What is Atypical HUS?
Atypical HUS (Hemolytic Uremic Syndrome) has historically fallen under the category of "kidney disease", as the kidney is the main organ involved.
But recent advances in understanding of the genetic condition now demonstrate it is more complex and can damage other organs, and even the brain.
How does it work?
Your body circulates blood continuously. It contains many proteins, red blood cells, platelets and other things as well. When your blood passes through your kidneys, small vessels (called nephrons) filter out waste.
In aHUS, the walls of your blood vessels can become clogged with platelets. So now your body is filtering blood, and the platelets are clotting up the kidney function. So when your red blood cells pass through, they are shattered. Therefore, your platelet and red blood cell counts start to decrease.
As a result, your kidney struggles to do its job and wastes such as creatinine are not filtered out properly, and your BUN (blood urea nitrogen) levels may increase too.
The clotting is caused because one of the proteins in the blood is not working properly.
Your genes are responsible for directing the proteins; so ultimately, one of your genes is not functioning properly.