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C1 Esterase Inhibitor: Uses, Dosing, and Side Effects
Summary of Key Points
C1 esterase inhibitor concentrate is a medication primarily used to treat and prevent various forms of angioedema, a condition characterized by sudden swelling in different areas of the body. This treatment works by replenishing a protein that helps control inflammation and swelling. Administered through an injection, it is effective for managing both acute episodes and long-term prevention. Always consult your healthcare provider before starting or adjusting your dosage of C1 esterase inhibitor concentrate to ensure safe and effective use.
Names of the Medication
US Brand Names: Berinert, Cinryze, Haegarda
Generic Name: C1 esterase inhibitor (human)
The generic name refers to the active ingredient in the medication and is listed in parentheses following the brand name.
Pharmacologic Category
- Complement Inhibitors
- Plasma Protein Fraction
Dosing Guidelines for C1 Esterase Inhibitor
C1 esterase inhibitor concentrate is prescribed to manage various types of angioedema. The dosage depends on the specific condition being treated. Always adhere to your healthcare provider’s instructions and consult them before making any changes to your treatment plan. For additional support, you can schedule a telemedicine appointment with one of our providers.
Hereditary Angioedema (HAE) – Acute Attack
Dose: 20 units per kilogram of body weight
Frequency: Administered as needed during an acute attack
Hereditary Angioedema (HAE) – Prophylaxis (Prevention)
Dose: 1,000 units every 3 to 4 days
Frequency: Regularly scheduled to prevent attacks
Acquired Angioedema
Dose: 20 units per kilogram of body weight
Frequency: Administered as needed during an acute attack
Angioedema Due to ACE Inhibitors
Dose: 20 units per kilogram of body weight
Frequency: Administered as needed during an acute attack
Allergic Angioedema
Dose: 20 units per kilogram of body weight
Frequency: Administered as needed during an acute attack
Idiopathic Angioedema
Dose: 20 units per kilogram of body weight
Frequency: Administered as needed during an acute attack
Post-Surgical Angioedema
Dose: 20 units per kilogram of body weight
Frequency: Administered as needed during an acute attack
Trauma-Induced Angioedema
Dose: 20 units per kilogram of body weight
Frequency: Administered as needed during an acute attack
Drug-Induced Angioedema
Dose: 20 units per kilogram of body weight
Frequency: Administered as needed during an acute attack
Angioedema Associated with Infections
Dose: 20 units per kilogram of body weight
Frequency: Administered as needed during an acute attack
Dosage Forms and Strengths
- Injection: 500 units per vial
- Injection: 1,000 units per vial
Administration Instructions
C1 esterase inhibitor concentrate is administered as an intravenous injection (into a vein). Typically, it is given by a healthcare professional, but some patients may be trained to self-administer the medication at home. If you are administering this medication yourself, follow your healthcare provider’s instructions closely. Always wash your hands thoroughly before handling the medication, and use a clean, sterile needle and syringe. If you have any questions or concerns about the administration process, consult your healthcare provider or schedule a telemedicine appointment for further guidance.
Adverse Reactions and Side Effects
As with any medication, C1 esterase inhibitor concentrate may cause side effects. While not everyone experiences them, it’s important to be aware of potential reactions. If you notice any severe or unusual symptoms, contact your healthcare provider immediately.
Common Side Effects (1-10%): Headache, nausea, and injection site reactions, such as pain, redness, or swelling.
Less Common Side Effects (Less than 1%): Dizziness, rash, and fever.
Serious Side Effects (Rare): Allergic reactions, including difficulty breathing, chest tightness, or swelling of the face, lips, or throat. Seek emergency medical attention if these occur.
Contraindications for C1 Esterase Inhibitor Concentrate
A contraindication refers to a specific condition or circumstance in which a medication should not be used because it may cause harm. For C1 esterase inhibitor concentrate, certain factors can make its use unsafe.
Severe allergic reaction (hypersensitivity) to C1 esterase inhibitor concentrate: If you have previously experienced a serious allergic reaction to this medication or any of its components, its use is contraindicated. Re-exposure could result in severe, potentially life-threatening reactions, such as anaphylaxis.
History of blood clotting disorders: C1 esterase inhibitor concentrate may increase the risk of blood clots. If you have a history of clotting conditions, such as deep vein thrombosis (DVT) or pulmonary embolism (PE), or if you are predisposed to clotting, this medication may not be appropriate for you.
It is crucial to share your complete medical history with your healthcare provider before starting any new medication. If you are uncertain whether C1 esterase inhibitor concentrate is suitable for you, consider scheduling a telemedicine consultation with one of our providers to discuss your options in detail.
Drug-to-Drug Interactions with C1 Esterase Inhibitor Concentrate
Like many medications, C1 esterase inhibitor concentrate can interact with other drugs, potentially reducing its effectiveness or increasing the risk of side effects. Be sure to inform your healthcare provider about all medications you are currently taking, including prescription drugs, over-the-counter medications, supplements, and herbal remedies.
Estrogen-containing medications: (e.g., ethinyl estradiol) – Estrogen can elevate the risk of blood clots, a risk that may be further heightened when combined with C1 esterase inhibitor concentrate.
Androgens: (e.g., danazol) – Androgens may also increase the likelihood of blood clots when used in conjunction with C1 esterase inhibitor concentrate.
ACE inhibitors: (e.g., lisinopril, enalapril) – These medications, often prescribed for high blood pressure, may worsen angioedema, a condition that C1 esterase inhibitor concentrate is used to treat.
If you are taking any of these medications, your healthcare provider may need to adjust your treatment plan to minimize risks. Discuss potential interactions with one of our telemedicine providers to ensure your medications are safe and effective when used together.
Using C1 Esterase Inhibitor Concentrate During Pregnancy
Is it safe to take C1 esterase inhibitor concentrate during pregnancy?
The safety of C1 esterase inhibitor concentrate during pregnancy has not been fully established. Although limited data is available on its use in pregnant women, animal studies have not shown harm to the fetus. If you are pregnant or planning to become pregnant, it is important to carefully weigh the potential benefits and risks. Your healthcare provider can help determine whether C1 esterase inhibitor concentrate is the most appropriate treatment for your condition. For personalized advice, consider booking a telemedicine appointment to explore your options.
Using C1 Esterase Inhibitor Concentrate While Breastfeeding
Is it safe to take C1 esterase inhibitor concentrate while breastfeeding?
There is limited information on whether C1 esterase inhibitor concentrate passes into breast milk. However, since this medication is a naturally occurring protein in the body, it is generally considered safe for breastfeeding. Even so, it is always best to consult your healthcare provider before starting or continuing this medication while nursing. Our telemedicine providers are available to help you make an informed decision based on your specific circumstances and health needs.
Estimated Cost of C1 Esterase Inhibitor Concentrate
The cost of C1 esterase inhibitor concentrate can vary depending on your location and the pharmacy you use. Without insurance, a 30-day supply typically costs between $10,000 and $15,000. However, using a GoodRx coupon or similar discount programs may help reduce the expense. Be sure to check with your pharmacy for the most accurate pricing and information on available discounts.
Possible Alternatives to C1 Esterase Inhibitor Therapy
If C1 esterase inhibitor concentrate is not a suitable option for you, or if you are considering other treatments, there are alternatives available. It is important to consult your healthcare provider before making any changes to your treatment plan. You can also schedule a telemedicine consultation with one of our providers to discuss these options in detail.
Hereditary Angioedema Treatment Options
For hereditary angioedema, alternative treatments include medications such as icatibant (Firazyr) or ecallantide (Kalbitor). In addition to medication, lifestyle modifications—such as avoiding known triggers—can play a key role in managing symptoms effectively.
Acquired Angioedema Management
Treatment for acquired angioedema often involves the use of corticosteroids, antihistamines, or androgens. Avoiding medications like ACE inhibitors, which are known to trigger angioedema, is also an important part of management.
Angioedema Due to ACE Inhibitors
For angioedema caused by ACE inhibitors, discontinuing the medication is the first step. Switching to a different class of blood pressure medication, such as an angiotensin II receptor blocker (ARB), may help resolve symptoms and prevent recurrence.
Allergic Angioedema Treatment
Allergic angioedema is typically treated with antihistamines and corticosteroids. In severe cases, epinephrine may be required to quickly reduce swelling and manage the allergic reaction.
Idiopathic Angioedema Management
When the cause of angioedema is unknown, treatment generally focuses on symptom control. Antihistamines and corticosteroids are commonly used to reduce swelling and provide relief.
Post-Surgical Angioedema
Post-surgical angioedema is managed with corticosteroids and careful monitoring. In some cases, avoiding certain surgical procedures may help prevent future episodes.
Trauma-Induced Angioedema
For trauma-induced angioedema, addressing the underlying injury is essential. Medications such as corticosteroids or antihistamines can help reduce swelling and support the healing process.
Drug-Induced Angioedema
The primary approach to treating drug-induced angioedema is to discontinue the medication responsible and switch to an alternative. Antihistamines and corticosteroids may also be prescribed to manage symptoms effectively.
Angioedema Associated with Infections
When angioedema is linked to an infection, treating the underlying infection is the priority. In some cases, antihistamines or corticosteroids may be used to alleviate swelling and other symptoms.
Recent Updates on C1 Esterase Inhibitor Concentrate Research
Recent advancements in research are focused on improving the delivery methods and overall effectiveness of C1 esterase inhibitor concentrate. For instance, studies are investigating subcutaneous (under the skin) administration as a more convenient alternative to traditional intravenous (IV) methods. Additionally, ongoing clinical trials are assessing the long-term safety of C1 esterase inhibitor concentrate in diverse patient populations, including children and individuals with acquired angioedema. These developments aim to expand the accessibility of C1 inhibitor therapy and enhance patient outcomes.
Staying informed about the latest research is crucial. If you have questions about how these updates might affect your treatment, consider scheduling a telemedicine consultation with one of our providers today.
