How am I doing?

Good question. September got in the way, I slipped into the back-to-school drowning pool and needed October to throw me the life preserver.

How am I doing? Exceedingly well thank you. If the question relates to results, I've got nothing for ya. Results in this study are determined by kidney volume. Mine will be measured again on June 2008. I tried my best to worm some info out of Lovely Lorie last week during my four month visit but she was giving nothing up. Nothing. No news, damn. My creatine level is irrelevant at this point. I'm normal and not tracking for failure in the distant future. Yes I know everyone is different but my kidneys are not that big, so really, I don't think they will blow in the next few years.

Symptom wise, no problem. I've always been a huge water drinker, we have a Arrowhead Water dispenser in the house and I now find myself guzzling half full drinks that the kids have left behind. I also grab the ecologically incorrect water bottles on my way to anywhere in the car. Problem solved. Since I don't work I can easily go as often as I want without significant disruption (not alot of buckles or embarrassing looks from co-workers). I've heard that some study participants cannot tolerate these side effects, but I'm pretty sure those individuals have never been pregnant. Yes boys, this means you. Suck it up, it may be worth it in the end.

I really do have to watch my hydration levels, especially when I want to enjoy some vino in the evening. I can get a REALLY BAD hangover on just two glasses of wine if I haven't grazed on all those half filled water glasses. One weekend the dispensor broke and sure enough, Sunday morning I felt like I had spent the previous evening gorging on jello shooters. After just two glasses of wine, not pretty.

So there you have it. I'm doing fine.

It took my breath away

Gossip. I have some.
It is medical gossip.
I can't verify it's truth.
Don't know the people who uttered it.
Couldn't tell you on which end of the telephone line this tidbit lies,
Lies? Dare I be the purveyer?


Sit closer and hear this......

In a chat room, someone said that
Lovely Lorie said that
a participant who had been on Tolvaptan for a year has
show a reduction in his kidney size,
a lessening of pain and a shrinkage of cysts.

CAN YOU STAND IT

I CAN'T

I CAN'T WAIT TO GET INTO THAT GIANT MAGNET AND HOLD MY BREATH
I CAN'T WAIT TO COMPARE MY MRI REPORTS

I'm 12 weeks +three dosage weeks into this thing. One quarter of the way through to my 2nd MRI. I CAN'T WAIT.

Using drugs to get off drugs

Tolvaptan is my methadone. When I started this trial my limited brain made a connection to my even more limited medical knowledge. If I'm taking a gigantic diuretic, then maybe I can get off my hideous ace inhibitors (that lower blood pressure). After all, diuretics were the old fashioned way of lowering BP, and I'm taking massive dose. Why not? (I'll explain why not later). So I did it, I dropped the little blue pills and withing two days I felt fabulous. I was back. No more mental fog, no more having to sit down, no more yawns. Now, we all know that following one's "feelings" is not exactly a mature, responsible way to live. I "feel" like pitching the kids out the window sometimes, but try to restrain myself. But this time, I "felt" that if I can get my heart rate up through daily exercise, lost the extra pounds, and ate like a caveman, I can stay off those nastys. So far - it has worked. I'm back to religiously taking my BP a couple times a day and can track the spikes/valleys. I can relate them to a little too much soy sauce, a lack of action at the gym. I'm running at an average of 124/82. Not ideal but I've got another 16 pounds to lose and can bump up my trips to the treadmill to daily.
No, I haven't' discussed this with my neph. I'm still pissed at him for not reading my Tolvaptan info. He'll probably say that I'm missing out on the other benefits of ACE inhibitors - prevention of the thickening of the heart walls, and that while 124/82 is good, its not the ideal 110/70 that we should all aspire too. But I'm almost there. I need the time to shed the pounds and rev up the heart. I need to be myself again, for a while.

Better Living Through Chemicals

I looooooooove drug companies. I admire corporations in general and find it hilarious that screen writers today can no longer come up with any politically correct villains other than large companies. Even the latest "Pirates of the Caribbean" writers couldn't find any new monsters other than the East Indian Trading Company. Having paid my $7.50 I expected some new and improved Kraken, some dazzling CG beast. Arrrrr, nope - monster replaced by corporation, very lazy on the writers part.

But enough about popular culture, I'm writing about my favorite things - drug companies. Without them I would be a vegetable in a nursing home somewhere. Without them my blood pressure would surge to such levels the my head would explode. That little blue pill in the morning changed my life and I am GRATEFUL. Sure it too two years to snap out of the funk that my exploratory chemical cocktail put me through. I did get exhausted in the evening, even with an exemplary diet and exercise standard (OK that has only been for the last few months). But consider the alternative........

Its like anything else, you don't appreciate them/it/that until you need it. And I needed that greedy, unfeeling, inhuman, publicly traded, fairy godmother. Her avaricious fairy dust saved my life. Her Asian sister is sprinkling new dust on my forehead. Arigato.

Those mighty mutant mice

Imagine, being created to be destroyed. That is the plight of the lowly PKD lab rat/mice. Once those PKD gene (genes) were identified in 1994/1995, PKD research took off. Why? Because some lab somewhere could infect the mice with those nasty genes and presto! PKD kidneys. Let the games begin.

I've never met one of these creatures but I have seen pictures of their kidneys. Pictures of the "Before" -bulging, bumpy PKD kidneys, and "After" - adorable, pink, solid, post Tolvaptan kidneys. A GREAT RECRUITING TOOL. It sold me on the stuff.

I'm sure the PETA people would be appalled. And it does give me pause, should we create/destroy these creatures just to further my life? Biblically I think we're covered. I'm not sure about the other religions, it might piss off the Hindus or the Buddhists, I'm ignorant of their theology. What about the Muslims? Where do they weigh in on this? Secularly, I'm thinking that we're forwarding the "survival of the fittest" aspect of life (if one takes their moral direction from Darwin). Keeping this species going, even when some of us have defective genes. Lets fix the less fittest. Isn't that what we as humans do? As civilized creatures we take care of our weakest. Our herd doesn't leave them (me) lying in the dust. But our path is paved with the lives of these small creatures, these PKD mice.

I think I'll be nicer to the dog.

Its a little late to be reading this article

Time Magazine April 22, 2002. The Cover reads "How Medical Testing has turned millions of us into....Human Guinea Pigs" Fortunately, Time's picture of the most horrendous offender, a Doctor McGee, looks nothing like my study Doctor - what a relief. Turns out Dr McGee "was a good surgeon and a decent man". Apparently he really believed that the study drug he was dolling out was a cure for malignant melanoma. The good Doctor didn't actually want to "run a study", but to "administer the drug" BIG DIFFERENCE. Worst yet, the university review board let him get away with numerous screw-ups. It wasn't until a nurse blew the whistle (the nurses know everything). An independent audit review found deficiencies "so severe that it is beyond the scope of this report to advise corrective actions". Here's the rub - 12 out of his 94 patients fought to keep the trial going. They loved this guy and wanted to goods. Talk about hope.

So where am I in this thinking? Given the risks of ingesting an unproven chemical for the next three years, one would think that I would have really done my due diligence on Dr. Lee (my study Doctor). And I made a minimal attempt. But really, I knew that if I liked the nurses, I was in. They remain our greatest protectors. Not the regulators, but those individuals who are doling out the pills and taking the fluids. If you get a good one (like my Lovely Lorie) you are golden.

Don't get me wrong, I know strong regulatory oversight is critical to insuring that such a large system is working. But as often as there are screw-up Doctors, there are golden nurses. I'm counting on the Lovely's of this world, we really need them. That's where my hope comes from.

But will Tolvaptan get her out of Jury Duty?

I'm in. After a week on the "high dose", I'm certain that I have the Tolvaptan. What a difference a little hope makes. Hope for the future - no dialysis, no transplant....and hope for the present - wean myself off the blood pressure medicines. That stuff keeps me down, literally. At my high dose of ACE Inhibitors, I'm often looking for a chair......and the mental fog is astonishing. My friends have been very patient with me, repeating whatever they just said as if I had Alzheimer's, because my drugged brain was unable to accept the info. I WANT OFF THAT STUFF.

Now, I could have taken steps to turn my body into a high blood pressure fighting machine - cardio trained heart, lithe muscles, and a digestive system that only accepts low sodium/antioxidanted foods grown locally. I could have done it then, but what was the point? My gems were shooting off so much of the high BP hormone that I gave up. Let the chemicals do the work, if I'm going to feel like crap I may as well enjoy a few chips and sushi.

That was the lifestyle I choose, but it wasn't much fun. I had to pace myself, in the evening my energy level was so low that I needed to decide what tasks to do each day, and exercise wasn't usually at the top of the list. No, a nap didn't help, the fatigue was relentless. And the YAWNING! So impolite to hold a conversation while trying to suppress a unsuppressable yawn, people thought that they were boring me. I couldn't help it, it was the drugs yawning!

Now I'm trying to change, let my body evolve to its new state: Tolvaptan, exercise, water and no sodium. What a life.

She's gone mental

A week of Tolvaptan, I think. My rings spin around even though I discontinued by diuretic, a sure sign? I've been very hesitant to accept that I received the prize. While I may be optimistic, I hate to be disappointed and I'm just not 100% sure. I've always drank gallons of water, I'm always thirsty and peeing, its just my nature. So every time I was thirsty over the last week I've wondered - is it the Tolvaptan talking? Today I go in for the "medium dose". I'll know with in 24 hours (or did I say that last week?). Mental. And the mental energy that has gone into this week's speculation is outrageous. I wish I could harness it and offer those BTUs to some energy utility to replace my carbon footprint. Talk about GREEN.....

Name your poison

Which one do I want? Seem silly, doesn't it? Taking a sugar pill for 3 and a half years. Today I get the Tolvaptan, or not. I should know within 24 hours whether it is the real deal. I'm somewhat grateful that this drug's side effects are visceral, I really hate not knowing. When I was a kid I used to unwrap my Christmas present (I knew where Mom hid them) just because I couldn't stand the suspense. This should bring rounds of peeing and ridiculous thirst, or not.
But which one do I want? I really can't answer that. Seems enough right now just to participate, be close to the heat. I actually feel like I'm doing something to fight this disease and that is the gift.

I'm convinced they rounded up.

I'm not excited any more, Lovely Lorie relayed the MRI count - 180 cysts on the right kidney and 220 on the left (my original ultrasound only spoke of "multiple" cysts, one the size of an orange). Plenty to qualify for the Tolvaptan study, hard to hear. I really wanted Lovely to tell me that mistakes had been made! That the cysts were on the outside of my gems, not inside. Instead I got numbers. And when asked, Lovely in her most professional and compassionate voice replied, "There were several participants in this Tempo study with many more cysts than that......" I'm sure she was lying. IT WAS HARD TO HEAR.

It took two years and I'm in. As the risks and reality of participating in a three and a half year clinical trial materialized before me, I got grumpy. I even had to yell at my kids (we no longer have a cat so I has nothing to kick). I suppose that facing one's demons is like grieving. Individuals are just that, reacting differently to facts, diagnosis, numbers. My bravado has deflated and I'm terrified. Maybe it isn't bravado but an inexplicable knack for plunging in and waking up later. I woke up grumpy. And then I remembered that I'm not supposed to drink caffeine, so now I'm even grumpier. On top of that, if I'm not careful I could have a really, really long hangover.......
Enough, I'll be fine.
I'm in.

The excitable nature of an optomist

OK, so I lifted that line from Michael Chabon, so what? He was just pointing out the obvious. I also admit to being an optomist, and LUCKY. Think about it - I was diagnosed with PKD (right place) at a time when research was funded at a historically high level and was showing promise for the first time (right time). I am genetically inclined toward investigation and have the personality to charm my way into situations above my station - like the time I elbowed my way into a cab with a principal PKD researcher from Harvard so we could "chat" on the way to the airport (LOVED that guy). Is that lucky? I think so. Did I make my own luck? Sure, I attended the PKD conference in the first place, heard him speak, had the nerve to introduce myself and inserted myself into this guy's space. I was excited, and our discussion confirmed my optimism.


And your point is? I'm really blogging this as a deversion because I can't stand waiting to hear if I qualify for something I've been waiting TWO years to get into.

I will know this week, and I'm excited. That would be me.

Tricky Business

40 minutes in that tube, that miraculous brownie box of the 21st century, the MRI machine. "Take a breath and hold" - I must have done that 30 times, trying to get the perfect picture of my jems, my kidneys. I really tried to behave, knowing that if I could provide the perfect pictures, the guys in Massachusetts would Pick Me! Pick Me! Arms above my head, gliding through imagined tropical waters with each breath, I can swim through this! Pick Me! Pick Me!

I won't know for two weeks. The pictures get shipped to Mass and then analysed by some research genius as to size and number or cysts. Lovely Lorie said that a gaggle of siblings were trying to get into the study, competing for the greatest number of cysts. I like that. Humor above all else.

Later that afternoon, the study doctor and other research tech (Lovely had the day off) said that I was the first person to describe to them the arduois MRI process. Leave it to me to be the biggest whinner. But I was interested in knowing how people who were sick or out of shape could handle such torture? I had two years of pilates training and could actually freeze up my midsection like a slab of granite, speaking to each can in my 6-pack and willing it to rigormorph (just know that my 6-pack lies under a mound of flab so don't think I am bragging). How did the other patients manage it?

Next time I will bring something to hold in my hands (which are raised above my head in side the tube). I will tell the Tech that I don't need to rest in between the 18th and 19th "Take a breath". I just want to power through it. Lets get my time down, I want a personal best of 38 minutes, maybe 37 minutes in the tube.

Lets hope I get the chance.

Obsessed

I'm reading the chat room posts at least three times a day. A test participant wrote that the Tolvaptan had caused her to lose her appetite. It is a miracle cure! Another participant posts that she hasn't had that effect, It isn't a miracle cure! I'm looking for volume here, I figure that if enough participants report this side effect then it really exists. Now I really want this magical elixir.

By now I should have been MRIed (that is a good word) and sweating about the size of my kidneys. These girls need to be big enough to measure plenty o'cysts, if not - I'm out. The Lovely Lorie (aka Gatekeeper) has worked diligently to secure my records from my neph. No records, no MRI, no elixir. Four weeks, no records. Lorie has identified the culprit, an overworked nursing assistant who claims she mailed them. Perhaps she did, perhaps she didn't realize that I can't get into this study until those records are received evidencing a diagnosis of PKD. That this is my life we're talking about. The intrepid Lovely has instead received only four pages of notes/labs. She proclaims - We have ENOUGH! Within those four pages have some slight reference to a PKD diagnosis. They should have just called my witness, he would have vouched for me.

I can now schedule my MRI.

Anything but THAT!

Admittedly, I am a bit of a highbrow. When I first heard of Dr. Lee's Tolvaptan trail center in Riverside I blanched - UCLA, USC, even Irvine - but Riverside? In this city of 12 million why did the study Dr have to be from Riverside? I googled Lee and the only thing I could come up with was a letter from the Feds citing Medicaid fraud. Figures.
Soooo I spend the time reading the fraud documents and found that maybe the Feds were a little hasty, after all - turns out that Dr Lee's nurses adamantly denied the Dr's wrongdoing, these gals defended his honor citing faulty paperwork not shoddy practices. And we all know that the nurses are the one who KNOW. I forged on......
I was a bit nervous on the drive, as you can imagine. Heading East of Los Angeles, and more East, and more East. Riverside. Of course I made the wrong turn up a side street, as I passed check cashing stores covered with graffiti and drive-thru tobacco outlets I tried not to panic. Wrong street! My relief at discovering my navigational error was visceral. Of course his office couldn't be here, its round the corner next to that massive, newly constructed hospital over there. Silly girl.....It would be fine, Riverside is a wonderful place, full of "cutting edge" medicine types. Then I say the sign....the hospital sign...it was a KAISER hospital. Groan, all was lost.
In case any of you haven't heard, Kaiser was forced to close two of its transplant centers in northern California due to sheer incompetence. What Kaiser put those patients through was criminal, and the Federal oversight of their operations was disgraceful. Fortunately, the intrepid LA Times pulled records and discovered what was going on and had the centers shut down. That is how bad it was.
Turns out Dr. Lee wasn't from Kaiser. Turns out he had applied for the study three years ago, and was accepted as one of the two centers on the West coast. Turns out he just got his Law degree, just because he could. Turns out I liked him and the lovely Lorie. I really liked them. I'm in.

I need some water

This is harder than I though. Today I'm going to my first appt with the study doctor. My brain has been occupied by the Tolvaptan impulse, I can't think of anything else. Should I? Why should I? and can't someone just make this decision for me? I'm needing kind words from loved ones, they are not exactly forthcoming. I need my doctors to spend a little time on this, it didn't happen.

Guess I'll have to get my own water.

Useless

I tried - it was the 3rd time I had spoken to my neph about this Tolvaptan trial. Two weeks prior to this appointment I even sent the guy an e-mail with links to info sources. I asked him to review the info so that we could speak of it at the next appt. He said he did read the e-mail but (and this is rich), doesn't have any info on the drug. As he spoke I knew he was useless - that another layer of security had vanished. Blah Blah Blah - "This is cutting edge" - Blah Blah Blah - "I'll read the stuff the company sends me" - Blah Blah Blah - Why can't he just admit that he doesn't want to take the time to evaluate the info? It is out of his realm, so admit it.

I'm on my own again. I don't like this.
Lucky for him I still think he is an amazing human being, just LIMITED.

I'm in

Got the call to set up the initial appt. Now I can panic.

Here is my first question: This drug interferes with the body's ability to respond to the hormone that allows our cells to retain water. Does that mean I will have a 3and a 1/2 year hangover? (This is what a hangover is - dehydration) and if this works, does that mean I'll have a lifelong hangover?

Shut Out

I feel like I'm applying for college. Get the app in (med records), tout your assets (I have a creatinin level of .8), get your recommendations in (my neph appt is on Wednesday) and GET IT ALL IN ON TIME. But in this case we don't know what the deadline is. "Lovely Lorie", the administrator at the Southern California clinical trial site, has said that there are 50 slots - "no problem getting in". Lovely does have a nice voice on the phone (hence my "Lovely" moniker) but is she the one, the admissions czar, THE one who can anoint me with the elixir? Or is she a clerk, doing her job.

My experience with the national registration line has been ridiculous. These operators present the great information wall to the diseased public - "yes, we have you in the system" "yes, we're going to send out notices" "no, we don't understand the 19 month delay". Never got the notice....only reason I know about Lovely Lorie is from a post on a PKD forum. Last week this forum also reported that Otsuka (the drug manufacturer) won't be having trials in Northern California at Stanford or UCSF because "they have enough participants". Shut out in Northern California.

Wish me luck, pray that I won't be in the same boat as the 15,000 students that got rejection letters from Duke - because at this point, it is my first and only choice.

Why the military reference?

"Syringe soldier", What the Hell is that? It is the impulse to enlist. To join the grunts, those poor infantrymen who are the first fodder on the battle field. Shot at, not by cannons, but with AKA-47 syringes filled with the enemy (enough hyperbole for ya?). At this point the new drug is the enemy, unproven, potentially deadly, but ready to be turned into an ally at the end of a long and arduous application of the scientific model. Before we can align ourselves with this chemical assailant we must determine it's loyalties. WE MUST USE HUMANS. Right out of a Michael Crichton novel.

My Cardiologist looked concerned, as he does when we talk about our respective children (but he never has that look when discussing me). "So many patients want to rush into this, they don't think about the risks, just the newest snake oil being marketed as the cure all". Talk about hyperbole, my radar went up with "they don't think about", a bit patronizing. I dutiful listened as he went on to describe how the drug companies have killed off test participants, how the FDA has become too lax in its oversight and THE RISKINESS of the endeavor.

He then went on to prescribe a new ACE inhibitor to reduce my soaring blood pressure. Raving about this drug, how everyone on the planet should be shooting this with their smoothies in the morning. This stuff will save my life. Geez, I was grateful, but to whom? Not my Dr., he was just the historian, knowledgeable about outcomes, battles, enemies. Granted, he had sifted through all the source material, original documents, archives. But who did the real work to get me there? Some poor medical doughboy in a hospital gown getting poked and analyzed on a chemical level, and then turned into a statistic. Risking their lives for us.

I decided that I wanted to suit up with the rest of them. And while you could say that I have an extreme vested interest in this drug's success, I could have let that other marine take the risk, waited a few more years (Tolvaptan has been fast-tracked by the FDA). After all, I've got a family to raise, three kids under 14. I like my life, love my husband. Why not wait?

It is because of those kids who have a 50/50 chance on inheriting this gene. I am willing to fall on that syringe for them. Lets just hope I qualify for the study.

Can I get a witness?

St. Patrick's Day 2007 - Everyone has their own physiological land mines. Sometimes these internal bombs can be seen, felt, discovered. Most times they can't, an errant gene, a hardening artery. I just happen to know my mine, my mines. I have two, aka my kidneys. I was diagnosed with Polycystic Kidney Disease in 2002. Yes, it rocked my world. More on that later.

Today, I'm trying to enter a clinical trial. "Tolvaptan", the first drug that has show the promise of stopping the cysts that are wreaking havoc on my kidneys.

I fill out my name on the medical release form. The first small step. Date of Birth, that's easy, but I flashed on the next logical question, date of death. Whoa, slow down Julie, you're losing it. That is a different form. Take a break. Let my mind massage a scenario about how I would argue with my "neph" (Nephrologist - kidney doctor) to release my medical forms. Let's just call. The Dr. Neph's office manager flatly stated "What do you want the forms for?" As if it mattered to her, those forms represented my life, my health, why did she want to know? Before those forms could go out, I needed to sign a release. Hold the phone, the release says I need a witness, a second signature on this critical document.

Can I Get A Witness?