November 3–8, 2025 is Hyperoxaluria Consciousness Week.
Major hyperoxaluria (PH) is a uncommon genetic illness that has three sorts: PH1, PH2 and PH3. Lower than 5,000 individuals within the U.S. have PH, lots of whom are youngsters.
Your liver produces a waste product known as oxalate — when you’ve got PH, you overproduce oxalate and it builds up, inflicting kidney stones. Sadly, many individuals who’ve the illness don’t understand it till their kidneys cease working. Eight out of 10 individuals with PH have PH1, which is essentially the most critical sort of main hyperoxaluria, and it causes essentially the most extreme signs.
Treating and managing main hyperoxaluria
Major hyperoxaluria can’t be cured, however when you can gradual the oxalate buildup, you may forestall or scale back the variety of kidney stones that type, defending your kidneys for so long as doable. The sooner you’re identified and handled, the higher.
Therapy choices embody:
In case you have PH, it’s vital to drink extra water and different fluids. The extra urine you make, the tougher it’s for oxalate to clump collectively and make kidney stones. The final suggestion is no less than 3 liters (somewhat over 12 cups) of water all through the day. If you happen to don’t wish to drink water, there are different methods you may get fluids, like by way of soups, fruits, greens and different meals.
Kids with PH want a whole lot of fluid too, however their HCP ought to inform you how a lot they want, relying on age and measurement. Infants and really younger youngsters may want a tube inserted by way of the nostril to the abdomen (nasogastric) or surgically inserted by way of a tiny opening within the stomach (gastrostomy) to provide the fluids.
As PH progresses, it might trigger kidney or coronary heart failure, which implies you may need to cut back how a lot fluid you drink. If this occurs, discuss along with your HCP about what your every day fluid consumption needs to be.
Oxalate is present in plant meals, which can embody many meals which are typically wholesome. A few of your staples could now be on the “needs to be averted” listing. A dietitian can assist you adapt your weight loss program, give concepts for alternative meals and supply help as you navigate this new method of consuming. If you happen to’re having bother affording the meals, ask your dietitian for native sources.
These are just some wholesome meals which are excessive in oxalate that you must keep away from:
- Beets
- Bran
- Peanut butter
- Spinach
- Tea
- Almonds, cashews and another nuts
And a few treats are excessive in oxalate, so additionally needs to be averted:
- Chocolate
- Darkish beer
- French fries
- Potato chips
Processed meals can even include oxalate, in order that’s one more reason to keep away from processed merchandise.
Including calcium-rich meals to your weight loss program is a good suggestion. Calcium helps by protecting the oxalate from getting absorbed into your bloodstream. Dairy is the very best meals group for calcium. (If you happen to’re lactose illiberal, you may typically discover lactose-free merchandise that also assist meet your calcium wants.) Your dietitian may give you different concepts for high-calcium meals as effectively.
Some dietary supplements may assist handle oxalate ranges. These can embody:
- Vitamin B6, additionally known as pyridoxine, helps about 3 out of 10 individuals with PH by decreasing oxalate ranges. The every day B6 suggestion for many adults is between 1.3 and 1.7 milligrams (mg) per day, however for individuals with PH, doses are a lot greater. Your HCP can assist inform you what dose you must take and comply with you carefully to ensure you don’t develop poisonous ranges.
- Dietary supplements which have citrates or magnesium can assist forestall oxalate from clumping collectively. They work in several methods however might be efficient for some individuals with PH.
Individuals with PH ought to keep away from any dietary supplements that embody vitamin C. Your physique converts extra vitamin C into oxalate, one thing you don’t need.
Your liver produces oxalate, so your HCP could prescribe considered one of two injected drugs that decrease the quantity it makes:
- Lumasiran (Oxlumo)
- Nedosiran (Rivfloza)
Diuretics, medication that makes you produce urine, aren’t normally a part of the first-line remedy as a result of they are often dehydrating. Nevertheless, you may want a diuretic — typically known as a water tablet — when you’ve got excessive calcium ranges in your urine.
Kidney stones could cause extreme ache and block urine move. If they will’t move naturally, the stones have to be eliminated. Extracorporeal shock wave lithotripsy, a noninvasive process that makes use of shock waves, is a typical process for breaking down kidney stones, however it’s typically not as efficient in individuals with PH.
In case you have massive or a number of stones, your HCP could advocate a surgical procedure known as percutaneous nephrolithotomy. In case you have smaller stones or they’re in your ureters, the skinny tubes that join your kidneys to your bladder, your HCP may do a ureteroscopy. This includes inserting a slender tube known as a ureteroscope into your urethra, by way of your bladder and into the ureter. The tube has a small basket on the finish to seize the stones.
Treating end-stage renal illness
PH1 and typically PH2 can progress to end-stage renal illness. If that occurs, your kidneys can’t do their job, which ends up in kidney failure and requires you to have dialysis a couple of instances every week. The commonest methodology is hemodialysis. A catheter is positioned in your arm and blood flows out right into a machine that cleans the toxins out of your blood. The cleaned blood then flows again into your physique. Sadly, this isn’t a treatment for kidney failure, and your HCP could ask you to think about having a liver transplant and presumably a kidney transplant if nonetheless wanted.Since your liver is chargeable for overproducing oxalate, a brand new one could cease the overproduction, and your kidneys may enhance after a liver transplant. If not, your HCP may advocate a kidney transplant at that time.
Life-style modifications can assist handle main hyperoxaluria
Though PH can’t be cured, adjusting your weight loss program and taking dietary supplements and/or prescription drugs could assist handle the illness. Working carefully along with your HCP and a dietitian can assist make the method somewhat simpler.
This instructional useful resource was created with help from Alnylam.
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