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)
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 based on how your PH1 is progressing.
Along the way, other specialists may play a part in managing your PH1.
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.
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.
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 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 starts in the liver, where an overproduction of oxalate occurs.
A transplant team can include a urologist specializing in kidney transplant, another surgeon specializing in liver transplant, and additional physicians who provide medical support during follow-up.
As you work with your care team, friends and family may also be able to provide invaluable emotional and practical support.
The goal of management is to slow disease progression
Current management options may reduce the amount of crystals formed due to oxalate overproduction and help to slow down kidney damage and reduce stone formation.
As part of your management plan, your healthcare provider may measure different things, including your oxalate levels (how much oxalate is in your urine and/or blood) and your kidney function (how well your kidneys are able to filter waste products). To determine your kidney function, blood tests will measure your glomerular filtration rate (GFR).
PH1 perspectives: understanding progression
Your healthcare provider may consider these management options for your PH1:
(drinking lots of water):
Helps dilute oxalate in your urine
May help the mutated enzyme try to work the way it should
Alkali citrate (potassium citrate or sodium citrate):
May help reduce crystal formation
Using a machine to remove oxalate from your body when your kidneys are no longer able to handle it on their own
- Your healthcare providers may manage your PH1 with multiple options at the same time, such as making sure you drink enough water, along with taking alkali citrate and vitamin B6, if you are a responder.
- Your PH1 management may shift or change over time, depending on how your disease is progressing.
- Most management options cannot keep up with the continuous oxalate overproduction. However, consistent management of PH1 may help you slow progressive loss of kidney function.
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 advanced disease progression. Only a liver transplant is curative, because it resolves oxalate overproduction in the liver. Most other management options cannot do this.
Today, liver transplantation is the only way to stop oxalate overproduction. Researchers are actively working to develop additional ways to
*If not fully responsive to vitamin B6.
A liver transplant is a surgical procedure in which a donor liver is taken from a person without PH1 and is transplanted into a person with PH1.
Because PH1 primarily causes damage to the kidneys, both the liver and the kidneys may need to be replaced, either at the same time or during separate surgeries. This is called a dual liver-kidney transplant.
Transplant surgeries are major surgeries that require preparation, long-term follow-up and lifelong medications. If your healthcare providers think that this is the right next step for you, ensure that you partner with them to create a detailed plan to help you recover from your surgery.