Norma,
I think it's sweet that your friends are concerned about you and your family. About all doctors learn in medical school (having talked to many current orthopedic residents, the young ones who just got out of school) is PKD is hereditary, dominant and recessive genes (mostly dominant gene), 50/50 chance, cerebral aneurysms,no treatment, no cure. Back in the 1940s, who knows what they learned about PKD, if it was even called PKD or Bright's disease. So your friends who are orthopods, who went to school years ago, really aren't off the mark because things haven't changed much at all since they were in school. And it's a very common misconception that transplanted kidneys will become cystic because in many other kidney diseases (and other organ transplant situations) the disease comes back to attack the transplanted organ after a period of time. PKD just isn't one of those diseases (thank goodness). Your friends aren't family doctors, or internists, let alone nephrologists or transplant surgeons; these simply aren't areas they specialize in or would have knowledge of. Medicine is much too vast to expect every doctor to know details about every single disease. Patients go to these doctors because they need an orthopedic surgeon, not a nephrologist. To quote my nephrologist last Tuesday (applicable to the conversation we were having), "I don't expect to know everything there is to know about surgery and I don't expect a surgeon to know everything there is to know about nephrology...and I don't want him to pretend he does."

Back in the 1940s PKD was still being called Bright's disease in places. We didn't have the imaging capabilities and kidney transplants didn't exist (kidney transplants are barely 50 years old). Dialysis, if it existed, was a last ditch effort to save a life and was a horrible experience that usually resulted in aluminum toxicity. There were no blood pressure medications.

By the late 1950s some nephrologist had figured out this disease ran in families, but that hadn't filtered down to the surgeons (to wit the surgeon who told my parents after my father had surgery for a cyst rupture, that his PKD was a quirk, not hereditary and thus I was conceived...my brother and I would not be here had my parents known PKD ran in families).

It's only in the last 20 years that they've found the actual genes (2 dominant, 1 recessive; and researchers suspect there's a third dominant gene, but no one has found it yet). But even the average nephrologist doesn't get a detailed, seriously in-depth education on PKD; it's just one of an incredible number of kidney diseases and other issues nephrologist deal with and one of the more frustrating because there is no treatment and there is no cure. It's patently unrealistic to expect your friends, who are in a completely different field, to be up-to-date on PKD. There's so much in their own field to keep up on. They rely on the intenists and nephrologist, cardiologist and pulmonologists to give their patients the medical clearance for the surgery and the anesthesiologist to manage the electrolyte balance during surgery (if they're even still practicing).

That said, yes, we need to interview our doctors. I always have during my first visit. If they're not on the wall I ask all about their credentials, where they went. I will also ask why they chose medicine and why this particular line of medicine (as well as why DO versus MD if they happen to be osteopaths), and especially why they chose military medicine (some have good reasons, be it paying for medical school, serving their country, being able to practice medicine without the restrictions of all the paperwork, whereas a few, usually the older doctor who was in private practice have refused to answer and as a result, I've fired them. I figured if they refused to answer a basic question, then they can't be trusted and were most likely one patient away from a malpractice suit; the military self-insures). I know enough about myself and what's happening with my body and what type of doctor I like to work with to get a good feel for a person in a visit or two (I generally give doctors at least a second chance in case they were having a really bad day the first time and I've been very happy I have on more than one occasion). But I've hired and fired doctors of all types, military and civilian. I've also recommended doctors of all types, military and civilian. I've shredded surgery consent forms in pre-op because the nurses seemed were incompetent and I just didn't have a good feeling about the situation. I've smacked a doctor who started to poke at my liver after being told not to touch me by the attending physician as well as me, banned him from my care and my room and then reported him for assualt. I've also hit a nurse (reflex) who was flickinig at a blown IV just because he could.

Please keep in mind that there really is nothing your doctor can do right now other than TREAT the SYMPTOMS. Your doctor isn't ignoring your by doing nothing when there is nothing he/she can do. PKD is a disase with no treatment and no cure and with the possible exception of Tolvaptan (which is still in clinical trials) will remain that way for a very long time.

So yes, interview your doctors. But I don't think the situation is anywhere near as dire as you make it out to be. Doctors are much more open to learning new information these days and we can come armed with our own medical information from scientific websites and journal articles about PKD, pain management and any other topic applicable to our health; we no longer have to rely on word of mouth or "I've heard that...". And we all have the power to hire and fire our doctors, even within an HMO or PPO. If educating your doctor or getting him started down the road toward educating himself and developing an interest in the details of PKD is what it takes, then that's what we do. But it's patently unrealistic to expect every doctor to be an expert in OUR disease; they have patients of all sorts, with PKD being just one of any number of diseases they're dealing with even within the nephrology arena. This is our life and it's up to us to advocate for ourselves. No one else is going to do it for us.

Best wishes,
Ruth

Norma/Ruth. I will interview my 2nd opinion urologist and nephrologist when I see them. It might be easier to get to know these 2 docs, hopefully I won't be running out of this urologists office upset over something that was said. We shall see what happens monday.

Lets see I have a few memories of my own Ruth, I'am rather over the hill if you recall. I remember taking my father to the only kidney doc in this area when I was 16 yrs. old. He was probably in his late 40's or something like that. I didn't go in with him, but the doctor came out to my car and wanted to talk with me. My mother was in the hospital dieing at that time and why she wasn't with us. He told me dad had Brights disease and that his kidneys were covered with cysts. I don't recall what they used in those days, probably just ordinary X-rays. He told me he was going to die and that I needed to prepare the family. He died when he was 66 of colon cancer and his kidney funtion was completely normal upon his death.
I was born in 1941, so this would have been in 57 or thereabouts and they were still referring to it as Brights disease then. My mother had one of the very first dialysis prcedures done when she was close to death. It was pretty much barbaric, but did the job temporarily that is, again in the 50's. A lot changed in the world during the 50's. Cars, people, technology zoomed. It was the last time people sat down at a table together as a family and ate a meal together, another family tradition gone down the toilet. The world as we knew it the 50's was gone, never to return. We played in the dirt, we played outside till dark, we knew to come home after that, we ate raw hamburger and didn't die, we ate lots of things and didn't die. The world has definitely changed. Oh and BTW, those doc's that I was talking to were not family friends, of mine anyway and they were not old, they were bearly out of their residencies and still knew nothing of PKD. If so many people have it, wouldn't you think that more doc's would be on the look out for it? I do and expect someone to be doing it. As far as I can see, no one is looking for it. It was no small miracle that my GP knew what I had when I was first diagnosed. No one else had ever head of it. He also called it PKD, not Bright's disease.
He put me on BP meds when I was 38, told me to stop drinking coffee and sent me to a kidney doctor to see if I had aneurysms, which as you know I did. This was a long time ago, he had to have been on top of things to have known that then, wouldn't you think. He was a former radiologist with 10 kids and came to Greene as we didn't have a GP in that small town. this was 38 years ago!! If 1 in every 1000 people have pkd, that we know of anyway, wouldn't you think that doctor's would have done a little more than they have? I do. we don't have Jerry Lewis campaining for us or any other celebrity and thats probably part of the problem. If I could verify that Joe Biden had PKD, wouldn't that be a big plus, but I can't verify it, only that he had brain aneurysms and has a kidney disease, no mention of pkd. Trying to cover it up? I think so, acutally would bet my last buck on it. Just look at the money people have spent on MS, Diabetes, and all the other big name diseases, why? Because they all have celebrities helping them. There's one that I can recall, he's a designer and has PKD. When asked to represent us, he said a flat NO. He's had two transplants because he didn't take care of himself for the first one, but of course got another one and fast. It pays to have big names behind you, but we can't see to get that and I wonder why?

Big question I would want answered is why aren't doctor's screening more people for PKD? they do it in Europe all the time, every year and pay for it too. Here in the good old US, we have to beg for healthcare, if people are poor or unemployed. I'll believe the new healthcare system when I see it in action, but I'am betting that I'll be dead and gone before that ever happens. I've spoken to many a committee in Washington and in Albany, what did it ever get me, a big fat nothing! We don't have the money or the big names and thats what it all boils down to.

good for you standing up for yourself, you have to because no one else is going to do it for you. I've thrown a few intern's out of my hospital room when they were going to do a spinal tap on me. Didn't take me long to figure out it was there first time and had no idea what they were doing. If we don't take care of ourselves, then we are a cooked goose and I mean that in every aspect of our lives. To abuse from family members, to husbands, to doctor's to attornies, to every area in our lives, we are the ones that are responsible, no one else and it's up to us to get it done!

Best wishes to you too...:)
Norma..

It can be very disheartning to speak with a Dr. when they don't seem to have knowlege about what seems so very basic to those of us who are dealing with the disease. I suppose the thing to do would be to offer the correct information so that they don't spread the "untruth" to others. Fortunately there are Dr.s out there that do know and are specialized in what we deal with every day. I am seeing a Neph that I think knows very little about PKD specifically. I believe he is a smart man, but just isn't as well versed as I would prefer him to be. Because of that, I am now seeing a specialist in Denver that can hopefully answer ALL my questions without hemming-and-hawing over what may or may not be. Each person deserves the treatment that they deem necessary. And if one Dr. doesn't fit that profile, then move on to the next. We each have to do what works for us as individuals.

Norma, I love to read your posts. I find them very enlightning and informational. Thank you!

God Bless!

To add...

I find it really disturbing that my 5 children should have to wait until they have symptoms or health insurance before they get screened for PKD. I certainly don't want them to have to be without insurance because of something they can't control. Until then, I tell them to keep an eye on their blood pressure and their creatine count at the annual Health fair. At least to be aware of where their health is. Thank you again Norma for your history lesson. I love to listen to you!

Hey Norma,
At least you educated a few doctors! I think I posted this before, but, my GYN was amazed I'm not considered in end stage renal failure w/a GFR of 40. I finally got the MRA/MRI issue straightened out w/my nephrologist. He is a good enough doc to tell me he had to consult w/a radiologist to learn the difference AND, he was man enough to tell me he had gotten an education from the radiologist and I am scheduled for an MRA to check for aneurysms. There's too much to know and they can't know it all, but, I understand your frustration - why aren't we a "glamorous disease?"
Ruth, I am going to take a lesson from you and the next time a doctor tries to probe my liver, they'd better watch out!
And for some more history: My grandfather died in 1944 at the age of 48 from "apoplexy, due to hypertension, due to congenital cystic kidneys." He was in and out of the sanatorium because they hoped rest and good food would shrink his kidneys and lower his blood pressure. My grandmother did not tell my mother any specifics because she was not told his disease was heritable and that's why my mom had six kids - half of whom have PKD. We all consider ourselves very lucky that great strides have been made in the treatment of this disease, even if it's just correct BP medication to keep us from dying (or being paralyzed like my mom was) from apoplexy.
Hugs and good night
Lisa H

Hi, Norma!

Ignorance is NOT BLISS!

In this day of the internet and gadgets, it is inexcusable for doctors to be so clueless.

Ignorance is understandable. None of us are born knowing about PKD. But when a doctor is introduced to PKD, it is inexcusable not to at least be generally informed as to the nature of the disease.

On the other side of the coin, the PKD Foundation's goal of "awareness" obviously still hasn't been achieved.

Thanks for sharing, Norma!

Peace and Blessings!
CoachRichie

Norma,
I'm sorry I missed the fact these doctors were IN their mid-40s; I though they went to med school IN the mid 40s. Nonetheless, the level of knowledge is the same as what I told you, very limited. They know bones and muscles and how to take them apart and put them back together again, but when it comes to PKD, when they learned in medical school is just the basics and with so much to keep up with in orthopedics the other fields fall by the wayside. Even nephrologists have a problem keeping up with all the papers being written on nephrology. And Norma, no, even at a rate of 1 in 1,000, I have no expectation of doctors or researchers having been able to do anything about PKD before now. It's not for a lack of trying; it's just that lab research hasn't translated to clinical trials and the actual patient until recently. We haven't been ignored, there's just been a lack of breakthoughs.

Yes, it would be nice to have a reasonable and rational celebrity who would testify before Congress and get us increased research funding. "Cojo", Stephen Cojocaru, the flaky stylist who found out he had PKD and had two kidney transplants wasn't the best role model ("kidneys are the new black"), but please, get your story straight. He lost his first kidney due to BK virus, a virus that can attack a transplanted organ and cause acute rejection; it was NOT due to lifestyle issues. And he didn't say no to the foundation; once he had recovered from his second translant he sponsored a walk team in LA.

What do we get by screening people for PKD? More people who are worried about having a potentialy life threatening disease is about it. They're not screening Europeans for PKD as a disease; they're simply screening for kidney disease via creatinine/BUN and GFR and as needed, referring them to a consultant as needed. No one is going around scanning entire populations.
Aneurysms run in families without PKD just as much as they do in families with PKD, so I doubt the VP has PKD (President and VP records are generally made quite public during the campaign) and we will most assuredly not know till long after he's out of office if ever if he actually does.
Rich,
Do you see a gynocologist and expect her to know all the details about prostate issues? Do you expect a psychiatrist to know the nuances and be able to perform transplant surgery? Or perhaps your expect your nephrologist to have the knowledge and technique down to remove the pituatary tumor from your brain using laser surgery? It's a valid question under you expectation that they're all doctors and "In this day of the internet and gadgets, it is inexcusable for doctors to be so clueless." Do you know everything there is to know about every possible aspect of your chosen career field, hobbies, etc.?

I would greatly prefer my nephrologist to focus on nephrology than surgery and psychiatry, despite his medical school exposure to them 14 years ago. And if perchance I come across a doctor who graduated back in the 1940s, I will be gracious, but not eager to be treated by him.

Norma's expereince are very different from mine. When her father was being diagnosed with Bright's disease, my father was being diagnosed with PKD, but being told it was a quirk, he'd live a long life, and to go forth and prosper and have children. My father died at 41 of a thoracic aortic aneurysm (as did my uncle at 43, 6 months earlier), a complication only recently linked to PKD genetically (although suspected to run in small family clusters for years). My grandmother died at age 47 of "natural causes" (no mention of any reason; NYC is still searching for her paperwork, buried in the bowels of some municipal records or public health due to her date of death).

Norma,

Sorry about the last post; it was not complete when the computer somehow decided to post it on it's own (my computer has its own mine lately which is driving me nuts).

It's late and I will try to clarify later. No negative comments or intent is meant.
Ruth

I guess you re read your last posting Ruth! Sometimes you are the most irritating person I have ever known in my life. somehow I find your perfection in every aspect of life to be rather tiring! Is there anything in this world that you are not the end all of knowledge? And I beg to differ with you on a few things, but know better than to argue with you.

No negative comments are meant or intended!