Hemlibra 105mg/0.7ml 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
Antihemophilic agent
Pharmacologic Class
Bispecific monoclonal antibody; Factor VIII-mimetic
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 episodes in people with hemophilia A. It works by helping the blood clotting process, similar to how Factor VIII works, but it's not Factor VIII itself.
How to Use This Medicine
Proper Administration of This Medication
To ensure safe and effective use, follow your doctor's instructions and read all accompanying 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. The solution should be colorless to faint yellow. If you notice any abnormalities, do not use the medication.
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 to maintain its potency. 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, return the medication to the refrigerator or discard it if it has been at room temperature for the entire 7-day period.
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. If you have any questions or concerns, consult your doctor or pharmacist.
To ensure safe and effective use, follow your doctor's instructions and read all accompanying 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. The solution should be colorless to faint yellow. If you notice any abnormalities, do not use the medication.
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 to maintain its potency. 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, return the medication to the refrigerator or discard it if it has been at room temperature for the entire 7-day period.
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. If you have any questions or concerns, consult your doctor or pharmacist.
Lifestyle & Tips
- Administer injections exactly as prescribed by your healthcare provider.
- Learn proper subcutaneous injection technique from your healthcare provider.
- Store Hemlibra in the refrigerator (2°C to 8°C / 36°F to 46°F) in its original carton to protect from light. Do not freeze.
- Do not shake the vial.
- Keep a record of your injections and any bleeding episodes.
- Carry identification indicating you have hemophilia A and are on Hemlibra.
- Discuss any planned surgeries or dental procedures with your healthcare provider.
- Avoid using activated prothrombin complex concentrate (aPCC) for breakthrough bleeding unless specifically instructed by your doctor, due to serious risks.
Available Forms & Alternatives
Dosing & Administration
Adult Dosing
Standard Dose:
Loading dose: 3 mg/kg once weekly for the first 4 weeks. Maintenance dose: 1.5 mg/kg once weekly OR 3 mg/kg every two weeks OR 6 mg/kg every four weeks.
Dose Range:
1.5 - 6 mg
Condition-Specific Dosing:
prophylaxis_hemophilia_A_with_inhibitors:
Loading dose: 3 mg/kg once weekly for 4 weeks. Maintenance: 1.5 mg/kg once weekly OR 3 mg/kg every 2 weeks OR 6 mg/kg every 4 weeks.
prophylaxis_hemophilia_A_without_inhibitors:
Loading dose: 3 mg/kg once weekly for 4 weeks. Maintenance: 1.5 mg/kg once weekly OR 3 mg/kg every 2 weeks OR 6 mg/kg every 4 weeks.
Pediatric Dosing
Neonatal:
Dosing established (weight-based, same as adult dosing for prophylaxis).
Infant:
Dosing established (weight-based, same as adult dosing for prophylaxis).
Child:
Dosing established (weight-based, same as adult dosing for prophylaxis).
Adolescent:
Dosing established (weight-based, same as adult dosing for prophylaxis).
Dose Adjustments
Renal Impairment:
Mild:
No dose adjustment necessary.
Moderate:
No dose adjustment necessary.
Severe:
No dose adjustment necessary (not renally cleared).
Dialysis:
No dose adjustment necessary (not renally cleared).
Hepatic Impairment:
Mild:
No dose adjustment necessary.
Moderate:
No dose adjustment necessary.
Severe:
No dose adjustment necessary (not hepatically cleared in the traditional sense).
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 activated factor VIII (FVIIIa), which is missing in patients with hemophilia A. It thereby promotes effective hemostasis.
Pharmacokinetics
Absorption:
Bioavailability:
80.4%
Tmax:
5-7 days
FoodEffect:
Not applicable (subcutaneous injection)
Distribution:
Vd:
5.6 L
ProteinBinding:
Not applicable (monoclonal antibody)
CnssPenetration:
Limited
Elimination:
HalfLife:
27-32 days
Clearance:
3.2 mL/kg/day
ExcretionRoute:
Catabolic degradation products
Unchanged:
Not applicable (protein degradation)
Pharmacodynamics
OnsetOfAction:
Within hours to days (clinical effect observed within first week of dosing)
PeakEffect:
Steady-state concentrations reached by approximately 4 weeks with weekly loading dose
DurationOfAction:
Extended due to long half-life (weekly, bi-weekly, or monthly dosing)
Safety & Warnings
Side Effects
Urgent Side Effects: Seek Medical Attention Immediately
While rare, some people may experience severe and potentially life-threatening side effects when 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, difficulty breathing, swallowing, or talking, unusual hoarseness, or swelling of the mouth, face, lips, tongue, or throat
- A change in skin color to black or purple
Other Possible Side Effects
Like all medications, this drug can cause side effects. Although 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, consult your doctor. For medical advice about side effects, you can also contact your doctor. Additionally, you can report side effects to the FDA at 1-800-332-1088 or online at https://www.fda.gov/medwatch.
While rare, some people may experience severe and potentially life-threatening side effects when 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, difficulty breathing, swallowing, or talking, unusual hoarseness, or swelling of the mouth, face, lips, tongue, or throat
- A change in skin color to black or purple
Other Possible Side Effects
Like all medications, this drug can cause side effects. Although 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, consult your doctor. For medical advice about side effects, you can also contact your doctor. Additionally, you can 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 bleeding: unusual bruising, nosebleeds, bleeding gums, blood in urine or stool, joint pain or swelling, prolonged bleeding from cuts.
- Signs of serious clotting problems (if aPCC is used): unusual bruising or small red spots on the skin (petechiae), extreme tiredness, pale skin, yellowing of skin or eyes, decreased urination, swelling, confusion, seizures, sudden severe headache, vision changes, chest pain, shortness of breath, pain/swelling/warmth/redness in an arm or leg.
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 information will help your doctor assess potential interactions between this medication and other substances.
* Any existing health problems, as they may affect the safety and efficacy of this medication.
To ensure your safety, it is crucial to verify that this medication can be taken with all your current medications and health conditions. Never start, stop, or adjust the dosage of any medication without first consulting your doctor. By sharing this information, you can help your doctor make informed decisions about your treatment and minimize potential risks.
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 information will help your doctor assess potential interactions between this medication and other substances.
* Any existing health problems, as they may affect the safety and efficacy of this medication.
To ensure your safety, it is crucial to verify that this medication can be taken with all your current medications and health conditions. Never start, stop, or adjust the dosage of any medication without first consulting your doctor. By sharing this information, you can help your doctor make informed decisions about your treatment and minimize potential risks.
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 normal 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. You 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 normal 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. You 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 thrombotic risk.
What to Do:
In case of suspected overdose, contact a poison control center (e.g., 1-800-222-1222) or seek emergency medical attention immediately. Management is supportive.
Drug Interactions
Major Interactions
- Activated prothrombin complex concentrate (aPCC): Concomitant use should be avoided unless no other treatment options are available for breakthrough bleeding. Increased risk of thrombotic microangiopathy (TMA) and thrombotic events.
Monitoring
Baseline Monitoring
Diagnosis of Hemophilia A
Rationale: Confirm indication for treatment.
Timing: Prior to initiation of therapy
Factor VIII inhibitor status
Rationale: Emicizumab is indicated for patients with or without FVIII inhibitors.
Timing: Prior to initiation of therapy
Routine Monitoring
Bleeding episodes
Frequency: Continuously
Target: Reduction in frequency
Action Threshold: Increased bleeding frequency may indicate need for dose adjustment or re-evaluation of treatment.
Signs/symptoms of thrombotic microangiopathy (TMA) or thrombotic events
Frequency: Continuously, especially if aPCC is used for breakthrough bleeding
Target: Absence of symptoms
Action Threshold: Any signs (e.g., atypical hemolysis, thrombocytopenia, acute kidney injury, weakness, altered mental status) require immediate medical attention and discontinuation of aPCC.
Liver function tests (ALT, AST, bilirubin)
Frequency: Periodically (e.g., every 6-12 months or as clinically indicated)
Target: Within normal limits
Action Threshold: Significant elevations may warrant investigation.
Renal function tests (creatinine, BUN)
Frequency: Periodically (e.g., every 6-12 months or as clinically indicated)
Target: Within normal limits
Action Threshold: Significant changes may warrant investigation.
Symptom Monitoring
- Signs of bleeding (e.g., joint pain, swelling, bruising, prolonged bleeding from minor cuts)
- Signs of thrombotic microangiopathy (TMA): unusual bruising, petechiae, fatigue, pale skin, jaundice, decreased urine output, swelling, confusion, seizures, stroke-like symptoms (if aPCC is used)
- Signs of thrombosis: pain, swelling, warmth, redness in a limb; chest pain, shortness of breath, sudden severe headache, vision changes (if aPCC is used)
Special Patient Groups
Pregnancy
Limited human data on emicizumab use in pregnant women. Animal studies have not shown direct harm to the fetus. IgG antibodies are known to cross the placental barrier, particularly in the third trimester. Use only if the potential benefit justifies the potential risk to the fetus.
Trimester-Specific Risks:
First Trimester:
Limited data, theoretical risk of immune-mediated effects on fetal development.
Second Trimester:
Limited data, theoretical risk of immune-mediated effects on fetal development.
Third Trimester:
Increased placental transfer of IgG antibodies, theoretical risk of immune-mediated effects on the fetus.
Lactation
It is unknown whether emicizumab is excreted in human milk. Maternal IgG is known to be present 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.
Infant Risk:
Low risk, but unknown. Large protein, likely poorly absorbed orally by infant.
Pediatric Use
Approved for use in pediatric patients of all ages. Dosing is weight-based and consistent with adult dosing. Clinical trials included pediatric patients as young as neonates.
Geriatric Use
No specific dose adjustment is required for geriatric patients. Clinical studies included patients 65 years and older, and no overall differences in safety or effectiveness were observed compared to younger patients.
Clinical Information
Clinical Pearls
- Emicizumab is a prophylactic treatment and does not replace factor VIII for acute bleeding episodes. Patients may still require on-demand treatment for breakthrough bleeds.
- Standard one-stage clotting assays (e.g., aPTT, FVIII activity) are affected by emicizumab and cannot be used to monitor FVIII activity or coagulation. Chromogenic assays using human factors are recommended if FVIII activity needs to be measured.
- The most critical drug interaction is with activated prothrombin complex concentrate (aPCC). Concomitant use significantly increases the risk of thrombotic microangiopathy (TMA) and thrombotic events. If aPCC is necessary for breakthrough bleeding, use the lowest effective dose and duration, and monitor closely.
- Patients and caregivers should be thoroughly educated on proper subcutaneous injection technique and the importance of adherence to the dosing schedule.
- Emicizumab has a long half-life, allowing for less frequent dosing (weekly, bi-weekly, or monthly), which can improve patient convenience and adherence.
Alternative Therapies
- Factor VIII concentrates (recombinant or plasma-derived, e.g., Advate, Eloctate, Jivi, Kogenate, Novoeight, Recombinate, Xyntha)
- Bypassing agents (for patients with inhibitors, e.g., activated prothrombin complex concentrate [aPCC] - FEIBA, recombinant Factor VIIa [rFVIIa] - NovoSeven RT)
- Gene therapy (e.g., Valoctocogene roxaparvovec - Roctavian)
- Non-factor replacement therapies (e.g., Fitusiran, Concizumab - investigational)
Cost & Coverage
Average Cost:
Highly variable, typically >$100,000 annually per year
Insurance Coverage:
Specialty Tier (requires prior authorization, often subject to high co-pays/coinsurance)
General Drug Facts
If your symptoms or health issues persist or worsen, it is essential to contact your doctor promptly. To ensure safe use, never share your medication with others or take someone else's medication. This medication is accompanied by a Medication Guide, which provides crucial information for patients. Please read this guide carefully and review it again whenever you receive a refill of this medication. If you have any questions or concerns about this medication, consult 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 quantity, and the time it occurred.