Norma,
How is your nephew doing by this week? My grandfather had a stroke due to PKD (on my mother's side) and it is not easy. But he had great determination to get better and keep moving forward. I admire him for that. I really hope your nephew is doing better and will continue to stay strong and keep moving forward.
God Bless,
K~

Thanks all for your good wishes and intent. My nephew is being transferred to a reabilitation hospitial today some time. He is about as recovered without rehab, as he will get. I think it's pretty doubtful he will regain any of his function, but time and determination will tell.
His daughter has her doctorate in physical therapy and has come home to oversee his rehab. If there is any recovery to make, I'am sure that she will make it happen.

I don't know Ruth, I was told the complete opposite about PKD aneurysums, that we were born with them and that rarely do they cause any symptoms other than drooping eyelids or eye brow, usually something in the face muscles are affected, if anything. I was told this by highly respected neusurgeons and have had follow up care with the same comments. I've also talked with the few other people on this forum that have had crainitomies, and we all pretty much had the same symptoms and care. It's my thoughts that everyone with PKD should be screened, especially in today's world where it can be done so easily, compared to the angeogram that I had to have done years ago. I am not 100% sure about that, but have been told that by people that know, not a physician.

We are just back from New York city again, with more good news from our precious Dr. Amin. This man is so great that I am just amazed with him. We can now go 2 months without seeing him for the first time since Jim's surgery. Everything looks great on Jim's vocal cords, no signs of it returning, which are more during the first year. It has been 6 months. We are tired...

Thanks again everyone, you are all so very special to me and my family. Best wishes to each and every one of you.

love,
Norma...

Norma,
Wonderful news about Jim! And I truly hope your nephew makes great strides in rehabilitation. Amazing things happen with stroke patients these days!

I don't know who gave you the information about PKD aneurysms, but they were flat out wrong. An aneurysms is an aneurysm, period. The only thing unique about those with PKD is that we are more prone to developing them than the rest of the population. But all other factors remain the same: they can develop at ANY time, can cause symptoms or no symptoms at all, and depending on size there is a high risk of rupture.

Here's one of my references for cerebral aneurysms (the others are my neurologist and neurosurgeon as I went through a cerebral aneursym scare due to an wildly overly conscientious radiologist who thought he saw aneurysms everywhere in my brain during when reading an MRI in 2002--it was a nightmare!)

http://emedicine.medscape.com/article/1161518-overview (Article title: Cerebral Aneurysms: eMedicine Neurology) Any comments within quote come from this article (although I have put some comments of my own in [brackets])

"Saccular, berry, or congenital [birth defect versus genetic; this can mean the aneurysms can develop at any time, not necessarily that it is present during fetal development] aneurysms constitute 90% of all cerebral aneurysms and are located at the major branch points of large arteries. Dolichoectatic, fusiform, or arteriosclerotic aneurysms are elongated outpouchings of proximal arteries that account for 7% of all cerebral aneurysms. Infectious or mycotic aneurysms are situated peripherally and comprise 0.5% of all cerebral aneurysms. Other peripheral lesions include neoplastic aneurysms, rare sequelae of embolized tumor fragments, and traumatic aneurysms. Traumatic injury also may result in dissecting [splitting of the lining of the blood vessel that eventually results in a full tear] aneurysms in proximal vessels. Microaneurysms of small perforating vessels may result from hypertension."

Saccular aneurysms are the type commonly known as "berry anerysms" and are given their name as they look like a berry sitting on or near where two branches meet (in this case two branches of an arteries or two arteries meet in the brain). In reality, they are the outpockings of the blood vessel (thus a sac, similar to the formation of a cyst) but if they leak or rupture (e.g try to break off) a bleed will occur (rather than a cyst form as occurs in the kidney or liver). There can be one or more berry aneurysm in the brain at any one time, but they rarely if ever appear in clusters in the same location. "85-95% of saccular [berry] aneuryms will be located in the anterior circulation region of the brain" (most commonly in an area of blood vessels called the Cirle of Willis). "Multiple saccular [berry] aneurysms are noted in 20-25% of patients" (this means that 20-25% of patients will have more than one, not that they are clustered together; each is usually located on/near a branch of where two blood vessels meet, but different branches in different areas, not a series right next to each other or even adjacent branches...the delusional radiologist saw at least 2 berry aneurysm on my MRI/MRA along with another 4 suspected berry aneurysms; argh!).

"The development of cerebral aneurysms remains a controversial topic. A multifactorial etiology is most likely, reflecting the interaction of environmental factors, such as atherosclerosis or hypertension, and a congenital predisposition associated with various vascular abnormalities. Abnormalities of the internal elastic lamina may be congenital or degenerative. Multiple conditions have been associated with cerebral aneurysms; they include the following:

Autosomal dominant inherited polycystic kidney disease..."

Note that ADPKD is the first disease listed as a possible cause of aneurysms (first amount 18 disease; they were NOT listed in alphabetical order but the order listing was not described or provided). There is also a familiar inheritence pattern in 2% of cases of cerebral aneurysms (without the presence of one of the diseases; we already know about 10% of families with PKD have a strong familial rate of aneurysms).

Causes for aneurysms may also be environmental, hypertension, artherosclerotic (all of these occur over time; they are not present at birth). Traumatic aneurysms may also occur (due to head trauma, again, not present at birth).

"Causes:

Congenital or familial inheritance [the risk may be there at birth, either due to defect (congenital) or genetics (familial inheritance) but the aneurysms will develop over time and new ones may develop even after an old one is repaired]

Atherosclerosis [hardening of the arteries, an event that occurs over time]

Hypertension [happens over the course of time and with can be also spike with specific events]

Autosomal dominant inherited polycystic kidney disease [although we are all born with the disease, the aneuryms, just like the cysts develop over time]

Vasculopathies [abnormalities in the blood vessels, can be present at birth AND occur over time]

Arteriovenous malformations [AVM; these can develop, change and grow over time; the tumors in my liver are a form of AVMs but they were most assuredly not present at birth]

Connective tissue disorders [not always present at birth and many change and get more severe over time]

Sickle cell anemia [present at birth, but flares and attacks occur over a lifetime]

Infections [can happen at any time and the damage can be cumulative]

Trauma [any place, any time]

Neoplasms [tumors, etc; occur over time]

Cigarette smoking [few infants start smoking at birth; this is the long-term use]

Illicit drug use [few infants start using illegal drugs in the bassinet; this is the long term user, not the infant born to an addicted mother]

Alcohol [this isn't fetal alchohol syndrome, but alcoholics with heavy use over time]"

Keep in mind that these are POSSIBLE causes and are by no means an exhaustive list. Some happen "just because" and there is never a known cause.

Under the workup section, it was interesting to see that they emphasis was on the NON-CONTRAST CT in the event of a severe headache or suspected subarrachnoid hemmorage (SAH) (aka ruptured aneurysms). They note that the use of contrast can actually obscure the detection of a SAH.(This is the WORST headache of your life scenario and the reason you go to the ER immediately and tell them you're having the WORST headache of your life and EMPHASIZE that you have PKD!) For diagnostic purposes a CTA (CT with contrast) can be used to determine if there are aneurysms present (in a non-emergent situation) and are often more precise than the MRI/MRA without contrast.

In my case, I had an MRI/MRA in 1993, which stated said I had no aneurysms, but was missing the left anterior cerebral artery. Strange, but okay. In 2002, at the very strong recommendation of the radiologist who was very well versed in PKD, and with the agreement of my internist (keep in mind that none of my doctors get paid to recommend medical tests or perform procedures; they all get the same salary no matter how many patients they see or procedures they perform; I had fired the Army nephrologist I had been assigned at the time for sheer stupidty--he thought 140/90 was just fine for blood pressure), I had a second MRI/MRA (no contrast). That was the study where the radiologist thought he saw everything but the man behind the curtain (I think he was delusional...he knew I had PKD and thus was bound and determinded to find something wrong in my brain!). He did find the left anterior cerebral artery; it had never been missing. However, he also stated I definitely had 2 berry aneurysms and identified 4 other suspicious areas (no one else saw them, the chief of neurosurgery spent 1.5 hours with me searching through the image trying to find anything out of th ordinary and only found one area that looked like it might be a tad unusual from one perspective, but was perfectly normal from all other perspectives...it was strange to see your brain's blood vessel's in 3D spinning on a screen!). The follow-up study to absolutely confirm the radiologist was wrong (since there was that one are that needed confirmation and I had gracefully declined the angiogram they offered) was the CTA (CT with contrast of my brain; this was before I had the allergic reaction to CT IV contrast) and that showed I had a perfectly normal brain, no aneurysms at all and no missing blood vessels! I've since had one more MRI/MRA of my brain, this after finding out that my first cousin died of a cerebral aneurysms and getting confirmation of the link between ascending aortic dissections (vascular abnormality that killed my father and uncle) and PKD. That too was just fine. In all cases the doctors (radiologist, neurosurgeons and my wonderful nephrologists) have emphasized that aneurysms can form at any time--they are insidious beasts that can sneak up on you silently without warning and can strike when you least expect it. The chance that I will develop one at this point in time is very slim (I'm nearly 50 and haven't had any problems to date; my BP is very well controlled; I don't drink alcohol; my lipid panel is excellent; and all other labs that would indicate any artherosclerosis are excellent) but I still need regular screening based on my family history just to be safe.

In your case, Norma, you already had one extremely large aneurysm fixed. Have you been screened since? That was a long time ago. One aneurysms tends to portend more aneurysms, so repeated screenings on a regular basis are essential!

I agree that everyone with PKD should be screened at least once (so few of us truly know our family history, so limiting the screening to only those with known first degree relatives (parents, siblings) with ruptured aneurysms is not reasonable (nowadays they may well be on BP meds early or not have been screened themselves and have an aneurysms but it just hasn't ruptured...yet!) but others in the family with the same gene, e.g. aunts/uncles, first cousins, may have aneurysms, which means your parents are at equal risk and so are you! Just because a parent never experienced a ruptured aneurysms before doesn't mean they dont' have one now or didn't have one before they died. Up to 40% of those who undergo autopsy are found to have one or more aneurysms present that didn't rupture, yet autopsies are rarely done these days when cause of death is so often well known. And since PKD is so often the "family secret", who knows what happened in previous generations anyhow?!

PKD sucks. But don't let bad advice from long ago make it even worse. Please get that screening done now!

Lots and lots of gentle hugs,
Ruth

ok Ruth, I give, I give!!! I admit that I probably was given wrong information, afterall, it was a few years ago! Lets see I think about 7 years ago. HAH, just kidding. You've got a few facts wrong about me, not about PKD, but me. I didn't have one very large aneurysm, I had NINE!! All really deep in the brain, I used to be able to identify where they all were, but can't anymore due to insomnia, displasia, anorexia, but mostly old age! I also can't be screened for them again becaues I have 9 metal clips in my head, you'll remember that now that I'am saying it. But I can have normal old x-rays which does show some of them and they are all still clipped and everything looks as copisetic as they can. I almost got the life sucked out of me one time when I experienced a lot of vertigo and they were going to do an MRI. The surgeon that did the surgery said it was ok, as the clips were all teflon. Jim asked him to confirm that with his sales rep. and guess what? They were not all made of Teflon, they were teflon coated!! No MRI's for me ever and no dye, so I'am cooked, guess anyway.

I told Jim that you are happy he's still doing well and he said to give you a big hug!! So here it is....((((((hug)))))))

thanks Sweetie,
Love, Norma..

Oh Norma,
OMG, I completely missed the part where someone had tried to put you even near an MRI machine KNOWING you had type of clips of any form in your head! Clips, especially back when you had your surgery (and even now) are all made from some form of metal! Teflon is just a coating in most cases and are used so they aren't seen a CT scan (although why you wouldn't want to see them in a CT and thus let anyone think there were no clips at all simply defies the imagination! (the 1977 article was found at http://www.springerlink.com/content/g25085753721855r/). But I could find no reference to any form of clips that are safe for use in an MRI (only plastic would be MRI safe and there are no clips for aneurysms that are made of plastic!).

Teflon is used in various surgical products (e.g. thread, coating on clips, in mesh for hernia repair, etc.). My brother had bilateral inguinal hernia repair using mesh 18 months ago. What the doctor neglected to tell him (but he thankfully found out, ironically from the TSA while going through the airport screening, was that she used titanium clips to hold it in place! The metal detector was blaring!). In his case the mesh and the clips are visible on the TSA screening tests; it was a rotten example of an inginual hernai repair and he just had to get the left side repaired yesterday (hopefully this time the doctor actually repaired the hernia FIRST before putting just putting mesh in place!). BTW, inguinal hernias in particular are also an extra-renal complication of PKD.

There must be some screening options left for you (just as there must be contrast options left for me despite my decreased kidney function and fatal allergy). PET scan? Triple helical CT without contrast (although I've always had that with contrast)? Nuclear imaging? I do know that X-rays will only show the hard structure of your brain (skull, sinus cavitities and your existing clips; it won't show any blood vessels, brain tissue (unless there are shadows of some sort (like the mass of your brain on the skull).

And Norma, you weren't wrong about what you were told; you were told what you were told. It's was simply that the information your received was errorneous (and was back before the internet too). A large part of why I wanted to ensure you understood how aneurysms form in those with PKD (or anyone else) is so everyone else understands how aneurysms form. I definitely don't want you to roll over and play dead by any stretch of the imagination! I want to make sure our whole PKD family stays safe and healthy!

All too often we get erroneous information from our doctors, either based on their lack of knowledge (it's outside their realm of knowledge or expertise; they don't bother doing the research needed to get the right information for their patient or they base everything on what they learned in medical school 40 odd years ago and have never cracked a medical journal, read an article and to keep their board certification, take the easiest possible continuing medical education courses (at most pay your fee, get your credit and it usually means no cost and a nice dinner by big pharma with a small, short presentation about some new drug and voila, they're good to go for another 10 years!). I've received bad info too over the years; I just do my research and call the doctor out on it (and them educate and/or fire them depending on the egregious nature of what was said and done). It get stuck in our minds and by golly, that's it, period, end of discussion! Or some trust their doctor, who may be as nice as the day is long, but equally as incompentent (some of the worst doctors in terms of actual medical knowledge and ability to practice safe or good medicine get the best ratings and grades from their patients because they are so nice!), so patients stick with them, much to their determinent and often despite any and all evidence to the contrary, because they like them personally. In your case, the info was just wrong, dangerously wrong (but may have been all that doctor knew at the time; the knowledge of PKD and medicine in general has progressed dramatatically since you had your surgery). Just consider the difference of what the "local yokels" (your term) WANTED to do to Jim last year compared to what Dr. Amin was ABLE to do for Jim, all thanks to your knowledge, skill and YOUR perserverance and persistance in searching for the RIGHT doctor and not just passively going by the opinion of the first or even the second, but local opinion, and thus you ensured he preserved not only his voice, but his throat and most importantly, his life!

Okay, now I just have to figure out that imaging challenge...one for you and one for me!

Lots of hugs back to Jim and he sure better give you big ones from me, too!
Ruth