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The days between November 3rd and 8th, 2025 are Hyperoxaluria Awareness Week.
Primary hyperoxaluria (PH) is a rare genetic disorder that has three types: HP1, HP2 and HP3. 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. If you suffer from HP, you will overproduce oxalate, which builds up and causes kidney stones. Unfortunately, many people who suffer from this disorder 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 control of primary hyperoxaluria
Primary hyperoxaluria has no cure, but if you can stop the buildup of oxalate, you could prevent or reduce the formation of kidney stones, thereby protecting your kidneys as much as possible. The sooner you are diagnosed and treated, the better.
Therapeutic options include:
- liquidsIf you have HP, it is important that you drink more water and other fluids. The more urine you pass, the harder it is for oxalate to clump together and cause kidney stones. The general recommendation is 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 consume liquids such as soups, fruits, vegetables, and other foods.
Children with PH also need plenty of fluids, but their doctor should tell them how much they need depending on their age and size. Infants and very young children may need to have a tube inserted through the nose into the stomach (nasogastric) or surgically inserted through a small opening in the abdomen (gastrostomy) to provide them with fluids.
As PH progresses, kidney or heart failure may occur, meaning you may need to reduce the amount of fluids you drink. In this case, talk to your doctor about how much fluid you should drink.
- NutritionOxalate is found in plant foods, including many foods that are generally considered healthy. Now you may need to avoid some of your staple foods. A nutritionist can help you change your diet, give you ideas for replacement foods, and support you as you transition to the new diet. If you’re having trouble paying for your groceries, talk to your dietitian about local resources.
There are few healthy foods that are high in oxalate that you should avoid:
- Beets
- bran
- Peanut butter
- spinach
- The
- Almonds, cashews and other nuts
And some treats are high in oxalates, so you should avoid them:
- Chocolate
- dark beer
- French fries
- Melt the french fries
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 is helpful in preventing oxalate from being absorbed into your bloodstream. Dairy products are the best food group when it comes to calcium (if you are lactose intolerant, you can find lactose-free products that will also help meet your calcium needs). Your dietitian may be able to give you other ideas for foods high in calcium.
- SupplementsSome supplements may be helpful in controlling oxalate levels. These can include:
- Vitamin B6, also called pyridoxine, is helpful for about three in ten people with PH by lowering their oxalate levels. The daily recommendation for vitamin B6 for most adults is between 1.3 and 1.7 milligrams (mg) per day, but for people with PH, recommended doses are much higher. Your doctor can help you determine the dosage you should take and should monitor you closely to make sure you are not experiencing toxic levels.
- Supplements containing citrates or magnesium may be helpful in preventing oxalate from clumping. They work in different ways but may be effective for some people with HP
People with PH should avoid all supplements that contain vitamin C. Your body converts excess vitamin C into oxalate, which is not practical.
- Prescription medicationsYour liver produces oxalate, so your doctor may prescribe one or two injectable medications that reduce oxalate production:
- Lumasiran (Oxlumo)
- Nedosiran (Rivfloza)
Diuretics, drugs that stimulate urine production, are usually not a first-line treatment because they can lead to dehydration. However, you may need a diuretic to eliminate fluids if your urine has high calcium levels.
- Removal of kidney stonesKidney 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 up kidney stones, but is often not as effective in people with PH.
If you have large stones, your doctor may recommend a surgery called a percutaneous nephrolithotomy. If you have smaller stones or they are located in your urethra, the thin tubes that connect your kidneys to your bladder, your doctor may perform a ureteroscopy. This involves inserting a thin tube called a ureteroscope into your urethra, through your bladder and into your uterus. The probe has a small basket at one end for collecting rocks.
Treatment of end-stage renal failure
HP1 and sometimes HP2 can progress to end-stage kidney failure. When this happens, your kidneys stop working properly, leading to kidney failure and you have to undergo dialysis several times a week. The most common method is hemodialysis. A catheter is inserted into your arm and blood is sucked into a machine that cleans the toxins in your blood. Clean blood returns to your body. Unfortunately, this is not a cure for kidney failure and your doctor may ask you to consider a liver transplant and, if necessary, a kidney transplant.
Because your liver overproduces oxalate, a new one could stop the overproduction and your kidney function could improve after a liver transplant. Otherwise, your doctor may recommend a kidney transplant at this time.
Lifestyle changes could be useful to control primary hyperoxaluria
Although there is no cure for PH, changing your diet and taking supplements or prescription medications can help manage the condition. Working closely with your doctor and a nutritionist can be helpful in making the process a little easier.
This educational resource was created with support from Alnylam.
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