Options For managing Chronic Granulomatous Disease (CGD)

The risk of infection for people with CGD is high. It makes it hard for their bodies to fight off infections. These infections can be severe and can come back often. Patients may even have to be hospitalized many times to treat these infections. The most important thing for someone with CGD to do is to prevent infection.1

Proper care calls for1,2:

  • Year-round prophylaxis with doctor-prescribed medicines to try to prevent infections
  • Careful lifestyle choices to avoid harmful activities and environments
  • Regular checkups with a healthcare provider to see when early treatment is necessary

Understanding your options for managing CGD

options for managing CGD

Healthcare providers can use a combination of 3 types of medicines, sometimes referred to as Triple Prophylaxis Therapy, or a 3-part preventive therapy, to protect against serious infections. This includes ACTIMMUNE® (Interferon gamma-1b), daily low-dose antibiotics, and antifungals. The goal is to try to keep patients as healthy and infection free as possible.3,4

What is Triple Prophylaxis Therapy?

ACTIMMUNE® is often recommended for patients with Chronic Granulomatous Disease (CGD), along with daily oral antibiotics and oral antifungal medicines. For many healthcare providers, this Triple Prophylaxis Therapy, which is a combination of 3 preventive medicines, is becoming a standard of care for treating patients with CGD.3,4 As with all medicines, it is important that you follow your healthcare provider’s instructions closely and take all medicines as directed.

Triple Prophylaxis Therapy has two goals. The first is improving immune function with ACTIMMUNE®. The second is preventing further infections with the use of antifungals and antibiotics.5,6 Here is a summary of the different medicines involved and how they work to help prevent and treat infections.

The 3 Parts of Triple Prophylaxis Therapy

1. ACTIMMUNE® (Interferon gamma-1b) is not a cure for CGD, but it can reduce the number and severity of serious infections associated with CGD. It is taken by injection under the skin, 3 times a week.6

Learn more about ACTIMMUNE® for CGD

The most common side effects of ACTIMMUNE® are “flu-like symptoms” that may decrease as treatment continues. Side effects may not be as bad if ACTIMMUNE® is taken at bedtime. Taking acetaminophen before the injection may also help.6

Learn more about side effects of ACTIMMUNE®

2. Antibiotics help to prevent bacterial infections, which can occur anywhere in the body but commonly in the lungs, bones, and blood.4,7

Of course, it is very important to talk about side effects of antibiotics with your healthcare provider. Side effects for antibiotics can include: diarrhea that is watery or bloody; flu-like symptoms such as fever, chills, swollen glands, body aches, sores in your mouth and throat coughing, light-headedness, easy bruising, and severe tingling; numbness; pale skin; or feeling light-headed.4,7

3. Antifungals, the third part of Triple Prophylaxis Therapy, help prevent fungal infections such as fungal pneumonia, an infection that causes fluid to build up in the lungs.4,7

As with the other parts of therapy, it is very important to talk with your healthcare provider about side effects of antifungal medicines. Side effects of antifungal medicine could include sensitivity to sunlight and, sometimes, high levels of liver enzymes. If you are on antifungals, you should also talk to your healthcare provider about taking tests to see how well your liver is working.4,7

Stem Cell Transplantation (HSCT)

For some patients, HSCT (also referred to as Bone Marrow Transplant or BMT) may be considered. BMT provides a possible cure for CGD, but there are risks, including rejection of the transplanted cells. Ask your healthcare provider for more information.4

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. After being diagnosed. Chronic Granulomatous Disease Association Web site. http://www.cgdassociation.org/afterdiagnosis.php. Accessed June 5, 2013. 3. 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. 4. Holland SM. Chronic granulomatous disease. Clin Rev Allergy Immunol. 2010;38(1):3-10. 5. 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. 6. ACTIMMUNE® (Interferon gamma-1b) Full Prescribing Information. Roswell, GA: Vidara Therapeutics Inc; 2013. 7. The Immune Deficiency Foundation. "Medications." Living with CGD (Chronic Granulomatous Disease): The Immune Deficiency Foundation Resource for the CGD Community. http://www.livingwithcgd.org/about-cgd/treatment/medications/. Accessed April 29, 2015.
living with 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.