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Most management options help with symptoms, but do not treat the cause, of primary hyperoxaluria (PH)

Here is what management of PH symptoms looks like today:

Oxalate and crystal reduction


Drinking a lot of water every day is very important for people with PH. This is called hyperhydration.


All that water dilutes the urine and reduces the chance for the formation of calcium oxalate crystals; these crystals can lead to kidney stones and kidney damage.

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How does hyperhydration work in infants and small children? Infants and small children struggling to drink enough water may need a gastrostomy tube, also known as a G-tube. Talk to your child’s healthcare provider to determine if a G-tube may be necessary.

How much water do you need to drink?

Want a hyperhydration guide for kids to print or save?

Download the guide

Want tips for managing the hydration routine for a child?

What parents can do

In people with PH, food isn’t the main source of high oxalate; oxalate levels increase within the body due to a genetic mutation that causes the liver to make too much oxalate.

Limiting the consumption of foods that contain high amounts of oxalate may have a small effect in reducing oxalate levels. To be safe, however, foods that are high in oxalate, including the following, should be eaten in moderation:

  • Beets
  • Chocolate
  • Miso
  • Nuts
  • Peanuts
  • Potatoes
  • Raspberries
  • Rhubarb
  • Spinach
  • Sweet potatoes
  • Swiss chard
  • Wheat bran

Be sure to have a conversation with your healthcare provider about if and what diet modifications are needed.

Want more information about following a low-oxalate diet? The University of Chicago is a great resource.

Crystal inhibitors

A crystal inhibitor is a treatment taken by mouth that may help prevent crystals in your kidneys and maintain your kidney function.

Examples of crystal inhibitors that your healthcare provider may prescribe include potassium citrate and sodium citrate.

Oxalate reduction – PH1 only

Vitamin B6

Also called pyridoxine, vitamin B6 pills can help reduce oxalate levels in certain people with PH1. Vitamin B6 can be gotten over the counter and is taken by mouth every day.

There are many different mutations that can cause PH. Research studies have shown that vitamin B6 may work in people with these specific PH1 mutations:

  • p.Gly170Arg (~30% of people with PH1)
  • p.Phe152Ile
  • p.Ile244Thr
RNAi therapy

RNAi therapy, also called RNA interference therapy, is a treatment that interferes with messenger RNA, the chemical messengers in your cells that give instructions for making proteins. By interfering with messenger RNA, RNAi treatments help prevent these proteins from being made.

An RNAi therapy for PH1 has been approved by the US Food and Drug Administration. RNAi therapy is also being investigated in PH2 and PH3.

Stone management

Nonsurgical kidney stone management

Kidney stones are common in people with PH, but they don’t always need to be treated with surgical procedures.

Depending on the size and location, you may be able to pass a kidney stone without surgery. Your healthcare provider may recommend pain medication and lots of fluids to help you pass it. They could also recommend medication that relaxes the ureter to help the stone pass. Stones may take up to 3 weeks to pass.

Surgical treatment for kidney stones

Sometimes stones are too big to pass on their own. In these cases, healthcare providers will recommend surgery. There are 3 procedures commonly used to remove stones, and they all require general anesthesia and a short hospital stay.


Ureteroscopy is a procedure using a very small instrument to gain access to the stone via the ureter. The instrument is then used to break up the stone mechanically or with a laser. Next, the pieces are flushed out or extracted through the ureter.

Learn more about ureteroscopy from the National Kidney Foundation and the Cleveland Clinic.

Percutaneous nephrolithotomy (PCNL) is a stone removal surgery in which a healthcare provider uses instruments to access the kidney through a small incision in the back. Then they break up the stone mechanically or with a laser and remove the pieces.

Learn more about PCNL from the National Kidney Foundation and the Cleveland Clinic.

Extracorporeal shock wave lithotripsy (ESWL) is a noninvasive procedure in which shock waves are used to break up the kidney stone. The stone fragments can then be passed in the urine.


Though it is still often used, ESWL is not the preferred treatment for people with PH. This is because PH kidney stones are harder than other kinds of kidney stones, so they are not broken up as well with ESWL. Because of this, ESWL could leave larger fragments behind, which can grow into new kidney stones. ESWL can also have a low success rate for removing stones in children.

Learn more about ESWL from the National Kidney Foundation and the Cleveland Clinic.

Advanced kidney disease


In people with PH, sometimes the kidneys have become so damaged by calcium oxalate buildup that they can’t do the job of filtering out waste products from the blood. Dialysis is a medical procedure that does the job of the kidneys and filters out the wastes your body doesn’t need.

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Remember: Not everyone with PH will need dialysis. Dialysis is for people who have severe kidney damage, where the kidneys are no longer able to filter out wastes on their own.

There are 2 kinds of dialysis:

  • Intermittent hemodialysis uses a machine to filter waste from the blood and may be required as often as 6 days per week for those with PH. Dialysis sessions last for about 4 hours 
  • Peritoneal dialysis fills the abdomen (belly) with a special solution to absorb waste from the blood. Many people on dialysis with PH may have peritoneal dialysis in addition to intermittent hemodialysis. Peritoneal dialysis may be done 3 to 5 times a day and each session takes about 30 to 40 minutes

Dialysis isn’t a cure; it won’t be able to flush out all of the excess oxalate in people with PH—but it can help reduce it, and that can be very important. In people with PH whose kidneys are severely damaged, dialysis may help delay or prevent systemic oxalosis.


Learn more about dialysis from the National Institutes of Health (NIH) or the American Association of Kidney Patients.

Kidney and liver transplant

A dual liver-kidney transplant is the only known cure for PH and may be recommended in severe cases for people with PH and advanced chronic kidney disease (CKD) who need (or will soon need) dialysis.


Transplanting both organs is usually required because both the liver and kidneys are affected in PH. A kidney transplant replaces the damaged kidney that is no longer functioning. The liver is often transplanted, as well, because otherwise the liver with PH will continue to make too much oxalate, eventually causing damage to the newly transplanted kidney.

Life after transplant

After your kidney transplant, your new kidney will filter your blood.


When you get a transplanted liver or kidney, there is a chance that your body will reject (fight) the new organ. To prevent your immune system from doing that, you’ll need to take drugs called immunosuppressants (antirejection medications). Even though immunosuppressants cause side effects, they are very necessary because they help keep your new kidney (and liver, if you have a liver transplant) healthy and working.


Getting a transplant is a major life change, so it’s normal to have all kinds of emotions afterward. Seeking the support of friends and family members can help you cope during this time. Your transplant team can also refer you to a mental health specialist if you need additional support.


Learn more about kidney transplants in adults from the American Association of Kidney Patients, the American Kidney Fund, and the National Kidney Foundation.


Learn more about kidney transplants in children from the American Kidney Fund.


Learn more about liver transplants from the Cleveland Clinic and the National Institute of Diabetes and Digestive and Kidney Diseases.

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    "[I]f you’ve ever had a kidney stone, it’s something you’ll never forget—let alone, if you have them chronically. When I have a kidney stone, it just drops me to the ground in pain.… All I know is I want to get out of my own body, and you never know when it’s going to hit.”

    – Diagnosed with PH1 at age 38

    How do I know the status of my kidneys?

    There are several tests that can be used to monitor your kidney function and oxalate levels.

    See tests

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