INTRODUCTION TO CHRONIC GRANULOMATOUS DISEASE (CGD)

With Chronic Granulomatous Disease (CGD), your body has a hard time fighting off infections. Finding out that you or someone you love has CGD is a hard thing to deal with. That’s why we’re here to give you the information you and your family need. Of course, you can always talk to your healthcare provider for information on CGD. Look through this site to find helpful information about:

  • CGD—what it is and how it is inherited
  • Treatment options for CGD, including
    Triple Prophylaxis Therapy
  • How to protect against infections while living your life
  • More help for you:
    • Groups and organizations to help you and your loved ones

What is Chronic Granulomatous Disease (CGD)?

CGD is a disease of the immune system. It is also known as a primary immunodeficiency disease, or PI. Primary means it is not caused by some other disease or disorder.1 CGD is a type of PI that causes children and adults to have infections that come back frequently and are unusually hard to cure. CGD is a rare PI that is usually discovered in childhood. It weakens the immune system and increases the chances of getting a serious or unusual infection, or an infection that keeps coming back.1-3

What happens in the body?

In people who have CGD, a type of white blood cell called a phagocyte is defective. These defective phagocytes cannot generate superoxide. This means that in people with CGD, their bodies can’t kill harmful microorganisms like bacteria and fungi. This weakens the immune system. In trying to fight off an infection, the body may form extra tissue in the form of a lump, called a “granuloma.” These granulomas can cause serious harm. They can block off the intestines, for example.2

  • While CGD causes infections, it can also cause inflammation or swelling. This can lead to problems all over the body2

Click to see parts of the body that can be
affected by CGD:

lung graphic lung lymph nodes liver bone skin stomach colon urinary tract

CGD was first identified in the 1950s. Since then, we have learned much about CGD. In the past, before current treatments became available, the disease was often fatal.3

Today, CGD is known to be a condition that patients can live with and manage. Studies suggest overall survival has improved over the last decade, with more patients living well into adulthood. They usually have to take medicine to help fight off infections, and they have to be careful not to do things that add to the risk of getting infections. About 1 out of 200,000 babies in the US is born with CGD.4,5

References: 1. Leiding JW, Holland SM. Chronic granulomatous disease. In: Pagon RA, Bird TD, Dolan CR, et al, eds. GeneReviews. Seattle, WA: University of Washington; 2012. 2. Song E, Jaishankar GB, Saleh H, et al. Chronic granulomatous disease: a review of the infectious and inflammatory complications. Clin Mol Allergy. 2011;9(1):10. doi:10.1186/1476-7961-9-10. 3. Mclean-Tooke ACP, Aldridge C, Gilmour K, Higgins B, Hudson M, Spickett GP. An unusual cause of granulomatous disease. BMC Clinical Pathology. 2007;7(1).doi:10.1186/1472-6890-7-1. 4. Holland SM. Chronic granulomatous disease. Clin Rev Allergy Immunol. 2010;38(1):3-10. 5. Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Asthma Immunol. 2005;94(5 suppl 1):S1-63.
who gets CGD

IMPORTANT SAFETY INFORMATION AND INDICATIONS

Important Information About ACTIMMUNE

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

ACTIMMUNE® (Interferon gamma 1-b) 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. You may also contact the Horizon Pharma Medical Information Department toll-free at 1-866-479-6742 or medicalinformation
@horizonpharma.com
.

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.