Alprolix 2000unit Inj 1 Vial
It is used to treat or prevent bleeding in people with hemophilia.
Drug Class
Antihemophilic Agent
Pharmacologic Class
Coagulation Factor IX, Recombinant, Fc Fusion Protein
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 your blood doesn't clot properly because you don't have enough of a protein called Factor IX. Alprolix replaces this missing protein, helping your blood to clot and stop bleeding. It's designed to stay in your body longer than some other Factor IX medicines, so you might not need to take it as often.
How to Use This Medicine
Proper Use of This Medication
To use this medication correctly, follow your doctor's instructions and read all the information provided. This medication is administered as an injection into a vein. If you will be self-administering the injection, your doctor or nurse will instruct you on the proper 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 medication should be used within 3 hours. After mixing, do not refrigerate the medication, and protect it from direct sunlight.
Inspecting the Medication
Before administering the medication, inspect the solution for any signs of cloudiness, leakage, or particles. Do not use the medication if the solution has changed color or exhibits any of these signs.
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.
Missed Dose
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 the information provided. This medication is administered as an injection into a vein. If you will be self-administering the injection, your doctor or nurse will instruct you on the proper 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 medication should be used within 3 hours. After mixing, do not refrigerate the medication, and protect it from direct sunlight.
Inspecting the Medication
Before administering the medication, inspect the solution for any signs of cloudiness, leakage, or particles. Do not use the medication if the solution has changed color or exhibits any of these signs.
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.
Missed Dose
If you miss a dose, contact your doctor to determine the best course of action.
Lifestyle & Tips
- Maintain regular communication with your healthcare provider regarding your bleeding episodes and treatment effectiveness.
- Carry your medication and emergency contact information at all times.
- Avoid activities that carry a high risk of injury or bleeding.
- Inform all healthcare providers, including dentists, that you have hemophilia B and are on Factor IX replacement therapy.
- Consider wearing a medical alert identification.
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 levels, bleeding episodes, and weight. Prophylaxis: 50 IU/kg once weekly or 100 IU/kg every 10 days. On-demand treatment: Dose depends on severity and location of bleeding.
Dose Range:
20 - 100 mg
Condition-Specific Dosing:
prophylaxis:
50 IU/kg once weekly or 100 IU/kg every 10 days
on_demand_minor_bleeding:
20-30 IU/kg to achieve 20-40% Factor IX activity
on_demand_major_bleeding:
40-80 IU/kg to achieve 60-100% Factor IX activity
Pediatric Dosing
Neonatal:
Not established (limited data, generally not indicated for neonates unless specific diagnosis of hemophilia B)
Infant:
Dosing individualized based on weight and Factor IX levels, similar principles to older children/adults.
Child:
Dosing individualized based on weight and Factor IX levels, similar principles to adults. Prophylaxis: 50 IU/kg once weekly or 100 IU/kg every 10 days.
Adolescent:
Dosing individualized based on weight and Factor IX levels, similar principles to adults. Prophylaxis: 50 IU/kg once weekly or 100 IU/kg every 10 days.
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 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 activated by Factor XIa in the intrinsic pathway and by Factor VIIa/tissue factor in the extrinsic pathway. Activated Factor IX (FIXa) forms a complex with Factor VIIIa, calcium, and phospholipid to activate Factor X. Activated Factor X (FXa) then converts prothrombin to thrombin, which in turn converts fibrinogen to fibrin, leading to clot formation. The Fc fusion protein extends the half-life of Factor IX by utilizing the neonatal Fc receptor (FcRn) salvage pathway, which protects immunoglobulins from lysosomal degradation.
Pharmacokinetics
Absorption:
Bioavailability:
100% (intravenous administration)
Tmax:
Not applicable (IV administration, peak activity is at the end of infusion)
FoodEffect:
Not applicable (IV administration)
Distribution:
Vd:
Approximately 100-120 mL/kg (similar to plasma volume)
ProteinBinding:
Not applicable (is a protein itself)
CnssPenetration:
Limited (large protein, does not cross intact blood-brain barrier)
Elimination:
HalfLife:
Approximately 82 hours (range 60-100 hours) in adults and adolescents; 60-70 hours in children 1-11 years.
Clearance:
Approximately 1.0-1.4 mL/hr/kg
ExcretionRoute:
Primarily catabolic degradation, not renal or hepatic excretion of intact protein.
Unchanged:
Not applicable (protein degradation)
Pharmacodynamics
OnsetOfAction:
Immediate (upon completion of infusion, Factor IX activity levels rise rapidly)
PeakEffect:
Immediately after infusion
DurationOfAction:
Extended due to prolonged half-life, allowing for less frequent dosing (up to 7-10 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
Unusual burning, numbness, or tingling sensations
Upset stomach or vomiting
Dizziness or fainting
Restlessness
Weakness on one side of the body
Trouble 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
+ Trouble 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 or seek medical help:
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
Unusual burning, numbness, or tingling sensations
Upset stomach or vomiting
Dizziness or fainting
Restlessness
Weakness on one side of the body
Trouble 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
+ Trouble 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 or seek medical help:
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: rash, hives, itching, facial swelling, difficulty breathing, chest tightness, dizziness, feeling faint.
- Signs of inhibitor development: increased bleeding episodes, bleeding that doesn't stop with your usual dose of Alprolix.
- Signs of blood clots: pain, swelling, warmth, or redness in an arm or leg; sudden shortness of breath; chest pain; sudden numbness or weakness on one side of the body; sudden confusion or trouble speaking.
- Any new or worsening bleeding that is not controlled by your usual dose.
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, its components, or other substances, such as foods or drugs. Be sure to describe the allergic reaction you experienced, including any symptoms that occurred.
All medications you are currently taking, including prescription and over-the-counter (OTC) drugs, natural products, and vitamins. This is crucial to ensure safe use and avoid potential interactions.
* Any existing health problems, as this medication may interact with other medical conditions or drugs.
To guarantee your safety, do not start, stop, or change the dosage of any medication without first consulting your doctor. It is vital to verify that it is safe to take this medication with all your other medications and health conditions. Your doctor and pharmacist will work together to ensure that all your medications, including this one, are safe and appropriate for you to take.
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, its components, or other substances, such as foods or drugs. Be sure to describe the allergic reaction you experienced, including any symptoms that occurred.
All medications you are currently taking, including prescription and over-the-counter (OTC) drugs, natural products, and vitamins. This is crucial to ensure safe use and avoid potential interactions.
* Any existing health problems, as this medication may interact with other medical conditions or drugs.
To guarantee your safety, do not start, stop, or change the dosage of any medication without first consulting your doctor. It is vital to verify that it is safe to take this medication with all your other medications and health conditions. Your doctor and pharmacist will work together to ensure that all your medications, including this one, are safe and appropriate for you to take.
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 has been associated with an increased risk of blood clots. If you have a history of blood clots, notify your doctor, and discuss the potential risks and benefits.
Regular monitoring of your blood work and other laboratory tests is crucial, as directed by your doctor.
If you find that the usual dose of this medication is not effective, contact your doctor immediately to discuss alternative options.
Before traveling, consult with your doctor to ensure you have an adequate supply of this medication for the duration of your trip.
If you are pregnant, planning to become pregnant, or are breastfeeding, inform your doctor. It is crucial to discuss the potential benefits and risks of this medication to both you and your baby, allowing for an informed decision about its use.
This drug has been associated with an increased risk of blood clots. If you have a history of blood clots, notify your doctor, and discuss the potential risks and benefits.
Regular monitoring of your blood work and other laboratory tests is crucial, as directed by your doctor.
If you find that the usual dose of this medication is not effective, contact your doctor immediately to discuss alternative options.
Before traveling, consult with your doctor to ensure you have an adequate supply of this medication for the duration of your trip.
If you are pregnant, planning to become pregnant, or are breastfeeding, inform your doctor. It is crucial to discuss the potential benefits and risks of this medication to both you and your baby, allowing for an informed decision about its use.
Overdose Information
Overdose Symptoms:
- No specific symptoms of overdose are known, but very high doses could theoretically increase the risk of thrombotic events (blood clots).
What to Do:
In case of suspected overdose, contact your healthcare provider or emergency services immediately. Call 1-800-222-1222 for poison control. Management would be supportive and focused on monitoring for and treating thrombotic complications if they arise.
Drug Interactions
Monitoring
Baseline Monitoring
Factor IX activity levels
Rationale: To establish baseline deficiency and guide initial dosing.
Timing: Prior to initiation of therapy.
Inhibitor (neutralizing antibody) screen
Rationale: To detect pre-existing inhibitors to Factor IX, which can render treatment ineffective.
Timing: Prior to initiation of therapy.
Routine Monitoring
Factor IX activity levels
Frequency: Periodically, especially during dose adjustments, major bleeding episodes, or surgery.
Target: Depends on clinical situation (e.g., >1% for prophylaxis, higher for bleeding/surgery).
Action Threshold: If expected Factor IX activity levels are not achieved or bleeding is not controlled, consider inhibitor development or inadequate dosing.
Inhibitor (neutralizing antibody) screen
Frequency: Regularly (e.g., every 3-6 months, or if bleeding is not controlled with expected doses).
Target: Negative
Action Threshold: Positive inhibitor titer requires alternative treatment strategies.
Signs and symptoms of allergic reaction
Frequency: During and after infusion.
Target: Absence of symptoms
Action Threshold: Presence of rash, hives, itching, facial swelling, dyspnea, chest tightness, or hypotension requires immediate discontinuation and treatment.
Signs and symptoms of thrombosis
Frequency: Periodically, especially in patients with risk factors for thrombosis.
Target: Absence of symptoms
Action Threshold: Presence of pain, swelling, redness in a limb, shortness of breath, chest pain, or neurological changes requires immediate medical evaluation.
Symptom Monitoring
- Signs of bleeding (e.g., joint pain/swelling, bruising, nosebleeds, blood in urine/stool, prolonged bleeding from cuts)
- Signs of allergic reaction (e.g., rash, hives, itching, facial swelling, dyspnea, chest tightness, hypotension)
- Signs of thrombosis (e.g., pain, swelling, redness in a limb, shortness of breath, chest pain, neurological changes)
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 cannot be ruled out due to lack of human data.
Second Trimester:
Risk cannot be ruled out due to lack of human data.
Third Trimester:
Risk cannot be ruled out due to lack of human data.
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. Consider the risk of Factor IX transfer to the infant.
Infant Risk:
Risk cannot be ruled out due to lack of human data. Large protein, likely minimal transfer into milk, and poor oral absorption by infant.
Pediatric Use
Alprolix is approved for use in pediatric patients of all ages. Dosing is individualized based on weight and Factor IX levels, similar to adults. Children, especially those under 12, may have a slightly faster clearance and shorter half-life compared to adults, which may necessitate more frequent dosing or higher doses per kg for prophylaxis in some cases, though the extended half-life benefit is still observed.
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. No specific dose adjustment is recommended based on age alone. Dosing should be individualized based on Factor IX levels and clinical response, as in younger adults.
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 days).
- Monitor for the development of Factor IX inhibitors, especially in previously untreated patients (PUPs) or those with severe hemophilia B, as this can lead to lack of efficacy.
- The dose of Alprolix is expressed in International Units (IU). It is crucial to ensure accurate dosing based on the patient's weight and desired Factor IX activity level.
- Patients should be educated on proper infusion technique if self-administering, and on recognizing signs of bleeding, allergic reactions, and potential thrombotic events.
- While rare, thrombotic events have been reported with Factor IX products, particularly in patients with risk factors or high doses. Monitor accordingly.
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 - etranacogene dezaparvovec)
- Bypassing agents (e.g., FEIBA, NovoSeven RT) for patients with inhibitors.
Cost & Coverage
Average Cost:
Highly variable, typically several thousand to tens of thousands of USD per vial depending on unit strength and supplier. per vial
Insurance Coverage:
Specialty Tier (requires prior authorization, often covered for diagnosed hemophilia B)
General Drug Facts
If your symptoms or health issues persist or worsen, it's 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 be sure to check with your pharmacist for more information. If you have any questions or concerns about this medication, don't hesitate to reach out to 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 details about the overdose, including the medication taken, the amount, and the time it occurred.