ADPKD

Autosomal Dominant Polycystic Kidney Disease aka 'ADPKD' is the fourth leading cause of chronic kidney disease, affecting about 1.6% of renal patients.

What does ADPKD mean?

Source: PKD Foundation

• Autosomal - it's an equal opportunity genetic mutation - it doesn't matter if you're male or female
• Dominant - you have a 50/50 chance of receiving it from a parent with ADPKD and you have a 50/50 chance of passing it along to a child.
• Polycystic - the disease creates many fluid-filled cysts
• Kidney - the affected organ and
• Disease (I think you have that one figured out)

So ADPKD is an insidious (emphasis my own) genetic mutation inherited from a parent that causes the formation of multiple cysts on your kidneys.

There is no cure for ADPKD. Other than controlling blood pressure and living a healthy lifestyle there's really nothing you can do to slow the disease's progression. Once you have it, you have it.

As the disease progresses, the cysts increase both in number and size. Eventually the cysts displace the actual kidney tissue which is why function will decrease. A normal kidney is about the size of an adult's fist. By the time you reach end stage renal disease, EACH kidney can be the size of a football, weighing up to 30 pounds.

Source: University of Kansas

Imagine carrying 60 extra pounds around your mid-section. Yikes!

Some people with ADPKD will get a 'PKD' belly which could be construed as a beer belly (if you're male) or a pregnant belly (if you're female).

While in the past there was really nothing you could do to try to slow down the progression of the disease, now we have Tolvaptan aka Jynarque (see page on Tolvaptan for more detail). After running the numbers on my own situation, I figure the drug should delay end stage for two to three years.

Mid-Stage 4 - right around eGFR of 20 - you should decide if you want to be place on a transplant list. If you do, find a transplant center that aligns with your needs and then set up an appointment to be reviewed for placement on their list. It's a serious affair and it takes the better part of a day. Some centers have age restrictions (it's better to be younger) as well as weight restrictions (it's best not to be overweight, obese or morbidly obese). Something else to consider is whether you want your native kidneys removed. Some centers won't do it. Or if they do, they won't remove them during the transplant surgery. Unlike with other forms of CKD, ADPKD kidneys don't shrivel after transplant. The cysts on PKD kidneys continue to grow. This is why I'll be choosing Mayo as my transplant center; (I believe that) they will remove the native kidneys as part of the transplant surgery.

Once you reach Stage 5 (eGFR of less than 15%) and haven't been fortunate to either be called up for transplant or found a live donor, you need to think about dialysis. From what I can tell there's no 'set in stone' criteria for when you would start treatment. But generally it's somewhere between an eGFR of 5 and 10.