Haegarda 2000unit Inj, 1 Vial
It is used to prevent swelling attacks in people with hereditary angioedema (HAE).
Drug Class
Agents for hereditary angioedema
Pharmacologic Class
C1 Esterase Inhibitor
Pregnancy Category
Not available
FDA Approved
Jun 2017
DEA Schedule
Not Controlled
Overview
What is this medicine?
Haegarda is a medicine made from human blood that contains a protein called C1 esterase inhibitor. People with a rare genetic condition called hereditary angioedema (HAE) don't have enough of this protein, which can cause sudden, painful swelling attacks. Haegarda helps replace this missing protein to prevent these swelling attacks.
How to Use This Medicine
Proper Administration of This Medication
To use this medication correctly, follow your doctor's instructions and read all accompanying information carefully. This medication is administered via injection into the fatty tissue under the skin. If you will be self-administering the injection, your doctor or nurse will provide guidance on the proper technique.
Before use, this medication must be mixed according to the instructions provided by your doctor. Do not shake the medication. If it has been stored in the refrigerator, allow it to reach room temperature before mixing. Avoid heating the medication.
Inspect the solution before use; do not administer if it appears cloudy, is leaking, or contains particles. Discard any remaining medication after the dose has been administered. Only use the medication if the solution is clear and colorless. Administer the dose within 8 hours of mixing.
To minimize the risk of injection site reactions, rotate the injection site with each administration. Dispose of used needles and syringes in a puncture-resistant container. Do not reuse needles or other injection materials. When the disposal container is full, follow local regulations for proper disposal. If you have any questions or concerns, consult your doctor or pharmacist.
Storage and Disposal
Store this medication at room temperature or in the refrigerator, avoiding freezing. Keep the medication in its original container to protect it from light. After mixing, do not refrigerate or freeze the medication.
Missed Dose Instructions
If you miss a dose, take it as soon as you remember. However, if the missed dose is near the time of your next scheduled dose, skip the missed dose and resume your regular dosing schedule. Do not take two doses at the same time or take extra doses to compensate for a missed dose.
To use this medication correctly, follow your doctor's instructions and read all accompanying information carefully. This medication is administered via injection into the fatty tissue under the skin. If you will be self-administering the injection, your doctor or nurse will provide guidance on the proper technique.
Before use, this medication must be mixed according to the instructions provided by your doctor. Do not shake the medication. If it has been stored in the refrigerator, allow it to reach room temperature before mixing. Avoid heating the medication.
Inspect the solution before use; do not administer if it appears cloudy, is leaking, or contains particles. Discard any remaining medication after the dose has been administered. Only use the medication if the solution is clear and colorless. Administer the dose within 8 hours of mixing.
To minimize the risk of injection site reactions, rotate the injection site with each administration. Dispose of used needles and syringes in a puncture-resistant container. Do not reuse needles or other injection materials. When the disposal container is full, follow local regulations for proper disposal. If you have any questions or concerns, consult your doctor or pharmacist.
Storage and Disposal
Store this medication at room temperature or in the refrigerator, avoiding freezing. Keep the medication in its original container to protect it from light. After mixing, do not refrigerate or freeze the medication.
Missed Dose Instructions
If you miss a dose, take it as soon as you remember. However, if the missed dose is near the time of your next scheduled dose, skip the missed dose and resume your regular dosing schedule. Do not take two doses at the same time or take extra doses to compensate for a missed dose.
Lifestyle & Tips
- Administer Haegarda subcutaneously (under the skin) twice a week as directed by your healthcare provider.
- Learn proper injection technique from your healthcare provider or nurse.
- Rotate injection sites to avoid skin irritation.
- Keep a diary of your HAE attacks (frequency, severity, triggers) to help your doctor assess the effectiveness of the treatment.
- Avoid known triggers for your HAE attacks, if identified.
- Carry an acute treatment for HAE attacks, even while on prophylaxis, as breakthrough attacks can occur.
- Report any signs of allergic reaction (hives, rash, difficulty breathing) or blood clots (chest pain, shortness of breath, leg swelling) immediately to your doctor.
Available Forms & Alternatives
Available Strengths:
Dosing & Administration
Adult Dosing
Standard Dose:
60 IU/kg subcutaneously twice weekly. For patients weighing less than 65 kg, a dose of 2000 IU is recommended. For patients weighing 65 kg or more, a dose of 3000 IU is recommended.
Dose Range:
2000 - 3000 mg
Condition-Specific Dosing:
routine_prophylaxis_of_hae:
60 IU/kg subcutaneously twice weekly. For patients weighing less than 65 kg, a dose of 2000 IU is recommended. For patients weighing 65 kg or more, a dose of 3000 IU is recommended.
Pediatric Dosing
Neonatal:
Not established
Infant:
Not established
Child:
For children 6 to <12 years: 60 IU/kg subcutaneously twice weekly. For children 12 to <18 years: 60 IU/kg subcutaneously twice weekly. For patients weighing less than 65 kg, a dose of 2000 IU is recommended. For patients weighing 65 kg or more, a dose of 3000 IU is recommended.
Adolescent:
For children 12 to <18 years: 60 IU/kg subcutaneously twice weekly. For patients weighing less than 65 kg, a dose of 2000 IU is recommended. For patients weighing 65 kg or more, a dose of 3000 IU is recommended.
Dose Adjustments
Renal Impairment:
Mild:
No adjustment needed
Moderate:
No adjustment needed
Severe:
No adjustment needed
Dialysis:
No specific recommendations; C1-INH is a large protein and not significantly removed by dialysis.
Hepatic Impairment:
Mild:
No adjustment needed
Moderate:
No adjustment needed
Severe:
No adjustment needed
Pharmacology
Mechanism of Action
C1 Esterase Inhibitor (Human) is a plasma-derived concentrate of C1-INH, a normal constituent of human plasma. It functions as a serine protease inhibitor, regulating the complement, contact, fibrinolytic, and coagulation systems. In patients with hereditary angioedema (HAE), a deficiency or dysfunction of C1-INH leads to uncontrolled activation of these systems, particularly the contact system, resulting in excessive bradykinin generation, which causes increased vascular permeability and angioedema attacks. Haegarda replaces the deficient C1-INH, thereby restoring the regulation of these pathways and preventing bradykinin overproduction.
Pharmacokinetics
Absorption:
Bioavailability:
Approximately 43% (subcutaneous)
Tmax:
Approximately 13 hours
FoodEffect:
Not applicable (subcutaneous administration)
Distribution:
Vd:
Not specifically reported for Haegarda, but generally distributes within the plasma and extracellular fluid compartments.
ProteinBinding:
Not applicable (it is a protein itself)
CnssPenetration:
Limited
Elimination:
HalfLife:
Approximately 70 hours
Clearance:
Not specifically reported for Haegarda, but eliminated via protein catabolism.
ExcretionRoute:
Not applicable (catabolized)
Unchanged:
Not applicable (catabolized)
Pharmacodynamics
OnsetOfAction:
Not immediately for acute attack resolution; for prophylaxis, steady-state levels are achieved over time.
PeakEffect:
Peak plasma levels reached around 13 hours post-SC administration.
DurationOfAction:
Provides sustained prophylactic effect due to its half-life and twice-weekly dosing.
Safety & Warnings
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. If you notice any of the following symptoms, contact your doctor or seek immediate medical attention:
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
Severe dizziness or fainting
Discoloration of the mouth, turning blue
Rapid heartbeat
Severe headache
Weakness on one side of the body, difficulty speaking or thinking, balance problems, drooping on one side of the face, or blurred vision
Symptoms of a blood clot, including:
+ Chest pain or pressure
+ Coughing up blood
+ Shortness of breath
+ Swelling, warmth, numbness, color change, or pain in a leg or arm
+ Difficulty speaking or swallowing
Other Possible Side Effects
Like all medications, this drug can cause side effects. Although many people do not experience any side effects or only have mild ones, it is essential to discuss any concerns with your doctor. If you experience any of the following side effects or any other symptoms that bother you or do not go away, contact your doctor or seek medical help:
Irritation at the injection site
Nose or throat irritation
Dizziness
This is not an exhaustive list of 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 immediate medical attention:
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
Severe dizziness or fainting
Discoloration of the mouth, turning blue
Rapid heartbeat
Severe headache
Weakness on one side of the body, difficulty speaking or thinking, balance problems, drooping on one side of the face, or blurred vision
Symptoms of a blood clot, including:
+ Chest pain or pressure
+ Coughing up blood
+ Shortness of breath
+ Swelling, warmth, numbness, color change, or pain in a leg or arm
+ Difficulty speaking or swallowing
Other Possible Side Effects
Like all medications, this drug can cause side effects. Although many people do not experience any side effects or only have mild ones, it is essential to discuss any concerns with your doctor. If you experience any of the following side effects or any other symptoms that bother you or do not go away, contact your doctor or seek medical help:
Irritation at the injection site
Nose or throat irritation
Dizziness
This is not an exhaustive list of 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 allergic reaction: hives, rash, itching, difficulty breathing, wheezing, swelling of the face, lips, tongue, or throat, dizziness, fainting.
- Signs of a blood clot: chest pain, shortness of breath, swelling or pain in an arm or leg, sudden vision changes, slurred speech.
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. To ensure safe use, disclose all prescription and over-the-counter (OTC) medications, natural products, and vitamins you are taking to both your doctor and pharmacist.
* All your health problems, as they may affect the safety of taking this medication.
To avoid potential risks, do not start, stop, or change the dosage of any medication without first consulting your doctor. It is crucial to verify that it is safe to take this medication in combination with your other medications and health conditions.
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. To ensure safe use, disclose all prescription and over-the-counter (OTC) medications, natural products, and vitamins you are taking to both your doctor and pharmacist.
* All your health problems, as they may affect the safety of taking this medication.
To avoid potential risks, do not start, stop, or change the dosage of any medication without first consulting your doctor. It is crucial to verify that it is safe to take this medication in combination with your other medications and health conditions.
Precautions & Cautions
It is essential to inform all your healthcare providers, including doctors, nurses, pharmacists, and dentists, that you are taking this medication. As a precautionary measure, your doctor will prescribe epinephrine, which you should carry with you at all times while taking this drug. Make sure you understand how and when to use epinephrine, and seek immediate medical attention after administering it.
There is a risk of blood clots associated with this medication. Your risk may be increased if you have a certain type of catheter or device in a vein, or if you are taking other medications such as estrogens or androgens. Additionally, your risk may be higher if you have a history of heart or blood vessel disease, stroke, thick blood, or a previous blood clot. Prolonged periods of immobility may also increase your risk. Discuss these factors with your doctor.
As this medication is derived from human plasma, there is a potential risk of transmitting viruses that can cause disease. Although the medication is thoroughly screened, tested, and treated to minimize this risk, it is crucial to discuss this with your doctor.
It is important to note that this medication is used to prevent certain health problems, but it will not treat acute HAE attacks. If you have questions about managing HAE attacks, consult your doctor.
Before traveling, consult your doctor to ensure you have an adequate supply of this medication. If you experience any changes in weight, inform your doctor, as your dosage may need to be adjusted.
If you are pregnant, planning to become pregnant, or breastfeeding, discuss the potential benefits and risks of this medication with your doctor to make an informed decision about your treatment.
There is a risk of blood clots associated with this medication. Your risk may be increased if you have a certain type of catheter or device in a vein, or if you are taking other medications such as estrogens or androgens. Additionally, your risk may be higher if you have a history of heart or blood vessel disease, stroke, thick blood, or a previous blood clot. Prolonged periods of immobility may also increase your risk. Discuss these factors with your doctor.
As this medication is derived from human plasma, there is a potential risk of transmitting viruses that can cause disease. Although the medication is thoroughly screened, tested, and treated to minimize this risk, it is crucial to discuss this with your doctor.
It is important to note that this medication is used to prevent certain health problems, but it will not treat acute HAE attacks. If you have questions about managing HAE attacks, consult your doctor.
Before traveling, consult your doctor to ensure you have an adequate supply of this medication. If you experience any changes in weight, inform your doctor, as your dosage may need to be adjusted.
If you are pregnant, planning to become pregnant, or breastfeeding, discuss the potential benefits and risks of this medication with your doctor to make an informed decision about your treatment.
Overdose Information
Overdose Symptoms:
- No specific symptoms of overdose have been reported with C1-INH. High doses may theoretically increase the risk of thromboembolic events, though this is not well-established for Haegarda.
What to Do:
In case of suspected overdose, contact a poison control center (e.g., 1-800-222-1222) or seek immediate medical attention. Management is generally supportive.
Drug Interactions
Monitoring
Baseline Monitoring
Diagnosis of Hereditary Angioedema (HAE)
Rationale: Confirm C1-INH deficiency or dysfunction (Type I or Type II HAE) before initiating therapy.
Timing: Prior to initiation of therapy
Baseline C1-INH functional activity and antigenic levels
Rationale: To confirm diagnosis and establish baseline for monitoring response.
Timing: Prior to initiation of therapy
Routine Monitoring
Frequency and severity of HAE attacks
Frequency: Regularly (e.g., monthly or quarterly patient assessment)
Target: Reduction in attack frequency and severity
Action Threshold: Persistent attacks or inadequate response may warrant re-evaluation of dose or alternative therapy.
Injection site reactions
Frequency: At each visit or patient self-monitoring
Target: Minimal to no reaction
Action Threshold: Severe or persistent reactions may require intervention or change in injection technique.
Signs/symptoms of hypersensitivity/allergic reactions
Frequency: Patient self-monitoring, especially during/after administration
Target: Absence of symptoms
Action Threshold: Development of hives, rash, pruritus, dyspnea, wheezing, angioedema, hypotension, or anaphylaxis requires immediate discontinuation and appropriate medical management.
Signs/symptoms of thromboembolic events (rare)
Frequency: Patient self-monitoring
Target: Absence of symptoms
Action Threshold: Symptoms like chest pain, shortness of breath, swelling/pain in a limb require immediate medical attention.
Symptom Monitoring
- Reduction in frequency of HAE attacks
- Absence of new or worsening angioedema symptoms
- Injection site reactions (pain, swelling, redness, bruising)
- Signs of allergic reaction (hives, rash, itching, difficulty breathing, wheezing, swelling of face/lips/tongue/throat, dizziness, fainting)
- Signs of thromboembolic events (chest pain, shortness of breath, swelling or pain in an arm or leg, sudden vision changes, slurred speech)
Special Patient Groups
Pregnancy
There are no adequate and well-controlled studies of Haegarda in pregnant women. Animal reproduction studies have not been conducted. Use during pregnancy only if clearly needed and the potential benefit outweighs the potential risk to the fetus. The decision to use Haegarda during pregnancy should be made on a case-by-case basis in consultation with a healthcare provider.
Trimester-Specific Risks:
First Trimester:
Risk unknown; C1-INH is a human protein, generally considered low risk for teratogenicity.
Second Trimester:
Risk unknown; generally considered low risk.
Third Trimester:
Risk unknown; generally considered low risk.
Lactation
It is unknown whether Haegarda is excreted in human milk. Human C1-INH is a normal component of human plasma. The developmental and health benefits of breastfeeding should be considered along with the mother’s clinical need for Haegarda and any potential adverse effects on the breastfed infant from Haegarda or from the underlying maternal condition. Proteins like C1-INH are generally digested in the infant's gastrointestinal tract if transferred into milk.
Infant Risk:
Low risk (L3 - Moderately safe, but data limited)
Pediatric Use
Haegarda is approved for routine prophylaxis of HAE attacks in children 6 years of age and older. Dosing is weight-based. Safety and effectiveness in children younger than 6 years have not been established.
Geriatric Use
Clinical studies of Haegarda did not include sufficient numbers of subjects aged 65 and over to determine whether they respond differently from younger subjects. In general, dose selection for an elderly patient should be cautious, usually starting at the low end of the dosing range, reflecting the greater frequency of decreased hepatic, renal, or cardiac function, and of concomitant disease or other drug therapy. However, no specific dose adjustments are recommended based on age alone.
Clinical Information
Clinical Pearls
- Haegarda is the first and only C1-INH concentrate approved for subcutaneous administration for routine prophylaxis of HAE attacks.
- Patients can self-administer Haegarda at home after proper training, offering convenience and reducing the need for frequent clinic visits.
- It is crucial for patients to continue carrying acute treatment for breakthrough HAE attacks, as prophylaxis does not eliminate all attacks.
- Ensure patients understand the importance of rotating injection sites to minimize local reactions.
- While rare, monitor for signs of hypersensitivity reactions and thromboembolic events, especially in patients with pre-existing risk factors.
Alternative Therapies
- Cinryze (C1 Esterase Inhibitor, Human, IV)
- Berinert (C1 Esterase Inhibitor, Human, IV)
- Takhzyro (lanadelumab, monoclonal antibody, SC)
- Orladeyo (berotralstat, oral)
- Ruconest (C1 Esterase Inhibitor, recombinant, IV - for acute attacks)
Cost & Coverage
Average Cost:
Highly variable, typically several thousand dollars per vial per 2000 IU 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 is essential to contact your doctor promptly. To ensure your safety, never share your medication with others or take someone else's medication. Some medications may come with an additional patient information leaflet, so it's a good idea to check with your pharmacist. If you have any questions or concerns about this medication, don't hesitate to discuss them with your doctor, nurse, 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.