Treatment and Management of Primary Hyperoxaluria

November 3–8, 2025 is Hyperoxaluria Awareness Week.

Primary hyperoxaluria (PH) is a rare genetic disease that has three types: PH1, PH2 and PH3. Less than 5,000 people in the U.S. have PH, many of whom are children.

Your liver produces a waste product called oxalate — if you have PH, you overproduce oxalate and it builds up, causing kidney stones. Unfortunately, many people who have the disease don’t know it until their kidneys stop working. Eight out of 10 people with PH have PH1, which is the most serious type of primary hyperoxaluria, and it causes the most severe symptoms.

​Treating and managing primary hyperoxaluria

Primary hyperoxaluria can’t be cured, but if you can slow the oxalate buildup, you might prevent or reduce the number of kidney stones that form, protecting your kidneys for as long as possible. The earlier you’re diagnosed and treated, the better.

Treatment options include:

• Fluids

If you have PH, it’s important to drink more water and other fluids. The more urine you make, the harder it is for oxalate to clump together and make kidney stones. The general recommendation is at least 3 liters (a little over 12 cups) of water throughout the day. If you don’t like to drink water, there are other ways you can get fluids, like through soups, fruits, vegetables and other foods.

Children with PH need a lot of fluid too, but their HCP should tell you how much they need, depending on age and size. Infants and very young children might need a tube inserted through the nose to the stomach (nasogastric) or surgically inserted through a tiny opening in the abdomen (gastrostomy) to give the fluids.

As PH progresses, it can cause kidney or heart failure, which means you might have to reduce how much fluid you drink. If this happens, talk with your HCP about what your daily fluid intake should be.

• Diet

Oxalate is found in plant foods, which may include many foods that are generally healthy. Some of your staples may now be on the “should be avoided” list. A dietitian can help you adapt your diet, give ideas for replacement foods and provide support as you navigate this new way of eating. If you’re having trouble affording the food, ask your dietitian for local resources.

These are just a few healthy foods that are high in oxalate that you should avoid:

• Beets
• Bran
• Peanut butter
• Spinach
• Tea
• Almonds, cashews and some other nuts

And some treats are high in oxalate, so also should be avoided:

• Chocolate
• Dark beer
• French fries
• Potato chips

Processed foods can also contain oxalate, so that’s another reason to avoid processed products.

Adding calcium-rich foods to your diet is a good idea. Calcium helps by keeping the oxalate from getting absorbed into your bloodstream. Dairy is the best food group for calcium. (If you’re lactose intolerant, you can often find lactose-free products that still help meet your calcium needs.) Your dietitian can give you other ideas for high-calcium foods as well.

• Supplements

Some supplements might help manage oxalate levels. These can include:

• Vitamin B6, also called pyridoxine, helps about 3 out of 10 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 HCP can help tell you what dose you should take and follow you closely to make sure you don’t develop toxic levels.
• Supplements that have citrates or magnesium can help prevent oxalate from clumping together. They work in different ways but can be effective for some people with PH.

People with PH should avoid any supplements that include vitamin C. Your body converts excess vitamin C into oxalate, something you don’t want.

• Prescription medications

Your liver produces oxalate, so your HCP may prescribe one of two injected medications that lower the amount it makes:

• Lumasiran (Oxlumo)
• Nedosiran (Rivfloza)

Diuretics, medicine that makes you produce urine, aren’t usually part of the first-line treatment because they can be dehydrating. However, you might need a diuretic — often called a water pill — if you have high calcium levels in your urine.

• Removing kidney stones

Kidney stones can cause severe pain and block urine flow. If they can’t 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 it’s often not as effective in people with PH.

If you have large or several stones, your HCP may recommend a surgery called percutaneous nephrolithotomy. If you have smaller stones or they’re in your ureters, the thin tubes that connect your kidneys to your bladder, your HCP might do a ureteroscopy. This involves inserting a narrow tube called a ureteroscope into your urethra, through your bladder and into the ureter. The tube has a small basket at the end to grab the stones.

​Treating end-stage renal disease

PH1 and sometimes PH2 can progress to end-stage renal disease. If that happens, your kidneys can’t do their job, which results in kidney failure and requires you to have dialysis a few times a week. The most common method is hemodialysis. A catheter is placed in your arm and blood flows out into a machine that cleans the toxins from your blood. The cleaned blood then flows back into your body. Unfortunately, this isn’t a cure for kidney failure, and your HCP may ask you to consider having a liver transplant and possibly a kidney transplant if still needed.Since your liver is responsible for overproducing oxalate, a new one may stop the overproduction, and your kidneys might improve after a liver transplant. If not, your HCP might recommend a kidney transplant at that point.

​Lifestyle changes can help manage primary hyperoxaluria

Although PH can’t be cured, adjusting your diet and taking supplements and/or prescription medications may help manage the disease. Working closely with your HCP and a dietitian can help make the process a little easier.

This educational resource was created with support from Alnylam.