Alphanate 500iu Vwf/hum Inj 1 Vl
It is used to treat or prevent bleeding in people with hemophilia.It is used to treat or prevent bleeding in people with von Willebrand disease.
Drug Class
Hemostatic agent
Pharmacologic Class
Blood coagulation factor; Factor VIII/von Willebrand Factor complex
Pregnancy Category
C
FDA Approved
Sep 1999
DEA Schedule
Not Controlled
Overview
What is this medicine?
Alphanate is a medicine made from human plasma that contains two important proteins: Factor VIII and von Willebrand Factor. These proteins are crucial for blood clotting. It is used to treat and prevent bleeding in people with Hemophilia A (a condition where the body doesn't make enough Factor VIII) and von Willebrand Disease (a condition where the body doesn't make enough von Willebrand Factor or it doesn't work properly). It is given as an injection into a vein.
How to Use This Medicine
Taking Your Medication
To use this medication correctly, follow your doctor's instructions and read all the information provided. This medication is administered as an intravenous injection. 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. Do not shake the mixture. The medication should be used within 3 hours of preparation. Check the solution for any changes in color; if it has changed, do not use it. After mixing, you may notice a few flakes or particles, but the filter used during preparation will remove these from the solution. However, if the solution appears cloudy, is leaking, or still contains flakes or particles after filtering, do not use it.
Disposing of Unused Medication and Supplies
After administering the injection, discard any remaining medication from the opened vial. Dispose of 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.
Storing Your Medication
Store the medication at room temperature, avoiding freezing. Keep it in its original container to protect it from light. Ensure that all medications are stored in a safe location, out of the reach of children and pets.
Missing a Dose
If you miss a dose, skip it and resume your regular dosing schedule. Do not take two doses at the same time or extra doses. If you are unsure about what to do in case of a missed dose, contact your doctor for guidance.
To use this medication correctly, follow your doctor's instructions and read all the information provided. This medication is administered as an intravenous injection. 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. Do not shake the mixture. The medication should be used within 3 hours of preparation. Check the solution for any changes in color; if it has changed, do not use it. After mixing, you may notice a few flakes or particles, but the filter used during preparation will remove these from the solution. However, if the solution appears cloudy, is leaking, or still contains flakes or particles after filtering, do not use it.
Disposing of Unused Medication and Supplies
After administering the injection, discard any remaining medication from the opened vial. Dispose of 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.
Storing Your Medication
Store the medication at room temperature, avoiding freezing. Keep it in its original container to protect it from light. Ensure that all medications are stored in a safe location, out of the reach of children and pets.
Missing a Dose
If you miss a dose, skip it and resume your regular dosing schedule. Do not take two doses at the same time or extra doses. If you are unsure about what to do in case of a missed dose, contact your doctor for guidance.
Lifestyle & Tips
- Always carry your medication and identification indicating your bleeding disorder.
- Avoid activities that carry a high risk of injury or bleeding.
- Avoid medications that interfere with blood clotting, such as aspirin and NSAIDs (e.g., ibuprofen, naproxen), unless specifically approved by your doctor.
- Maintain good dental hygiene to prevent gum bleeding.
- Follow your doctor's instructions for regular infusions (prophylaxis) to prevent bleeding episodes.
- Learn how to self-administer the medication if recommended by your healthcare provider.
Available Forms & Alternatives
Available Strengths:
- Alphanate 250iu Vwf/hum Inj 1vl
- Alphanate 500iu Vwf/hum Inj 1 Vl
- Alphanate 1000iu Vwf/hum Inj, 10ml
- Alphanate 1500iu Vwf/hum Inj, 10ml
- Alphanate 2000iu Vwf Human Inj,10ml
- Alphanate 250iu Vwf/hum Inj 1vl
- Alphanate 500iu Vwf/hum Inj 1vl
- Alphanate 1000iu Vwf/hum Inj, 1vl
- Alphanate 1500iu Vwf/hum Inj, 1vl
- Alphanate Vwf Cmplx Human Inj, 1 Vl
Dosing & Administration
Adult Dosing
Standard Dose:
Highly individualized based on indication, severity of bleeding, and patient's weight and baseline factor levels. Administered intravenously.
Condition-Specific Dosing:
Hemophilia A (Factor VIII deficiency):
Dose (IU) = Body Weight (kg) x Desired FVIII Rise (% of normal) x 0.5 (IU/kg per % rise). Administer as needed for bleeding or prophylaxis. For major surgery, target FVIII activity 80-100% pre-op, maintain >50% for 1-2 weeks.
von Willebrand Disease (VWD):
Dose (IU VWF:RCo) = Body Weight (kg) x Desired VWF:RCo Rise (% of normal) x 0.5 (IU/kg per % rise). Initial dose typically 40-80 IU VWF:RCo/kg for major bleeding/surgery. Subsequent doses 20-40 IU VWF:RCo/kg every 12-24 hours as needed. Target VWF:RCo >50% and FVIII >50% for major bleeding/surgery.
Pediatric Dosing
Neonatal:
Dosing based on weight and clinical need, similar principles as older children/adults. Consult hematologist.
Infant:
Dosing based on weight and clinical need, similar principles as older children/adults. Consult hematologist.
Child:
Dosing based on weight and clinical need, similar principles as adults (e.g., for Hemophilia A: Dose (IU) = Body Weight (kg) x Desired FVIII Rise (% of normal) x 0.5).
Adolescent:
Dosing based on weight and clinical need, similar principles as adults.
Dose Adjustments
Renal Impairment:
Mild:
No specific dose adjustment recommended.
Moderate:
No specific dose adjustment recommended.
Severe:
No specific dose adjustment recommended. Monitor for fluid overload.
Dialysis:
No specific dose adjustment recommended. Monitor for fluid overload and clinical response.
Hepatic Impairment:
Mild:
No specific dose adjustment recommended.
Moderate:
No specific dose adjustment recommended.
Severe:
No specific dose adjustment recommended.
Pharmacology
Mechanism of Action
Alphanate provides exogenous Factor VIII (FVIII) and von Willebrand Factor (VWF) to temporarily correct the coagulation defect in patients with Hemophilia A or von Willebrand Disease. FVIII is an essential cofactor in the intrinsic coagulation pathway, facilitating the activation of Factor X by Factor IXa. VWF mediates platelet adhesion to the subendothelium at sites of vascular injury and serves as a carrier protein for FVIII, protecting it from proteolytic degradation and prolonging its half-life.
Pharmacokinetics
Absorption:
Bioavailability:
100%
Tmax:
End of infusion (intravenous administration)
FoodEffect:
Not applicable (intravenous administration)
Distribution:
Vd:
FVIII: Approximately 40-60 mL/kg; VWF: Larger, primarily intravascular.
ProteinBinding:
FVIII binds extensively to von Willebrand Factor.
CnssPenetration:
Limited
Elimination:
HalfLife:
FVIII: Approximately 10-18 hours; VWF:RCo: Approximately 8-12 hours (can be longer for VWF:Ag).
Clearance:
Variable, depends on individual patient characteristics and baseline factor levels.
ExcretionRoute:
Metabolites excreted via renal and biliary pathways.
Unchanged:
Not applicable (protein degradation)
Pharmacodynamics
OnsetOfAction:
Immediate (upon completion of intravenous infusion)
PeakEffect:
At the end of infusion
DurationOfAction:
Dependent on the half-life of FVIII and VWF, typically 12-24 hours for clinical effect, requiring repeat dosing for sustained activity.
Safety & Warnings
Side Effects
Serious Side Effects: Seek Medical Help Immediately
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 medical attention right away:
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 certain infections (parvovirus B19, hepatitis A), including:
+ Fever or chills
+ Headache
+ Feeling very sleepy
+ Runny nose
+ Rash
+ Joint pain
+ Tiredness
+ Poor appetite
+ Upset stomach or vomiting
+ Diarrhea
+ Stomach pain
+ Yellow skin or eyes
Flushing
Severe dizziness or fainting
Abnormal burning, numbness, or tingling sensations
Stinging
Swelling
Severe upset stomach or vomiting
Restlessness
Dark urine or yellow skin or eyes
Rapid heartbeat
Blue discoloration of the mouth
Weakness on one side of the body
Trouble speaking or thinking
Balance problems
Drooping on one side of the face
Blurred vision
Signs of a blood clot, including:
+ Chest pain or pressure
+ Coughing up blood
+ Shortness of breath
+ Swelling, warmth, numbness, change of color, 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 attention:
Upset stomach
Constipation
Dizziness
Fatigue
Weakness
Headache
* Back pain
Reporting Side Effects
If you have questions or concerns about side effects, 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 medical attention right away:
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 certain infections (parvovirus B19, hepatitis A), including:
+ Fever or chills
+ Headache
+ Feeling very sleepy
+ Runny nose
+ Rash
+ Joint pain
+ Tiredness
+ Poor appetite
+ Upset stomach or vomiting
+ Diarrhea
+ Stomach pain
+ Yellow skin or eyes
Flushing
Severe dizziness or fainting
Abnormal burning, numbness, or tingling sensations
Stinging
Swelling
Severe upset stomach or vomiting
Restlessness
Dark urine or yellow skin or eyes
Rapid heartbeat
Blue discoloration of the mouth
Weakness on one side of the body
Trouble speaking or thinking
Balance problems
Drooping on one side of the face
Blurred vision
Signs of a blood clot, including:
+ Chest pain or pressure
+ Coughing up blood
+ Shortness of breath
+ Swelling, warmth, numbness, change of color, 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 attention:
Upset stomach
Constipation
Dizziness
Fatigue
Weakness
Headache
* Back pain
Reporting Side Effects
If you have questions or concerns about side effects, 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 an allergic reaction: hives, rash, itching, swelling of the face/lips/tongue/throat, difficulty breathing, wheezing, dizziness, fainting.
- Signs of a blood clot: chest pain, shortness of breath, pain/swelling/warmth in an arm or leg, sudden vision changes, slurred speech.
- Signs of inhibitor development (in Hemophilia A): increased bleeding episodes, bleeding that doesn't respond to treatment as expected.
- 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, 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 taking
Natural products and vitamins you are using
* Any health problems you have
It is crucial to verify that it is safe to take this medication in conjunction with your other medications and health conditions. Never 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 taking
Natural products and vitamins you are using
* Any health problems you have
It is crucial to verify that it is safe to take this medication in conjunction with your other medications and health conditions. Never 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. Regular blood tests and laboratory checks should be conducted as directed by your doctor to monitor your condition.
If you experience unexplained bruising, bleeding, or if bleeding does not stop after taking this medication, contact your doctor immediately. This could be an indication that your current dosage is not effective.
There is a risk of blood clots associated with this medication. If you have a history of blood clots, discuss this with your doctor to assess the potential risks.
As this medication is derived from human plasma, there is a possibility of viral contamination, which could lead to disease transmission. Although the medication undergoes rigorous screening, testing, and treatment to minimize infection risk, it is crucial to discuss this with your doctor.
Before traveling, consult your doctor to ensure you have an adequate supply of this medication for the duration of your trip.
If you are 65 years or older, exercise caution when using this medication, as you may be more susceptible to side effects.
If you are pregnant, planning to become pregnant, or breastfeeding, inform your doctor to discuss the potential benefits and risks of this medication to both you and your baby.
If you experience unexplained bruising, bleeding, or if bleeding does not stop after taking this medication, contact your doctor immediately. This could be an indication that your current dosage is not effective.
There is a risk of blood clots associated with this medication. If you have a history of blood clots, discuss this with your doctor to assess the potential risks.
As this medication is derived from human plasma, there is a possibility of viral contamination, which could lead to disease transmission. Although the medication undergoes rigorous screening, testing, and treatment to minimize infection risk, it is crucial to discuss this with your doctor.
Before traveling, consult your doctor to ensure you have an adequate supply of this medication for the duration of your trip.
If you are 65 years or older, exercise caution when using this medication, as you may be more susceptible to side effects.
If you are pregnant, planning to become pregnant, or breastfeeding, inform your doctor to discuss the potential benefits and risks of this medication to both you and your baby.
Overdose Information
Overdose Symptoms:
- Increased risk of thrombotic events (blood clots) due to excessively high Factor VIII and von Willebrand Factor levels.
- Fluid overload (due to large volume of infusion).
What to Do:
In case of suspected overdose, contact your healthcare provider or emergency services immediately. Call 911 or your local emergency number. For general overdose information, you can call a poison control center at 1-800-222-1222. Management may involve stopping the infusion, supportive care, and potentially anticoagulant therapy if thrombosis occurs.
Drug Interactions
Moderate Interactions
- Antifibrinolytic agents (e.g., aminocaproic acid, tranexamic acid)
Monitoring
Baseline Monitoring
Factor VIII (FVIII) activity
Rationale: To establish baseline levels and guide initial dosing for Hemophilia A.
Timing: Prior to first infusion.
von Willebrand Factor ristocetin cofactor (VWF:RCo) activity
Rationale: To establish baseline levels and guide initial dosing for VWD.
Timing: Prior to first infusion.
von Willebrand Factor antigen (VWF:Ag)
Rationale: To establish baseline levels and aid in VWD diagnosis/monitoring.
Timing: Prior to first infusion.
Activated Partial Thromboplastin Time (aPTT)
Rationale: To assess intrinsic pathway coagulation status.
Timing: Prior to first infusion.
Complete Blood Count (CBC)
Rationale: To assess overall hematologic status and monitor for bleeding complications.
Timing: Prior to first infusion.
FVIII inhibitor screen (Bethesda assay)
Rationale: To detect pre-existing FVIII inhibitors, which can impact treatment efficacy.
Timing: Prior to first infusion, especially in Hemophilia A patients.
Routine Monitoring
Factor VIII (FVIII) activity
Frequency: Before and after infusion (to confirm peak levels), and periodically (e.g., daily) to maintain target levels during acute bleeding or surgery.
Target: Dependent on indication (e.g., >50% for major bleeding/surgery, 1-5% for mild prophylaxis).
Action Threshold: Adjust dose or frequency if levels are outside target range or clinical response is inadequate.
von Willebrand Factor ristocetin cofactor (VWF:RCo) activity
Frequency: Before and after infusion (to confirm peak levels), and periodically (e.g., daily) to maintain target levels during acute bleeding or surgery.
Target: Dependent on indication (e.g., >50% for major bleeding/surgery).
Action Threshold: Adjust dose or frequency if levels are outside target range or clinical response is inadequate.
FVIII inhibitor assay (Bethesda assay)
Frequency: Periodically (e.g., every 3-6 months or annually) in Hemophilia A patients, or if there is a lack of expected clinical response to treatment.
Target: Negative (<0.6 Bethesda Units/mL)
Action Threshold: If positive, consider alternative treatments or immune tolerance induction.
Signs and symptoms of thrombosis
Frequency: Continuously during treatment, especially with high doses or concurrent antifibrinolytics.
Target: Absence of signs/symptoms
Action Threshold: Discontinue infusion and initiate appropriate management if thrombosis is suspected.
Signs and symptoms of allergic/anaphylactic reactions
Frequency: During and immediately after infusion.
Target: Absence of signs/symptoms
Action Threshold: Stop infusion immediately and provide supportive care.
Symptom Monitoring
- Unusual bleeding or bruising
- Joint pain or swelling (hemarthrosis)
- Muscle pain or swelling
- Headache (intracranial hemorrhage)
- Blood in urine or stool
- Nosebleeds
- Excessive bleeding after injury or surgery
- Hives or rash
- Itching
- Wheezing or difficulty breathing
- Chest tightness
- Dizziness or lightheadedness
- Swelling of face, lips, tongue, or throat
- Signs of thrombosis: chest pain, shortness of breath, swelling/pain in a limb, sudden vision changes, slurred speech.
Special Patient Groups
Pregnancy
Use during pregnancy only if clearly needed and the potential benefit outweighs the potential risk to the fetus. Factor VIII and von Willebrand Factor levels naturally increase during pregnancy, but some women with VWD or Hemophilia A may still require treatment, especially around delivery. Close monitoring of coagulation parameters is essential.
Trimester-Specific Risks:
First Trimester:
Limited data, but generally considered low risk for congenital malformations.
Second Trimester:
Limited data, but generally considered low risk.
Third Trimester:
Increased risk of bleeding around delivery may necessitate treatment. Monitor factor levels closely.
Lactation
It is generally considered safe to use during lactation. Factor VIII and von Willebrand Factor are large proteins and are unlikely to be excreted into breast milk in significant amounts that would harm the infant. However, caution is advised, and the decision should be made in consultation with a healthcare provider.
Infant Risk:
Low risk.
Pediatric Use
Dosing is weight-based and similar to adults. Children, especially infants, may have different pharmacokinetic profiles (e.g., faster clearance) requiring more frequent dosing or higher per-kg doses. Close monitoring of factor levels and clinical response is crucial. Risk of FVIII inhibitor development is higher in previously untreated Hemophilia A patients (PUPs).
Geriatric Use
No specific dose adjustments are generally required based on age alone. However, elderly patients may have an increased risk of cardiovascular disease and thrombosis, so careful monitoring for thrombotic events is warranted, especially with high doses or prolonged treatment.
Clinical Information
Clinical Pearls
- Alphanate is a plasma-derived product; while highly purified and viral inactivated, patients should be informed of the theoretical risk of pathogen transmission.
- Individual patient response to factor replacement can vary; monitor FVIII and VWF:RCo levels to ensure adequate therapeutic concentrations.
- For Hemophilia A, monitor for the development of FVIII inhibitors, especially in previously untreated patients (PUPs) or those with severe disease.
- In VWD, the FVIII component of Alphanate can accumulate with repeated dosing, potentially leading to excessively high FVIII levels and an increased risk of thrombosis. Monitor FVIII levels in addition to VWF:RCo.
- Administer intravenously at a rate comfortable for the patient, typically 10 mL/minute or as tolerated. Do not exceed 10 mL/minute.
- Reconstitute immediately before use and use within 3 hours. Do not refrigerate after reconstitution.
- Ensure proper training for home infusion to minimize complications.
Alternative Therapies
- Recombinant Factor VIII (for Hemophilia A, e.g., Advate, Eloctate, Hemlibra (emicizumab) for prophylaxis)
- Desmopressin (DDAVP) (for mild Hemophilia A and Type 1 VWD)
- Antifibrinolytic agents (e.g., tranexamic acid, aminocaproic acid) (adjunctive therapy for bleeding control)
- Other VWF-containing products (e.g., Vonvendi (recombinant VWF))
Cost & Coverage
Average Cost:
Extremely high, typically thousands to tens of thousands of USD per vial depending on strength and market. per vial (e.g., 500 IU)
Insurance Coverage:
Specialty Tier (requires prior authorization, often covered by major medical insurance and government programs for bleeding disorders)
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 your 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 medical attention. When seeking help, be prepared to provide details about the medication taken, the amount, and the time it happened.