Hemlibra 30mg/ml Inj, 1 Vial

Manufacturer GENENTECH Active Ingredient Emicizumab(em i SIZ ue mab) Pronunciation HEM-lee-brah (Emicizumab: em i SIZ ue mab)
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
Hemostatic agent
🧬
Pharmacologic Class
Bispecific Factor IXa- and Factor X-directed antibody
🀰
Pregnancy Category
Not available
βœ…
FDA Approved
Nov 2017
βš–οΈ
DEA Schedule
Not Controlled

Overview

ℹ️

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 clot properly, even if you have developed inhibitors to other hemophilia treatments.
πŸ“‹

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 subcutaneous injection into the fatty tissue of the thigh, abdomen, 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. Rotate the injection site with each dose to minimize the risk of local reactions.

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, 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. Use the medication immediately after removing it from the vial, and do not shake the vial. After administering the dose, discard any remaining medication in the opened vial and dispose of the needle in a sharps disposal container. Do not reuse needles or other injection equipment.

Storage and Disposal

Store this medication in the refrigerator to maintain its potency. Protect the medication from light by storing it in its original container. If necessary, unopened vials can be stored at room temperature for up to 7 days, after which they must be returned to the refrigerator or discarded. Do not store this medication at room temperature for more than 7 days in total. If a vial has been at room temperature for 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.
πŸ’‘

Lifestyle & Tips

  • Follow your prescribed dosing schedule carefully.
  • Learn proper subcutaneous injection technique from your healthcare provider.
  • Keep a record of your bleeding episodes and any side effects.
  • Avoid activities that put you at high risk for injury or bleeding.
  • Carry your medical identification card or information about your condition.
  • Discuss any planned surgeries or dental procedures with your healthcare provider.
  • Do not use activated prothrombin complex concentrate (aPCC) for bleeding treatment unless directed by your doctor, and if used, monitor closely for serious side effects.

Dosing & Administration

πŸ‘¨β€βš•οΈ

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, administered subcutaneously.
Dose Range: 1.5 - 6 mg

Condition-Specific Dosing:

Hemophilia A with inhibitors: Same as standard dosing.
Hemophilia A without inhibitors: Same as standard dosing.
πŸ‘Ά

Pediatric Dosing

Neonatal: Not established
Infant: Same as adult dosing (3 mg/kg loading, then 1.5 mg/kg weekly or 3 mg/kg every 2 weeks or 6 mg/kg every 4 weeks) for patients of all ages, including infants.
Child: Same as adult dosing (3 mg/kg loading, then 1.5 mg/kg weekly or 3 mg/kg every 2 weeks or 6 mg/kg every 4 weeks).
Adolescent: Same as adult dosing (3 mg/kg loading, then 1.5 mg/kg weekly or 3 mg/kg every 2 weeks or 6 mg/kg every 4 weeks).
βš•οΈ

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 not renally cleared).
Dialysis: No dose adjustment necessary.

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 not hepatically cleared).

Pharmacology

πŸ”¬

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), which is necessary for effective hemostasis. It mimics the cofactor function of FVIIIa by bringing FIXa and FX into close proximity, thereby facilitating the activation of FX to FXa and promoting thrombin generation.
πŸ“Š

Pharmacokinetics

Absorption:

Bioavailability: 80.4% (subcutaneous)
Tmax: 5-7 days
FoodEffect: Not applicable (subcutaneous administration)

Distribution:

Vd: 10.4 L (central compartment)
ProteinBinding: Not applicable (monoclonal antibody)
CnssPenetration: Limited

Elimination:

HalfLife: 27.2 days (approximately 4 weeks)
Clearance: 0.271 L/day
ExcretionRoute: Not applicable (catabolized)
Unchanged: Not applicable
⏱️

Pharmacodynamics

OnsetOfAction: Rapid, within hours to days of first dose, with steady-state concentrations achieved by 4 weeks.
PeakEffect: Steady-state concentrations achieved by 4 weeks with weekly dosing.
DurationOfAction: Prolonged due to long half-life, allowing for weekly, bi-weekly, or monthly dosing.
Confidence: High

Safety & Warnings

⚠️

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
- 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, consult your doctor. For medical advice about side effects, contact 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:

  • Severe headache, nausea, vomiting, stomach pain, or unusual tiredness (could be signs of a serious blood clotting problem called TMA).
  • Swelling, pain, or redness in an arm or leg (could be signs of a blood clot).
  • New or worsening bleeding despite treatment.
  • Severe injection site reactions (e.g., large area of redness, swelling, blistering).
πŸ“‹

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 your safety, 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 health problems you have

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 modify the dose 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 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 notify 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 will need to discuss the potential benefits and risks of this medication to both you and your baby.
πŸ†˜

Overdose Information

Overdose Symptoms:

  • Symptoms of overdose are not well-established. Given its mechanism, an overdose might theoretically increase the risk of thrombotic events, but clinical data are limited.

What to Do:

In case of suspected overdose, contact a healthcare professional immediately or call a poison control center (e.g., 1-800-222-1222). Management would likely be supportive and focused on monitoring for and treating potential thrombotic complications.

Drug Interactions

πŸ”΄

Major Interactions

  • Activated prothrombin complex concentrate (aPCC, e.g., FEIBA): Concomitant use with emicizumab may increase the risk of thrombotic microangiopathy (TMA) and thrombotic events. Avoid routine prophylactic use of aPCC. If aPCC is required, monitor for signs of TMA and thrombosis.
🟑

Moderate Interactions

  • Recombinant Factor VIIa (rFVIIa): While not contraindicated, caution is advised. Doses of rFVIIa may need to be lower or less frequent when used with emicizumab. Monitor for signs of thrombosis.
⚠️

Confidence Interactions

Monitoring

πŸ”¬

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 emicizumab is effective with or without inhibitors).

Timing: Prior to initiation of therapy.

πŸ“Š

Routine Monitoring

Bleeding episodes (frequency, severity, location)

Frequency: Continuously, at each clinical visit.

Target: Reduction in bleeding episodes.

Action Threshold: Persistent or increased bleeding may indicate need for re-evaluation or adjunctive therapy.

Injection site reactions

Frequency: At each clinical visit, and patient self-monitoring.

Target: Minimal to no reaction.

Action Threshold: Severe or persistent reactions may require intervention or re-evaluation of administration technique.

Signs/symptoms of thrombotic microangiopathy (TMA) or thrombosis (e.g., headache, nausea, vomiting, abdominal pain, fatigue, swelling, pain, redness in limbs)

Frequency: Especially when concomitant bypassing agents are used; patient education for self-monitoring.

Target: Absence of symptoms.

Action Threshold: Any suspicion of TMA or thrombosis requires immediate medical evaluation and discontinuation of bypassing agents.

Laboratory coagulation assays (e.g., aPTT, FVIII activity)

Frequency: Not routinely recommended for monitoring emicizumab efficacy, as emicizumab significantly shortens aPTT and renders FVIII activity assays unreliable. If FVIII activity is needed, chromogenic assays using bovine FIXa and FX are recommended.

Target: Not applicable for routine monitoring of emicizumab effect.

Action Threshold: Not applicable for routine monitoring of emicizumab effect.

πŸ‘οΈ

Symptom Monitoring

  • Unusual bleeding or bruising (despite treatment)
  • Injection site reactions (redness, swelling, pain, itching)
  • Headache, nausea, vomiting, abdominal pain, fatigue (potential signs of TMA)
  • Swelling, pain, or redness in an arm or leg (potential signs of thrombosis)
  • Dizziness or lightheadedness
  • Rash or hives

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. Human IgG antibodies are known to cross the placental barrier.

Trimester-Specific Risks:

First Trimester: Risk unknown. Animal studies did not show direct harm, but human data are lacking.
Second Trimester: Risk unknown. Human IgG antibodies are known to cross the placental barrier, particularly in the second and third trimesters.
Third Trimester: Risk unknown. Potential for fetal exposure and impact on fetal hemostasis is possible.
🀱

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.

Infant Risk: L3 (Moderate risk) - Large molecular weight suggests limited excretion into milk, but potential for oral absorption by infant is low. Monitor breastfed infant for adverse effects.
πŸ‘Ά

Pediatric Use

Approved for use in pediatric patients of all ages, including infants. Dosing is weight-based. Safety and efficacy established in clinical trials.

πŸ‘΄

Geriatric Use

No specific dose adjustment is required for geriatric patients. Clinical studies included patients up to 75 years of age. No overall differences in safety or efficacy were observed between older and younger patients, but greater sensitivity of some older individuals cannot be ruled out.

Clinical Information

πŸ’Ž

Clinical Pearls

  • Emicizumab significantly shortens activated partial thromboplastin time (aPTT) and renders FVIII activity assays unreliable. If FVIII activity is needed, use a chromogenic assay with bovine FIXa and FX.
  • Patients on emicizumab may still require on-demand treatment with bypassing agents (e.g., aPCC, rFVIIa) for breakthrough bleeds. Extreme caution is advised with aPCC due to the risk of thrombotic microangiopathy (TMA) and thrombosis.
  • Educate patients and caregivers on the signs and symptoms of TMA and thrombosis, especially if bypassing agents are used.
  • Hemlibra is administered subcutaneously, allowing for home administration after proper training.
  • The long half-life allows for flexible dosing schedules (weekly, bi-weekly, or monthly), which can improve patient adherence and quality of life.
πŸ”„

Alternative Therapies

  • Factor VIII (FVIII) replacement therapy (for hemophilia A without inhibitors)
  • Bypassing agents (e.g., Activated Prothrombin Complex Concentrate (aPCC), Recombinant Factor VIIa (rFVIIa)) (for hemophilia A with inhibitors, or on-demand for breakthrough bleeds)
  • Gene therapy (e.g., Valoctocogene roxaparvovec, Etranacogene dezaparvovec) (emerging therapies for hemophilia A)
  • Non-factor therapies (e.g., Fitusiran - investigational RNAi therapeutic)
πŸ’°

Cost & Coverage

Average Cost: Highly variable, typically several hundred thousand dollars per year. per year
Insurance Coverage: Specialty Tier / Tier 4 or 5 (requires prior authorization, often subject to high co-pays or co-insurance)
πŸ“š

General Drug Facts

If your symptoms or health issues persist or worsen, it's 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, a patient fact sheet that provides crucial information. 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, 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.