Firazyr (Icatibant)

Firazyr (icatibant) is the first FDA-approved drug given subcutaneously to treat hereditary angioedema. It is a selective competitive B2 bradykinin receptor antagonist and an immunomodulator. Firazyr can be used alone or with other medications, depending on the patient’s condition and disease.

Before starting the medication, make sure to inform your doctor if:

• You are pregnant or planning to become pregnant.
• You are breastfeeding.
• You have any allergies to medication or food.
• You are taking any other prescription drugs, herbal medicines, or vitamins.
• You have a disease history. For example, if you are suffering from any disease, such as diabetes, kidney disorders, hypertension, or any other chronic illness, let your doctor know.

What Is Firazyr?

Firazyr is an immunomodulator prescription medication also known by its generic name icatibant. It is indicated for the treatment of acute attacks of hereditary angioedema (HAE) in adults aged 18 and older. 

What Is Hereditary Angioedema (HAE)?

Hereditary angioedema is a disorder that causes recurrent episodes of severe swelling, known as angioedema. Rapid tissue swelling happens due to fluid accumulation outside of the blood vessels, which blocks the normal flow of bodily fluids such as blood or other lymphatic fluids. The common areas of the body where swelling tends to develop include the skin, face, airways, limbs, and gastrointestinal tract. 

HAE is a rare hereditary disorder characterized by unpredictable swelling and is estimated to affect anywhere between 1 in 10,000 and 1 in 150,000 individuals worldwide. The disease can be potentially life-threatening.

How Does Firazyr Work? 

Patients who have HAE have an absence or defectiveness of C1-esterase inhibitors in their system, which leads to the increased production of bradykinin. Bradykinin is a potent vasodilator that increases vascular permeability. This leads to the formation of edema.

Bradykinin binds to bradykinin B2 receptors located on the vascular endothelium, which stimulates the release of prostaglandins (PG), which in turn stimulates substance P release, initiating angioedema. Hence, bradykinin plays a leading role in causing attacks of angioedema.

Firazyr inhibits the binding of bradykinin to the bradykinin B2 receptors, thus inhibiting bradykinin activity and treating the symptoms of hereditary angioedema attacks.

How Is Firazyr Used? 

Firazyr is injected subcutaneously into the abdominal area, about 2 to 4 inches below the belly button, over a period of at least 30 seconds. Patients may self-administer the medication after receiving training from a healthcare professional.

Firazyr may be taken with or without food. As the drug is administered subcutaneously, food and drinks do not affect the action of this medicine.

It is not known if Firazyr is present in breast milk. Talk to your physician if you are breastfeeding while taking Firazyr.

Information related to the use of icatibant in pregnancy is limited. Therefore, it is important to talk to your physician if you are pregnant or planning to get pregnant while taking Firazyr.

Firazyr Storage and Administration 

• Firazyr doesn’t need to be refrigerated. It should be stored in its original container at a temperature between 36°F to 77°F until the time of administration.
• The solution should be clear and colorless.
• Prior to administration, inspect Firazyr visually for any particulate matter or discoloration. If present, do not administer and discard the Firazyr injection.
• Before administration, disinfect the injection site and inject over at least 30 seconds by subcutaneous injection.
• A 25 gauge needle should be attached to the syringe hub and screwed on securely.
• If patients wish to self-administer the medication, they should first receive proper training under the guidance of a healthcare professional prior to administration.

How Long Does It Take for Firazyr To Work?

As Firazyr is administered subcutaneously (injected under the skin), it can relieve a lot of angioedema symptoms, like swelling and pain in the skin or stomach. Relief should start about 2 hours after injection, but it may take up to 8 hours for the patient to feel completely better. If the symptoms remain or recur, the Firazyr dose may be repeated every 6 hours (maximum of 3 doses per 24 hours). 

Side Effects of Firazyr 

Side effects vary from patient to patient. The common side effects of Firazyr include:

• 恶心
• 头晕
• 发烧
• 皮疹
• 头痛
• 疲劳
• Injection site reactions
• Increase in the liver enzyme ALT
• Antibody development
• Urticaria

These side effects are usually minor and go away on their own within a few days as the body gets used to the drug. But if they become severe or persistent, consult your doctor to switch the medication.

Do not drive, operate machinery, or perform activities that require alertness, as dizziness and drowsiness are reported from the use of Firazyr.

Serious side effects of Firazyr include:

• Difficulty breathing
• 麻疹
• Severe dizziness
• Swelling in the lips, face, throat, or tongue

These are severe side effects requiring immediate assistance from a medical professional as they may indicate an allergic reaction to Firazyr which can be serious.

Firazyr Dosage Form

Icatibant is available as a solution for subcutaneous use in one strength: 30 mg/3ml.

剂量 

• The usual dose of Firazyr is 30 mg, given subcutaneously in the abdominal area.
• If symptoms recur after the 1st dose or if there is an inadequate response, another dose can be given after an interval of at least 6 hours.
• Only three doses of Firazyr injection can be given in a 24-hour period.

禁忌症 

There are no contraindications for Firazyr.

Firazyr Cost

Firazyr is a prescription medication indicated for hereditary angioedema. The average cost of Firazyr is $11,307 for 3 ml of 10 mg/ml subcutaneous solution. However, the price may vary at different pharmacies. Copay and financial assistance may be available to some patients. 

Pros and Cons of Firazyr

You should not avoid or skip medication based on the pros and cons below. Firazyr is a prescription medicine and should be administered if the doctor has prescribed it for treating your disease. 

Pros:

• It comes in a pre-filled syringe (fewer steps for preparation).
• It doesn’t need to be refrigerated (ease of storage).
• It can be injected at home (time-saving and convenient).

Cons:

• The medication may cause swelling, bruising, and irritation at the injection site. In case these signs appear, stop the dose and consult your doctor and get medical help if needed.
• The injection can be painful.
• Proper training is required for self-administration.

结论

Firazyr is a prescription-based immunomodulator and a bradykinin receptor antagonist. It is used to treat acute attacks of hereditary angioedema in adults aged 18 and above. 

Hereditary angioedema is a rare disease that causes severe swelling in the face, skin, gastrointestinal tract, limbs, and airways because of increased activity of bradykinin in the body. Firazyr works to block bradykinin activity by inhibiting its binding to the B2 receptors.

Firazyr is administered subcutaneously in the abdominal area over 30 seconds. Not more than 3 doses of injection should be administered within 24 hours to the patient. In case of any severe side effects, or signs of an allergic reaction such as bruising, hives, or breathing difficulties, consult your doctor immediately and seek medical help.