Hemlibra 150mg/ml Inj, 1 Vial
WARNING: Severe side effects have happened when this drug was used with another drug called activated prothrombin complex concentrate (aPCC). The risk of this drug interaction may last for up to 6 months after the last dose of this drug. Side effects have included blood clots and injury to blood vessels. Call your doctor right away if you feel confused, faint, sick, or weak. Call your doctor right away if you have back or stomach pain, change in eyesight, chest pain or pressure, coughing up blood, eye pain or swelling, fast heartbeat, numbness in the face, pain in the leg or arm, shortness of breath, swelling in the arms or legs, trouble passing urine, upset stomach or throwing up, very bad headache, or yellow skin or eyes. @ COMMON USES: It is used to prevent bleeding in people with hemophilia A.
Drug Class
Coagulation Factor
Pharmacologic Class
Bispecific Factor IXa- and Factor X-Directed Antibody
Pregnancy Category
Not available
FDA Approved
Nov 2017
DEA Schedule
Not Controlled
Overview
What is this medicine?
Hemlibra is a medicine given by injection under the skin to help prevent bleeding in people with hemophilia A. It works by helping the blood to clot properly, even if you have developed antibodies against other hemophilia treatments.
How to Use This Medicine
Proper Administration of This Medication
To ensure safe and effective use, follow your doctor's instructions and read all provided information carefully. This medication is administered via injection into the fatty tissue of the skin, typically on the top of the thigh, belly area, or upper arm. If you will be self-administering the injection, your doctor or nurse will provide guidance on the proper technique.
When preparing the dose, you may need to use more than one vial. However, do not combine vials of different strengths in a single injection. To minimize the risk of injection site reactions, rotate the injection site with each dose.
Important Handling Instructions
Do not shake the vial.
Use the medication immediately after removing it from the vial.
Check the solution for any changes in color, cloudiness, or particles. Do not use if the solution appears discolored, cloudy, or contains particles.
Avoid injecting into moles, scars, or skin that is irritated, tender, bruised, red, hard, or broken.
Refrain from applying pressure (e.g., using a waistband or belt) on the injection site.
Do not draw the medication into a syringe and store it for future use.
Dispose of any unused portion of the opened vial after administration.
Properly dispose of needles in a designated needle/sharp disposal box. Do not reuse needles or other items. When the box is full, follow local regulations for disposal.
Storage and Disposal
Store this medication in a refrigerator at all times, unless otherwise specified. Do not freeze. Keep the medication in its original container to protect it from light. If necessary, unopened vials can be stored at room temperature for a maximum of 7 days. After 7 days at room temperature, the medication must be discarded. If you have any questions or concerns, consult your doctor or pharmacist.
Missed Dose Instructions
If you miss a dose, take it as soon as you remember and resume your regular schedule. However, if you do not remember the missed dose until the day of your next scheduled dose, skip the missed dose and continue with your regular schedule. Do not take two doses on the same day.
To ensure safe and effective use, follow your doctor's instructions and read all provided information carefully. This medication is administered via injection into the fatty tissue of the skin, typically on the top of the thigh, belly area, or upper arm. If you will be self-administering the injection, your doctor or nurse will provide guidance on the proper technique.
When preparing the dose, you may need to use more than one vial. However, do not combine vials of different strengths in a single injection. To minimize the risk of injection site reactions, rotate the injection site with each dose.
Important Handling Instructions
Do not shake the vial.
Use the medication immediately after removing it from the vial.
Check the solution for any changes in color, cloudiness, or particles. Do not use if the solution appears discolored, cloudy, or contains particles.
Avoid injecting into moles, scars, or skin that is irritated, tender, bruised, red, hard, or broken.
Refrain from applying pressure (e.g., using a waistband or belt) on the injection site.
Do not draw the medication into a syringe and store it for future use.
Dispose of any unused portion of the opened vial after administration.
Properly dispose of needles in a designated needle/sharp disposal box. Do not reuse needles or other items. When the box is full, follow local regulations for disposal.
Storage and Disposal
Store this medication in a refrigerator at all times, unless otherwise specified. Do not freeze. Keep the medication in its original container to protect it from light. If necessary, unopened vials can be stored at room temperature for a maximum of 7 days. After 7 days at room temperature, the medication must be discarded. If you have any questions or concerns, consult your doctor or pharmacist.
Missed Dose Instructions
If you miss a dose, take it as soon as you remember and resume your regular schedule. However, if you do not remember the missed dose until the day of your next scheduled dose, skip the missed dose and continue with your regular schedule. Do not take two doses on the same day.
Lifestyle & Tips
- Follow your prescribed dosing schedule carefully.
- Learn proper subcutaneous injection technique from your healthcare provider.
- Keep a record of your injections and any bleeding episodes.
- Avoid activities that carry a high risk of injury or bleeding.
- Carry identification indicating your hemophilia and medication.
- Discuss any planned surgeries or dental procedures with your healthcare provider well in advance.
Available Forms & Alternatives
Dosing & Administration
Adult Dosing
Standard Dose:
Loading dose: 3 mg/kg subcutaneously once weekly for the first 4 weeks. Maintenance dose: 1.5 mg/kg once weekly OR 3 mg/kg every 2 weeks OR 6 mg/kg every 4 weeks, all subcutaneously.
Dose Range:
1.5 - 6 mg
Condition-Specific Dosing:
Hemophilia A with Factor VIII Inhibitors:
Loading dose: 3 mg/kg once weekly for 4 weeks. Maintenance dose: 1.5 mg/kg once weekly OR 3 mg/kg every 2 weeks OR 6 mg/kg every 4 weeks.
Hemophilia A without Factor VIII Inhibitors:
Loading dose: 3 mg/kg once weekly for 4 weeks. Maintenance dose: 1.5 mg/kg once weekly OR 3 mg/kg every 2 weeks OR 6 mg/kg every 4 weeks.
Pediatric Dosing
Neonatal:
Not established
Infant:
Not established (safety and efficacy established in pediatric patients from birth to <18 years of age, but specific neonatal/infant dosing not differentiated from general pediatric)
Child:
Loading dose: 3 mg/kg subcutaneously once weekly for the first 4 weeks. Maintenance dose: 1.5 mg/kg once weekly OR 3 mg/kg every 2 weeks OR 6 mg/kg every 4 weeks, all subcutaneously.
Adolescent:
Loading dose: 3 mg/kg subcutaneously once weekly for the first 4 weeks. Maintenance dose: 1.5 mg/kg once weekly OR 3 mg/kg every 2 weeks OR 6 mg/kg every 4 weeks, all subcutaneously.
Dose Adjustments
Renal Impairment:
Mild:
No dose adjustment necessary.
Moderate:
No dose adjustment necessary.
Severe:
No dose adjustment necessary (limited data, but not expected to be renally cleared).
Dialysis:
No specific recommendations; not expected to be removed by dialysis.
Hepatic Impairment:
Mild:
No dose adjustment necessary.
Moderate:
No dose adjustment necessary.
Severe:
No dose adjustment necessary (limited data, but not expected to be hepatically cleared).
Pharmacology
Mechanism of Action
Emicizumab is a bispecific monoclonal antibody that bridges activated factor IX and factor X to restore the function of activated factor VIII, which is missing or impaired in patients with hemophilia A. It mimics the cofactor function of activated factor VIII, thereby promoting effective hemostasis.
Pharmacokinetics
Absorption:
Bioavailability:
80.4% (subcutaneous)
Tmax:
5-7 days
FoodEffect:
Not applicable (subcutaneous administration)
Distribution:
Vd:
6.0 L (central volume of distribution)
ProteinBinding:
Not applicable (monoclonal antibody)
CnssPenetration:
Limited
Elimination:
HalfLife:
27.8 days (approximately 4 weeks)
Clearance:
0.271 L/day
ExcretionRoute:
Not specifically excreted; catabolized and components recycled.
Unchanged:
Not applicable (catabolized)
Pharmacodynamics
OnsetOfAction:
Within hours to days (clinical effect observed with loading dose)
PeakEffect:
After loading dose period (around 4 weeks)
DurationOfAction:
Long, due to long half-life (allows for weekly, bi-weekly, or monthly dosing)
Safety & Warnings
BLACK BOX WARNING
Thrombotic microangiopathy and thrombotic events have been reported when activated prothrombin complex concentrate (aPCC) was administered to patients receiving Hemlibra. Consider the benefits and risks of aPCC use. If aPCC is necessary, monitor for signs and symptoms of thrombotic microangiopathy and thrombotic events.
Side Effects
Urgent Side Effects: Seek Medical Attention Immediately
Although rare, this medication can cause severe and potentially life-threatening side effects. If you experience 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, or swelling of the mouth, face, lips, tongue, or throat
- Change in skin color to black or purple
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. Contact your doctor or seek medical help if you experience any of the following side effects or if they bother you or do not go away:
- Pain, redness, itching, or irritation at the injection site
- Headache
- Joint pain
- Diarrhea
Reporting Side Effects
This list does not include all possible side effects. If you have questions or concerns about side effects, contact your doctor for medical advice. You can also report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
Although rare, this medication can cause severe and potentially life-threatening side effects. If you experience 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, or swelling of the mouth, face, lips, tongue, or throat
- Change in skin color to black or purple
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. Contact your doctor or seek medical help if you experience any of the following side effects or if they bother you or do not go away:
- Pain, redness, itching, or irritation at the injection site
- Headache
- Joint pain
- Diarrhea
Reporting Side Effects
This list does not include all possible side effects. If you have questions or concerns about side effects, contact your doctor for medical advice. 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 thrombotic microangiopathy (TMA): confusion, weakness, swelling, yellowing of skin or eyes, decreased urination, chest pain, shortness of breath, severe headache, vision changes.
- Signs of blood clots (thrombosis): pain, swelling, warmth, or redness in an arm or leg; sudden chest pain, shortness of breath, or coughing up blood; sudden severe headache, weakness on one side of the body, or problems with speech.
- Severe allergic reactions: difficulty breathing, swelling of face/lips/tongue/throat, severe rash, dizziness.
- Unusual or persistent bleeding despite treatment.
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.
All medications you are currently taking, including prescription and over-the-counter (OTC) drugs, natural products, and vitamins. This is crucial to avoid potential interactions between this medication and other substances.
* Any existing health problems, as this medication may interact with certain conditions.
To ensure your safety, it is vital to verify that it is safe to take this medication with all your current medications and health conditions. Always consult your doctor before starting, stopping, or changing the dose of any medication, including this one.
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.
All medications you are currently taking, including prescription and over-the-counter (OTC) drugs, natural products, and vitamins. This is crucial to avoid potential interactions between this medication and other substances.
* Any existing health problems, as this medication may interact with certain conditions.
To ensure your safety, it is vital to verify that it is safe to take this medication with all your current medications and health conditions. Always consult your doctor before starting, stopping, or changing the dose of any medication, including this one.
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 will be necessary, as directed by your doctor, to monitor your condition. Be sure to discuss any concerns or questions with your doctor.
Please note that this medication may interfere with certain laboratory tests, and this effect may persist for up to 6 months after your last dose. It is crucial to inform your doctor and laboratory personnel that you are taking this medication to ensure accurate test results.
If you find that your usual dose is not providing the expected relief, contact your doctor immediately. Additionally, if you experience any changes in weight, consult with your doctor, as your dosage may need to be adjusted.
To prevent pregnancy while taking this medication, use a reliable form of birth control. If you are pregnant, planning to become pregnant, or breastfeeding, inform your doctor promptly. You and your doctor will need to discuss the potential benefits and risks of this medication to both you and your baby.
Please note that this medication may interfere with certain laboratory tests, and this effect may persist for up to 6 months after your last dose. It is crucial to inform your doctor and laboratory personnel that you are taking this medication to ensure accurate test results.
If you find that your usual dose is not providing the expected relief, contact your doctor immediately. Additionally, if you experience any changes in weight, consult with your doctor, as your dosage may need to be adjusted.
To prevent pregnancy while taking this medication, use a reliable form of birth control. If you are pregnant, planning to become pregnant, or breastfeeding, inform your doctor promptly. You and your doctor will need to discuss the potential benefits and risks of this medication to both you and your baby.
Overdose Information
Overdose Symptoms:
- Symptoms of overdose are not well established, but theoretical risks could include increased risk of thrombotic events.
What to Do:
Contact your healthcare provider or emergency services immediately. Treatment is supportive. Call 1-800-222-1222 for Poison Control.
Drug Interactions
Major Interactions
- Activated prothrombin complex concentrate (aPCC): Concomitant use with emicizumab may increase the risk of thrombotic microangiopathy (TMA) and thrombotic events. If aPCC is needed, closely monitor for signs of TMA and thrombosis.
Moderate Interactions
- Recombinant Factor VIIa (rFVIIa): Concomitant use may increase the risk of thrombotic events, though less data than with aPCC. Monitor for signs of thrombosis.
Confidence Interactions
Monitoring
Baseline Monitoring
Baseline bleeding frequency and severity
Rationale: To establish a baseline for assessing treatment efficacy.
Timing: Prior to initiation of therapy
Factor VIII inhibitor status
Rationale: To confirm diagnosis and guide treatment strategy (though Hemlibra is effective in both inhibitor and non-inhibitor patients).
Timing: Prior to initiation of therapy
Routine Monitoring
Signs and symptoms of bleeding
Frequency: Continuously
Target: Reduction in bleeding episodes
Action Threshold: New or worsening bleeding may require additional hemostatic treatment.
Signs and symptoms of thrombotic microangiopathy (TMA) or thrombotic events
Frequency: Especially when co-administering with aPCC or rFVIIa; otherwise, routinely
Target: Absence of TMA/thrombosis
Action Threshold: If suspected, discontinue aPCC/rFVIIa immediately and consider discontinuing emicizumab. Evaluate for TMA/thrombosis.
Platelet count, serum creatinine, LDH, haptoglobin, bilirubin
Frequency: If TMA is suspected, or if aPCC is used
Target: Normal ranges
Action Threshold: Abnormalities may indicate TMA.
Liver function tests (ALT, AST)
Frequency: If aPCC is used
Target: Normal ranges
Action Threshold: Elevations may indicate liver injury associated with TMA.
Symptom Monitoring
- Unusual bleeding or bruising
- Signs of thrombotic microangiopathy (e.g., confusion, weakness, swelling, yellowing of skin/eyes, decreased urination, chest pain, shortness of breath, severe headache, vision changes)
- Signs of thrombosis (e.g., pain, swelling, warmth, redness in a limb; sudden chest pain, shortness of breath, coughing blood; sudden severe headache, weakness on one side of body, speech changes)
- Injection site reactions (e.g., redness, swelling, pain, itching)
Special Patient Groups
Pregnancy
There are no adequate and well-controlled studies of Hemlibra in pregnant women. Use during pregnancy only if the potential benefit justifies the potential risk to the fetus. Human IgG antibodies are known to cross the placental barrier, and emicizumab may be transmitted from the mother to the developing fetus.
Trimester-Specific Risks:
First Trimester:
Limited data; potential for fetal exposure.
Second Trimester:
Limited data; potential for fetal exposure.
Third Trimester:
Limited data; potential for fetal exposure, particularly in late pregnancy.
Lactation
It is not known whether emicizumab is excreted in human milk. Consider the developmental and health benefits of breastfeeding along with the mother’s clinical need for Hemlibra and any potential adverse effects on the breastfed infant from Hemlibra or from the underlying maternal condition.
Infant Risk:
Risk unknown; large protein, likely minimally absorbed by infant if present in milk. Monitor for adverse effects.
Pediatric Use
Safety and efficacy established in pediatric patients from birth to <18 years of age. Dosing is weight-based. No specific dose adjustments needed for age within this range.
Geriatric Use
Clinical studies did not include sufficient numbers of subjects aged 65 and over to determine whether they respond differently from younger subjects. No dose adjustment is required based on age.
Clinical Information
Clinical Pearls
- Emicizumab is a prophylactic treatment and does not replace on-demand treatment for acute bleeding episodes. Patients may still require bypassing agents (e.g., aPCC, rFVIIa) for breakthrough bleeds.
- Careful monitoring for thrombotic microangiopathy (TMA) and thrombotic events is crucial, especially if aPCC is used for breakthrough bleeding. Discontinue aPCC immediately if TMA/thrombosis is suspected.
- Emicizumab affects activated partial thromboplastin time (aPTT) and other clotting assays that use activated factor IX as a reagent, leading to falsely shortened aPTT results. Use chromogenic assays with human coagulation factors for factor VIII activity measurement.
- Patients should be educated on the signs and symptoms of TMA and thrombosis and instructed to seek immediate medical attention if they occur.
- The long half-life allows for flexible dosing schedules (weekly, bi-weekly, or monthly), which can improve adherence and quality of life for patients.
Alternative Therapies
- Factor VIII replacement therapy (recombinant or plasma-derived)
- Bypassing agents (e.g., activated prothrombin complex concentrate [aPCC], recombinant Factor VIIa [rFVIIa]) for patients with inhibitors
- Gene therapy (e.g., valoctocogene roxaparvovec, etranacogene dezaparvovec)
- Non-factor therapies (e.g., fitusiran, concizumab - under development/review)
Cost & Coverage
Average Cost:
Highly variable, often >$25,000 per vial for 150mg/ml per 150mg/ml vial
Insurance Coverage:
Specialty Tier (requires prior authorization, often subject to high co-pays or co-insurance)
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.
This medication is accompanied by a Medication Guide, which provides crucial information about its use. Please read this guide carefully and review it again whenever you receive a refill. If you have any questions or concerns about this medication, don't hesitate to discuss them with your doctor, 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 reporting the incident, be prepared to provide details about the medication taken, the amount, and the time it occurred.
This medication is accompanied by a Medication Guide, which provides crucial information about its use. Please read this guide carefully and review it again whenever you receive a refill. If you have any questions or concerns about this medication, don't hesitate to discuss them with your doctor, 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 reporting the incident, be prepared to provide details about the medication taken, the amount, and the time it occurred.