Chronic granulomatous disease (CGD) is a disorder that damages the immune system. It makes your body susceptible to infections caused by particular fungi and bacteria. It causes granulomas, which are collections of immune cells that form at sites of inflammation or infection.
The only cause of CGD is inheriting it through genetics. It mostly affects men, but there are also forms of CGD that affect both sexes.
People with CGD typically have at least one serious bacterial or fungal infection every three to four years.
CGD can involve any organ system or tissue in the body, but infections are usually found in these areas:
- lymph nodes
- brain (though only occasionally)
People with CGD may also find they have trouble healing if they acquire a wound, and an inflammatory condition known as granuloma can develop.
Pneumonia that has been caused by a fungus is a red flag for CGD and often results in people being tested for the condition. Pneumonia is the most common infection to be contracted by people with CGD.
Life expectancy and prognosis
The life expectancy of individuals with CGD is slightly reduced due to repeated episodes of infection and inflammation. However, if people with CGD have the right treatment, most will live into mid to late adulthood.
The best treatment plan for CGD is prevention from infection. The following tips can help prevent infection:
- Avoid swimming in water other than chlorinated pools. Fresh or salt water may contain organisms that, while safe for healthy people, may cause infection in people with CGD.
- Avoid using mulch when gardening. Exposure to garden mulch can cause a life-threatening form of pneumonia from Aspergillus infection.
- Turning compost piles, repotting plants, cleaning cellars, and going on hayrides are other activities that should be avoided by people with CGD.
When an infection does occur, treatment can include prophylactic antibiotics and antifungal medications. An infection in someone with CGD generally requires a long period of time to treat.
Bone marrow transplant is another treatment option for some people with severe symptoms of CGD. Bone marrow transplantation can cure CGD. However, this therapy is extremely complex, and transplant candidates and donors must be carefully chosen.
CGD in children
CGD may become apparent at any time from birth right up to late adulthood. The vast majority of affected individuals are diagnosed before the age of 5. Children with CGD are often healthy at birth but develop severe infections in infancy or early childhood.
If you’re a parent with CGD, ask your doctor about seeking diagnosis for your child.
In CGD, aside from the defective function of certain immune system cells, the rest of the immune system is normal. People with CGD can be generally healthy until they become infected with one of the germs that those defective cells can’t fight. The severity of these infections can often lead to prolonged hospitalizations for treatment.
Research confirms that the severity of CGD is not the same for all, and the long-term life expectancy for people with CGD in now greatly increased. However, it’s suggested that people who begin to experience the symptoms of CGD earlier may be likely to have a shorter life span.