Treatment guidelines for CGD
Prevention of serious infections is vital for patients with CGD. Serious infections can be difficult to manage and may require lengthy hospitalizations.
CGD treatment guidelines recommend combination immunomodulatory and prophylaxis therapy1,2
For the treatment of active infections, using appropriate antimicrobials based on suspected pathogens or identification is recommended.3
Information on the management of CGD
For more information on the medical management of CGD, please see the recommendations provided by these organizations:
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 associated with potential morbidity and mortality, usually related to graft-versus-host disease. As with any invasive procedure, the risks and benefits of HSCT in the appropriate patient should be carefully considered.4
CGD lifestyle modifications
Infections caused by bacteria and fungi can occur from many 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 certain places and environments.
Share these recommendations with your patients to help them avoid infections:
- Refrain from engaging in activities involving leaf raking or being close to such activities
- Avoid entering barns, caves, basements, and other locations characterized by damp, musty conditions
- Don’t participate in hay rides to minimize exposure to potential allergens and microorganisms
- Ensure all abrasions and lacerations are promptly and thoroughly cleansed with soap and water to prevent infection
- Consult with a medical professional regarding any wounds or the onset of fever to ensure appropriate care and intervention
- Abstain from smoking and avoid exposure to smoke to maintain lung health
Connect your patients with an expert
Learn more about treatment options for CGD
References:
1. 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. 2. Holland SM. Chronic granulomatous disease. Hematol Oncol Clin North Am. 2013;27(1):89-99. 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; 1993-2022. 4. 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.