Prevention of severe infections is vital for patients with chronic granulomatous disease (CGD)
Severe infections can be difficult to manage and may require lengthy hospitalizations.
CGD treatment guidelines recommend combination immunomodulatory and prophylaxis therapy*
For the chronic medical management of CGD, immunomodulatory therapy in combination with prophylactic antimicrobials is recommended by the1,2:
For the treatment of active infections, using appropriate antimicrobials based on pathogen likelihood or identification is recommended.3
Recommended chronic
treatment paradigm4,5:
*This does not include all options for managing CGD. Adapted from Gallin JI, et al (2003).4
Treatment of acute infections
Patients with CGD can have life-threatening infections and need to be monitored for signs of infection. Early diagnosis and treatment are critical. Infections should be cultured, then patients can be treated empirically for gram-negative, gram-positive, and fungal infections until the pathogen is determined. Intravenous antimicrobial therapy is usually required and is often followed by prolonged oral therapy.
Hematopoietic stem cell transplant
Because of the risk of life-threatening infections, a hematopoietic stem cell transplant (HSCT) may be considered. HSCT provides the recipient with the donor’s immune system, which has neutrophils with functional nicotinamide adenine dinucleotide phosphate (NADPH) and superoxide anion production. It is a potentially curative treatment for patients with CGD but is a procedure with potentially significant risk associated with morbidity and mortality, usually related to graft-versus-host disease. The risks and benefits of an HSCT in the appropriate patient should be carefully considered. The indications for an HSCT in patients with CGD are still being debated due to the difficulty of weighing the risks and benefits.6
CGD lifestyle modifications
Infections caused by bacteria and fungi can occur from many natural sources. Infectious bacteria and fungi may live in places like gardens (in mulch) or playgrounds (in wood chips), and in lakes and ponds. People with CGD can still enjoy outdoor activities, but they should be cautioned to stay away from these places.
Here are some other tips to help your patients avoid infections:
- Don’t rake leaves or be around someone raking leaves
- Stay out of barns, caves, and other musty places
- Don’t go on hay rides
- Wash all cuts and scrapes with soap and water
- Make sure to talk to your doctor about any cut or fever
- Don’t smoke or inhale smoke
- Vaccinate all pets
Access educational materials for you and your patients
Learn more about a treatment option for CGD
References:
1. Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136(5):1186-1205. 2. Leiding JW, Malech HL, Holland SM. Clinical Focus on Primary Immunodeficiencies: Chronic Granulomatous Disease. Immune Deficiency Foundation; 2013. 3. Leiding JW, Holland SM. Chronic granulomatous disease. In: Pagon RA, Adam MP, Ardinger HH, et al, eds. GeneReviews®. Seattle, WA: University of Washington, Seattle; 1993-2018. 4. Gallin JI, Alling DW, Malech HL, et al. Itraconazole to prevent fungal infections in chronic granulomatous disease. N Engl J Med. 2003;348(24):2416-2422. 5. Holland SM. Chronic granulomatous disease. Hematol Oncol Clin North Am. 2013;27(1):89-99. 6. Connelly JA, Marsh R, Parikh S, Talano JA. Allogeneic hematopoietic cell transplantation for chronic granulomatous disease: controversies and state of the art. J Pediatric Infect Dis Soc. 2018;7(suppl 1):S31-S39.