KUB (stands for kidney, ureters, bladder) x-ray = nothing. (yes, x-ray not CT Scan. Cystine stones don't show on x-rays -_-) Blood test = nothing (yay!) Ultrasound = one tiny stone on the left. My pain is on the right, so nothing.

That's great, but I'm still in effing pain in one spot.

At least my kidneys are fine.

Wow...how long has it been? I didn't actually look at the blog before pulling up this form, but it's been a looooong. Time. More importantly, I don't think I've felt this kind of pain since I was living in Japan. I'm in school now, and this is my second week of the spring semester. Hopefully this will go away on its own, because I can't miss school. This sucks.

All week long I thought I had a nasty UTI. Go figure. (Hey, it's been a while.) But check out the size of this baby! It was 8mm x 4mm. Any wider...*shudder*

I had my CT scan done last Tuesday, and before I left Stanford, I grabbed a copy of it on CD. The scan was underwhelming to say the least. I saw a couple new stones in my right kidney, (shoot - was it my left? I always forget if the sides are reversed on film and I don't have it open right now). Either way, my right kidney is killing me today. It's gotten worse since yesterday.

Okay, I jumped ahead. There were 4 or 5 stones in the right kidney and one teeny one in the other. It looks like two on the right are 6mm. Despite all of the ureter pain I've been feeling, I didn't see anything in my ureter, meaning, I might have been wrong for the first time since I was diagnosed. So what is this, phantom pain?? I'm still waiting on my urologist to call me back to be sure. I've always sucked at reading ureters on film, so idk. I saw a couple of bright spots down by my bladder, but I don't think it's where the ureters would have hit the bladder. They are so faint. :(

So back to my kidney. Docs will tell you can only feel kidney pain if they are swollen. You're not supposed to feel stones up in the kidney. So where is this pain coming from? The ureter area still hurts a little, but this is fo' real kidney pain. Oh - one of the new stones is flat in shape! It might be one of the kinds that get stuck at the corner output area of a lobe. I forget what that's all called, but those suck, because docs have a hard time reaching them without eswl or cutting into your kidney. meh. I'll have to look it up. It was pretty cool looking; I've never seen a flat stone before.

So that's it for now. I might google the type of stone shape I'm talking about - I've seen it before. I'll update after I hear back from the doc, which will be any minute.

They don't call you at 9pm to see how you're feeling. This is why I love my Stanford doctors.

It was either that, or Monday night at 8:30pm. I don't know, I'm in tons of pain, so I might change it to Monday, but pain alters my driving, and I didn't want to go all the way down to Stanford that late at night. On the other hand, the results should be in first thing in the morning.

It will be interesting to see how big the stones have gotten since February. Hopefully my pipes won't get blocked before then. At least my classes are almost over for the semester. I'll have exactly one month to get in and out of the OR and get that damn stent out.

One good thing about being in a tremendous amount of pain is that I have nothing better to do than study. Today was a productive day. I spent most of it studying up on Japanese so I won't be behind when the semester starts (I'm skipping Elem. Japanese 1 since I lived in Japan, but children's stories are a bitch to understand), and I finished my astronomy homework.

TriWest is great, but they don't have a sense of urgency when it comes to authorizing CT Scans. It could take until Monday to get set up for a same day or next day appointment. Maybe Tuesday. Meanwhile, my urine is getting bloodier and bloodier, and my right and left sides seem to have created a sport out of which side is trying to hurt me more. Wah, right? I know...sorry.

I'm thinking about looking into supplemental insurance - something light that covers all of Tricare's gaps. Something tells me having two insurers will create more of a headache in the end. Like each company fighting over who won't pay for what. Anyone know of a good company to go with? I need a company where the billing lady just has to look at the company's name to know CT scans and other tests don't need an authorization letter. Like, blanket coverage.

P.S. OUCH!!!!

On the way to school this evening, I was listening to one of my Rufus Wainwright CDs. A song came on that I hadn't heard in forever, and it gave me a flashback to the day my kidneys crapped out after a procedure. On the way to the ER that evening, I was listening to "Beautiful Child" in the car. While symbolism in songs isn't my forte, to me that song is about dying without fear. Even though it was one of my favorite songs eva, I didn't catch on until I was faced with my own mortality that night, and it gave me chills. I was approaching the Naval Hospital gate with a high fever, sick, bloated, and in tons of pain because my kidneys had completely stopped working much earlier in the day and I was like, "Oh shit. What if this is the last song I ever hear?" That moment was filled with irony, and I thought to myself, "I want this song played at my funeral."

Before I go on, I know even in the worst case scenario it was nothing a little dialysis and/or a transplant couldn't fix, but it was still too...weird, and it felt real.

Anyway, despite tons of previous trips to the OR, I had never been afraid of something going wrong, most likely due to my unconditional love of medicine. When faced with the possibility of death (because I really did feel that sick) I wasn't afraid. I felt a huge imagery-rush of sadness about possibly leaving my kids too soon, but other than that, I was ready. I was heading to a safe place with a doctors and nurses who would take care of me, and I wouldn't have to worry about being strong and fighting the sickness anymore. My husband was home safe with the kids (it was the middle of the night). All was good. I haven't been burdened with the fear of the unknown since around middle school, so perhaps what I experienced is typical of other steadfast atheists. I was the mythical atheist in the foxhole in that moment, but really, it just wasn't an issue at all. I had a total, "It is what it is" moment while stumbling into the ER, I surrendered to the situation and felt at peace (although there's nothing particularly peaceful about a foley cath in a dry bladder #$^&!T@).

Okay I'm starting to ramble, and I hope all that didn't sound too melodramatic, but how many people get to experience that feeling? Obviously I'm still here, and all went well that evening, but his song left a huge mark on me that's still surreal today.

I'm an obsessed certifiable OCD germaphobe away from home. I've been this way for a couple years now, but thanks to my constant grossedouttedness with the public's lack of good hygiene, I haven't had a cold in two years. Between Lysol wiping my desks at school and washing my hands several times a day, I've kept both cold germs and angry kidneys at bay.

No doctor has ever suggested to me that colds wear out stressed kidneys, but in the past (ever since I was first diagnosed) colds and fevers would bring about the beginnings of acute kidney failure - maybe not the cold itself, but the way I handle colds with the perseverance of a toddler. When feverish and bed-ridden, I let myself get dehydrated - probably as much as anyone else with a bad cold. Technically, anyone who lets his or herself get seriously dehydrated will eventually go into kidney failure, but does that really happen to people with healthy kidneys and the flu? Not that I know of; so maybe our stressed kidneys just can't handle colds and dehydration as gracefully as normal folks.

So, that's my observation for the day. If you have kidney disease or are prone to acute kidney failure, give Lysol wipes a try and wash your damn hands (lol). All I know is that it works for me.

It's about waist-level now. Oy vey.

No real pain to complain of today! I took a Flomax last night, so maybe that had something to do with it. I really hope I can pass this beast on my own. I also hope not going to the doc doesn't bite me in the kidney. I can't be bothered with fitting a drive to Stanford into my schedule, but boy would I be pissed if I get a kidney blockage because of neglecting this thing. Meh.

I have a stone on the left still, and it's reeeally high up, which is why I said kidney pain, even though I'm sure it's the ureter or renal pelvis. Thankfully the pain is intermittent, but the real pain is that I have about 3 weeks left until finals, and I really can't miss any of this. This is the worst stone pain I've had since I was in South Carolina. Last time I went in for surgery, it had nothing to do with stone pain. I had that first visit with my Stanford doc, and he got all giddy when he saw that honkin' stone up there. I didn't even know I had it, but I think he was worried about a blockage. That was over a year ago. I think. Cursed stent. :/

So I have some old-ass Flomax and lots of crappy pain meds. I would kill for some pot right now. Seriously, thanks to the government, I can't take an instant pain reliever that will be out of my system in like 4 hours (effect-wise), but I can take Vicodin or Percocet, which will leave me a puking mess for like 8 hours. Really? People are so ignorant.

Anyway, this will be my first trip to the ER or OR since Dave left the Marines. Retirees and their families only pay $30 for an ER visit, and $25 for ambulatory surgery. How cool is that?

Well the zofran should have kicked in by now. The pain made me puke but I think I'm ready for some wonderful pain meds. -_-

Congratulations to Dr. Goldfarb, et al, for their combined research leading to the discovery of two different cystine growth inhibitors. These new growth inhibitors not only keep cystine molecules from binding, it is said that new treatments containing these inhibitors will be free from side-effects. This is wonderful news for cystinuria patients!

Photo credits: Zhihua An and Timothy G. Bromage/New York University

Kidney failure in cystinurics, doesn't only occur when kidney stones stop proper kidney function, or if a post-op blood clot or other complication is present. It is the nature of cystinuria, that one day our kidneys will fail, and possibly even several times over many years before they finally konk out for good. I've experienced this sort of acute kidney failure three times - once due to a 7mm stone, once due to a post-op blood clot, and once just for the fun of it. I'd like to focus on the one that came out of nowhere.

First, please remember I'm no kidney doc. What I know comes from personal experience and years of reading up and consultations. Please go to your doctor if you think you're experiencing symptoms related to acute or even chronic kidney failure.

In the cystinuria community, it's well known that we all have a disposition to kidney failure. Most doctors outside of the loop don't know this. Hell, most doctors have only heard about cystinuria in passing or in a specialist school (even my nephrologist had never met an actual cystinuric, but it was on his final. :/). When you need to be seen by someone other than a kidney specialist, you might be given some grief, but you need to take control of the conversation, and say exactly why you feel you're going into possible kidney failure. This is easier said than done, because when you see your doctor, you're usually not feeling well and "all there".

First, here are the symptoms of acute kidney failure taken from the Mayo Clinic:

• Decreased urine output, although occasionally urine output remains normal
• Fluid retention, causing swelling in your legs, ankles or feet
• Drowsiness
• Shortness of breath
• Fatigue
• Confusion
• Nausea
• Seizures or coma in severe cases
• Chest pain or pressure

 Now, here are the symptoms of regular ol' chronic kidney failure from the National Institutes of Health:

• General ill feeling and fatigue
• Generalized itching and dry skin
• Headaches
• Weight loss without trying to lose weight
• Appetite loss
• Nausea

Symptoms when kidney function has worsened:

• Abnormally dark or light skin
• Bone pain
• Brain and nervous system symptoms
  • Drowsiness and confusion
  • Problems concentrating or thinking
  • Numbness in the hands, feet, or other areas
  • Muscle twitching or cramps
• Breath odor (*that can extend to entire body odor - that kidney rot, as I call it)
• bleeding, or blood in the stool
• Excessive thirst
• Frequent hiccups
• Low level of sexual interest and impotence
• Menstrual periods stop 
• Sleep problems, such as insomnia, restless leg syndrome, and obstructive sleep apnea
• Swelling of the feet and hands 
• Vomiting, typically in the morning (huh - had no idea, but when it does down, I promise vomiting can occur anytime.)

Okay, now that the basic symptoms of kidney failure are out of the way, here's how kidney failure relates to those of us with cystinuria. It is said that kidney failure is rare in cystinurics, but is also said that because of chronic kidney stones and the stress they put on the nephrons themselves, kidney failure does happen, and that we are definitely predisposed to it. One website simply stated, "A person cystinuria may develop kidney failure, which can shorten the person's lifespan."Another website mentioned, "Depending on the level, number of stones, physical stress, and trauma of one's Cystinuria, there is a small possibility that progressive kidney failure will result." 

This month, Science Magazine stated, "...Much less common are stones that develop from crystals of the amino acid L-cystine. The latter only occurs in patients with cystinuria, a condition with great morbidity due to the early age of onset, high frequency of stone recurrence, and increased risk of chronic kidney damage (1). The rarity of the disease—it occurs in about 1 in 15,000 in the United States—has deprived the field of clinical trials to assess treatment options. Treatment has not changed in over 20 years and remains unsatisfactory for many patients."


Here is a JAMA article about one patient who's cystinuria led him to End Stage Renal Failure at the ripe old age of 28. They measured his cystine and amino acid levels post-transplant, and the transplant led to a cure.

(Click image to enlarge.)




So the important take away from this, is that we can and do experience renal failure at one time or another, but seemingly not all cystinurics do experience random acts of acute kidney failure. We know our bodies better than anyone, and I doubt that any of us (post-diagnosis) were proven wrong when it came to the awareness of stones or kidney pain. The same must go for acute and chronic kidney failure. Don't believe the websites when they say it doesn't happen, or it's extremely rare. Shoot, we are rare, and up until recently, few statistics specifically about cystinura and kidney failure have been published.

I will be sure to update this post when I discover more information on the topic.

I just f-ed up my entire doctor's appt. I had never met the doctor I saw, and I had to name my condition and the type of stones I had 3 or 4 times before he literally said, "OOooOOooh....I get it. I understand." Hmmm, are you sure?

So, before long I was completely flustered, and even though I was able to rattle off everything I was experiencing, he basically wrote up a kidney stone work-up. CT scan with contrast. I was so wigged out, I was like, "Uh, I've had like two this year...but okay." Can you believe I was that complacent? He did order a urine dip stick thing to make sure I didn't have a UTI. Duh, that was clear. I totally forgot to ask for a BUN draw!! GRRRRR. My social anxiety wasn't helping clear my mind either.

Then I had a run-in with some crazy Marine-hating motorcycle dude in San Carlos on the way to the pharmacy. Had to call the police and everything. (Note to self. Always have pepper spray at the ready.) So they didn't catch him, I gave my police report, finally came home after stopping by CVS for some much-needed Zofran, and my regular doctor calls. Uh oh...all of a sudden I knew what this was going to be about.

She was basically irritated because that doctor ordered my 3rd or 4th CT scan of the year. She asked what I told him, I replayed the scene, and then she was like, "Well all you needed was a creatinine (sp) check, right?" My brain was foggy and I couldn't find the words to tell her about the BUN too, but I was totally whatever at this point. Totally felt like an asshole-hypochondriac.

So then she placed the order and out of concern, asked if I would drive down to Stanford since all the clinics were closed. I tried to go, but I just had to write her to say my hubby will have to drive me down first thing in the morning. For now I'll be keeping an eye on my urine output. I'm too freaked out to drive, and my husband is exhausted. Tomorrow morning, it is.

Oh - when I checked in, I had a low, 100.1 fever too. Temperature is something I totally forgot to check. Duh. I guess I can hold off until it gets up to 103 like it did last time they shut down.

So that's my crappy story. I wish I could rewind today and totally start over. I love my GP to bits, and this has more to do with my social anxiety and meeting new doctors, but I really wish I didn't have to deal with doctors as often as I do. Teach me how to give myself a bolus IV at home (I've read lots of people with cystinuria do this), magically make Zofran an OTC drug, magically make BUN/creatinine testing as easy as a home diabetic blood sugar test, give me pre-drawn shots of Toradol, and I can take care of myself until I need to go to the ER.

So here I am, back to gulping liquids and my hands are starting to feel stiff again - my precursor to swelling. Dr. G. said to go to the ER if I start swelling up again. Hopefully it will stop here. Need to check my ankles. Seriously though I'd rather croak in the comfort of my own bed than go back on the road tonight. Ugh.