Hemlibra 60mg/0.4ml 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 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 more effectively, similar to how a missing clotting factor would. It is given as an injection under the skin.
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 in the thigh, abdominal 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.
Before administering the injection, inspect the solution for any changes in color, cloudiness, or particulate matter. The solution should be colorless to faintly yellow; do not use if it has changed color or appears cloudy, leaky, or contains particles. Avoid injecting into moles, scars, or skin that is irritated, tender, bruised, red, hard, or broken.
After removing the medication from the vial, use it immediately and do not shake the vial. Do not apply pressure to the injection site using a waistband or belt. Additionally, do not draw the medication into a syringe and store it for future use. After administering the dose, discard any remaining medication in the opened vial and dispose of the needle in a designated sharps container. Do not reuse needles or other injection equipment.
Storage and Disposal
Store this medication in the refrigerator to maintain its potency. Keep it in the 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. If a vial has been at room temperature for a total of 7 days, discard it.
Missed Dose
If you miss a dose, take it as soon as you remember and resume your regular dosing 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 in the thigh, abdominal 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.
Before administering the injection, inspect the solution for any changes in color, cloudiness, or particulate matter. The solution should be colorless to faintly yellow; do not use if it has changed color or appears cloudy, leaky, or contains particles. Avoid injecting into moles, scars, or skin that is irritated, tender, bruised, red, hard, or broken.
After removing the medication from the vial, use it immediately and do not shake the vial. Do not apply pressure to the injection site using a waistband or belt. Additionally, do not draw the medication into a syringe and store it for future use. After administering the dose, discard any remaining medication in the opened vial and dispose of the needle in a designated sharps container. Do not reuse needles or other injection equipment.
Storage and Disposal
Store this medication in the refrigerator to maintain its potency. Keep it in the 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. If a vial has been at room temperature for a total of 7 days, discard it.
Missed Dose
If you miss a dose, take it as soon as you remember and resume your regular dosing 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
- Continue to avoid activities that carry a high risk of injury or bleeding.
- Maintain regular communication with your healthcare team regarding any bleeding episodes or new symptoms.
- Carry your Hemlibra dose and supplies with you when traveling.
- Follow proper injection technique as instructed by your healthcare provider.
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 subcutaneously once weekly OR 3 mg/kg subcutaneously every 2 weeks OR 6 mg/kg subcutaneously every 4 weeks.
Dose Range:
1.5 - 6 mg
Condition-Specific Dosing:
Hemophilia A with factor VIII inhibitors:
Initial indication, same dosing.
Hemophilia A without factor VIII inhibitors:
Approved for this indication, same dosing.
Pediatric Dosing
Neonatal:
Not established (safety and efficacy in neonates have not been established).
Infant:
Dosing is weight-based, same as adult dosing (3 mg/kg loading, then 1.5 mg/kg weekly or alternative maintenance schedules).
Child:
Dosing is weight-based, same as adult dosing (3 mg/kg loading, then 1.5 mg/kg weekly or alternative maintenance schedules).
Adolescent:
Dosing is weight-based, same as adult dosing (3 mg/kg loading, then 1.5 mg/kg weekly or alternative maintenance schedules).
Dose Adjustments
Renal Impairment:
Mild:
No dose adjustment recommended.
Moderate:
No dose adjustment recommended.
Severe:
No dose adjustment recommended (not renally eliminated).
Dialysis:
No specific recommendations; not expected to be removed by dialysis.
Hepatic Impairment:
Mild:
No dose adjustment recommended.
Moderate:
No dose adjustment recommended.
Severe:
No dose adjustment recommended (not hepatically metabolized via CYP450).
Pharmacology
Mechanism of Action
Emicizumab is a bispecific antibody that bridges activated factor IX (FIXa) and factor X (FX) to restore effective factor Xa generation, mimicking the cofactor function of activated factor VIII (FVIII). It facilitates the intrinsic coagulation pathway, promoting hemostasis in patients with hemophilia A.
Pharmacokinetics
Absorption:
Bioavailability:
80.4%
Tmax:
5 to 7 days
FoodEffect:
Not applicable (subcutaneous administration)
Distribution:
Vd:
6.0 L (central volume of distribution)
ProteinBinding:
Not applicable (antibody)
CnssPenetration:
Limited
Elimination:
HalfLife:
27.2 days (approximately)
Clearance:
0.19 L/day (approximately)
ExcretionRoute:
Not renally or hepatically eliminated in the traditional sense; catabolized into small peptides and amino acids.
Unchanged:
Not applicable (antibody catabolism)
Pharmacodynamics
OnsetOfAction:
Within hours to days (hemostatic effect observed within days of first dose)
PeakEffect:
Steady-state concentrations reached by approximately 4 weeks with weekly 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: Cases of thrombotic microangiopathy (TMA) and thrombotic events, including cerebral venous sinus thrombosis (CVST), have been reported when activated prothrombin complex concentrate (aPCC) was administered to patients receiving Hemlibra. Monitor for signs and symptoms of TMA and thrombotic events. If these events occur, discontinue aPCC and interrupt Hemlibra. Continue treatment for TMA and thrombotic events as clinically indicated.
Side Effects
Urgent Side Effects: Seek Medical Help Right Away
While rare, some people may experience severe and potentially life-threatening side effects when taking this medication. Immediately contact your doctor or seek medical attention if you experience any of the following symptoms, which may indicate a serious allergic reaction:
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
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 persist or bother you:
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. Your doctor can provide medical advice and guidance on managing side effects.
While rare, some people may experience severe and potentially life-threatening side effects when taking this medication. Immediately contact your doctor or seek medical attention if you experience any of the following symptoms, which may indicate a serious allergic reaction:
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
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 persist or bother you:
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. Your doctor can provide medical advice and guidance on managing side effects.
Seek Immediate Medical Attention If You Experience:
- Signs of a serious blood clot (thrombosis): pain, swelling, warmth, or redness in an arm or leg; sudden chest pain; shortness of breath; sudden numbness or weakness on one side of your body; sudden severe headache; problems with vision or speech.
- Signs of thrombotic microangiopathy (TMA): confusion, weakness, swelling, yellowing of skin or eyes, decreased urine output, chest pain, shortness of breath, vision changes, slurred speech.
- Any new or worsening bleeding episodes.
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 and pharmacist assess potential interactions between this medication and other substances you are taking.
* Any existing health problems, as this medication may interact with certain conditions or exacerbate underlying health issues.
To ensure your safety, it is crucial to verify that this medication can be taken safely with all your other medications and health conditions. Never start, stop, or adjust the dosage of any medication without first consulting your doctor.
It is essential to inform your doctor about the following:
Any allergies you have, including allergies to this medication, 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 and pharmacist assess potential interactions between this medication and other substances you are taking.
* Any existing health problems, as this medication may interact with certain conditions or exacerbate underlying health issues.
To ensure your safety, it is crucial to verify that this medication can be taken safely with all your other medications and health conditions. Never start, stop, or adjust the dosage of any medication without first consulting your doctor.
Precautions & Cautions
It is essential to inform all your healthcare providers, including doctors, nurses, pharmacists, and dentists, that you are taking this medication. Regular blood tests will be necessary, as directed by your doctor, to monitor your condition. Be sure to discuss any concerns or questions you have 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 effective, 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 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 effective, 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 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 could theoretically include an increased risk of thrombotic events or other adverse reactions.
What to Do:
Seek immediate medical attention. Contact your doctor or poison control center (1-800-222-1222). Management would be supportive and symptomatic.
Drug Interactions
Major Interactions
- Activated prothrombin complex concentrate (aPCC) at doses >100 U/kg/day for >24 hours (increased risk of thrombotic microangiopathy and thrombotic events).
Monitoring
Baseline Monitoring
Factor VIII inhibitor status
Rationale: To confirm diagnosis and guide treatment strategy.
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.
Platelet count and renal function
Rationale: To establish baseline for monitoring potential adverse events like thrombotic microangiopathy.
Timing: Prior to initiation of therapy.
Routine Monitoring
Signs and symptoms of bleeding
Frequency: Regularly (e.g., daily patient self-monitoring, clinic visits)
Target: Reduction in bleeding episodes
Action Threshold: Increased bleeding frequency or severity may indicate need for additional treatment or re-evaluation.
Signs and symptoms of thrombotic microangiopathy (TMA) or thrombosis
Frequency: Regularly (e.g., patient education for self-monitoring, clinic visits)
Target: Absence of symptoms
Action Threshold: New or worsening symptoms such as confusion, weakness, swelling, chest pain, shortness of breath, or changes in vision. Laboratory abnormalities (e.g., decreased platelet count, elevated creatinine, elevated LDH, decreased haptoglobin, schistocytes on blood smear) warrant immediate investigation.
Coagulation assays (e.g., aPTT, FVIII activity)
Frequency: Not routinely recommended for monitoring emicizumab activity
Target: Not applicable
Action Threshold: Emicizumab affects intrinsic pathway assays, leading to falsely shortened aPTT and falsely high FVIII activity levels. Use chromogenic FVIII assays with bovine FIXa and FX for FVIII activity if needed, or non-activated aPTT for inhibitor testing. Do not use one-stage FVIII assays.
Symptom Monitoring
- Unusual bleeding or bruising
- Signs of thrombotic microangiopathy (TMA): confusion, weakness, swelling, yellowing of skin/eyes, decreased urine output, chest pain, shortness of breath, vision changes, slurred speech, numbness/weakness on one side of body.
- Signs of thrombosis: pain, swelling, warmth, redness in a limb; chest pain, shortness of breath, rapid heart rate, dizziness.
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.
Trimester-Specific Risks:
First Trimester:
Limited data, theoretical risk of fetal exposure.
Second Trimester:
Limited data, theoretical risk of fetal exposure.
Third Trimester:
Limited data, theoretical risk of fetal exposure, particularly as IgG transfer increases in the third trimester.
Lactation
It is unknown whether emicizumab is excreted in human milk. Maternal IgG is known to be present in human milk. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for Hemlibra and any potential adverse effects on the breastfed child from Hemlibra or from the underlying maternal condition.
Infant Risk:
Risk is generally considered low for large protein molecules like antibodies, but specific data are lacking. Monitor breastfed infants for adverse reactions.
Pediatric Use
Safety and efficacy have been established in pediatric patients (birth to <18 years) with hemophilia A. Dosing is weight-based and similar to adults. No specific dose adjustments are needed based on age within the pediatric population.
Geriatric Use
Clinical studies of Hemlibra 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 for geriatric patients.
Clinical Information
Clinical Pearls
- Emicizumab is a prophylactic treatment and is NOT intended for the treatment of acute bleeding episodes. Bypassing agents (e.g., aPCC, rFVIIa) are still needed for acute bleeds.
- Coagulation assays (e.g., aPTT, FVIII activity using one-stage assays) are significantly affected by emicizumab and cannot be used to monitor its activity or FVIII levels. Use chromogenic FVIII assays with bovine FIXa and FX for FVIII activity if needed, or non-activated aPTT for inhibitor testing.
- Patients and caregivers must be educated on the signs and symptoms of thrombotic microangiopathy (TMA) and thrombosis, especially if concomitant use of aPCC is required.
- Ensure patients understand the importance of reporting all concomitant medications, especially bypassing agents, to their healthcare provider.
- The long half-life allows for flexible dosing schedules (weekly, bi-weekly, or monthly) after the initial loading phase, which can improve patient adherence and quality of life.
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, etranacogene dezaparvovec)
- Non-factor replacement therapies (e.g., fitusiran, concizumab - under development/review)
Cost & Coverage
Average Cost:
Highly variable, typically >$400,000 - $500,000 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 your safety, never share your medication with others or take someone else's medication. This medication is accompanied by a Medication Guide, which is a crucial patient fact sheet. Please read it carefully and review it again whenever you receive a refill. If you have any questions or concerns about this medication, we encourage you 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 seeking help, be prepared to provide detailed information about the overdose, including the medication taken, the amount, and the time it occurred.