Alphanate 250iu Vwf/hum Inj 1vl
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
Antihemophilic Agent; von Willebrand Factor Replacement
Pharmacologic Class
Coagulation Factor; Blood Product
Pregnancy Category
Category C
FDA Approved
Aug 1986
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 needed for your blood to clot properly. It is used to treat and prevent bleeding in people with hemophilia A (a bleeding disorder where Factor VIII is missing or low) and von Willebrand disease (a bleeding disorder where von Willebrand Factor is missing or not working correctly). It is 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 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.
Preparation and Administration
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.
Disposal and Storage
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.
Storage Instructions
Store this medication at room temperature, avoiding freezing. Keep it in the original container to protect it from light. Store all medications in a safe location, out of the reach of children and pets.
Missed Dose Instructions
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.
Preparation and Administration
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.
Disposal and Storage
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.
Storage Instructions
Store this medication at room temperature, avoiding freezing. Keep it in the original container to protect it from light. Store all medications in a safe location, out of the reach of children and pets.
Missed Dose Instructions
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 supplies with you, especially when traveling.
- Follow your doctor's instructions for dosing and administration carefully.
- Keep a detailed record of all infusions, including date, time, dose, and lot number.
- Avoid activities that carry a high risk of injury or bleeding (e.g., contact sports) unless approved by your doctor.
- Avoid medications that can increase bleeding risk, such as aspirin, ibuprofen (Advil, Motrin), naproxen (Aleve), and other NSAIDs, unless specifically instructed by your doctor.
- Inform all healthcare providers (including dentists) that you have a bleeding disorder and are on this medication.
- Consider wearing a medical alert bracelet or carrying an identification card.
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 factor levels, type of bleeding, and patient weight. Dosing is expressed in International Units (IU).
Condition-Specific Dosing:
Hemophilia A (on-demand treatment):
Initial dose: 20-40 IU FVIII/kg. Subsequent doses and frequency based on clinical response and FVIII levels. Target FVIII levels vary by bleed severity (e.g., 40-60% for minor, 80-100% for major).
Hemophilia A (prophylaxis):
25-50 IU FVIII/kg 2-3 times per week, or as determined by physician.
von Willebrand Disease (VWD) - Type 1 (minor bleeding):
Initial dose: 40-60 IU VWF:RCo/kg. Subsequent doses as needed to maintain VWF:RCo levels >50% and FVIII levels >50%.
von Willebrand Disease (VWD) - Type 1 (major bleeding/surgery):
Initial dose: 60-80 IU VWF:RCo/kg. Maintain VWF:RCo levels >50% and FVIII levels >50% for several days.
von Willebrand Disease (VWD) - Type 2/3:
Dosing is more complex and often higher, guided by VWF:RCo and FVIII levels. Initial dose 50-80 IU VWF:RCo/kg, repeated as needed.
Pediatric Dosing
Neonatal:
Not established, but used in neonates with severe bleeding disorders. Dosing is weight-based and individualized.
Infant:
Dosing is weight-based (IU/kg) and individualized based on factor levels and clinical need, similar to adults.
Child:
Dosing is weight-based (IU/kg) and individualized based on factor levels and clinical need, similar to adults.
Adolescent:
Dosing is weight-based (IU/kg) and individualized based on factor levels and clinical need, similar to adults.
Dose Adjustments
Renal Impairment:
Mild:
No specific adjustment recommended.
Moderate:
No specific adjustment recommended.
Severe:
No specific adjustment recommended.
Dialysis:
No specific adjustment recommended. Not significantly cleared by dialysis.
Hepatic Impairment:
Mild:
No specific adjustment recommended.
Moderate:
No specific adjustment recommended.
Severe:
No specific adjustment recommended.
Pharmacology
Mechanism of Action
Alphanate provides replacement of deficient coagulation factors, Factor VIII (FVIII) and von Willebrand Factor (VWF). In hemophilia A, it replaces the missing FVIII, which is essential for the intrinsic coagulation pathway. VWF acts as a carrier protein for FVIII, protecting it from degradation and localizing it to the site of injury. VWF also mediates platelet adhesion to the subendothelium and platelet aggregation, which are crucial for primary hemostasis in von Willebrand disease.
Pharmacokinetics
Absorption:
Bioavailability:
100%
Tmax:
Immediately after intravenous infusion
FoodEffect:
Not applicable (intravenous administration)
Distribution:
Vd:
Approximately plasma volume (for FVIII and VWF)
ProteinBinding:
FVIII binds to VWF; VWF circulates as multimers
CnssPenetration:
No
Elimination:
HalfLife:
FVIII: 8-12 hours; VWF:RCo: 8-12 hours (may vary depending on VWD type and individual patient)
Clearance:
Not available (complex biological clearance)
ExcretionRoute:
Metabolized and eliminated via protein degradation pathways
Unchanged:
Not applicable (protein degradation)
Pharmacodynamics
OnsetOfAction:
Immediate (upon completion of infusion)
PeakEffect:
Immediately after infusion
DurationOfAction:
FVIII activity: 8-12 hours; VWF activity: 8-12 hours, but may be longer for VWF due to endogenous FVIII stabilization and release from endothelial cells.
Safety & Warnings
Side Effects
Urgent Side Effects: Seek Medical Attention 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 help 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), such as:
+ 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 and 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, such as:
+ Chest pain or pressure
+ Coughing up blood
+ Shortness of breath
+ Swelling, warmth, numbness, color change, or pain in a leg or arm
+ Trouble speaking or swallowing
Other Possible Side Effects
Like all medications, this drug can cause side effects. Many people may not experience any side effects or may only have mild ones. If you notice any of the following side effects or any other unusual symptoms, contact your doctor or seek medical help:
Upset stomach
Constipation
Dizziness, tiredness, or weakness
Headache
* Back pain
This is not an exhaustive list of possible side effects. If you have questions or concerns about side effects, consult 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 help 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), such as:
+ 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 and 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, such as:
+ Chest pain or pressure
+ Coughing up blood
+ Shortness of breath
+ Swelling, warmth, numbness, color change, or pain in a leg or arm
+ Trouble speaking or swallowing
Other Possible Side Effects
Like all medications, this drug can cause side effects. Many people may not experience any side effects or may only have mild ones. If you notice any of the following side effects or any other unusual symptoms, contact your doctor or seek medical help:
Upset stomach
Constipation
Dizziness, tiredness, or weakness
Headache
* Back pain
This is not an exhaustive list of possible side effects. If you have questions or concerns about side effects, consult 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, or throat, chest tightness, wheezing, difficulty breathing, dizziness, feeling faint, low blood pressure. Seek immediate medical attention.
- Signs of a blood clot (for VWD patients, especially with high doses): pain, swelling, warmth, or redness in an arm or leg; sudden shortness of breath; chest pain; sudden headache or vision changes. Seek immediate medical attention.
- Signs that the medication is not working: increased frequency or severity of bleeding episodes, bleeding that does not stop with treatment, new or unusual bleeding.
- Signs of infection (though rare with modern purification): fever, chills, fatigue.
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 symptoms you experienced as a result of the allergy.
Potential interactions with other medications or health conditions. This medication may affect or be affected by other drugs or health problems, so it is crucial to disclose this information.
To ensure safe treatment, 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 existing health problems
Carefully review your medications and health conditions with your doctor to confirm that it is safe to take this medication. Do not initiate, discontinue, 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, 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 affect or be affected by other drugs or health problems, so it is crucial to disclose this information.
To ensure safe treatment, 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 existing health problems
Carefully review your medications and health conditions with your doctor to confirm that it is safe to take this medication. Do not initiate, discontinue, 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 evaluations, as directed by your doctor, are crucial to monitor your condition.
If you experience unexplained bruising, bleeding, or prolonged bleeding that 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, a component of blood, there is a theoretical risk of transmitting viruses that could cause disease. Although the medication undergoes rigorous screening, testing, and treatment to minimize this risk, it is crucial to discuss this with your doctor.
Before traveling, consult with your doctor to ensure you have an adequate supply of this medication. It is also important to discuss any travel plans with your doctor to receive personalized advice.
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. This will enable your doctor to provide personalized guidance and recommendations.
If you experience unexplained bruising, bleeding, or prolonged bleeding that 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, a component of blood, there is a theoretical risk of transmitting viruses that could cause disease. Although the medication undergoes rigorous screening, testing, and treatment to minimize this risk, it is crucial to discuss this with your doctor.
Before traveling, consult with your doctor to ensure you have an adequate supply of this medication. It is also important to discuss any travel plans with your doctor to receive personalized advice.
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. This will enable your doctor to provide personalized guidance and recommendations.
Overdose Information
Overdose Symptoms:
- High doses, especially in VWD patients, may increase the risk of thrombotic events (blood clots) due to elevated FVIII and VWF levels.
- Symptoms of thrombosis as listed above.
What to Do:
If an overdose is suspected or symptoms of thrombosis occur, discontinue the infusion immediately and seek emergency medical attention. Call 911 or your local emergency number. For general overdose information, call a poison control center at 1-800-222-1222.
Drug Interactions
Monitoring
Baseline Monitoring
Diagnosis confirmation (e.g., FVIII activity, VWF:RCo, VWF:Ag, FVIII binding to VWF, VWF multimer analysis)
Rationale: To confirm diagnosis of Hemophilia A or von Willebrand Disease and guide initial dosing.
Timing: Prior to initiation of therapy
Inhibitor screen (FVIII inhibitor titer)
Rationale: To detect pre-existing FVIII inhibitors, which can render therapy ineffective.
Timing: Prior to initiation of therapy
Baseline CBC, liver and renal function tests
Rationale: General health assessment.
Timing: Prior to initiation of therapy
Routine Monitoring
FVIII activity levels
Frequency: During acute bleeding episodes: before and after initial dose, then as needed to maintain target levels. For prophylaxis: periodically to ensure adequate trough levels.
Target: Varies by bleed severity (e.g., 40-60% for minor, 80-100% for major); for prophylaxis, typically >1% or higher trough levels.
Action Threshold: If levels are not within target range or bleeding persists, adjust dose or frequency.
VWF:RCo and FVIII activity levels (for VWD)
Frequency: During acute bleeding episodes: before and after initial dose, then as needed to maintain target levels. For surgery: daily for several days post-op.
Target: Maintain VWF:RCo and FVIII levels >50% for minor bleeding/surgery; >80-100% for major bleeding/surgery.
Action Threshold: If levels are not within target range or bleeding persists, adjust dose or frequency.
Clinical response (cessation of bleeding)
Frequency: Continuously during treatment of bleeding episodes.
Target: Resolution of bleeding symptoms.
Action Threshold: If bleeding persists or recurs, re-evaluate dose, frequency, or consider inhibitor development.
Inhibitor screen (FVIII inhibitor titer)
Frequency: Periodically (e.g., every 3-6 months or annually) or if clinical response to treatment is suboptimal.
Target: <0.6 Bethesda Units (BU)
Action Threshold: If inhibitor develops (>0.6 BU), treatment strategy needs to be re-evaluated (e.g., higher doses, bypassing agents).
Signs of thrombosis (for VWD patients, especially with high doses or prolonged use)
Frequency: Monitor regularly, especially in patients with risk factors.
Target: Absence of thrombotic events.
Action Threshold: If signs of thrombosis occur, discontinue infusion and manage appropriately.
Symptom Monitoring
- Signs of bleeding (e.g., joint pain/swelling, bruising, prolonged bleeding from cuts, nosebleeds, blood in urine/stools)
- Signs of allergic or hypersensitivity reactions (e.g., hives, rash, itching, facial swelling, chest tightness, wheezing, dizziness, faintness, hypotension)
- Signs of inhibitor development (e.g., increased bleeding frequency, bleeding that does not respond to usual doses)
- Signs of thrombosis (e.g., pain, swelling, redness in a limb, shortness of breath, chest pain)
Special Patient Groups
Pregnancy
Use with caution during pregnancy only if clearly needed. Bleeding disorders can pose significant risks during pregnancy and childbirth. Factor replacement therapy may be necessary to manage or prevent bleeding complications. The decision to use Alphanate should be made by a physician after careful consideration of the potential benefits and risks.
Trimester-Specific Risks:
First Trimester:
No specific data on first-trimester risks. Generally, factor replacement is used as needed to prevent bleeding complications.
Second Trimester:
No specific data on second-trimester risks. Continued monitoring and management of bleeding disorder.
Third Trimester:
Increased risk of bleeding during labor and delivery. Factor levels should be monitored closely and maintained at appropriate levels to prevent hemorrhage.
Lactation
It is not known whether Alphanate is excreted in human milk. However, as a large protein, it is unlikely to be transferred in significant amounts. The benefits of breastfeeding should be weighed against the potential risks. Generally considered compatible with breastfeeding when medically indicated for the mother.
Infant Risk:
Low risk of adverse effects to the breastfed infant due to the large molecular size and likely poor oral bioavailability of proteins.
Pediatric Use
Alphanate is approved for use in pediatric patients. Dosing is weight-based (IU/kg) and individualized based on the specific bleeding disorder, severity, and target factor levels. No specific age-related adjustments are typically needed beyond weight-based calculations. Close monitoring of factor levels and clinical response is crucial.
Geriatric Use
Clinical studies did not include sufficient numbers of subjects aged 65 and over to determine whether they respond differently from younger subjects. Dosing should be individualized based on factor levels and clinical response, similar to younger adults. Caution should be exercised in elderly patients with pre-existing cardiovascular risk factors due to the theoretical risk of thrombosis, especially with high doses or prolonged use in VWD.
Clinical Information
Clinical Pearls
- Alphanate is a plasma-derived product, meaning it comes from human blood. While highly purified and viral inactivated, patients should be informed of the theoretical risk of pathogen transmission, though this risk is extremely low with modern manufacturing processes.
- Patients with von Willebrand Disease, especially Type 3, may require higher and more frequent doses due to rapid clearance of VWF and FVIII.
- Monitor for the development of FVIII inhibitors in hemophilia A patients, as this can lead to treatment failure. If an inhibitor is suspected, specialized assays are needed.
- In VWD patients, particularly those receiving high or repeated doses, monitor FVIII:C levels to avoid excessive FVIII elevation, which can increase the risk of thrombosis.
- Reconstitution and administration must be done carefully according to package insert instructions to ensure sterility and proper dosing.
- Patients should be educated on self-administration techniques if applicable, and proper storage of the product.
Alternative Therapies
- Recombinant Factor VIII (for Hemophilia A, e.g., Advate, Eloctate, Jivi, Novoeight, Nuwiq, Xyntha)
- Recombinant von Willebrand Factor (for VWD, e.g., Vonvendi)
- Desmopressin (DDAVP) (for Type 1 VWD and mild Hemophilia A, if responsive)
- Antifibrinolytic agents (e.g., tranexamic acid, aminocaproic acid) (adjunctive therapy for bleeding)
- Bypassing agents (e.g., FEIBA, NovoSeven RT) (for Hemophilia A patients with inhibitors)
Cost & Coverage
Average Cost:
Very high (e.g., thousands to tens of thousands of USD per vial depending on strength) per vial
Insurance Coverage:
Specialty Tier (requires prior authorization, often covered by major medical and pharmacy benefit plans for approved indications)
General Drug Facts
If your symptoms or health issues persist or worsen, it is essential to contact your doctor promptly. To ensure safe and effective treatment, 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 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.