Journey Overview

Focal segmental glomerulosclerosis (FSGS) is a type of kidney damage that causes scarring and hardening in the glomeruli. Glomeruli are networks of tiny blood vessels that act as filtering units. As blood passes through the kidneys, glomeruli remove excess fluid, cellular waste products, and unneeded substances, which become urine and are directed to the bladder.

When glomeruli are damaged, this process breaks down and problems occur. Signs of FSGS can include high amounts of protein in the urine (proteinuria), foamy urine, fluid accumulation in the feet and legs (edema), chronically elevated blood pressure (hypertension), elevated cholesterol, and weight gain. FSGS can progress to kidney failure.

FSGS can have different causes. Some forms are genetic, some occur as a result of a medical condition or medication side effect (secondary FSGS), and some occur for no identifiable reason (primary FSGS). Distinguishing between primary, secondary, and genetic causes is a critical step in diagnosis and treatment.

If you or a loved one is living with FSGS, this guide provides information to help you better understand the healthcare providers that you will work with, the questions you should be asking at appointments, and how FSGS can be managed.

Your Multidisciplinary Team

People who are living with FSGS will work with a team of healthcare providers with different specialties. This team can include:

  • Nephrologist

    Treatment for FSGS will typically be overseen by a nephrologist, a medical doctor who specializes in diseases that affect the kidneys. Nephrologists are sometimes called “kidney doctors”. This specialist will oversee treatment and coordinate with other members of a healthcare team.

  • Primary Care Provider

    Your primary care provider is the healthcare provider you see for checkups, care when you are sick or injured, and preventive care like vaccinations. This provider may be an MD (Doctor of Medicine), DO (Doctor of Osteopathic Medicine), Nurse Practitioner (NP), or Physician Assistant (PA).

  • Renal Dietitian

    You may need to make changes to your diet to protect kidney function and reduce complications. Dietary changes can include limiting the amount of sodium and protein you consume. You may work with a registered dietitian who specializes in kidney disease (called a renal dietitian).

  • Additional team members

    Nurses play an important role in treatment, including coordinating care, patient education, and counseling. Depending on the underlying cause of FSGS or co-existing conditions, people living with FSGS may also work with other specialists to treat those conditions.

FSGS Treatment

  • Treatment will depend on the underlying cause (if any), kidney function, and symptoms.
  • The overall goal is to protect kidney function. Treatment often focuses on improving proteinuria, which is associated with better outcomes.
  • Treatment can include medications to control blood pressure and lower protein levels in the urine (such as ACE inhibitors or ARBs), and drugs to manage fluid retention, cholesterol, and blood clots.
  • Steroid medications and immunosuppressive drugs may be used in the treatment of primary FSGS but are potentially harmful to people with other types.
  • Novel therapies or clinical trials may be recommended.
  • A healthcare team may recommend changes to diet, exercise, taking certain vitamins, and quitting smoking (if you smoke).
  • If FSGS progresses to end-stage kidney failure, it will need to be treated with dialysis and/or kidney transplantation.

FSGS is a rare condition, and it’s important to work with healthcare providers who are experienced in treating rare kidney diseases. It’s also important to learn as much as you can about your kidney biopsy results, your diagnosis, and the medications you are taking for FSGS.

The Conversation: Questions for your healthcare team

Good communication with your healthcare team will help you get the most from treatment. Keep a symptom journal. Prepare for your appointments by writing down a list of questions and topics you want to cover. These questions can help you get started:

What are the most important things for me to know about my diagnosis? Where can I learn more about my diagnosis?

What do these numbers mean and why are they important to my kidneys? For example, “How do these numbers affect my risk of kidney failure?”

  • Glomerular filtration rate (GFR)
  • Urine albumin level
  • Blood pressure

Ask your provider to explain the results of any tests, including:

  • Blood tests
  • Urine tests
  • Kidney biopsy
  • Genetic testing

What treatment do you currently recommend? Ask these questions for every therapy you are prescribed.

  • Why do you recommend this treatment?
  • How does this treatment work?
  • What is the goal of this treatment?
  • How long will I be on this treatment?
  • What are the potential side effects?

What can I do to prevent damage to my kidneys?

  • What things should I limit, avoid, or change?
  • Where can I learn more about how to care for my kidneys?

What new symptoms, changes in symptoms, and complications do I need to watch for?

  • When do I need to call you?
  • When do I need to seek emergency care?

Are there other healthcare providers I should be working with?

Will you coordinate with my other healthcare providers? Ask what you can do to make sure your primary care provider and any specialists you are seeing are up to date on your current diagnosis and treatment.

Who can I talk to if I have concerns about the cost of treatment?

Your appointment is an opportunity to learn more about your diagnosis. If you have a question, ask. If something isn’t clear, ask for an explanation.

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