Chronic granulomatous disease
This is a type of primary immunodeficiency disorder (PID) - genetically-based minority immune system disorders - which affects the granulocytes, the cells that are essential to eliminate microbes.
Cause
Neutrocytes or phagocytes are cells that usually destroy bacteria, but with this disease they are incapable of eliminating them. With this disease, the phagocytes look for reinforcement from the surrounding cells, creating a “ball” (called a granuloma) to try to contain the infection.
How is it diagnosed?
With a specific blood test:
- An oxidation test to see if the phagocytes are working properly.
- A genetic study of the patient and their family.
What can happen?
- Lung infections like pneumonia or pulmonary abscess
- Inflammation of the ganglia in the neck
- Skin infections
It can also cause:
- Persistent diarrhoea
- Canker sores in the mouth
- height and weight alterations
How is it treated?
- Medication for the infections (antibiotics, antifungal medication).
- Surgery to drain the abscesses
- Hematopoietic stem cell transplant (HPSCT) to cure the patient.
How can I look after myself?
- Wash your skin twice a day with disinfectant soap.
- Keep your nails short.
- Good oral hygiene.
- Do not handle earth.
- Avoid places where building work is under way.
- Make sure you take the medication to prevent infection diligently.
