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To prevent bacterial infections, many patients with Chronic Granulomatous Disease (CGD) receive a daily regimen of antibiotic medication, such as cotrimoxazole (brand names Bactrim or Septra). Cotrimoxazole is active against most bacteria that may infect patients with CGD, but this medication does not suppress the helpful bacteria that normally live in the bowel. That is what makes cotrimoxazole a good choice for long term CGD prophylaxis.
A study in the Journal of Infectious Disease found that when patients were given Bactrim at a dose of 5mg/kg/day of trimethoprim divided twice daily, the frequency of major infections from one episode every year were reduced to one every 3.5 years.3 Occasionally patients are allergic or otherwise intolerant to cotrimoxazole, and in that case there are other antibiotics that may be used as an alternative, such as cephalexin.
Since the infections that are important in CGD are in the environment and not carried in our bodies normally, the effect of chronic antibiotics is to theoretically build a wall around the patient. Some antibiotics can also be given by injection if cultures indicate a need for such.
Patients need to be aware of side effects from antibiotics and discuss them with their physician
To reduce the frequency of fungal infections, many patients with CGD take itraconazole (Sporanox), or other antifungal medications.4 Daily doses of the oral antifungal drug itraconazole can reduce fungal infections in CGD.
Maximum infection prophylaxis for CGD involves treatment with twice-daily oral doses of cotrimoxazole and twice daily itraconazole, voriconazole or posaconazole, plus three times weekly injections of gamma interferon. With these prophylactic treatments, the average incidence of severe infections in CGD is less than once every four years. Of course, individual factors will influence this frequency as well.
Patients need to be aware of antifungal side effects and discuss them with their physician. Some side effects could include photosensitivity from Voriconazole and sometimes elevated liver enzymes from any of the imidazoles. Liver function tests should be monitored.
To reduce the frequency and severity of serious infections associated with CGD, many patients receive interferon gamma (ACTIMMUNE), a biologically manufactured protein that is similar to a protein your body makes naturally. The exact way this drug works is not fully understood, but it is thought to work by affecting the function of many different types of cells including cells in the immune system and those that help form your bones.
Although not a cure for CGD, interferon gamma can reduce the frequency and severity of serious infections associated with CGD. Typically, it is taken by under the skin injections three times a week. The most common side effects are “flu-like” that may decrease in severity as treatment continues. If side effects develop, they may be minimized by bedtime administration, and acetaminophen taken before the injection may help.
In a large international multicenter randomized placebo-controlled trial, interferon gamma was effective at reducing the number of serious infections (those requiring hospitalizations) by 70% regardless of inheritance pattern, sex or use of chronic antibiotics. 5
Recommended Prophylaxis for Patients with CGD
Alternatives to itraconazole include voriconazole or posaconazole
Children with CGD should receive routine vaccinations as recommended by the American Academy of Pediatrics including live virus vaccines. Patients with CGD do not have any defect in immunity to viruses, so they are able to receive live virus vaccines without adverse effect. Many countries in Europe and Asia vaccinate children with the tuberculosis live bacterial vaccine, BCG, but this is not recommended practice in the U.S. Children with CGD should never receive the BCG live bacterial vaccine as it can result in a severe life-threatening systemic BCG infection. Salmonella vaccine also to be avoided.
3 Margolis DM, Melnick DA, Alling DW, Gallin JI. Trimethoprim-sulfamethoxazole prophylaxis in the management of chronic granulomatous disease. The Journal of infectious diseases 1990;162:723-6.
4 Gallin JI, Alling DW, Malech HL, et al. Itraconazole to prevent fungal infections in chronic granulomatous disease. N Engl J Med 2003;348:2416-22.
5 A controlled trial of interferon gamma to prevent infection in chronic granulomatous disease. The International Chronic Granulomatous Disease Cooperative Study Group. N Engl J Med 1991;324:509-16.
Source: Clinical Focus on Primary Immunodeficiencies: Chronic Granulomatous Disease, Issue 15, June 2013.