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What is polycystic kidney disease (PKD), and how does it cause kidney failure?
In patients with polycystic kidney disease, the kidney is replaced by these bubbles of fluid. After many years, the sacks of fluid displace all the normal kidney tissue, causing the kidneys to stop working.
How is PKD diagnosed?
PKD is mostly a familial problem that gets passed down from generation to generation. There are some sporadic cases. Typically if someone in the family has had PKD, the rest of the family knows about it and gets screened. Primary care physicians play an important role in helping diagnose PKD by getting ultrasounds of their patients' abdomens. Unfortunately, there is nothing we can do to stop the progression of PKD.
What is life like for patients with symptomatic PKD?
Patients get very large abdomens because their kidneys have grown as large as footballs or bigger. They don't look very thin. PKD can lead to problems with shortness of breath because the kidneys take up so much room in the abdomen. Patients can also develop problems with feeling full very quickly because there is not enough room in stomach.
Those discomforts are reasons enough to take out the kidneys, even if a patient will need to remain on dialysis while they wait for a transplant. Sometimes patients can have bleeding from the kidneys because the cysts rupture, which is also painful. Finally, some patients with PKD can have also have polycystic liver disease.
What are the medical management options for PKD?
Right now there are none. You just follow the patients and manage their case so they don't exacerbate the situation. Usually they have high blood pressure. The job of the doctors and patients before transplantation is to do everything we can to get the maximum time out of their native kidneys.
Do all patients with PKD eventually need a transplant?
No, not all. Some forms of PKD are less severe. Some people have PKD that make it to their 70s or later with their own kidneys. But many patients do experience kidney failure and require a transplant.
When do patients become eligible for transplant?
We start evaluating for transplantation when the creatinine clearance gets below 20. Normal creatinine clearance is between 80-120.
How does the University of Maryland's treatment for PKD differ from other transplant centers?
We remove both failed kidneys and transplant a new kidney in one operation, usually from a living donor. This is very important from a patient perspective because you don't have to undergo two anesthetics and recover from two separate surgeries.
Taking out these enlarged 8-10 pound kidneys is a big operation in and of itself. To combine that with a kidney transplant is an even bigger operation. When we do a kidney transplant with bilateral nephrectomy [removing both kidneys], we put the new kidney in the abdomen and we have to do certain maneuvers to make sure it doesn't twist and stays were we want it. We started doing this a long time ago and have excellent technique. We avoid a lot of the pitfalls because of our experience and volume.
This is a big operation that is very technically challenging, and many centers don't have the depth of team to support such a complex surgery. We have that advantage as a large transplant team that does hundreds of transplants each year.
Does the transplant team do double nephrectomies and a transplant if the kidney is from a deceased donor, or just from living donors?
Normally we just do double nephrectomies with living donors because we have the time to prepare the team and schedule the time for a major surgery. This is a major operation that requires many surgeons and a lengthy operation.
What's the largest kidney you have ever removed from a patient with polycystic kidney disease?
Eighteen inches easily. Some have been approaching two feet in length.
Do you or a loved one have PKD? Please call our transplant team at 410-328-5408.