Autosomal Dominant Polycystic Kidney Disease: Your GoodRx Guide

ADPKD is a genetic kidney condition that you can inherit from one of your parents. There’s another type of polycystic kidney disease (PKD) that happens when you inherit the condition from both parents. This is autosomal recessive polycystic kidney disease — but this is much less common.

ADPKD is the most common form of PKD. Up to 1 in 1,000 people have this type of PKD. And it’s sometimes referred to as “adult PKD.”

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Causes

ADPKD is caused by a mutation (change) in a gene. Most of the time, this is a gene defect passed down from one parent. In rare cases, the gene mutation isn’t inherited, and the gene just mutates on its own. 

When one parent passes the genetic defect to a child, there’s a 50% chance of developing the disease. When both parents pass along the gene mutation, there’s a 75% chance each child will have ADPKD.

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Symptoms

Symptoms of ADPKD can vary widely from person to person. And people with ADPKD can go many years without any symptoms. The cysts in the kidneys may be present most of someone’s life. But symptoms are more likely to occur as the cysts grow larger. Symptoms more commonly develop after the age of 30, but sometimes they occur earlier. 

Typical symptoms of adult PKD include:

• High blood pressure

• Blood in the urine

• Back or flank pain

• Recurrent urinary tract infections (UTIs)

• Kidney stones

Adult PKD can eventually lead to kidney failure. This usually occurs by the age of 60 and is the result of the kidneys getting bigger. ADPKD causes about 10% of cases of kidney failure in the U.S. 

People with ADPKD are also at higher risk for some other complications. And these conditions can cause symptoms of their own. They include: 

• Brain aneurysm

• Aortic aneurysm

• Cysts in the liver

• Heart valve abnormalities

• Abdominal wall hernia

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Diagnosis

PKD is diagnosed by ultrasound, which can detect cysts in the kidney. But the presence of a kidney cyst isn’t enough to make the diagnosis. Healthcare professionals need to take several other factors into account, including:

• Your age

• Your family history

• The number of cysts in each kidney

You may also have other testing to help with diagnosis. This may include:

• Genetic testing: If you have a family history of PKD, this can help predict how likely you are to develop PKD in the future.

• CT scan: This imaging test can show the kidneys in greater detail than an ultrasound. 

• MRI: This test may help to track the size of your cysts and kidneys.

• Blood tests: Labs like a basic metabolic panel are useful in monitoring kidney function.

• Urinalysis: This helps to monitor for signs of kidney disease.

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Medications

Medications for ADPKD help to prevent the kidney disease from worsening. They’re also used to treat the symptoms and complications of PKD.

For ADPKD, Jynarque (tolvaptan) is the only FDA-approved treatment to preserve kidney function. It works by keeping the cysts from growing. This slows the rate of kidney decline in people who’re most at risk.

Other medications commonly used to treat the symptoms and complications of ADPKD include:

• Medications to lower blood pressure (like ACE inhibitors and ARBs)

• Pain relievers to control pain (like acetaminophen and aspirin)

• Antibiotics to treat infection (like ciprofloxacin)

• Cholesterol-lowering medication to keep blood vessels healthy (like statins)

Your healthcare team will likely include several specialists to help care for the kidneys, heart and blood vessels, and liver. They’ll work together to coordinate your care and medications. 

Research is ongoing for gene therapies and other innovative treatments that may help to effectively treat ADPKD. At this point, research has only been in lab and animal models, but it looks promising. Human clinical trials are still needed to determine the effectiveness of these new treatment approaches.

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Treatments

Treating ADPKD and preventing complications includes paying attention to other organs too. A healthy heart and blood vessels can help keep your kidneys as healthy as possible. In addition to medication, other treatments for PKD may include:

• Restricting your intake of sodium (salt)

• Quitting smoking

• Drinking enough water

• Exercising regularly

Sometimes, you may need procedures to treat the complications of ADPKD. In some cases, surgery to remove kidney cysts can help improve pain. This usually doesn’t improve kidney function, but it can improve your quality of life. 

In those who get kidney failure, dialysis or kidney transplant are treatment options. Dialysis uses a machine to filter the blood and do the work of healthy kidneys. A kidney transplant involves a donor kidney replacing kidneys damaged by ADPKD.

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