Hemlibra 300mg/2ml Inj, 2ml
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 used to prevent bleeding episodes in people with hemophilia A. It works by helping your blood clot properly, similar to how a missing blood clotting factor (Factor VIII) would work, but in a different way. It is given as an injection under the skin.
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 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 instruct you 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. Rotate the injection site with each dose to avoid tissue damage.
Important Administration Instructions
Do not shake the vial before use.
Use the medication immediately after removing it from the vial.
Check the solution for color and clarity before use. The solution should be colorless to faintly yellow. Do not use if the solution has changed color, is cloudy, leaking, or contains particles.
Avoid injecting into moles, scars, or skin that is irritated, tender, bruised, red, hard, or broken.
Do not apply pressure to the injection site using a waistband or belt.
Do not draw the medication into a syringe and store it for future use.
Dispose of any unused medication from an opened vial after administration.
Throw away needles and syringes in a designated sharps disposal container. Do not reuse needles or other equipment.
Storage and Disposal
Store this medication in the refrigerator to maintain its potency. Keep it in its original container, protected from light. If necessary, unopened vials can be stored at room temperature for up to 7 days, after which they should be returned to the refrigerator or discarded. Do not store this medication at room temperature for more than 7 days. If a vial has been at room temperature for 7 days, discard it.
Missed Dose Instructions
If you miss a dose, take it as soon as you remember and resume your regular schedule. However, if you don't remember until the day of your next dose, skip the missed dose and continue with your regular schedule. Do not take two doses on the same day. If you have any questions or concerns, consult your doctor or pharmacist.
To use this medication correctly, follow your doctor's instructions and read all the information provided. 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 instruct you 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. Rotate the injection site with each dose to avoid tissue damage.
Important Administration Instructions
Do not shake the vial before use.
Use the medication immediately after removing it from the vial.
Check the solution for color and clarity before use. The solution should be colorless to faintly yellow. Do not use if the solution has changed color, is cloudy, leaking, or contains particles.
Avoid injecting into moles, scars, or skin that is irritated, tender, bruised, red, hard, or broken.
Do not apply pressure to the injection site using a waistband or belt.
Do not draw the medication into a syringe and store it for future use.
Dispose of any unused medication from an opened vial after administration.
Throw away needles and syringes in a designated sharps disposal container. Do not reuse needles or other equipment.
Storage and Disposal
Store this medication in the refrigerator to maintain its potency. Keep it in its original container, protected from light. If necessary, unopened vials can be stored at room temperature for up to 7 days, after which they should be returned to the refrigerator or discarded. Do not store this medication at room temperature for more than 7 days. If a vial has been at room temperature for 7 days, discard it.
Missed Dose Instructions
If you miss a dose, take it as soon as you remember and resume your regular schedule. However, if you don't remember until the day of your next dose, skip the missed dose and continue with your regular schedule. Do not take two doses on the same day. If you have any questions or concerns, consult your doctor or pharmacist.
Lifestyle & Tips
- Follow your doctor's instructions for dosing and administration carefully.
- Keep a record of your bleeding episodes and any other symptoms.
- Carry your medical identification card or information about your condition and medication.
- Avoid activities that carry a high risk of injury or bleeding.
- Discuss any planned surgeries or dental procedures with your healthcare provider.
- Do not stop taking Hemlibra without consulting your doctor.
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:
Standard dosing as above.
Hemophilia A without Factor VIII inhibitors:
Standard dosing as above.
Pediatric Dosing
Neonatal:
Not established
Infant:
Dosing is weight-based, similar to adults. Safety and efficacy established in pediatric patients from birth to 17 years of age.
Child:
Dosing is weight-based, similar to adults. Safety and efficacy established in pediatric patients from birth to 17 years of age.
Adolescent:
Dosing is weight-based, similar to adults. Safety and efficacy established in pediatric patients from birth to 17 years of age.
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 require adjustment as it is a monoclonal antibody.
Dialysis:
No dose adjustment necessary. Not expected to be dialyzable.
Hepatic Impairment:
Mild:
No dose adjustment necessary.
Moderate:
No dose adjustment necessary.
Severe:
No dose adjustment necessary. Limited data, but not expected to require adjustment as it is a monoclonal antibody.
Pharmacology
Mechanism of Action
Emicizumab is a bispecific monoclonal antibody that bridges activated factor IX (FIXa) and factor X (FX) to restore the function of missing activated factor VIII (FVIIIa) that is necessary for effective hemostasis. It mimics the cofactor function of FVIIIa, thereby promoting efficient generation of activated factor X (FXa) and subsequent thrombin generation, which is deficient in hemophilia A.
Pharmacokinetics
Absorption:
Bioavailability:
80.4% (subcutaneous)
Tmax:
3-4 days
FoodEffect:
Not applicable (subcutaneous administration)
Distribution:
Vd:
10.4 L (central compartment)
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 into small peptides and amino acids.
Unchanged:
Not applicable (catabolized)
Pharmacodynamics
OnsetOfAction:
Rapid, within hours to days (thrombin generation increases quickly)
PeakEffect:
Within 4 weeks (after loading doses)
DurationOfAction:
Long, due to long half-life (allows for weekly, bi-weekly, or monthly dosing)
Safety & Warnings
BLACK BOX WARNING
Thrombotic microangiopathy and thromboembolism have been reported with emicizumab when activated prothrombin complex concentrate (aPCC) was administered. Consider the benefits and risks of emicizumab prior to initiating aPCC. If aPCC is required, monitor for signs and symptoms of thrombotic microangiopathy and thromboembolism.
Side Effects
Urgent Side Effects: Seek Medical Help Right Away
If you experience any of the following symptoms, call your doctor immediately or seek emergency medical attention, as they may be signs of a severe and potentially life-threatening reaction:
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
Change in skin color to black or purple
Other Possible Side Effects
While many people may not experience any side effects or only minor ones, it's essential to be aware of the following potential side effects. If you experience any of these or any other side effects that bother you or do not go away, contact your doctor for advice:
Pain, redness, itching, or irritation at the injection site
Headache
Joint pain
Diarrhea
Reporting Side Effects
This list is not exhaustive, and you may experience other 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.
If you experience any of the following symptoms, call your doctor immediately or seek emergency medical attention, as they may be signs of a severe and potentially life-threatening reaction:
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
Change in skin color to black or purple
Other Possible Side Effects
While many people may not experience any side effects or only minor ones, it's essential to be aware of the following potential side effects. If you experience any of these or any other side effects that bother you or do not go away, contact your doctor for advice:
Pain, redness, itching, or irritation at the injection site
Headache
Joint pain
Diarrhea
Reporting Side Effects
This list is not exhaustive, and you may experience other 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 a blood clot: pain, swelling, warmth, or redness in an arm or leg; sudden chest pain or shortness of breath; sudden numbness or weakness on one side of the body; sudden severe headache; vision changes.
- Signs of kidney problems: decreased urine output, swelling in legs or feet.
- Signs of low red blood cells (anemia): unusual tiredness, pale skin, shortness of breath, dizziness.
- Severe headache, confusion, seizures.
- Injection site reactions that are severe or do not go away.
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 minimize 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 modify the dosage of any medication without first consulting your doctor. It is your responsibility to verify that it is safe to take this medication in conjunction with your other medications and health conditions. Always consult your doctor and pharmacist to confirm the safe use of this medication.
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 minimize 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 modify the dosage of any medication without first consulting your doctor. It is your responsibility to verify that it is safe to take this medication in conjunction with your other medications and health conditions. Always consult your doctor and pharmacist to confirm the safe use of this medication.
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. Discuss the details of these tests with your doctor to ensure you understand the schedule and any required preparations.
This medication may interfere with certain laboratory tests, and this effect can persist for up to 6 months after your last dose. It is crucial that both your doctor and laboratory personnel are aware that you are taking this drug to avoid any misinterpretation of test results.
If you find that the usual dose of this medication is not providing the expected relief, contact your doctor immediately.
Any significant changes in your weight should be reported to your doctor, as your dosage may need to be adjusted accordingly.
To prevent pregnancy while taking this medication, use a reliable form of birth control. If you are pregnant, planning to become pregnant, or are breastfeeding, inform your doctor. You and your doctor will need to discuss the potential benefits and risks of this medication to both you and your baby to make an informed decision.
Regular blood tests will be necessary, as directed by your doctor. Discuss the details of these tests with your doctor to ensure you understand the schedule and any required preparations.
This medication may interfere with certain laboratory tests, and this effect can persist for up to 6 months after your last dose. It is crucial that both your doctor and laboratory personnel are aware that you are taking this drug to avoid any misinterpretation of test results.
If you find that the usual dose of this medication is not providing the expected relief, contact your doctor immediately.
Any significant changes in your weight should be reported to your doctor, as your dosage may need to be adjusted accordingly.
To prevent pregnancy while taking this medication, use a reliable form of birth control. If you are pregnant, planning to become pregnant, or are breastfeeding, inform your doctor. You and your doctor will need to discuss the potential benefits and risks of this medication to both you and your baby to make an informed decision.
Overdose Information
Overdose Symptoms:
- Symptoms of overdose are not well established, but could potentially include increased risk of thrombotic events if excessive procoagulant activity occurs.
What to Do:
Contact your healthcare provider or seek emergency medical attention immediately. Call 1-800-222-1222 (Poison Control Center).
Drug Interactions
Major Interactions
- Activated prothrombin complex concentrate (aPCC; e.g., FEIBA): Increased risk of thrombotic microangiopathy (TMA) and thromboembolism. Avoid concomitant use unless no other treatment options are available. If aPCC is required, monitor for signs and symptoms of TMA and thromboembolism.
Moderate Interactions
- Recombinant Factor VIIa (rFVIIa; e.g., NovoSeven RT): Potential for increased thrombotic risk, though less pronounced than with aPCC. Monitor for signs of thrombosis if co-administered.
Monitoring
Baseline Monitoring
Factor VIII inhibitor status
Rationale: To confirm diagnosis and guide initial treatment strategy (though emicizumab is effective regardless of inhibitor status).
Timing: Prior to initiation of therapy
Baseline bleeding frequency and severity
Rationale: To establish a baseline for assessing treatment efficacy.
Timing: Prior to initiation of therapy
Liver and renal function tests
Rationale: General health assessment, though emicizumab is not primarily metabolized or excreted by these organs.
Timing: Prior to initiation of therapy
Routine Monitoring
Clinical assessment of bleeding episodes
Frequency: Regularly (e.g., at each follow-up visit)
Target: Reduction in bleeding frequency
Action Threshold: Persistent or increased bleeding may indicate need for re-evaluation or adjunctive therapy.
Signs and symptoms of thrombotic microangiopathy (TMA)
Frequency: Especially when concomitant bypassing agents are used; otherwise, periodically.
Target: Absence of TMA symptoms (e.g., microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury, neurological symptoms)
Action Threshold: Immediate investigation and discontinuation of emicizumab and/or bypassing agent if TMA is suspected.
Signs and symptoms of thromboembolism
Frequency: Especially when concomitant bypassing agents are used; otherwise, periodically.
Target: Absence of thromboembolic events (e.g., DVT, PE, arterial thrombosis)
Action Threshold: Immediate investigation and management if thromboembolism is suspected.
Laboratory coagulation assays (aPTT, one-stage FVIII activity)
Frequency: As needed for acute bleeding or surgical procedures
Target: Not reliable for monitoring emicizumab activity; may be falsely shortened or elevated.
Action Threshold: Use chromogenic FVIII activity assays or specific emicizumab-insensitive assays for FVIII activity if needed. Do NOT rely on aPTT or one-stage FVIII assays for monitoring.
Symptom Monitoring
- Unusual bleeding or bruising
- Signs of thrombotic microangiopathy (e.g., fatigue, pallor, jaundice, decreased urine output, confusion, seizures, swelling)
- Signs of thromboembolism (e.g., pain, swelling, warmth in a limb; sudden chest pain, shortness of breath; sudden weakness or numbness)
- Injection site reactions (e.g., redness, swelling, pain)
- Headache
- Fever
- Joint pain
Special Patient Groups
Pregnancy
There are no adequate and well-controlled studies of emicizumab in pregnant women. Use during pregnancy only if the potential benefit justifies the potential risk to the fetus. IgG antibodies are known to cross the placental barrier.
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. IgG antibodies are actively transported across the placenta, with increasing amounts as pregnancy progresses.
Lactation
It is unknown whether emicizumab is excreted in human milk. Consider the developmental and health benefits of breastfeeding along with the mother’s clinical need for emicizumab and any potential adverse effects on the breastfed infant from emicizumab or from the underlying maternal condition. Maternal IgG is known to be present in human milk.
Infant Risk:
L3 (Moderately Safe - No human data, but animal studies suggest low risk or drug is large molecule unlikely to pass into milk in significant amounts. Monitor infant for adverse effects.)
Pediatric Use
Safety and efficacy have been established in pediatric patients from birth to 17 years of age. Dosing is weight-based. The safety profile is generally consistent with adults.
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 specifically recommended based on age, but monitor for age-related comorbidities.
Clinical Information
Clinical Pearls
- Emicizumab provides prophylactic treatment for hemophilia A, regardless of Factor VIII inhibitor status, by mimicking FVIIIa's cofactor function.
- Crucial to understand that emicizumab interferes with one-stage, aPTT-based coagulation assays, leading to falsely shortened aPTT and falsely high FVIII activity levels. Chromogenic FVIII assays using bovine FIXa and FX are recommended for monitoring FVIII activity in patients on emicizumab.
- The most significant drug interaction is with activated prothrombin complex concentrate (aPCC) due to the risk of thrombotic microangiopathy (TMA) and thromboembolism. Avoid concomitant use if possible; if necessary, monitor closely.
- Patients and caregivers should be educated on the signs and symptoms of TMA and thromboembolism and instructed to seek immediate medical attention if they occur.
- Subcutaneous administration offers convenience and improved adherence compared to intravenous Factor VIII replacement therapies.
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 or acute bleeds
- Gene therapy (e.g., valoctocogene roxaparvovec)
- Non-factor therapies (e.g., concizumab, fitusiran - under development or recently approved)
Cost & Coverage
Average Cost:
Highly variable, typically >$400,000 annually for maintenance per year
Insurance Coverage:
Specialty Tier (requires prior authorization, often subject to step therapy)
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. 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 consult 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.