What is Chronic Granulomatous Disease?

Granulomatous

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The immune system is incredibly complicated. But it performs a vital function in keeping you healthy. And when even one small part of the system breaks down, it can affect the way your entire body functions. Just look at a condition like Chronic Granulomatous Disease.

Chronic Granulomatous Disease (CGD) is a condition where a single type of immune cell ceases to function correctly. And it puts you at risk of near constant infection and can even be life-threatening. So, what exactly causes chronic granulomatous disease? What are the symptoms and risks? And what can you do to treat it?

What Is Chronic Granulomatous Disease?

CGD is caused by an inherited immunodeficiency disease. Essentially, it leads to a type of cell called phagocytes being unable to do its job. Phagocytes are a type of immune cell that attacks and destroy foreign cells like bacteria and fungal spores. And when you have CGD, the phagocytes are unable to effectively handle these kinds of cells, which means that you suffer from repeated infections.

The condition is passed down from parent to child and is linked to the sex chromosomes. The sex chromosomes are involved in determining the sex of a child when they are in the womb. There are two kinds of sex chromosomes, called the X and Y chromosomes. Boys have a single X chromosome and a single Y chromosome. But girls have two X chromosomes. Because CGD is linked to the X chromosome, boys are more likely to develop the condition than girls. They lack the extra X chromosome to make up for a single one that has the condition tied to it.

WHen you have CGD, the majority of your immune system functions properly. That means that unlike other immunodeficiency diseases, you will generally be healthy, since your immune system can fight off most viruses. The problem comes when you are infected with the specific type of bacteria or fungal infections that the phagocytes help to fight. These sorts of infections are generally very mild in most people. But when you have CGD, they are much more serious. They last longer and are harder to treat than they would normally by.

Symptoms And Risks

CGD causes no obvious symptoms until you are infected with a bacterial or fungal infection. These infections can cause serious complications. They can even lead to abscesses or granulomas in the organs and bones.

Granulomas and abscesses are a natural mechanism of the immune system where the body forms inflammations of tissue to block off pockets of infection. These growths of inflammation form pockets of pus in the tissue which can serve as vectors of infection. And they can also interfere with the function of organs like the kidneys and lungs.

In addition, CGD can lead to a variety of skin infections like dermatitis, sores on the face, and pus-filled lumps in the skin. And it can also lead to general infection symptoms like persistent diarrhea and swollen lymph nodes in the neck.

How Is It Treated?

It’s often difficult to diagnose CGD because of the lack of symptoms. Doctors will usually notice the fact that the patient is getting recurrent infections, which will necessitate a series of tests including tests of the blood to check for the presence of immune cells and how well they function. In addition, doctors can test the genetic composition to see if the sex chromosomes have evidence of carrying CGD.

There’s no easy way to cure CGD, since it is caused by the genes. A bone marrow transplant can help to modulate the way the immune system functions, but it is often a risky and painful procedure. So, the majority of treatment is focused on managing the symptoms of recurrent infections.

The most common method of treatment is antibiotics. These can help your immune system fight off infections by killing off bacteria and fungus. In addition, doctors can prescribe a type of drug called interferon, that helps your immune system fight off future infections.

Usually, these medications are enough to help manage the infections that come with CGD, but there is still the problem of abscesses forming on the organs or bones. Much of the time, these abscesses heal on their own, but sometimes surgery is necessary to treat particularly large ones.

So, what do you think? Do you have CGD? What do you do to manage it? Do you have any advice about what to expect? Would you like to see more research into the condition? Let us know in the comments.