What to Do:
Seek immediate medical attention. Management may include stopping the infusion, supportive care, and monitoring for signs of thrombosis or DIC. Call 1-800-222-1222 (Poison Control) for advice.
Drug Interactions
Monitoring
Baseline Monitoring
Factor IX activity levels
Rationale: To establish baseline deficiency and guide initial dosing.
Timing: Prior to first administration.
Inhibitor (neutralizing antibody) screen
Rationale: To detect pre-existing inhibitors to Factor IX, which can impact treatment efficacy.
Timing: Prior to first administration.
Clinical assessment of bleeding history
Rationale: To determine severity of hemophilia B and guide treatment strategy.
Timing: Prior to first administration.
Routine Monitoring
Factor IX activity levels
Frequency: Periodically, especially when initiating therapy, changing dose, or in response to bleeding episodes.
Target: Depends on clinical situation (e.g., 20-40% for minor bleed, 75-100% for major bleed).
Action Threshold: If levels are not therapeutic or if bleeding persists, adjust dose or frequency.
Inhibitor (neutralizing antibody) screen
Frequency: Regularly (e.g., every 3-6 months, or more frequently in patients at high risk for inhibitor development, such as previously untreated patients or those with severe hemophilia B). Also, if expected Factor IX activity levels are not achieved or if bleeding is not controlled.
Target: Negative
Action Threshold: Positive inhibitor titer requires alternative treatment strategies (e.g., bypassing agents).
Signs and symptoms of allergic reactions
Frequency: During and after each infusion.
Target: Absence of symptoms
Action Threshold: Stop infusion immediately if signs of hypersensitivity occur.
Signs and symptoms of thrombosis
Frequency: Periodically, especially in patients with risk factors for thrombosis.
Target: Absence of symptoms
Action Threshold: Investigate and manage promptly if symptoms occur.
Symptom Monitoring
- Bleeding episodes (frequency, severity, location)
- Signs of allergic reaction (hives, rash, swelling, chest tightness, wheezing, dizziness, faintness)
- Signs of thrombosis (pain, swelling, warmth, redness in a limb; shortness of breath, chest pain, rapid heart rate)
- Joint pain or swelling (indicating hemarthrosis)
Special Patient Groups
Pregnancy
There are no adequate and well-controlled studies of Alprolix in pregnant women. Animal reproduction studies have not been conducted. Use only if clearly needed and the potential benefit outweighs the potential risk to the fetus. Hemophilia B is rare in women, but management during pregnancy is critical.
Trimester-Specific Risks:
First Trimester:
Risk unknown. Factor IX is a large protein, unlikely to cross placenta significantly.
Second Trimester:
Risk unknown. Factor IX is a large protein, unlikely to cross placenta significantly.
Third Trimester:
Risk unknown. Factor IX is a large protein, unlikely to cross placenta significantly.
Lactation
It is not known whether Alprolix is excreted in human milk. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for Alprolix and any potential adverse effects on the breastfed infant from Alprolix or from the underlying maternal condition. Factor IX is a large protein, unlikely to be absorbed intact by an infant.
Infant Risk:
Low (L3 - Moderately safe, but no human data)
Pediatric Use
Approved for use in pediatric patients of all ages. Dosing is individualized based on weight, Factor IX levels, and clinical response. Pharmacokinetic parameters are generally similar to adults on a weight-adjusted basis. Close monitoring for inhibitor development is crucial, especially in previously untreated patients.
Geriatric Use
Clinical studies of Alprolix did not include sufficient numbers of subjects aged 65 and over to determine whether they respond differently from younger subjects. In general, dose selection for an elderly patient should be cautious, reflecting the greater frequency of decreased hepatic, renal, or cardiac function, and of concomitant disease or other drug therapy. No specific dose adjustments are recommended based on age alone.
Clinical Information
Clinical Pearls
- Alprolix offers an extended half-life compared to conventional Factor IX products, allowing for less frequent prophylactic infusions (e.g., once weekly or every 10-14 days).
- Individualize dosing based on Factor IX activity levels and clinical response. Regular monitoring of Factor IX activity is crucial, especially when initiating therapy or adjusting doses.
- Patients should be educated on the signs and symptoms of allergic reactions and thrombosis, and instructed to seek immediate medical attention if these occur.
- Inhibitor development is a serious complication in hemophilia B. Patients should be regularly monitored for inhibitors, especially if bleeding is not controlled or Factor IX levels are unexpectedly low.
- Reconstitute Alprolix with the provided diluent and administer intravenously. Do not mix with other medicinal products.
Alternative Therapies
- Other recombinant Factor IX products (e.g., BeneFIX, Rixubis, Idelvion, Rebinyn)
- Plasma-derived Factor IX products (e.g., AlphaNine SD, Mononine)
- Gene therapy for hemophilia B (e.g., Hemgenix)
- Bypassing agents (e.g., FEIBA, NovoSeven RT) for patients with inhibitors.
Cost & Coverage
Average Cost:
Highly variable, typically thousands of dollars per vial depending on unit strength and quantity. per vial
Insurance Coverage:
Specialty Tier (requires prior authorization, often covered by major medical and pharmacy plans for hemophilia B)
General Drug Facts
If your symptoms or health issues persist or worsen, it is essential to contact your doctor for further guidance. To ensure your safety, never share your medication with others or take someone else's medication. Some medications may come with an additional patient information leaflet, so it's a good idea to check with your pharmacist. If you have any questions or concerns about this medication, don't hesitate to discuss them with your doctor, nurse, pharmacist, or other healthcare provider. In the event of a suspected overdose, immediately call your local poison control center or seek emergency medical attention. When seeking help, be prepared to provide detailed information about the overdose, including the medication taken, the amount, and the time it occurred.