Autosomal dominant polycystic kidney disease (ADPKD) is a lifelong condition that leads to irreversible kidney damage. Over time, the strain on your kidneys from ADPKD can cause chronic kidney disease (CKD). 

From the community: “i want to tell you some information about PKD to provent further step. most people detect PKD in their 30s or 40s, which indicates that the cysts develop quickly at that time in most cases. if your cysts enlarge quickly, please find a treatment to control them. if your condition progresses slowly, you are very luky one. in some patients with pkd, their cysts did not grow bigger at then end of their lives. i sincerely hope you are the luky one.” – Inspire member 

Slowing ADPKD progression

While there isn’t a cure for ADPKD, treatments can keep your kidneys healthy for as long as possible while easing ADPKD symptoms. These treatments may include medications to control blood pressure and dietary changes. 

In 2018, the U.S. Food and Drug Administration (FDA) approved the first drug specifically designed to treat ADPKD. The drug slows the effects of the disease on the kidneys, helping to keep them functioning longer.

Stages of chronic kidney disease and ADPKD

When your kidney function decreases for three consecutive months or longer, you have chronic kidney disease (CKD). Diabetes and high blood pressure are the main causes of CKD, but people with ADPKD also develop CKD.

There are five stages of CKD. The stages indicate how well your kidneys can function and filter toxins from your blood. 

• Stage 1: You have mild kidney disease with 90% or higher kidney function.

• Stage 2: You have mild kidney disease with 60% to 89% kidney function. 

• Stage 3: You have moderate kidney disease with 30% to 59% kidney function.

• Stage 4: You have severe kidney disease with 15% to 29% kidney function.

• Stage 5: You have kidney failure, which means your kidneys function at less than 15% of capacity.

ADPKD and kidney failure

When kidney failure occurs, it means your kidneys can’t filter wastes from your blood. This is their primary job. Toxins start to build up in your body, causing a condition called uremia. Without treatment, uremia and kidney failure are life-threatening. Kidney failure is also called renal failure and end-stage renal disease (ESRD). 

When your kidneys begin to fail, you may experience these symptoms:

• difficulty breathing or shortness of breath

• fatigue and weakness

• insomnia or sleep problems

• itchy skin

• lack of appetite

• metallic taste in your mouth

• muscle cramps

• nausea and vomiting

• peeing a lot or not making enough urine

• swollen legs and feet

From the community: “I am on stage 3 FSGS and have polycystic kidney disease. I can relate to your symptoms. I also feel dizzy & nauseated & dont know what is making me feel like this. I went to the ER last week with severe kidney pain on my left side & I was throwing up, but the docs came back to tell me that I had a kidney stone.  When I went for a follow up visit with my neph, he told me that my xray showed no sign of kidney stones & doesnt know why the would tell me that. Well my pain has gotten better, but I am still having nausea. Its a never ending cycle with this disease.  I lost my career due to the housing market, at times I am depressed for my career, but then when I start having my symptoms, I am glad I can stay home to deal with it.  I remember how hard it was to get up in the morning & go to work feeling crappy & having difficulty concentrating.  I can ony commend you for your efforts to continue to work to help put your wife through college. I will keep you in my prayers & Good Luck with your dialysis.  Keep us posted on your progress.” – Inspire member

Kidney failure and ADPKD treatments

When kidney failure occurs, you need another way for your body to filter wastes and prevent uremia. Your treatment options include:

• Dialysis: Hemodialysis and peritoneal dialysis are two different ways to remove wastes and excess fluids from your blood. Hemodialysis uses a dialysis machine and a dialyzer (artificial kidney). Peritoneal dialysis uses a cleansing solution and your peritoneum (abdominal lining) to filter out impurities.

• Kidney transplant: During this surgery, doctors remove one or both of your diseased kidneys. You receive a healthy kidney from a living or deceased organ donor.

ADPKD and life expectancy

ADPKD affects everyone differently. For some people, the disease progresses slowly. They may have smaller, less numerous cysts that cause fewer symptoms. For others, the disease is more severe. 

The type of ADPKD plays a role in disease progression and prognosis. People with changes to the PKD1 gene (ADPKD type 1) often need dialysis for kidney failure by the time they’re in their 50s. People with ADPKD type 2 (changes to the PKD2 gene) often don’t experience kidney failure until their 70s.

These factors may indicate a faster disease progression and worse prognosis:

• blood or protein in your urine (hematuria)

• enlarged kidneys or enlarged heart

• high blood pressure

• liver cysts (hepatic cysts) in females

• male gender

• three or more pregnancies for women

• UTIs in men

• young age at the onset of symptoms 

Steps to improve kidney health

While you can’t prevent or cure ADPKD, these steps can help protect the health of your kidneys:

• aim for at least 30 minutes of physical activity every day

• drink lots of water and cut back or eliminate caffeinated and alcoholic beverages

• manage high blood pressure with diet, lifestyle changes, and medications

• lose weight if needed, and maintain a healthy weight

• keep blood sugar levels in the healthy range via diet, exercise, and medications (if needed)

• follow a low-salt, low-fat, kidney-friendly diet

• get help to give up smoking and nicotine products

Sources

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

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

• Halvorson CR, et al. Polycystic kidney disease: Inheritance, pathophysiology, prognosis, and treatment. International Journal of Nephrology and Renovascular Disease. June 2010.

• 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.

• Polycystic kidney disease. National Kidney Foundation. 

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

• What is ADPKD? PKD Foundation. June 2021.

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.