November 3–8, 2025 is Hyperoxaluria Awareness Week.
Main hyperoxaluria (PH) is a uncommon genetic illness that has three sorts: PH1, PH2 and PH3. Less than 5,000 people in the U.S. have PH, a lot of whom are youngsters.
Your liver produces a waste product referred to as oxalate — if in case you have PH, you overproduce oxalate and it builds up, inflicting kidney stones. Sadly, many individuals who’ve the illness don’t comprehend it till their kidneys cease working. Eight out of 10 individuals with PH have PH1, which is probably the most critical sort of main hyperoxaluria, and it causes probably the most extreme signs.
Treating and managing main hyperoxaluria
Main hyperoxaluria can’t be cured, however in the event you can sluggish the oxalate buildup, you may stop or scale back the variety of kidney stones that type, defending your kidneys for so long as doable. The sooner you’re recognized and handled, the higher.
Remedy choices embody:
When you have PH, it’s necessary to drink extra water and different fluids. The extra urine you make, the more durable it’s for oxalate to clump collectively and make kidney stones. The final suggestion is at the least 3 liters (just a little over 12 cups) of water all through the day. Should you don’t prefer to drink water, there are other ways you can get fluids, like by means of soups, fruits, greens and different meals.
Youngsters with PH want a number 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 means of the nostril to the abdomen (nasogastric) or surgically inserted by means of a tiny opening within the stomach (gastrostomy) to present the fluids.
As PH progresses, it will possibly trigger kidney or coronary heart failure, which suggests you might need to scale back how a lot fluid you drink. If this occurs, discuss together with your HCP about what your day by day fluid consumption must be.
Oxalate is present in plant meals, which can embody many meals which can be typically wholesome. A few of your staples might now be on the “must be averted” checklist. A dietitian may also help you adapt your food plan, give concepts for alternative meals and supply assist as you navigate this new manner of consuming. Should you’re having hassle affording the meals, ask your dietitian for native assets.
These are just some wholesome meals which can be excessive in oxalate that it’s best to keep away from:
- Beets
- Bran
- Peanut butter
- Spinach
- Tea
- Almonds, cashews and another nuts
And a few treats are excessive in oxalate, so additionally must be averted:
- Chocolate
- Darkish beer
- French fries
- Potato chips
Processed meals may also comprise oxalate, in order that’s another excuse to keep away from processed products.
Including calcium-rich meals to your food plan is a good suggestion. Calcium helps by maintaining the oxalate from getting absorbed into your bloodstream. Dairy is the perfect meals group for calcium. (Should you’re lactose intolerant, you may usually discover lactose-free merchandise that also assist meet your calcium wants.) Your dietitian may give you different concepts for high-calcium meals as properly.
Some dietary supplements may assist handle oxalate ranges. These can embody:
- Vitamin B6, additionally referred to as pyridoxine, helps about 3 out of 10 individuals with PH by decreasing oxalate ranges. The day by 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 increased. Your HCP may also help inform you what dose it’s best to take and comply with you intently to ensure you don’t develop poisonous ranges.
- Dietary supplements which have citrates or magnesium may also help stop oxalate from clumping collectively. They work in numerous methods however may be efficient for some individuals with PH.
Folks 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 might prescribe considered one of two injected medicines that decrease the quantity it makes:
- Lumasiran (Oxlumo)
- Nedosiran (Rivfloza)
Diuretics, drugs that makes you produce urine, aren’t often a part of the first-line therapy as a result of they are often dehydrating. Nonetheless, you may want a diuretic — usually referred to as a water tablet — if in case you have excessive calcium ranges in your urine.
Kidney stones may cause extreme ache and block urine circulation. If they will’t move naturally, the stones should be eliminated. Extracorporeal shock wave lithotripsy, a noninvasive process that makes use of shock waves, is a typical process for breaking down kidney stones, but it surely’s usually not as efficient in individuals with PH.
When you have giant or a number of stones, your HCP might suggest a surgical procedure referred to as percutaneous nephrolithotomy. When 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 entails inserting a slender tube referred to as a ureteroscope into your urethra, by means 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 generally PH2 can progress to end-stage renal illness. If that occurs, your kidneys can’t do their job, which leads to kidney failure and requires you to have dialysis a number of occasions every week. The most typical technique 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 might ask you to contemplate having a liver transplant and probably a kidney transplant if nonetheless wanted.Since your liver is liable for overproducing oxalate, a brand new one might cease the overproduction, and your kidneys may enhance after a liver transplant. If not, your HCP may suggest a kidney transplant at that time.
Life-style modifications may also help handle main hyperoxaluria
Though PH can’t be cured, adjusting your food plan and taking dietary supplements and/or prescription medicines might assist handle the illness. Working intently together with your HCP and a dietitian may also help make the method just a little simpler.
This academic useful resource was created with assist from Alnylam.
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