management
Managing PH1 takes a team
Primary hyperoxaluria type 1 (PH1) requires lifelong management and monitoring. A well-coordinated care team may help you approach the different aspects that managing PH1 can involve.
Since PH1 is a personal experience and affects everyone differently, you and your care team will work together to create a personalized plan. The makeup of your care team may include the healthcare providers listed below.
Your care team in your corner:
Some of the specialists you may meet in your journey with primary hyperoxaluria type 1 (PH1)
Nephrologist
Kidney specialist
A nephrologist specializes in diseases like PH1 that affect the kidneys, and may have a special focus in either children or adults. You and your nephrologist will create a personalized management plan tailored to your PH1 symptoms and progression.
Along the way, other specialists may play a part in managing your PH1.
Nephrologist
Urologist
A surgeon who specializes in disorders of the urinary tract. For some people with PH1, their urologist may treat only their kidney stones and refer them to a nephrologist to help manage PH1 as a whole.
Though some kidney stones may require medical attention, there is a lot more to managing PH1 beyond removing stones when you have them.
Urologist
Genetic Counselor
A specialist who can answer questions you might have about genetic testing and interpret your results. Genetic counselors also help those who may be at risk for or be affected by PH1 understand the risks and make decisions, including whether other family members should consider being tested.
Genetic Counselor
Dialysis Nurse
A nurse who is specially trained to assist and monitor a person undergoing dialysis. Dialysis nurses work in hospitals or in separate dialysis centers. Healthcare providers at dialysis centers may not be familiar with PH1 since they are primarily responsible for conducting the dialysis process, not for treating diseases that create the need for dialysis. You can play an important role in educating others by memorizing a 1-2 sentence description of PH1. Here are some examples:
- PH1 is a rare condition where the liver makes too much oxalate, which can damage the kidneys over time
- PH1 mostly affects the kidneys, and the most common symptom is kidney stone formation. But PH1 actually involves the overproduction of oxalate, which occurs in the liver
Dialysis Nurse
Transplant Team
A transplant team can include transplant surgeons, transplant nephrologists, and other physicians who provide medical support during follow-up.
Transplant Team
As you work with your care team, friends and family may also be able to provide invaluable emotional and practical support.
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Management of PH1
Your healthcare provider may consider these management options for your PH1:
Alkali citrate or vitamin B6:
May be recommended or prescribed by your doctor to help reduce the formation of oxalate crystals or decrease the amount of oxalate your body produces.
Staying hydrated:
Your doctor may recommend adequate hydration to help dilute oxalate in the urine.
Dialysis:
Dialysis may be needed to remove oxalate and other waste products from the blood.
RNAi therapies:
Your doctor may prescribe RNAi therapies, which work by reducing the amount of oxalate your body is producing.
Developing a management plan with your doctors and closely following this regimen is important to staying as healthy as possible. Patients with PH1 should always talk to their doctor before making changes to their disease management plan.
PH1 perspectives: understanding progression
Thinking ahead: when management is no longer enough
Oxalate overproduction can cause permanent damage, and transplant surgery may eventually be necessary if your body reaches a certain point of disease progression.
Although PH1 primarily damages the kidneys, it is a metabolic defect of the liver, and patients may need a liver and kidney transplant to stop the overproduction of oxalate and replace the damaged kidney. Organ transplantation is a surgical procedure in which a damaged or nonfunctioning organ is replaced with a healthy one.
A liver transplant from a donor without PH1 is a cure. Transplant surgeries are major surgeries that require preparation, long-term follow-up, and life-long medications.
Talk to your healthcare provider to understand if transplant surgery is a treatment option for you.
What is living with PH1 like?
Living with PH1 is more than just a management plan. Discover ideas on how you can make your care team's management plan part of your life.
Living with PH1