Wednesday, April 01, 2015

Hydrocholorizaytoiuwoiet2tkadn and super powers

I'm pretty sure the title of my post is the name of my new medicine.  Or, you know, pretty close.

It's a very long name for a very tiny pill.  Put simply, it's a water pill.  My neph prescribed it to help lower my diastolic numbers without having to drop my systolic numbers too low.  Or maybe it's to drop my systolic number without dropping my diastolic number too low.  I'm not sure.  There are altogether too many tolics in my life.

Put another way, it's a pill that makes me pee.  A lot.

It's hard not to have a strong relationship with urine when you have a kidney disease (and hard not to read about urine when you are on a blog about kidney disease.  If pee makes you queasy, you might want to go watch some cat videos, or something.  Although cats pee on EVERYTHING.).  It sort of comes with the territory---monitoring urine for color/odor changes, making sure that urine samples are "clean catch" samples, and the oh so appetizing jug 'o pee in the fridge when you are collecting a 24 hour urine sample.

The one perk of having PKD is that my urine output has always been, well, pretty low.  One time I gave so little urine to my neph for a 24 hour urine collection that she said that if she didn't know it was me, she would assume that I did it wrong.

I call this a perk because it means that I never have to find a bathroom.  The usefulness of this skill was brought home to me when I traveled last year with my extended family, particularly my older sister, who ALWAYS has to pee.  She said that everywhere she goes, the first thing she looks for is the bathroom.  She can't get through a movie without having to pee.  Twice.  It sort of blew my mind how often she had to pee, and I realized that having to pee only twice a day was sort of like my super power.

And now I have my kryptonite.

This pill makes me pee all the time.  I mean, all the time!! I even had to leave a movie the other day in the middle because I had to pee.  What?  Me, missing a movie to go to the bathroom?? Never!

Granted, I was drinking a large Diet Coke at the time, so I had the extra added bonus of another diuretic, but I'm usually quite good at being able to hold it until the end of the movie.  Having to miss part of whatever movie I was watching made me feel

(I'll admit that since I can't even remember what movie I was watching, I probably didn't miss much.  Still, it's the principle, people.  It's the principle.)

And I also hate that I have to be on another med.  I know this disease is progressive, I know that keeping my BP low is key, and I know that all of this is important, especially now that my GFR is hovering around 50 (which, for those who aren't obsessed with medical lab reports, is not a good GFR.  It's a lousy GFR.  Totally crappy.). But every time I'm reminded that this disease is progressing, it makes me swear under my breath a little and I go sit in the car and call my husband and cry a little, and then we go eat lunch together while he tells me that I'm wearing the"I hate PKD" face.  (I apparently also have  a stressed about my job face, a worried mom face, and a stressed about people at church face.  I guess stress makes my face uber expressive.)

But now when we eat our I Hate PKD lunch, I'll have to pee afterwards, like a normal person. Oh how the mighty have fallen.

Monday, March 02, 2015


I remember the last time my father held me on his hip.  I was 8 or 9 years old, too old to be held on his hip, and old enough to remember that I was too old.  It was after a performance I was in with a children's singing/dancing group.  There was some kind of reception after the performance (old enough to remember I was too old, too young to remember what we were doing, exactly).  I was tired, and whining, and I wanted to go home.  I pressed myself against my dad, whimpering a little--I might have even wrapped my arms around his legs.

You have to know that my father is very tall, and all of his height is in his legs.

He was patting me on the head, trying to get me to calm down, and then he reached down and hooked his arms under my armpits.  My mom asked, "What are you doing?"  He said, "Heather is tired.  I'm picking her up."

And so he picked me up and perched me on his hip.

It was awesome and embarrassing, all at the same time.

Again, my father is tall, so perched on his hip made me the tallest person in the room, easily.  It meant I got a completely different view of the room, and I was suddenly no longer tired.  Instead of adult legs flashing by, I could look down on their heads.  I could see completely across the entire room.  I was giggling, flushed with excitement of being so high up, and also hoping that none of my friends would see that my dad was holding me.

Indeed, one of my friends did see, and I have a distinct memory of her looking up at me and shaking her finger, saying, "Heather, YOU ARE TOO BIG to be up there!"

I think I stuck my tongue out at her.

But as she said it, I knew it was true.  I reluctantly slid down, and satisfied my childish need for closeness to my parent by holding my dad's hand instead.

Even as a kid, it kind of made me sad to know that time was moving forward, and time inevitably shifts a child's relationship with a parent.

My father, now 81, has been recently diagnosed with pancreatic cancer.  Like most of these types of things, it came as a shock, and my family has been thrown into a new world, my parents especially.  Talking to my siblings, we are all in varying stages of grief, which is to be expected.  But part of me realizes that even though 81 is a little younger than we all expected, and pancreatic cancer moves faster than we'd like, the truth is that the time when we would have to live without our parents was coming closer, because people don't live forever.  Time kind of stinks that way.

We've been lucky in that both of my parents have always been in excellent health, with no major health issues.  Nope, not even PKD has slowed anybody down.  We know that my father carries the gene, which I inherited, but his cysts are small, he has low blood pressure, his kidneys are of regular size.  When he asked my nephrologist if he should be taking measures to slow the progression of his disease, the neph told him he'd be dead before PKD could kill him.  At the time, 8 years ago, it was funny.  The joke now falls a little flat.

And now time seems to be the question of the day.  How much time will we have before he dies?  Will the treatment/chemo buy us more time?  Did we catch the tumor in time?  How much time before we know if the treatment is working?  I was talking to my sister about summer plans, and she said, "Does Dad have that long?"

Time.  It's a tricky, tricky thing.

I was with my father when he got the biopsy, when it was starting to really look like he had cancer.  I quipped, "Well, now YOU have a life threatening disease.  Welcome to the club."  He gave a rueful laugh, and said something like, "Nice club."

Yeah.  What a  club.

Saturday, January 31, 2015

January 31, 2006

This was the day I was diagnosed.  9 years ago today.

I was diagnosed via MRI.  9 years ago today, I underwent my first MRI, this one a back MRI ordered by my doctor, who did not suspect PKD.  He suspected I had a spinal tumor that was causing my back and abdominal pain.   He was wrong, of course, but at least he took me seriously when I told him something was off about my body.

I had never had an MRI before, and, not gonna lie, I found it terrifying.  It was a closed MRI, and I went in head first.

(This picture makes it look like there is a comfy amount of space between the person's body and the top of the tube.  Trust me, that space is not comfy.  Also, I went in further than that, because it was a back MRI.)

There was no button to push, no squishy ball to squeeze if I wanted out, no mirror to see anybody in the room.  I asked the tech what I should do if I panicked and wanted out, and she shrugged and said, "I dunno.  Wiggle your legs.  I'll see that and come back into the room."

And then she left me alone in a cold room, stuck inside a tube.

I breathed.  I hummed.  I sang songs in my head.  I imagined terrible, terrible, terrible things happening to the hospital while I was stuck in the tube, like an earthquake hitting and killing everybody who knew I was down there, or an electrical fire that cut out all the power to the building, so I would be stuck there in a fire AND without any power to the machine to get me out.  Can the jaws of life cut through an MRI machine?

These are not calming thoughts.  FYI.

I've had several MRIs since then, including a brain MRI a week after my diagnosis where they strapped my head into a CAGE to immobilize it before they sent me into that tiny tube.  That was also terrifying, and I knew I had to do some different kind of mental mind games to get through it.  This time, instead of pretending that the whole hospital was going to collapse around me, I tried to pretend I was in a rocket ship, heading to the moon, or the space station.  I read an article once about how astronauts had to stay in a tight, close position the whole way to the International Space Station, so I pretended I was John Glenn, and that this was just another space mission.

Then I accidentally pretended I was a French prisoner, trapped in an iron mask forever.  This unwelcome image was courtesy of the 15 minutes of Leondardo DiCaprio's movie "Man in the Iron Mask" that I happened to watch in high school, to my detriment.

Rocket ship images?  Kinda fun and cool.  French prison images? Not fun or cool, and very unhelpful when you are trapped in a face cage in a tube.

Anyway, that was 9 years ago.  Like I said, I've had several MRIs since, and while they are never pleasant, I no longer need intense mental stamina to get through them.

(I still sometimes pretend I'm an astronaut, though, just because, you know, space.  It's cool.  And I'm kind of a dork.)

More has happened than just MRIs.  I went from a normal BP to needing 35 mg of Lisinopril to keep me from stroking out.  I had an almost flat stomach when I was diagnosed, and last week somebody asked me if I was pregnant.  My husband would often tell me how cute my belly button was.  Now I have an umbilical hernia that often resembles a hard apricot that I have to manually shove back into my body, and I can't do sit ups anymore--doctor's orders.  The disease.  It progresses.

But there are really good things that have happened in those 9 years too.  My daughter was born.  My husband got a new job that moved us to a city that we love.  We got a sweet dog that have taught my children to love animals.  I'm in the thick of learning a new career that is challenging and fun. My children and husband are healthy and thriving, which I know is not a given.

So this life.  It progresses too.  And it delivers rich abundance even on those days when I have to lie curled up in a ball for the pain.  (And just FYI, speaking of Leondardo DiCaprio, "Titanic" is a great one to watch when you are in so much pain you think you might die.  It's boring enough that you don't have to pay close attention, and if you are in enough pain that you are hating the world, the drowning scene is kind of satisfying.)

(And you can also yell things at the screen like, "That ticket brought you DEATH, you stupid git!"  That helps too.)

Sunday, January 25, 2015

It's twins!

The title of this post is what I should have said to the lady who said to me yesterday, "Oh my goodness, Heather, are you preggo?"

What actually  happened was much different. I blinked at her, touched my "baby", and said, "No, I'm not pregnant, those are my kidneys. "  She looked confused, and since she had interrupted me in the middle of a conversation/task with another person, I didn't have time to explain, so I simply said to my husband, who was standing behind her, "Can you explain it to her please?" while I turned back to what I was doing.

It was an awkward moment, to be sure, and I half listened to my husband's explanation as I worked.  I didn't hear everything he said to her, but I did see her face later.  She was sort of gawking at me, a little amazed and still a little confused.  I smiled at her and we went along our way.

I can't blame her---it does sound incredulous to say, "Oh, no, I'm not pregnant, those are my kidneys."  If you don't know what PKD is, and have never even heard of it, and your knowledge of anatomical structures of the body is sketchy at best (and my husband has to remind me that most people's knowledge of where things are in their body is sketchy at best), then it is, at the least, an odd statement. At worst, it's incredibly alarming.

I asked my husband later what exactly he told her about PKD.  We talked about it, and then he said, "How do you feel that she called you pregnant?"

I considered that, and said, "Fine, actually."

And I was surprised to find that it was true.  I've been dreading this day for a while, knowing that my body was changing in more obvious ways.  I've cringed in the mirror and moaned to my husband and friends (and readers!) about what my body looks like, hoping that nobody else is paying as much attention to my body as I am, that most people just don't notice.   And I've always wondered what it would feel like if somebody asked me if I was pregnant, thinking I would be mortified.

(Turns out, I'm really rather vain.)

But suddenly, when it came, I thought, "Well, there's nothing I can do about it, this is it, people" and I found that giving up that control was far easier than I thought it would be.

I can't exercise this baby bump away.  I can't diet it away.  I can't spanx it, or suck it in, or tummy tuck it.  In some ways, that's been incredibly hard, having no control over it.

Yesterday, it suddenly felt really easy.

I have no control.  It is what it is, and I have a feeling I will be fielding more questions about my pending bundle of joy in the future as my kidneys continue to grow and my body continues to change.  Expending large amounts of emotional energy on the shape of my body will not help.  Best to let that go and spend that energy on more productive things.

It WOULD have been fun to tell her I was expecting twins, though.  Oh well. Maybe next time.

Monday, December 29, 2014

HALT PKD, GFR, and Stage 3

I got the official transcript of the HALT PKD study the other day, fresh from the presses at the New England Journal of Medicine.  I was surprised to see that Dr. Steinman was not first author, that it was Dr. Schrier.  I've never heard of Dr. Schrier. I don't know why it bothered me.  It just did.  What can I say, I wanted to be able to point to the top of the list of doctors and say, "I KNOW HIM!"

Clearly, kidney disease has made me prideful about stupid things.

I read through the abstract, and then skimmed through the study.  I have one semester of research methods under my belt, and another semester of statistics, and just had to review for a gnarly test that covered my entire academic career of learning about speech language pathology. All of this means that I was able to understand the study only slightly better than my mother.

Basically, here is what the study said:

1) Combining two different types of blood pressure meds doesn't really do much in terms of slowing down growth of the cysts, which is measured via MRI as total kidney volume.  So taking one pill of Lisinopril is just as effective as taking one pill of Lisinopril and one pill of Telmisarten, the "study drug", which is really just another type of blood pressure medication.

2) Keeping blood pressure low, below standard levels, is more effective at keeping the growth of the cysts down than keeping blood pressure at standard  120-130/80-90.

That part is actually super helpful and super concrete, and gives nephrologists at least something to work with, I think.  It's not like my nephrologist was letting my blood pressure run amok or anything, but at least now there is concrete evidence that aggressive treatment of blood pressure is a good move.

(I love my local neph, by the way.  It had been a long time since I had seen her, and being in her office again was like greeting an old friend. That sounds super weird, I know.  It IS weird.  But so is having cysts growing in your body, so, you know, back off.)

The study results also included my own personal study information (although I still don't know if I was on the placebo or not.  Not that it matters, I guess, since the whole thing turned out to be unnecessary).

(Wow, that sounded bitter.  I'm not bitter.)

(Well, maybe just a little bitter because being in the study was a pain and now it turns out it didn't do anything?)

(It's like when my diabetic sister found out that she had been getting the placebo in her study, and she was upset.)

 (I told her she was a data point, and that's just part of medical research.)

(She probably should have told me to shut up.)

My own personal statistics showed that my GFR is now 54, down from 79 when I started the study 6 years ago, which puts me squarely in the Stage 3 category of kidney disease.

Which means, of course, that I spent the evening googling GFR and Stage 3 kidney disease.

Are you interested in what Google told me?

(Of course you are.  If you weren't, why would you be here, am I right?)

GFR stands for Glomerular Filtration Rate.  Basically, it means how much blood your kidneys are filtering.  There is a complicated formula that involves your creatnine number (the lower the better--if creatnine is leaking from your kidneys, you're in trouble) and some other stuff that I've already forgotten, and then you plug all this stuff into a formula and come up with a GFR number.  And then that number corresponds to a graph of the 5 stages of kidney disease which tells you just exactly how screwed you are.

Turns out, I am officially moderately screwed.

I am squarely in the Stage 3 portion of the graph, which is characterized by moderate damage to the kidney.

I use the words minimum and moderate in my work all the time.  "Client will produce target phoneme with minimum to moderate cuing".  "Client will attend to task for 5 minutes with minimum to moderate redirection".  It's almost a useless classification---what is moderate vs. minimum?  I use the phrase deliberately when I don't know how much it's going to take to get a patient to do what I want him to do.  If he can do it without me physically doing it for him, then I classify the performance as a moderate assist.  But if I have to cue him more than once, I also classify the whole thing as a moderate assist.

Moderate is a big, broad word.

It's not as bad as severe, or maximum.  Maximum---well, there's nothing above that, right?  That's it, the highest, the limit.  Maximum damage means there is no more damage to be done.

Severe damage doesn't carry the same punch as maximum, but severe is still pretty bad.  It seems like nobody wants to say "maximum" because that means there's nothing left. Severe damage might mean you have a nephron or two still kicking and so the limits haven't been reached yet.  I suppose this is why stage 4 is categorized as moderate to severe damage, and stage 5 is categorized as severe and requires dialysis, because I suppose that if your kidneys are still functioning at 15% capacity, there is still not maximum damage done, because if there was maximum damage done, one would then, naturally, be dead.

And then you are not moderately screwed.  Then you are just dead.

After I read the study, I tried on the label of "moderately impaired", and I found it difficult to process.  I don't feel moderately impaired--in fact, since coming off the Telmisarten, I feel pretty good, better than I have in years.  And there is a lot about my life that is going really really well, and I'm pretty excited about my future. So if this moderately impaired, well, I guess I'll take it.

I'll let you know when I become moderate to severe.

Here is a link to the abstract, if you're interested:

HALT PKD results

Monday, December 08, 2014

Riding and balance

I'm learning to ride a  horse.  Not just, you know, hop on, giddyap and go Bessie, but actually learning how to do tricky things like post a trot and canter.  It's all part of my master plan to eventually become a therapeutic horseback riding instructor so I can do speech therapy with kids while they are riding a horse.

Yes, that's totally a thing.  No, I didn't make it up.  Seriously.

But the problem is, riding a horse is hard.  And I'm not very good at it.  I mean, it seems like it's not all that difficult, just sit up and tall and move, but there is actually a lot involved.  And sometimes my brain short circuits because it is trying to process too many things.  The other day, my trainer asked me to fix how I was holding the reins in my right hand, and I pretty much forgot everything else I was doing and thought about my right hand.  She sort of called to me and said, 'Whoa, what happened to the circle, and what happened to your trot?"  I called back and said, "Sorry, I forgot all about that stuff because I was trying to fix my right hand!"  She laughed and said, "You have to KEEP RIDING while you're fixing stuff!  Put it all together!"

Because, you know, horses are actually kinda lazy, and if you stop riding, they stop moving.  Except when they don't.  Sometimes if you stop riding, they think, "Hey look, HAY!" and off they go.

Neither one of these makes a person look like a professional.  Or even very dignified.

So it's humbling to learn a new skill.  I have to trust that my trainer knows what she's doing (she does). I have to train my own body to develop new muscle memories.  I have to make sure I'm continuing to ride even as I am panicking at the speed of the horse as she pulls up in a canter.  I have to be humble enough to be teachable. And I have to trust my core.

Which is hard, because my core has issues.

Since entering this training program, I just don't have time to exercise the way I used to, but I still try to squeeze short yoga sessions in a few times a week, and today I tried some balancing moves.  Most of what goes into riding a horse involves balance and core strength, and I want to improve both of those things.

I discovered today that my balance kind of stinks.  And it feels like it's getting worse.

I wondered aloud to my sister if my hernia and kidneys were to blame.  It's one thing to get fat and weak--fat packs on the padding around the muscles, but the muscles can still be strong under the fat.  It's another thing to have your muscles being stretched from the inside by ever enlarging kidneys, like a balloon filling up with air, getting structurally weaker as it gets bigger.

I worry that I'm getting weaker, and I'm not sure what to do about it.

I'm also getting dumber, but I know there's nothing I can do about that.  I have several tests coming up that I have to study for---tests about speech, tests about horses, tests about speech AND horses, and I feel my brain complaining, creaking to life, like that old man in UP when he gets out of bed.

This is also how my brain feels:

And I'm not even on drugs.

(Well, not THOSE kinds of drugs, anyways.)

I want to look like this when I ride:

 I am, however, fairly certain that if I tried the above maneuver, I would look like this:

I'm getting way too excited about videos and pictures.  I'll stop now.

Thursday, October 30, 2014

Bodies are not like houses

Our house is old.  Not cool old, like historical mansion type old, but rather annoying, built in the 80's with cruddy construction old.  We like the layout, we LOVE the neighborhood, but the house itself is, in a lot of ways, a piece of crap.

Over the 8 years we've lived in it, we've had to replace, well, a lot of things.  We've replaced the carpet, tile, and hardwood on the main floor.  My husband had to rebuild our porch because it literally fell off the house.  We've had to replace both the front storm door and the back door, because they, again, fell off.  We've had to replace the roof, the siding, and in the not too distant future, we will have to replace the pipes as well.  The pipes are a ticking time bomb, we know, but we've learned as homeowners that unless you want to be buried under a mound of debt, you have to just take one thing at a time, and currently we have a broken garage door to fix (why is it always doors???).  And we've learned the hard way that replacing worn out things is almost always better than trying to repair them.

I read an article once about aging, and how there is a medical doctor out there who is trying to reverse the process, and how he thinks that's the new frontier of medicine.  With an aging population and healthcare costs skyrocketing, it seems kind of legit that keeping people healthier longer would be worth some research investment.  But the story got kind of wacky when the doctor in question started talking about how the possibility of immortality was not so far fetched, that we could, in theory, keep a body going forever.

"We do it with houses all the time," he explained.

And it was there that he lost me.  Because bodies are not like houses.

When I explain PKD to somebody for the first time, there are two inevitable questions that they ask:

"Are you on a donor list yet?"

No, I explain, you can't really be on a donor list until your kidneys fail. Insurance won't cover donor testing, etc, until the patient is at 15% or 20% function (I hear different numbers from different people, but it's around there).

And then there's the second question:

"Why wouldn't you just put in a new kidney now?  Get it taken care of?"

And then I explain that putting in a new kidney is one giant freakin' big deal.

Usually I leave it at that (and I say it politely and snark free), but if the person pursues the notion (and I never mind when people pursue the topic---I think about PKD almost all the time, so I'm happy to talk about something that I'm constantly thinking about), I go into the issues that accompany a transplant---risk of rejection,  how long a transplanted kidney can last, the regimen of immunosuppressant meds, the increased risk of cancer, and oh yeah, the surgery where they kind of like rip you open and then sew you up again.

I also learned today from my nephrologist that patients with PKD have an increased risk of kidney cancer on TOP of the general population's risk for skin cancer and lymphoma after organ transplant, because the cysts can continue to cause issues, because basically, cysts are jerks.  Obviously the cystic kidneys can be (and sometimes are) removed, but again, that is also a big deal.  I'm not sure why---I haven't needed to explore that part of the journey yet, but I heard a nephrologist at the PKD convention say, "It's just a huge deal to remove the kidneys.  Trust us on this."

I trust him.

So a person would do well to do whatever she can do to maintain her current level of function, and keep her own organs in her body for as long as those organs will do the job, no matter how badly they are doing it.  Because until they quit completely, bad kidneys that are still your own and are still functioning at a decent level are better than a new kidney that is not your own.

Replacing a kidney is not like replacing a door.  Or pipes.  Or a worn out washing machine.  It's a giant freakin' big deal.  Not the biggest deal in the world, and not the scariest, and not even the most unpleasant.  But still, it's something that is best put off as long as possible.

And last time I checked, they don't sell kidneys at Home Depot.