Autosomal dominant polycystic kidney disease (ADPKD) is a lifelong condition that causes multiple cysts to form in your kidneys. When you have this genetic condition, the kidney cysts don’t tend to develop into kidney cancer. But over time, these cysts can affect your kidney function. You can develop irreversible kidney damage and chronic kidney disease (CKD).

There isn’t a way to stop kidney cysts from forming when you have ADPKD. But there are treatments to ease symptoms and improve your kidney function for as long as possible.

From the community: “Yesterday I got approved for my kidney transplant! I was pretty sure I would but it was exciting to hear anyway. My brother and sister are testing to see if they are a match to be donors (it's like a competition to them that they each want to win) and I feel so blessed to have such wonderful family and friends. I have had so many people volunteer to donate a kidney that's it's emotionally overwhelming to feel so cared about. My family is so supportive in every way that I feel so blessed but at the same time I'm not a person to show my emotions. So often I feel on the verge of tears. Part I believe from exhaustion and part from the enormity of it all. I'm not scared to get the transplant. I'm sad that I feel so tired all the time, that my quality of life is getting less and less. Sad that I must rely on others and sad to see my parents worry about something they have no control over.” – Inspire member 

Who treats ADPKD?

In addition to your kidneys, ADPKD can affect other organs like your heart, brain, liver, and pancreas. Depending on your symptoms and the severity of the disease, you may see a team of specialists, including:

• nephrologist, a medical doctor who specializes in kidney disease

• radiologist, a medical doctor who uses radiation therapy to treat cysts that cause pain or other problems

• genetics expert, who can determine if you carry the gene change that causes ADPKD, as well as your risk of passing it along to your children 

• dietitian, an expert who can help you follow a kidney-friendly diet

How is ADPKD treated?

Until 2018, there wasn’t a drug specifically designed to treat ADPKD. That year, the U.S. Food and Drug Administration (FDA) approved tolvaptan (Jynarque®). You take this pill twice a day to slow the growth of cysts. The medication keeps your kidneys functioning longer.

Over-the-counter pain relievers can ease pain from enlarged cysts, but certain ones may harm the kidneys by reducing blood flow. You should always check with your doctor before taking any medication or supplement to ensure it’s safe for your kidneys. For people with kidney disease, doctors typically recommend acetaminophen instead of nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen.

If pain medicines don’t help, your doctor may perform a minimally invasive surgical procedure using a needle to drain and shrink some of the larger kidney cysts. In rare instances, you may need surgery to remove a problematic cyst.

People with ADPKD are also at risk for other problems. You may need to take:

• medications to control high blood pressure

• antibiotics to treat a bladder infection, kidney infection, or urinary tract infection (UTI) 

Does ADPKD lead to chronic kidney disease (CKD) and kidney failure?

Having ADPKD increases your risk of chronic kidney disease. Chronic kidney disease occurs when your kidney function decreases for at least three consecutive months. 

Doctors measure chronic kidney disease in stages from 1 to 5. Kidney failure (stage 5) occurs when less than 15% of your kidneys are working. Approximately half of people with ADPKD eventually develop kidney failure. Because your kidneys can’t filter wastes from your blood, toxins build up in your body. You may have trouble breathing, itchy skin, muscle cramps, and other symptoms. Kidney failure (also called end-stage renal disease or ESRD) can be fatal without proper treatment. 

When should you start treatment for kidney failure?

Your doctor will consider these factors to determine when you should start treatments for kidney failure brought on by ADPKD:

• other health conditions, such as liver cysts, diabetes, and high blood pressure

• your symptoms and how they impact your quality of life

• the stage of the kidney disease and how well your kidneys filter your blood

• your diet and lifestyle 

How is kidney failure treated?

Your body needs another way to get rid of toxins when you have kidney failure. Your treatment options include:

• hemodialysis 

• peritoneal dialysis

• kidney transplant

Hemodialysis

Dialysis is a way to filter wastes and excess fluids from your blood. With hemodialysis, a machine and a dialyzer filter (sometimes called an artificial kidney) perform these tasks. The system then returns the filtered blood to your body.

Before you start hemodialysis, your doctor will perform a minor surgical procedure to create easier access to a blood vessel. Hemodialysis access provides a way for your blood to travel more easily through the machine and back to your body. 

How often you need hemodialysis depends on your doctor’s prescription and where you choose to get the dialysis:

• At a hospital or dialysis center, hemodialysis takes place three days a week. Each session takes about four hours. 

• Home hemodialysis may take place three days a week for up to four hours. Or you may do the process more frequently (five to seven days a week), but for a shorter amount of time (about two hours). You may also choose to do dialysis for six to eight hours at night while you sleep.

From the community: “there are a great many people who do home hemodialysis here and I will soon be one of them. I have talked to many people and visited their homes to see the machine and learn and all say that its a breeze. They do it overnight while they sleep, which sounds fine to me. There was an old lady who was 87 y/o in hospital with me last time I went who did PD at home, so I think they are not gibing you the whole story. Apparently it is much easier on your heart if you do HD over a longer period, say 8 hours as opposed to the 4 or 5 hours they do it over in hospital.” – Inspire member 

Peritoneal dialysis

Peritoneal dialysis is a treatment that cleanses your blood while it’s inside your body. Before starting this type of dialysis, you undergo a minor surgical procedure to place a thin, flexible tube called a catheter into the lining of your abdomen (peritoneum). 

There are two ways to do peritoneal dialysis. For continuous ambulatory peritoneal dialysis (CAPD), you perform these steps every day:

1. Connect the catheter to a bag with a tube that holds dialysis solution.

2. Use the catheter to fill your peritoneal (abdominal) cavity with the solution.

3. Disconnect the bag, and place a cap on the end of the catheter.

4. Resume daily activities for the next 30 to 40 minutes while the solution absorbs waste and excess fluids from your blood.

5. Take the cap off the catheter, connect the catheter to a tube, and drain the fluid out of your abdomen and into a new bag.

6. Repeat these steps three to five times within a 24-hour period.

7. Sleep with the solution in your abdomen and empty the fluid in the morning.

Some people prefer to do automated peritoneal dialysis (APD) at night. While you sleep, a machine called a cycler fills and empties the peritoneal cavity with the cleansing solution. With this method, you don’t have to do CAPD during the day.

From the community: “Hello,I went on dialysis in June… I do home dialysis every night for 8 hrs of peritoneal home Dialysis and I sleep well and have so much more energy. The days are free to do what ever unfilled 10 pm when I. Start. My treatment. I would ask if you could change to home dialysis and if you could change to peritoneal Dialysis. I know you would be so much happier.” – Inspire member

Kidney transplant

A kidney transplant is a major surgical procedure. You receive one healthy kidney from a living or deceased organ donor. Your body functions quite well with only one kidney. Some people need dialysis as they wait to get a kidney transplant. 

During a kidney transplant, your surgeon may remove one or both of your diseased kidneys if they’re causing pain or bleeding, or if the organs take up a lot of space. This procedure is a nephrectomy. 

Otherwise, your surgeon disconnects the diseased kidneys from your bladder and blood vessels, leaving the organs in place inside your body. Without blood flow, the kidneys eventually shrink. Your doctor places the donor kidney in your groin area and connects it to your bladder and blood vessels. To prevent your body from rejecting the donor kidney, you need to take immunosuppressants for life.

Can you cure ADPKD?

A kidney transplant won’t cure ADPKD or get rid of the gene change that causes the disease. But it greatly improves your kidney function, helping you enjoy a better quality of life for longer. While the transplanted organ won’t develop kidney cysts, you can still develop liver cysts and other disease-related complications. 

Your prognosis depends on many factors like:

• type of ADKPD

• related conditions like liver cysts, enlarged heart, and high blood pressure 

• your age when diagnosed

What changes can you make to manage ADPKD?

These steps can slow the development and growth of kidney cysts:

• avoid caffeinated and alcoholic beverages

• cut back on sodium (salt)

• drink lots of water every day

• don’t smoke or use nicotine products

• manage high blood pressure and diabetes

• stay physically active and maintain a healthy weight

Sources

• Kidney failure (ESRD) causes, symptoms, and treatments. American Kidney Fund.

• Polycystic kidney disease (PKD) symptoms, treatments, and causes. American Kidney Fund.

• Hope for PKD patients: First-ever treatment approved by FDA. American Kidney Fund. April 2018.

• Dialysis. Cleveland Clinic. August 2021.

• Kidney transplant procedure. Cleveland Clinic. March 2019.

• Kidney cysts. Mayo Clinic. July 2020.

• Autosomal dominant polycystic kidney disease National Institute of Diabetes and Digestive and Kidney Diseases. January 2017.

• Choosing a treatment for kidney failure. National Kidney Foundation.

• Autosomal dominant polycystic kidney disease. Genetic and Rare Diseases Information Center. February 2020.

• Dialysis. National Kidney Foundation. 2015.

• Hemodialysis. National Kidney Foundation.

• Hemodialysis access. National Kidney Foundation. 2015.

• Home hemodialysis. National Kidney Foundation. 2015.

• Kidney transplant. National Kidney Foundation.

• Peritoneal dialysis: What you need to know. National Kidney Foundation. June 2021.

• Pain medicine (analgesics). National Kidney Foundation. 

• Polycystic kidney disease. National Kidney Foundation. 

• What are the stages of ADPKD? PKD Foundation. June 2021.

• What is ADPKD? PKD Foundation. June 2021.

• Kidney transplant. Polycystic Kidney Disease Charity (UK). 

• Polycystic kidney disease. University of California San Francisco Department of Surgery. 

Disclaimer

Member comments are lightly edited for length and to remove identifying information but are otherwise reproduced as they appear in the community as part of public posts.

This content is for general informational purposes only and does not necessarily reflect the views and opinions of any organization or individual. The content should not be used as a substitute for professional medical advice, diagnosis, or treatment. Please consult your healthcare provider about any questions you may have regarding a medical condition.