There's currently no cure for autosomal dominant polycystic kidney disease (ADPKD), and it's not possible to stop cysts forming in the kidneys.
But there are some potentially useful medications, such as tolvaptan, that can sometimes be used to reduce the growth rate of cysts.
The various problems associated with ADPKD, such as high blood pressure (hypertension), pain and kidney stones, can also be treated.
If you're diagnosed with ADPKD, you'll usually see a kidney specialist, who can help draw up a suitable treatment plan.
The plan will also include what you'd want to do if your kidneys stop working sufficiently (kidney failure).
Medication is usually used to treat high blood pressure in people with ADPKD.
Angiotensin-converting enzyme (ACE) inhibitors and angiotensin-2 receptor blockers (ARBs) are the 2 medications most widely used.
There are also some lifestyle changes you can make to help reduce your blood pressure, such as cutting your salt intake to less than 6g a day (6g of salt is about 1 teaspoonful).
In many cases of ADPKD, any pain you experience can be relieved by treating the underlying cause, such as kidney stones or a urinary tract infection (UTI).
If you need to take a painkiller, paracetamol is the best medication to try first.
If your pain is particularly severe, you may be prescribed a stronger painkiller, such as tramadol or oxycodone.
It's usually recommended that non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, are avoided.
This is because they could disrupt kidney function and interfere with medications prescribed to control your blood pressure.
But a short course of NSAIDs may sometimes be safe for people with well-controlled blood pressure and relatively normal kidney function.
Antidepressants or anticonvulsants, which are normally used to treat epilepsy, may be prescribed for long-term (chronic) pain. These medications can also be used to relieve some types of pain.
Occasionally, large cysts can be drained to help relieve the pain caused by the increased pressure.
Small kidney stones pass out of your body when you urinate. If needed, you may be given a strong painkiller and medication to stop you feeling or being sick.
Drinking plenty of water will increase the flow of urine, which will help flush the stone into the bladder.
If a kidney stone is too big to be passed naturally, you may need treatment to help remove it.
Possible treatment options include:
Urinary tract infections (UTIs) can often be treated with a 7- to 14-day course of antibiotic tablets.
You should drink plenty of fluids during the course of the infection to keep you hydrated.
Paracetamol can be used to relieve pain and bring down your temperature.
It's important to see your GP as soon as possible if you develop symptoms of a UTI, as it could spread to the cysts in your kidneys if left untreated.
Infection in the cysts is harder to cure because it can be difficult for the antibiotics to penetrate them.
If the infection persists despite antibiotic treatment, the infected cysts may need to be drained during surgery or using a needle inserted through your skin.
If you have severe, persistent or frequently recurring UTIs, you may need surgery to remove 1 or both of your kidneys, followed by dialysis or a kidney transplant.
You'll have blood tests at varying intervals to monitor your kidney function.
You should discuss with your doctors what treatment you'd like if your condition reaches a stage where your kidneys stop working altogether (kidney failure).
The 2 main treatment options for kidney failure are:
You only need 1 kidney to survive. This means that unlike other types of organ donation, a living person can donate a kidney.
Close relatives usually make the best match, so you may want to see if a relative would consider being tested to find out whether they're a suitable donor.
A few people with kidney failure decide not to have dialysis or a kidney transplant, preferring simple treatment of their symptoms.
For example, a person may choose this option if it's unlikely that dialysis will significantly prolong their life or improve their quality of life.
If this choice is made, supportive treatment to help control symptoms will be provided to make the end of life as comfortable as possible.
Tolvaptan is a medication that's recommended by the National Institute for Health and Care Excellence (NICE) to treat ADPKD in adults.
It can be used to slow down the growth of cysts, reducing overall kidney growth and preserving kidney function for longer.
But tolvaptan can only be used in adults who have:
Tolvaptan comes in tablet form and is taken twice a day as a split dose.
Common side effects include:
Chemical-related liver damage (hepatotoxicity) has also been reported in some people taking tolvaptan for ADPKD.
If you're taking tolvaptan, you'll need to initially be monitored frequently through hospital clinics.
You can read more about the use of tolvaptan for treating ADPKD on the BNF website.
If you have ADPKD, your kidneys will be more vulnerable to injury. For example, a sudden knock or blow to your kidneys could cause the cysts to split and bleed, leading to severe and intense pain.
Avoiding all types of contact sport, such as rugby and football, is usually recommended.