Treatments for Primary Hyperoxaluria Type 1

When you have primary hyperoxaluria type 1 (PH1), too much of a natural substance called oxalate collects in your kidneys. To prevent the serious kidney damage that can result, your doctor will try to reduce the buildup of oxalate in your body. 

Your doctor will probably start by telling you to drink plenty of liquids. People with PH1 need fluids to flush out their kidneys and help stop oxalates from building up.

Your doctor may also recommend one or more of these treatments.

Medications

Vitamin B6 ( pyridoxine) . When taken in prescription-level doses, this vitamin can greatly reduce the amount of oxalate in your urine. It doesn’t work for everyone, though.

Potassium citrate or pyrophosphate. These salts work by making your urine more alkaline (shifting the pH balance). This makes oxalate stones less likely to form. Your doctor will probably tell you to take one or more of them several times throughout the day. 

Thiazide diuretics. In some cases, your doctor might prescribe these "water pills" to make you pee more often. When your doctor prescribes a diuretic, it's especially important to drink lots of fluids so you don't get dehydrated.

Procedures

Gastrostomy. Babies and young children may need this procedure to make sure they're getting enough fluids to keep their urine well-diluted and prevent kidney damage. In this operation, a doctor puts a feeding tube into the child's stomach through a small cut in their belly. This allows fluids, formula, or medicine to pass directly into their body. 

IV fluids. If you have an illness that keeps you from drinking enough liquids, you may need to get fluids through an IV.

Dialysis and Transplants

If these treatments don't stop your condition from getting worse, you might eventually need dialysis or a combined kidney and liver transplant. 

Dialysis. When medication and lifestyle changes don't help enough, your kidneys eventually stop working. You may need to get hemodialysis, perhaps for several hours a day. It helps to remove the extra oxalate from your blood, as well as clean the blood and remove fluid like your kidneys would normally do.

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This type of dialysis removes more oxalate than peritoneal dialysis, in which a cleansing fluid runs through a tube in your abdomen.

Liver transplant. The only cure for primary hyperoxaluria type 1 is a liver transplant. A transplanted liver from a healthy donor will make the enzymes your body needs to break down oxalate.

Because PH1 often damages the kidneys, too, some people may need both a liver and kidney transplant. These operations can be done at the same time, or you can have a liver transplant followed by a kidney transplant. 

A transplant has risks, though:

  • Your body might reject the transplanted organ.
  • You could get an infection after surgery.
  • You could have side effects from the drugs you take to help your body accept the organ.

Your doctor can help you weigh the pros and cons. 

WebMD Medical Reference Reviewed by Minesh Khatri, MD on May 06, 2020

Sources

SOURCES:

Clinical Journal of American Society of Nephrology: "Vitamin B6 in Primary Hyperoxaluria I: First Prospective Trial after 40 Years of Practice."

Gene Reviews: "Primary Hyperoxaluria Type 1."

Mayo Clinic: “Hyperoxaluria and Oxalosis.”

National Institutes of Health Genetics Home Reference: “Primary Hyperoxaluria.”

National Institutes of Health Genetic and Rare Diseases Information Center: " Primary hyperoxaluria type 1."

National Organization for Rare Diseases: “Primary Hyperoxaluria.”

Oxalosis & Hyperoxaluria Foundation: "Diagnosis and Treatment."

Rare Diseases Network: "Rare Kidney Stone Diseases in Depth."

UpToDate: “Primary Hyperoxaluria.”

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