Hyperoxaluria Awareness Week is November 3-8, 2025.
Primary hyperoxaluria (PH) is a rare genetic disorder that has three types: PH1, PH2 and PH3. Fewer than 5,000 people in the United States suffer from PH, many of whom are children.
Your liver produces a waste product called oxalate. When you have PH, you produce too much oxalate, which builds up and causes kidney stones. Unfortunately, many people who suffer from this disease don’t realize it until their kidneys stop working. Eight out of ten people with PH have PH1, the most severe form of primary hyperoxaluria, which causes the most severe symptoms.
Treatment and management of primary hyperoxaluria
Primary hyperoxaluria cannot be cured, but if you can slow oxalate formation, you may be able to prevent or reduce kidney stone formation, thereby protecting your kidneys for as long as possible. The sooner you are diagnosed and treated, the better.
Treatment options include:
If you have PH, it is important to drink more water and other fluids. The more urine you produce, the harder it is for oxalate to clump together and form kidney stones. The general recommendation is to drink at least 3 liters (just over 12 cups) of water throughout the day. If you don’t want to drink water, there are other ways to get fluids, such as soups, fruits, vegetables, and other foods.
Children with PH also need plenty of fluids, but their doctor should tell you how much they need depending on their age and size. In infants and very young children, a tube may need to be inserted through the nose into the stomach (nasogastric) or surgically inserted through a small opening in the abdomen (gastrostomy) to administer the fluid.
As PH progresses, kidney or heart failure may occur, meaning you may need to drink less fluids. In this case, talk to your doctor about what your daily fluid intake should be.
Oxalate is found in plant foods, which can include many generally healthy foods. Some of your staple foods may now be on the list to avoid. A nutritionist can help you adjust your diet, provide ideas for food replacements, and support you as you transition to this new diet. If you’re having trouble affording food, ask your dietitian about local resources.
These are just a few healthy foods high in oxalates that you should avoid:
- Beets
- bran
- Peanut butter
- spinach
- tea
- Almonds, cashews and some other nuts
And some treats contain a lot of oxalate and should therefore also be avoided:
- Chocolate
- Dark beer
- French fries
- Potato chips
Processed foods can also contain oxalate, which is another reason to avoid processed products.
It’s a good idea to add calcium-rich foods to your diet. Calcium helps by preventing oxalate from being absorbed into your bloodstream. Dairy products are the best food group for calcium. (If you have lactose intolerance, you can often find lactose-free products that still help meet your calcium needs.) Your dietitian can also give you other ideas for calcium-rich foods.
Some supplements can help control oxalate levels. These can include:
- Vitamin B6, also called pyridoxine, helps about three in ten people with PH by lowering oxalate levels. The daily B6 recommendation for most adults is between 1.3 and 1.7 milligrams (mg) per day, but for people with PH, doses are much higher. Your doctor can help you determine the dose you should take and monitor you closely to make sure you don’t develop toxic levels.
- Supplements containing citrates or magnesium can help prevent oxalate from clumping. They work in different ways but may be effective for some people with PH.
People with PH should avoid all supplements that contain vitamin C. Your body converts excess vitamin C into oxalate, which you don’t want.
Your liver produces oxalate, so your doctor may prescribe one of two injected medications that lower the amount produced:
- Lumasiran (Oxlumo)
- Nedosiran (Rivfloza)
Diuretics, i.e. drugs that stimulate urine production, are usually not part of the initial treatment because they can have a dehydrating effect. However, you may need a diuretic – often called a water pill – if your urine has high calcium levels.
Kidney stones can cause severe pain and block the flow of urine. If they cannot pass naturally, the stones must be removed. Extracorporeal shock wave lithotripsy, a noninvasive procedure that uses shock waves, is a common procedure for breaking down kidney stones, but is often not as effective in people with PH.
If you have large or multiple stones, your doctor may recommend a surgery called percutaneous nephrolithotomy. If you have smaller stones or they are located in your ureters, the thin tubes that connect your kidneys to your bladder, your doctor may perform a ureteroscopy. This involves inserting a narrow tube called a ureteroscope into your urethra, through your bladder and into the ureter. At the end of the tube there is a small basket to hold the stones.
Treatment of end-stage kidney disease
PH1 and sometimes PH2 can lead to end-stage kidney disease. When this happens, your kidneys cannot do their job, leading to kidney failure and forcing you to undergo dialysis several times a week. The most common method is hemodialysis. A catheter is inserted into your arm and the blood flows into a machine that cleanses your blood of toxins. The purified blood then flows back into your body. Unfortunately, this is not a cure for kidney failure, and your doctor may ask you to consider a liver transplant and possibly a kidney transplant if still necessary. Because your liver is responsible for overproducing oxalate, a new one can stop the overproduction and your kidney function could improve after a liver transplant. If not, your doctor may recommend a kidney transplant at this point.
Lifestyle changes can help treat primary hyperoxaluria
Although PH cannot be cured, adjusting your diet and taking nutritional supplements and/or prescription medications can help manage the disease. Working closely with your HCP and a nutritionist can help make the process a little easier.
This educational resource was created with support from Alnylam.
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