Narrator VO:

What is chronic granulomatous disease?

Patient VO:

Hi my name is Muris.

I live in Illinois with my brother, dad and mom.

In my free time, I like to play outside, play football and basketball with my friends.

I was diagnosed with CGD when I was 2 months old.

When I was old enough to understand what CGD was I felt nervous and scared.

I had a lot of questions too, like how long would I be on the medication, and what should I stay away from…

...but over time I learned a lot about CGD and how to manage my condition.

You may also have questions like I did…

...so let’s take a closer look to see what happens inside the body when you have CGD.

Narrator VO:

Chronic Granulomatous Disease, or CGD, is a rare, genetic disease that affects the immune system.

The immune system is a network of cells, tissues, and organs that help the body fight infection.

Blood cells called white blood cells are an important part of the immune system that help attack foreign Invaders…

...like germs. Normally, when germs enter the body…

...the immune system activates white blood cells to rush to the site of infection and attack the germs.

There are several different types of white blood cells.

One type is called a phagocyte. Phagocytes work by ingesting the germs and trapping them inside.

The phagocyte then uses a special killing mechanism called an “oxidative burst” to destroy the germs.

A healthy immune system usually prevents infections from becoming serious.

But people with CGD are at a greater risk of serious and potentially life-threatening bacterial and fungal infections because their immune system can’t fight them off as well.

In people with CGD, the phagocytes can still trap the germs…

...but their killing mechanism doesn’t work properly, so they can’t completely destroy bacteria and fungi.

The immune system sends more... ...and more white blood cells to fight the infection. But the infection doesn’t clear, and can become serious and life threatening.

The white blood cells just pile up forming a hard lump called a granuloma.

Granulomas can affect many parts of the body including the skin, urinary tract, stomach, and lymph nodes, resulting in complications like stomach pain, diarrhea, and weight loss.

Persistent, recurring infections are often serious and even life threatening...

...and can lead to pneumonia, liver and skin abscesses, inflammation of the colon - or colitis - and bone infections - or osteomyelitis.

Patient VO:

It’s important to remember that CGD is a serious condition and should be managed by working with your doctor. Now watch the next video to learn about available treatment options.

Narrator VO: For more information, speak with your doctor and visit ACTIMMUNE.com.

IMPORTANT SAFETY INFORMATION

What is ACTIMMUNE® (Interferon gamma 1-b) used for?

ACTIMMUNE is part of a drug regimen used to treat Chronic Granulomatous Disease, or CGD. CGD is a genetic disorder, usually diagnosed in childhood, that affects some cells of the immune system and the body’s ability to fight infections effectively. CGD is often treated (though not cured) with antibiotics, antifungals, and ACTIMMUNE.

ACTIMMUNE is also used to slow the worsening of severe, malignant osteopetrosis (SMO). SMO is a genetic disorder that affects normal bone formation and is usually diagnosed in the first few months after birth.

When should I not take ACTIMMUNE?

Don’t use ACTIMMUNE if you are allergic to interferon-gamma, E coli-derived products, or any ingredients contained in the product.

What warnings should I know about ACTIMMUNE?

At high doses, ACTIMMUNE can cause (flu-like) symptoms, which may worsen some pre-existing heart conditions.

ACTIMMUNE may cause decreased mental status, walking disturbances, and dizziness, particularly at very high doses. These symptoms are usually reversible within a few days upon dose reduction or discontinuation of therapy.

Bone marrow function may be suppressed with ACTIMMUNE, and decreased production of cells important to the body may occur. This effect, which can be severe, is usually reversible when the drug is discontinued or the dose is reduced.

Taking ACTIMMUNE may cause reversible changes to your liver function, particularly in patients less than 1 year old. Your doctor should monitor your liver function every 3 months, and monthly in children under 1 year.

In rare cases, ACTIMMUNE can cause severe allergic reactions and/or rash. If you experience a serious reaction to ACTIMMUNE, discontinue it immediately and contact your doctor or seek medical help.

What should I tell my healthcare provider?

Be sure to tell your doctor about all the medications you are taking.

Tell your doctor if you:

• are pregnant or plan to become pregnant or plan to nurse
• have a cardiac condition such as irregular heartbeat, heart failure, or decreased blood flow to your heart
• have a history of seizures or other neurologic disorders
• have, or have had, reduced bone marrow function. Your doctor will monitor these cells with blood tests at the beginning of therapy and at 3-month intervals on ACTIMMUNE therapy

What are the side effects of ACTIMMUNE?

The most common side effects with ACTIMMUNE are “flu-like” symptoms such as fever, headache, chills, muscle pain, or fatigue, which may decrease in severity as treatment continues. Bedtime administration of ACTIMMUNE may help reduce some of these symptoms. Acetaminophen may be helpful in preventing fever and headache.

What other medications might interact with ACTIMMUNE?

Some drugs may interact with ACTIMMUNE to potentially increase the risk of damage to your heart or nervous system, such as certain chemotherapy drugs. Tell your doctor about all other medications you are taking.

Avoid taking ACTIMMUNE at the same time as a vaccination.

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.

The risk information provided here is not comprehensive. To learn more, talk about ACTIMMUNE with your healthcare provider or pharmacist. The FDA-approved product labeling can be found at http://www.ACTIMMUNE.com or 1-866-479-6742.

Live action: In the last episode we learned about what CGD is and what happens inside the body when you have the condition. Along with staying healthy and being cautious about your environment treatments like Actimmune are available to help manage CGD

Narrator VO: Treatments are available to help manage chronic granulomatous disease, including antifungals, antibiotics, and ACTIMMUNE® (Interferon gamma-1b). Let’s talk about how it works.

ACTIMMUNE is the only FDA-approved biologic therapy that modifies the immune system for people with CGD.

ACTIMMUNE helps activate and strengthen other parts of the immune system to fight infection.

Antibiotics help prevent and fight infection caused by bacteria.

Antifungal medicines can help prevent and fight infection caused by fungi.

So how does ACTIMMUNE work?

ACTIMMUNE contains a protein called interferon gamma, which is naturally made by the body to fight infections.

Interferon-gamma activates several parts of the immune system.

The first thing it does...is it super charges the white blood cells ...to break down germs.

They also activate the other immune cells, helping them to attack and destroy the infection.

The effects of interferon-gamma also make the germ or infected cell easier for the immune system to find and attack.

ACTIMMUNE is given with a small needle as an injection... into the fatty layer right under the skin three times a week. ACTIMMUNE is given as an injection instead of a pill because stomach acid would break down the medicine before it could get to where it needs to be.

Remember, ACTIMMUNE can lead to fewer serious infections, which means less time in the hospital and more time to do the things you enjoy.

Live Action: It’s important that I manage my CGD so I created a schedule that works for me. For my treatment, I take my pills twice a day every day and I take my Actimmune 3 times a week. I set a phone reminder so that I don’t forget. I also know that if I ever have any questions I have a support team, like my doctor and my family to help me. And remember, stay strong and take it one day at a time.

Narrator VO: For more information, speak with your doctor and visit ACTIMMUNE.com.

IMPORTANT SAFETY INFORMATION

What is ACTIMMUNE® (Interferon gamma 1-b) used for?

ACTIMMUNE is part of a drug regimen used to treat Chronic Granulomatous Disease, or CGD. CGD is a genetic disorder, usually diagnosed in childhood, that affects some cells of the immune system and the body’s ability to fight infections effectively. CGD is often treated (though not cured) with antibiotics, antifungals, and ACTIMMUNE.

ACTIMMUNE is also used to slow the worsening of severe, malignant osteopetrosis (SMO). SMO is a genetic disorder that affects normal bone formation and is usually diagnosed in the first few months after birth.

When should I not take ACTIMMUNE?

Don’t use ACTIMMUNE if you are allergic to interferon-gamma, E coli-derived products, or any ingredients contained in the product.

What warnings should I know about ACTIMMUNE?

At high doses, ACTIMMUNE can cause (flu-like) symptoms, which may worsen some pre-existing heart conditions.

ACTIMMUNE may cause decreased mental status, walking disturbances, and dizziness, particularly at very high doses. These symptoms are usually reversible within a few days upon dose reduction or discontinuation of therapy.

Bone marrow function may be suppressed with ACTIMMUNE, and decreased production of cells important to the body may occur. This effect, which can be severe, is usually reversible when the drug is discontinued or the dose is reduced.

Taking ACTIMMUNE may cause reversible changes to your liver function, particularly in patients less than 1 year old. Your doctor should monitor your liver function every 3 months, and monthly in children under 1 year.

In rare cases, ACTIMMUNE can cause severe allergic reactions and/or rash. If you experience a serious reaction to ACTIMMUNE, discontinue it immediately and contact your doctor or seek medical help.

What should I tell my healthcare provider?

Be sure to tell your doctor about all the medications you are taking.

Tell your doctor if you:

• are pregnant or plan to become pregnant or plan to nurse
• have a cardiac condition such as irregular heartbeat, heart failure, or decreased blood flow to your heart
• have a history of seizures or other neurologic disorders
• have, or have had, reduced bone marrow function. Your doctor will monitor these cells with blood tests at the beginning of therapy and at 3-month intervals on ACTIMMUNE therapy

What are the side effects of ACTIMMUNE?

The most common side effects with ACTIMMUNE are “flu-like” symptoms such as fever, headache, chills, muscle pain, or fatigue, which may decrease in severity as treatment continues. Bedtime administration of ACTIMMUNE may help reduce some of these symptoms. Acetaminophen may be helpful in preventing fever and headache.

What other medications might interact with ACTIMMUNE?

Some drugs may interact with ACTIMMUNE to potentially increase the risk of damage to your heart or nervous system, such as certain chemotherapy drugs. Tell your doctor about all other medications you are taking.

Avoid taking ACTIMMUNE at the same time as a vaccination.

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.

The risk information provided here is not comprehensive. To learn more, talk about ACTIMMUNE with your healthcare provider or pharmacist. The FDA-approved product labeling can be found at http://www.ACTIMMUNE.com or 1-866-479-6742.