The human immune system is made up of a complex network of highly specialized cells and organs that work in concert to protect the body from disease. When any part of the network is faulty, the immune system is compromised and the body’s susceptibility to disease is increased.
Chronic granulomatous disease comprises a group of inherited disorders of the immune system caused by defects in immune cells called phagocytes. The primary function of phagocytes is to recognize and destroy microbial pathogens. In patients with CGD, phagocytes fail to generate sufficient levels of the enzymes responsible for killing ingested microorganisms. This defect leaves patients vulnerable to severe recurrent bacterial and fungal infections and chronic inflammatory conditions like gingivitis, enlarged lymph glands, and tumor-like masses called granulomas. While not malignant, granulomas can cause serious problems by reducing airflow in the lungs, obstructing the passage of food through the gastrointestinal system and preventing the flow of urine through the kidneys and bladder.1 It is because granulomas are a major recurring feature of this condition that it is called chronic granulomatous disease.
How ACTIMMUNE® works to prevent infection
The active ingredient in ACTIMMUNE® is interferon gamma-1b, a bioengineered form of human interferon gamma. Interferon gamma is a protein that occurs naturally in the human body; its primary function is to stimulate the immune system to respond to injury or infection.
Interferon gamma-1b has many properties thought to be helpful to patients with CGD. While the precise mechanism(s) through which it reduces the risk of infection in CGD is uncertain, recent studies suggest that interferon gamma-1b enhances the microbial killing activity of phagocytes.2
Starting ACTIMMUNE® Treatment
Unlike antibiotic or antifungal medications which are used to treat infections once they occur, ACTIMMUNE® is meant to prevent infections by enhancing the body’s ability to recognize and remove potential pathogens.3 Therefore, ACTIMMUNE® should be taken regularly according to the recommended dosing instructions.
ACTIMMUNE® is administered as a subcutaneous injection three times per week (for example, Monday, Wednesday, and Friday). A small thin needle is used to inject the medication into the fatty layer of tissue just beneath the skin. Patients and/or their caregivers can be trained to prepare and administer the injections at home.
Managing Side Effects
The most common side effects of ACTIMMUNE® therapy are fever, headache, rash, and chills.2 The majority of reactions are mild and usually become less noticeable over time. Additionally, the following strategies may be helpful in minimizing common side effects:
- Follow instructions for the proper handling of vials, needles, and syringes
- Take ACTIMMUNE® at bedtime
- Take acetaminophen or ibuprofen half an hour before injections
- Use a different injection site each time to minimize skin irritation
- Call your healthcare provider if you experience any side effects not described here
More Information
- Click here to learn more about ACTIMMUNE® efficacy
- Click here to learn more about ACTIMMUNE® safety in CGD treatment
The most common adverse events observed in patients with CGD were flu-like symptoms (e.g., headache, fatigue, fever, myalgia, and rigors). Similar adverse events were observed in patients with severe malignant osteopetrosis. Please refer to the ACTIMMUNE® Safety section on this Web site for more complete safety information
The health information contained herein is provided for educational purposes only and is not intended to replace discussions with a healthcare provider. All decisions regarding patient care must be made with a healthcare provider, considering the unique characteristics of patients.
For U.S. resident use only. The product discussed herein may have different product labeling in different countries. Please see the full U.S. Prescribing Information (pdf file).
References
1. Forrest CB, Forehand JR, Axtell RA, Roberts RL, Johnston RB. Clinical features and current management of chronic granulomatous disease. Hematology/Oncology Clinics of North America. 1988;2:253-266.
2. The International Chronic Granulomatous Disease Cooperative Study Group. A controlled trial of interferon-γ to prevent infection in chronic granulomatous disease. N Engl J Med. 1991;324:509–516.
3. Schroder K. Interferon-gamma: an overview of signals, mechanisms and functions. J Leukoc Biol. 2004 Feb;75(2):163-89.
