Focal Segmental Glomerulosclerosis: What It Can Mean for You and Your Family

This article is for people who have or are at risk for focal segmental glomerulosclerosis (FSGS), or anyone who wants to learn more about it. The goal of this activity is to help you understand more about FSGS and how it can affect you and your family.

You will learn about:

• What FSGS is

• Who can have FSGS and what may cause it

• FSGS symptoms

• How FSGS is diagnosed or identified

• How FSGS may impact you and your family

• Questions to ask your doctor and healthcare team

Your kidneys are important organs that clean your blood and remove toxins and waste through urine. Most people have 2 kidneys, and each of those kidneys has about 1 million small filters called glomeruli.

Focal segmental glomerulosclerosis (FSGS) is a condition that affects the tiny blood vessels in these glomeruli. Over time, these blood vessels can become scarred, making it hard for your kidney to filter your blood properly. FSGS can also spread to other parts of the kidneys and lead to kidney damage and failure where your kidneys stop working.

FSGS can happen in adults or children, at any age. But it tends to happen more in men, adults older than 45, and people of color, including more often in people who are Black/African American or Latino/Hispanic than people who are white or Asian.

FSGS can have many causes, including your genetics (traits you inherit from your family). In addition, health conditions like diabetes, high blood pressure, and obesity can cause problems in the kidneys. And if you have one of these conditions plus a serious infection or are taking medicine that can affect your kidneys, this may cause injury and stress on your kidneys, leading to FSGS.

There are 3 types of FSGS:

• Primary means that the cause is unknown

• Secondary is caused by an infection by a virus, health condition, or medicine:

  o  Viral infection like COVID-19, HIV, Epstein-Barr virus, cytomegalovirus (CMV), hepatitis B, parvovirus B19

  o  Health conditions like sickle cell disease or lupus

  o  Medicines such as steroids or certain ones used to help treat for osteoporosis (bone thinning)

• Genetic is when FSGS is passed down through your family. There are 2 gene changes or mutations known to cause FSGS: APOL1 and MYH9. People with APOL1 mutation typically have more severe FSGS

In the early stages of FSGS, people usually don’t have any symptoms. But as FSGS develops, symptoms may include:

• Swelling in the legs, ankles, or around the eyes

• Weight gain

• Foamy urine

• High blood cholesterol

• High blood pressure

• Protein in your blood or urine

• Blood in your urine

As FSGS gets worse and kidney failure starts to happen, some people can have symptoms such as:

• Fatigue (tiredness)

• Poor appetite

• Headache

• Itchy skin

• Shortness of breath

• Nausea

There are different tests may help your doctor diagnosis, or find, FSGS, such as:

• A urine test to measure how much protein or blood there is in a sample of your urine

• A blood test to measure protein and cholesterol levels in your blood and how well your kidneys are working (your glomerular filtration rate)

• A biopsy where a special needle is used to take a tiny piece of your kidney to look at it under a microscope. This test is often used to confirm an FSGS diagnosis

• A genetic test using blood or saliva (spit) to check for a mutation like APOL1 and MYH9

Based on the diagnosis and type of FSGS you have, you and your doctor can make a treatment plan together.

Your doctor may recommend changes to your diet and taking certain medicines a part of your treatment plan to help slow down FSGS. For some people, this can work well and FSGS may improve or not get worse for some time.

Diet changes can include eating foods that have less salt and protein in them. Medicines may include those to help manage high blood pressure, high cholesterol, and other health conditions like diabetes, and those to help calm your immune system (your body’s natural defense system) to help stop it from attacking your kidneys by mistake.

Once parts of your kidneys become scarred, the damage cannot be reversed. Over time, damage may lead to kidney failure where you’d need either lifelong dialysis to help filter your blood or a kidney transplant.

When you’re living with FSGS, your kidneys aren’t functioning well, and, over time, FSGS can get worse and may lead to kidney failure. If you need dialysis, you may go a few times a week, but can still likely carry on with most of your daily activities, family life, and work.

But FSGS can affect adults and children and may affect your family if there is a genetic mutation for it. If you have FSGS, your family should also get tested. If they have FSGS, they can get the right treatment to help prevent kidney disease early on.

Questions you can ask your doctor and healthcare team about FSGS can include:

• Am I at risk for FSGS? What about my family?

• Should my family and I take a genetic test for FSGS?

• What should I do to help manage FSGS so that my kidney disease doesn’t get worse?

• What kind of treatment is available for FSGS, and what are the side effects?

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You have successfully completed the program Focal Segmental Glomerulosclerosis: What It Can Mean for You and Your Family

View Additional Materials on this topic that you may find useful:

FSGS

Focal Segmental Glomerulosclerosis -- National Library of Medicine

Diabetes and Kidney Disease: What to Eat?

Dialysis Safety

Taking Care of Your Kidneys