Treatment

There's currently no cure for autosomal dominant polycystic kidney disease (ADPKD), and it isn't possible to stop cysts forming in the kidneys.

However,there are some potentially useful medications, such as tolvaptan, that can sometimes be used to reduce the growth rate of cysts(see below).

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 would want to do if your kidneys stop working sufficiently (kidney failure).

Some of the main treatments are described below.

High blood pressure

Medication is usually used to treat high blood pressurein people with ADPKD. Angiotensin-converting enzyme (ACE) inhibitors and angiotensin-2 receptor blockers (ARBs) are the two 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 (0.2oz) a day (6g of salt is about one teaspoonful).

If your pain is particularly severe, you may be prescribed a stronger painkiller, such as codeine or tramadol.

It's usually recommended that non-steroidal anti-inflammatory drugs (NSAIDs) , such as ibuprofen , are avoided because they could disrupt kidney function and interfere with medications prescribed to control your blood pressure. However, 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.

Kidney stones

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 sick or vomiting. 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 needtreatment to help remove it. Possible treatment options include:

• using energy waves to break the stone into smaller pieces this is known as extracorporeal shock wave lithotripsy (ESWL)
• passinga thin telescopic instrument called aureteroscope up your urethra (the tube that carries urine out of the body)to remove or break up the stone

Paracetamol can be used to relieve pain.

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 usinga needle inserted through your skin.

If you have severe, persistent or frequently recurring UTIs, you may need surgery to remove one or both of your kidneys, followed by dialysis or a kidney transplant (see below).

Kidney failure

You'll have blood tests at varying intervals to monitor your kidney function. You should discuss with your doctors what treatment you'd likeif your condition reaches a stage where your kidneys stop working altogether (kidney failure).

The two main treatment options for kidney failure are:

• dialysis where a machine replicates some of your kidneys' functions
• kidney transplant where a healthy kidney is removed from a living or recently deceaseddonor and implanted into someone with kidney failure

You only need one kidney to survive. Therefore, 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 optionif it's unlikely thatdialysis 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

Tolvaptan is a medication that's recommended by the National Institute forHealth 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.However, tolvaptan can only be usedin adults who have:

• chronic kidney disease (stage two or three)at the start of treatment
• evidence of rapidly progressing kidney disease

Tolvaptan comes in tablet form and is taken twice a day as a split dose. Common side effects include:

• thirst
• passing more than three litres (just over five pints)of urine a day(polyuria)
• the need to urinate frequently more than four or five times during the day and more than once at night (pollakiuria)
• the need to pass urine at night (nocturia)

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 NICE website.