Firazyr (Icatibant) - Subcutaneous

What Is Firazyr?

Firazyr (icatibant) is a prescription medication used for the acute treatment of hereditary angioedema (HAE) attacks (flare-ups) in adults.

As a bradykinin receptor antagonist, Firazyr blocks bradykinin B2 receptors (binding sites). By blocking bradykinin B2 receptors, a type of protein called bradykinin—that widens blood vessels—can’t attach to these binding sites to cause the typical HAE symptoms of swelling and pain.

Firazyr is available as an injectable to be given under the skin (subcutaneous).

What Is Firazyr Used For?

Firazyr is a prescription treatment option for HAE attacks. Although HAE is a serious genetic medical condition, it is rare—affecting 1 in 50,000 people in the United States. Many people with HAE will start experiencing symptoms during childhood.

Some common HAE symptoms include a non-itchy rash and swelling of the face, arms, legs, hands, feet, digestive system, and throat during a flare-up. If you experience swelling in the digestive system, additional symptoms may include severe stomach upset, nausea, and vomiting. As for swelling of the throat, you can have trouble breathing, which can be life-threatening. Without treatment, these flare-ups can occur every one to two weeks and last for three to four days with each attack.

There are three types of HAE:

• Type I: In type I HAE, the alteration in the SERPING1 gene causes a low amount of the C1 inhibitor protein. With less C1 inhibitor protein in the body, bradykinin amounts rise. 
• Type II: In type II HAE, there is also a change in the SERPING1 gene. Although there are normal amounts of C1 inhibitor protein in type II HAE, the C1 inhibitor protein is low-functioning. As a result, there are also high amounts of bradykinin in the body.
• Type III: In type III HAE, there is a variation in the F12 gene, leading to an abnormally active factor XII protein. This changed factor XII protein also encourages high amounts of bradykinin in the body.

In general, in all three types of HAE, a high amount of bradykinin is due to a modified gene made of deoxyribonucleic acid (DNA).

How to Use Firazyr

The following are general directions to use Firazyr. If you have any questions, please ask your healthcare provider or pharmacist. You will take your medication once you recognize the onset of an HAE attack.

1. Before using Firazyr, wash your hands with soap and water.
2. Take out the Firazyr syringe and the needle from the container or packaging. The medication inside the syringe should be clear and colorless.
3. Peel off the circular paper-like seal from the needle. Make sure that the needle is still protected inside the plastic-like cap.
4. Unscrew the protective cap from the hub of the Firazyr syringe.
5. Attach and screw the needle to the hub of the Firazyr syringe. Again, make sure that the needle remains protected inside the plastic-like cap.
6. Use an alcohol wipe to clean the injection site, which should be about 2 to 4 inches below the belly button on either side of your stomach. The site should also be at least 2 inches away from any scars. Avoid bruised, swollen, or painful areas.
7. Remove the protective plastic-like cap from the needle without pulling on the syringe plunger.
8. With one hand, gently pinch a fold of skin in the cleaned area of the injection site.
9. With the other hand, hold the syringe with the needle at a 45-degree to a 90-degree angle to the injection site.
10. Continue to pinch a fold of the skin while inserting the needle into the injection site and pushing the syringe's plunger over 30 seconds until there is no more medicine in the syringe.
11. Gently stop pinching the fold of your skin and remove the needle.
12. Throw away the syringe with the attached needle in one of the following containers: a red biohazard (sharps) container, a hard plastic container (e.g., a detergent bottle), or a metal container (e.g., an empty coffee can). Talk with your pharmacist for additional details on how to appropriately and legally dispose of your syringes and needles.
13. If your symptoms worsen, you can follow steps one through 12 after six hours from the first injection. A third dose can be used six hours after the second dose if necessary. Don’t use more than three injections within 24 hours.

If your HAE symptoms are different or Firazyr isn’t providing enough relief, get medical help right away. If your HAE symptoms include swelling of your throat or tongue, seek immediate medical attention—even after the first Firazyr dose.

Storage

Firazyr is a non-controlled prescription medication. As a result, your healthcare provider can authorize refills for up to one year from the originally written date on the prescription. However, your healthcare provider might give you fewer refills so they can regularly visit with you and monitor how you are doing on Firazyr.

After you receive Firazyr from the pharmacy, store the medication between 36 degrees to 77 degrees Fahrenheit (F). This is a wide range that includes standard refrigerator and room temperature conditions. However, don’t freeze this medication.

If you plan to travel with Firazyr, be prepared by knowing the regulations of your final destination. Keep the medication in its original container or packaging—with your name—from the pharmacy. Also, have a copy of your Firazyr prescription readily available.

Off-Label Uses

Angiotensin-converting enzyme inhibitors (ACE inhibitors) are used for several medical conditions, including high blood pressure. Although rare, a severe side effect of ACE inhibitors is angioedema.

Off-label use means a drug is prescribed for a medical condition that it is not approved by the Food and Drug Administration (FDA) to treat. Icatibant has been used off-label for ACE inhibitor-induced angioedema. However, effectiveness data is conflicting.

What Are the Side Effects of Firazyr?

This is not a complete list of side effects and others may occur. A healthcare provider can advise you on side effects. If you experience other effects, contact your healthcare provider. You may report side effects to the FDA at fda.gov/medwatch or 1-800-FDA-1088.

Common Side Effects

The most common side effects are the following injection site reactions—which are side effects in the area where you injected the medication:

• Bruising
• Burning or warmth
• Irritation
• Itching
• Numbness
• Pain
• Pressure
• Redness
• Swelling

Other common side effects include:

• Abnormal liver labs 
• Dizziness
• Fever
• Headache
• Nausea
• Rash

Severe Side Effects

Firazye does not typically cause severe side effects. However, you should get medical help immediately if any of your HAE symptoms involve swelling of the throat. Tell your healthcare provider if your symptoms bother you or don’t go away.

Long-Term Side Effects

Daily injections of Firazyr at high doses in rats and dogs were linked to wasting away of ovaries, uterus, and testicles.

In nonhuman studies, Firazyr also caused negative effects on the heart during or shortly after a heart attack, unstable angina (chest pain), or stroke. Since there is little safety data on Firazyr in humans for these situations, the manufacturer recommends weighing the benefits and risks of using this medication during or shortly after these medical events.

Dosage: How Much Firazyr Should I Take?

Modifications

Using Firazyr during pregnancy isn’t linked to negative effects on the fetus.

As for nursing, there is little safety data about Firazyr’s effects on nursing babies. Weigh the benefits and risks with your healthcare provider before using this medication while breastfeeding.

Missed Dose

Firazyr is typically used on an as-needed basis during an HAE attack. There are usually no scheduled doses to miss.

Once you notice an onset of an HAE attack, don’t delay using Firazyr. The medication is meant to prevent the worsening of an HAE attack. Early use of Firazyr is very important to control your symptoms.

Overdose: What Happens If I Use Too Much Firazyr?

The side effects at a high dose of 90 milligrams were similar to the standard one-time injection of 30 milligrams when given under the skin. However, another possible side effect might include low blood pressure.

Precautions

What Other Medications Interact With Firazyr?

Use caution when taking ACE inhibitors. Firazyr might lower the effectiveness of ACE inhibitors.  ACE inhibitors are used to lower blood pressure and include therapies such as Accupril (quinapril), Altace (ramipril), Zestril (lisinopril), and others.

What Medications Are Similar?

Firazyr is the only bradykinin B2 receptor antagonist. However, the following are other medication options for treating an HAE flare-up:

• Kalbitor (ecallantide): People with HAE have high amounts of kallikrein, which leads to high amounts of bradykinin in the body. As a kallikrein inhibitor, Kalbitor attaches to kallikrein and prevents it from making more bradykinin.
• Berinert (human C1 esterase inhibitor) and Ruconest (recombinant C1 esterase inhibitor): People with HAE have low amounts of C1 esterase inhibitor (C1NH). By using Berinert or Ruconest, the additional C1NH helps deactivate kallikrein and factor XIIa, which leads to lower amounts of bradykinin.

Although experts consider all four on-demand options effective and safe, there are some differences to keep in mind. Berinert and Ruconest are both given as an infusion through the vein. As for Kalbitor, like Firazyr, it is conveniently given under the skin. However, due to the potential of a severe allergic reaction, you will need to go to the healthcare provider’s office to receive Kalbitor. Firazyr is the most convenient option compared with the other three acute HAE treatment choices.

These medications are not typically used together. However, some people might benefit from more than one of these acute options in certain cases. If you have questions, talk with your healthcare provider.

How Can I Stay Healthy While Using Firazyr?

In addition to the physical symptoms, living with HAE can understandably take a toll on your emotions. A mental health professional can talk with you about some coping strategies to help change how you feel, think, or react to living with HAE.

It’s also important to have a strong support network with family, friends, and support groups. Consider taking the first step by sharing your medical condition with loved ones so that, if a flare-up occurs, your loved ones won’t feel as worried or afraid.

It’s also important to prepare for an HAE attack. Always have your Firazyr medication nearby to use if you sense an HAE flare-up immediately.

Staying in touch with your healthcare team will help address any questions or concerns that you may have.