Patient education: Polycystic kidney disease (The Basics) – UpToDate
What is polycystic kidney disease? — Polycystic kidney disease (PKD) is a condition that affects the kidneys. When people have PKD, abnormal fluid-filled sacs called “cysts” grow in the kidneys.
The cysts cause the kidneys to get bigger than normal. The cysts can also keep the kidneys from working normally. This can lead to problems, such as high blood pressure, kidney infections, and kidney failure. Kidney failure is when the kidneys stop working completely. Besides kidney problems, PKD can cause problems in other parts of the body.
PKD usually runs in families.
What are the symptoms of PKD? — Some people with PKD have no symptoms. When people do have symptoms, they can have:
●Pain in the lower half of the back or on the side, with or without a fever
●Pain in the belly
●Blood in the urine
●Kidney stones – These are small, stone-like objects that form inside the kidneys. They can cause belly or side pain, or blood in the urine.
PKD can also cause problems in other parts of the body, such as:
●A bulging blood vessel in the brain – If the blood vessel bursts, it can cause a sudden, severe headache and nausea and vomiting. A burst blood vessel can lead to brain damage and even death.
●Cysts in the liver – These can cause belly pain.
●A weak area in the belly muscles (called a “hernia”) – This can cause an area of the belly to bulge out.
●Heart problems – These do not usually cause symptoms.
Is there a test for PKD? — Yes. To find out if you have PKD, your doctor can do:
●An imaging test, such as an ultrasound, CT, or MRI scan – Imaging tests create pictures of the inside of the body.
●Blood tests to check for the abnormal genes that cause the disease
How is PKD treated? — If PKD is causing high blood pressure, your doctor will probably treat that first. This can help your kidneys stay healthy for longer. Treatment usually involves lifestyle changes, diet changes, and medicines.
If you have other symptoms or problems, you might need other treatments, too. For example, doctors can:
●Treat kidney infections with antibiotic medicines
●Treat pain with pain-relieving medicines
●Do surgery to fix a bulging blood vessel in the brain
●Do surgery to fix a hernia
In some cases, a medicine called tolvaptan (brand name: Jynarque) might help slow down PKD. It can also help with pain. But doctors do not recommend this medicine for everyone. It also comes with side effects. Your doctor can talk to you about your treatment options.
What happens if my kidneys stop working completely? — If your kidneys stop working completely, you will need treatment that takes over the job of your kidneys. Normally, the kidneys make urine by removing waste and excess salt and water from the blood.
There are 2 treatments for people whose kidneys stop working completely. They are:
●A procedure called dialysis – There are 2 types of dialysis, but most people with PKD have a type called “hemodialysis.” During hemodialysis, a machine removes waste and excess salt and water from the blood. People who get hemodialysis need to be hooked up to a machine for a few hours at least 3 times a week. They will need hemodialysis for the rest of their life or until they can get a kidney transplant.
●Kidney transplant surgery – During this surgery, a doctor replaces your diseased kidney with a healthy kidney. That way, the new kidney can do the job of your kidneys. (People need only one healthy kidney to live.)
If you have questions about the different options, talk with your doctor or nurse.
Should my family members get tested? — If you have PKD, your adult family members should talk with their doctor about getting tested for it. There are benefits and downsides to getting tested.
Doctors do not usually recommend that children get tested unless they have symptoms. But children should see their doctor or nurse every year to have their blood pressure checked.