SIGNS AND SYMPTOMS

A healthcare provider may suspect Chronic Granulomatous Disease (CGD) if they see infections that are unusual or in an unusual place for a healthy child. A person may begin to show signs of CGD at any time, but most of the time patients are diagnosed before 5 years of age.1

Healthcare providers may suspect CGD if a child’s infection fits this description2,3:

  1. Unusual infection
  2. Unusual place
  3. Unusual age

If you think your child may have CGD, you should talk to your pediatrician.

The signs and symptoms of CGD may include1:

  • Slow growth in childhood
  • Infections from specific types of bacteria or fungi that affect the lungs, lymph nodes, liver, bones, or skin. These are often serious and come on suddenly, and keep coming back
  • A collection of tiny lumps, called “granulomas,” mostly in the bladder and intestines
    • May also cause wounds to heal abnormally, with lumps in the tissue
  • Colitis (inflammation of the colon)

Talk to your healthcare provider for more information about bacterial infections.

This picture shows where patients with CGD often get infections—and the type of infections patients have to watch out for1:

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

Testing for Chronic Granulomatous Disease (CGD)

 dhr test

In most cases, healthcare providers will use a DHR test to find out if a person has CGD. This is a blood test, and it is very accurate. White blood cells (phagocytes) are exposed to the chemical dihydrorhodamine (DHR). The cells are measured using a process called flow cytometry. Normal white blood cells will give off a fluorescent light, which means the cells produce the chemical needed to kill harmful microorganisms. If the cells do not give off a fluorescent light, it means that the cells do not have any, or enough, of the chemical needed to kill the microorganisms. This would mean that the patient may have CGD.1

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. 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. 3. Holland SM. Chronic granulomatous disease. Clin Rev Allergy Immunol. 2010;38(1):3-10.
managing 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.