How does FIRAZYR work for patients?

NARRATOR: Hereditary angioedema, or HAE, is a rare disease. It is estimated to affect between 1 in 10,000 and 1 in 50,000 people worldwide.

About 75% of HAE cases are passed down through families. This leaves approximately 25% of people with HAE who have no family history at all. These cases result from a genetic change that happens spontaneously. Once the spontaneous change occurs, it can be passed on to children. A substance called bradykinin is considered to be responsible for the characteristic HAE symptoms of swelling and pain.

FIRAZYR®, or icatibant injection, is a prescription medicine used to treat acute attacks of HAE in adults 18 years and older. It is not known if FIRAZYR is safe or effective for children under 18 years of age.

FIRAZYR is the only HAE treatment that blocks bradykinin from activating the receptor. FIRAZYR is also the first and only injection you give yourself just under the skin as soon as you recognize symptoms, after being trained by a healthcare professional.

Attacks of the throat, also called laryngeal attacks, can become life threatening. If you have an HAE attack of the throat, or laryngeal attack, inject FIRAZYR and then go to the nearest hospital emergency room right away.

For additional safety information about FIRAZYR, please see the Important Safety Information included in this video and full Prescribing Information available at www.firazyr.com or from a Shire representative.

The most common symptom of HAE is the occurrence of repeated attacks of swelling that can be extremely painful. The number of attacks that people with HAE have can vary. Some people may have an attack every week, while others may have only 1 or 2 attacks per year.

Attacks can occur in many different parts of the body, including the abdomen or trunk area, throat, and skin, on the face, or extremities.

Abdominal attacks of swelling can be painful and may cause other symptoms like nausea, vomiting, and diarrhea.

An attack can start in the face or mouth and then spread to the throat. Attacks of the throat can be life threatening. If you have an attack of the throat, you should seek immediate medical attention.

The duration of an attack can vary. When untreated, HAE attacks usually worsen over 24 hours and gradually get better during the next few days, but some attacks, such as those that involve swelling of the extremities, can last for several days.

Some HAE attacks can be triggered by stress, medical and dental procedures, hormonal changes, certain foods, and illness. Swelling may also result from other physical activities, including activities such as prolonged sitting or prolonged standing.

However, many attacks happen without any trigger at all. For this reason, it is very difficult to predict how severe an HAE attack will be, or when or where an attack will happen.

People with HAE either do not have enough of a protein called C1-esterase inhibitor (C1-INH) or this protein does not work as it should.

Because there is not enough C1-INH or it is not working the right way, the body makes too much of another protein called bradykinin. Having too much bradykinin allows fluids to leak out of the blood vessels and into areas of the body where they shouldn't.

Bradykinin is thought to be responsible for the characteristic HAE symptoms of swelling and pain.

Let's take a closer look at what happens inside the body during an HAE attack.

Under normal circumstances the cells that hold the blood vessels together are tightly linked. Think of it like interlocking the fingers of your hands. Blood and other fluids flowing through the blood vessels are unable to leak out into surrounding tissues.

During an HAE attack, there is an increase in bradykinin inside the blood vessels.

Picture bradykinin as a key that attaches to a lock. This "lock" is called a receptor.

An increase in bradykinin means there is more bradykinin available to attach to the receptors in the blood vessels, or more keys that fit into the locks.

The increase in bradykinin attaching to receptors causes the cells that hold the blood vessels together to loosen, similar to what happens when you start to pull apart your interlocked fingers.

When the cells loosen, plasma leaks out of the blood vessels and into surrounding tissues.

The leaking plasma causes the symptoms of swelling during an HAE attack.

FIRAZYR is the only HAE treatment that blocks bradykinin from binding to its receptor.

FIRAZYR has a structure that is similar to bradykinin. Imagine FIRAZYR as a key that is similar to bradykinin and can fit into the same lock.

Due to this similarity, FIRAZYR can attach to the same receptor as bradykinin.

During an HAE attack, FIRAZYR competes with bradykinin for attachment to the receptors in the blood vessel.

When this happens, bradykinin is blocked from attaching to the receptors.

When FIRAZYR attaches to the receptor, bradykinin is blocked from activating the receptor, and the overall effect it has on the walls of the blood vessel is reduced.

This causes the cells to tighten and slows the leakage of plasma into surrounding tissues.

The most common side effects of FIRAZYR include redness, bruising, swelling, warmth, burning, itching, irritation, hives, numbness, pressure, or pain at the injection site, fever, too much of an enzyme called transaminase in your blood, dizziness, nausea, headache, and rash.

The safety and efficacy of FIRAZYR were studied in 223 adults with HAE. FIRAZYR was evaluated in 3 controlled clinical trials, and a range of patient responses was observed for abdominal and skin attacks. Across the 3 clinical trials, patients taking FIRAZYR had a median time to 50% reduction in attack symptoms ranging from 2.0 to 2.3 hours. Sixty patients who were treated with FIRAZYR for attacks of the throat had similar symptom relief compared with patients who were treated with FIRAZYR for abdominal and skin attacks. Attacks of the throat, also called laryngeal attacks, can become life-threatening. If you have an HAE attack of the throat, or laryngeal attack, inject FIRAZYR and then go to the nearest hospital emergency room right away.

These clinical trials showed that FIRAZYR can be used to treat acute HAE attacks that occur in different parts of the body, including the skin; abdomen, or trunk area; and throat.

FIRAZYR is available by prescription only. FIRAZYR does not need to be reconstituted or mixed; it is supplied in a prefilled, single-use syringe. A healthcare provider will teach you or your caregiver how to inject FIRAZYR.

FIRAZYR is given by slow injection over at least 30 seconds, in a fold of skin about 2 to 4 inches below your belly button on either side, with each dose delivering 30 mg of FIRAZYR.

If your symptoms continue or come back, you may repeat your FIRAZYR injection at least 6 hours apart. Do not use more than 3 doses of FIRAZYR in a 24-hour period. Tiredness, drowsiness, and dizziness have been reported following the use of FIRAZYR. If this occurs, do not drive a car, use machinery, or do anything that needs you to be alert.

FIRAZYR® is a treatment you can carry with you and give yourself as soon as you recognize the symptoms of an acute HAE attack, after being trained by a healthcare professional.

When considering FIRAZYR as an acute treatment for your HAE attacks, talk to your doctor about the benefits and risks, including the following Important Safety Information for FIRAZYR:

Important Safety Information

Laryngeal attacks can become life threatening. If you have an HAE attack of the throat (laryngeal attack), inject FIRAZYR and then go to the nearest hospital emergency room right away

The most common side effects of FIRAZYR include:

  • redness, bruising, swelling, warmth, burning, itching, irritation, hives, numbness, pressure, or pain
  • at the injection site
  • fever
  • too much of an enzyme called transaminase in your blood
  • dizziness
  • nausea
  • headache
  • rash

The safety profile of FIRAZYR in patients who gave themselves the injection was similar to that in patients who had the injection given by a healthcare professional

Tell your healthcare provider if you have any other medical conditions, if you are breastfeeding or plan to breastfeed, or if you are pregnant or planning to become pregnant. FIRAZYR has not been evaluated in pregnant or nursing women. You and your healthcare provider will decide if FIRAZYR is right for you

If your symptoms continue or come back, you may repeat your FIRAZYR injection at least 6 hours apart

Do not use more than 3 doses of FIRAZYR in a 24-hour period

Tiredness, drowsiness, and dizziness have been reported following the use of FIRAZYR. If this occurs, do not drive a car, use machinery, or do anything that needs you to be alert

Tell your healthcare provider if you have any side effect that bothers you or that does not go away. For more information about FIRAZYR, ask your healthcare provider. For further information, please see the full Prescribing Information, including Patient Information. You can also call Shire at 1-800-828-2088.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088.

Please see the full Prescribing Information available at www.firazyr.com or from a Shire representative.