What is FSGS?

FSGS, or Focal segmental glomerulosclerosis…

    • Wow, that’ a mouthful!!

    • Focal… glumeral…. I can’t say that!

    • Say what!?!?!

Those are some of the things people say when I tell them the name of the disease my wife has. Her name is Laura Torres and I’ve setup this site to tell her story.

So what is FSGS?
Each person has two kidneys and each kidney is made up of tiny filters called “glomeruli.” These function like a coffee filter but instead of keeping the coffee grounds from leaving the filter, they keep cells and protein in the blood. When glomeruli become damaged, protein begins leaking into the urine (this is called proteinuria). Proteinuria causes fluid to accumulate in the body, which causes severe swelling. Prolonged leakage can lead to kidney damage and can eventually lead to kidney (renal) failure.

Focal Segmental Glomerulosclerosis (FSGS) is a rare disease that attacks the kidney’s filtering system (glomeruli) causing serious scarring. FSGS is one of the causes of a serious condition known as Nephrotic Syndrome.

There are five variants of FSGS. The collapsing variant being the most aggressive of all. It does not typically respond to therapy and the scarring quickly affects the entire filter, causing it to collapse.  Most patients that are diagnosed with the Collapsing variant will require dialysis or a kidney transplant within one to two years despite treatment.  Unfortunately, this is the variant that has been diagnosed in my wife, Laura.

So what does Focal Segmental Glomerulosclerosis mean?
Sclerosis means “Scarring.” There are millions of tiny, microscopic filters in the kidneys called “glomeruli.” They are filters much like a sieve you might find in your kitchen, and they filter the blood, taking out the water-like part that becomes urine and leaving the protein in the blood. If the glomeruli become scarred, then they stop doing a good job filtering the blood and protein starts leaking into the urine instead of staying in the blood. The word “focal” is added because in FSGS, only some of the glomeruli filters become scarred. “Segmental” means that only some sections of the glomerulus becomes scarred, just parts of them. So Focal Segmental Glomerulosclerosis means:

Focal = some
Segmental = sections
Glomerulo = of kidney filters
Sclerosis = are scarred

So what causes FSGS? 
FSGS is “idiopathic,” which means it arises without a known cause, so researchers are actively trying to learn more.

So how is FSGS treated? 
Currently there are no FDA approved treatments, but usually a steroid called prednisone or prednisolone,is given to control proteinuria.

Share this on...Share on FacebookShare on Google+Tweet about this on TwitterShare on RedditEmail this to someone