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Presently, the mainstay of CGD therapy is prompt and aggressive treatment of infections with appropriate antibiotics. In addition, may patients are given oral antibiotics prophylacttically (as a preventive messure) to reduce the number and severity of infections.
A recently completed international study carried out at multiple medical centers indicated that interferon-gamma, a substance produced by the body's immune system, reduces the number and severity of infections in patients with CGD.
These studies were especially dramatic in children less than 10 years old. These are very exciting results, and, when interferon is given with prophylactic antibiotics, the outlook is particulary promising.
It is extremely important that P>
It is extremely important that physicians be contacted promptly for suspected problems so that treatments can be initiated early, thereby minimizing the extent and duration of required therapy. The more serious infections are treated with antibiotics given intravenously over a period of months, which may require a prolonged hospital stay. Such long-term therapy is necessary to prevent recurrences of the infections, which can happen if the drugs are stopped after a short period of time. Steroids and other anti-inflammatory drugs are sometimes given to shrink granulomas.
It is often necessary to take a tissue sample surgically (biopsy) from an infected area to determine the cause of an infection. Sometimes infected tissue must be removed or drained if the infection is not responding to antibiotic therapy. Drainage tubes placed in the chest, abdomen or other sites must sometimes remain in place for several weeks. Patients usually stay in the hospital during that time.
If antibiotics and surgery do not effectively combat serious infections, or if an infections is rapidly worsening, patients may be given transfusions of white blood cells. These tranfusions contain normal phagocytes that can curb the infection. Because white blood cell transfusions can prompt some serious side effects that worsen with each transfusion, this technique cannot be used routinely to treat infections.
Patients often experience a len experience a loss of appetite during treatment. When this happens, They may be fed intravenously to ensure proper nourishment. Sometimes intravenous fluids are administered through a tube (often called a Hickman catheter) surgically inserted in the subclavin or jugular vein in the neck or chest. With proper training on how to use the catheter and with help from family members, patients may be able to be fed at home at avoid a long hospital stay. The catheter can also be used at home to administer antibiotics intravenously and for obtaining blood samples for laboratory evaluation. Even when a patient is well, it is advisable to have frequent blood and urine tests, X-rays, and scans so that any abnormalities can be detected early.
Since early treatment of infections is important, patients should consult with their doctors even when experiencing minor symptoms. CGD patients should be on the lookout for the following signs of infections:
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U.S. DEPARTMENT OF HEALTH AND HUMAN SERVICES
Public Health Service
National Institute of Health
National Institute of Allergy and Infectious Diseases
Division of Intramural Research
Prepared by the NIAID
Office of Communications