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.

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Round 2 deja vu

My doctor decided to keep me at the initial 45/15 mg dose for another months so I'm anticipating a similar water consumption. Here are the first four of seven (?) or eight (?) five gallon bottles of water. My local grocery store has a water bottle fill station for an extremely reasonable 39 cents a gallon. The frugal side of me thinks I should invest in reusable water bottles and take advantage of the savings. The lazy side of me thinks that it'd be a total hassle to wash the bottles, keep bugs and dust from falling into them before I'm able to refill them, remember to take them with me to the store (I can't even remember those reusable shopping bags) and then stockpile a source of bottle caps. It's just so much easier to buy these recyclable five gallon bottles. Lazy beats frugal.

Artificial kidney

I have a somewhat fanciful thought of what an artificial kidney would look like. I see a device that is shaped like a kidney (of course) but is completely clear so you could see all the blood circulating through it. Granted, once it's implanted no one's going to see it but - hey - it's my vision. The closest picture I can find of what's in my mind's eye is this image. Granted, that's a picture of a kidney-shaped paper weight and it's a little cloudy, but I know you can picture it too. My, oh my. My imagination is not even close to reality. According to a post from the American Journal of Kidney Diseases , there are three new approaches being explored right now: the automated wearable artificial kidney ('AWAK'), the wearable artificial kidney ('WAK') and the implantable artificial kidney ('IAK'). My money's on the IAK and you'll understand why once I show you the concept pictures of the other two. The AWAK is said to ...

A happy liver

I'm two weeks into my Tolvaptan adventure and I'm happy to report that liver tested with no change to the pre-medication levels. A shout out to my liver. Way to go! One of the potential side effects of the medication is liver damage (which has been proven to be reversible as long as it's caught early). Discontinuation of the drug causes the reversal. Fortunately, the reported incidence of liver damage is relatively low at only 4-5%. More recently, in large registration trials of long term therapy in patients with ADPKD, serum aminotransferase elevations occurred in 4% to 5% of patients on tolvaptan, compared to only 1% of controls. Furthermore, clinically apparent liver injury occurred in approximately 0.1% of treated patients. The time to onset of illness ranged from 3 to 7 months (Case 1), but occasionally arose during long term therapy (Case 2: after 3 years of intermittent use). National Institute of Health Crossing my fingers that we'll be able to incr...

Truly blessed

For all the medical issues I have, for all the uncertainty that exists in my future, for all the modifications I need to make to my lifestyle... I feel honestly and truly blessed. Yes. I have an incurable disease that can possibly lead me down a path towards dialysis, organ transplant, lifelong immunosuppressive drugs and early death. But... I don't have diabetes. I don't have a history of aneurysms in my family. My blood pressure is well-managed. My risk for cardiovascular events is low. I work for a company with good healthcare. My job allows me the flexibility to go to doctor appointments and labs. I have friends and family who love and support me. Could be much, much worse.

Test tube kidneys?

While I'm sure that Miromatrix would shudder at my description, it looks like they're developing the equivalent of a test tube kidney which could be used for transplant. While the science and bioengineering behind it is extremely complex, this is how I understand it. Miromatrix uses decellularization and recellularization technology to create a transplantable kidney. Huh? They take an dead organ - it doesn't even need to be human - and using detergents (I'm guessing they're stronger than Dawn ;-) ) they strip away all the cells. What remains is the kidney's extracellular matrix (collagen, enzymes and glycoproteins) and all its chemical cues. Then the "generic" organ is repopulated with the transplant candidate's cells, eliminating the risk it will be rejected upon implantation. Early studies have shown that it takes from four to eight days to culture the transplantable kidney and, even better, the new organ produces urine when tested. B...

My Two Gallon Habit

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