Oh, hey.

I’m not exactly updating this site any longer, but I’m keeping it up since my stats tell me people are dropping by pretty much daily in search of info on hyperaldosteronism. I do hope they are finding what I’ve written here to be helpful.

It’s been almost 4.5 years since my adrenalectomy. While I continue to have some post-surgical issues, what I don’t have are regrets. I still have pain (allegedly from scar tissue) on the side with the incisions. I still have low potassium if I don’t take potassium supplements. I’ve got a really screwed-up back and core thanks to the fact that physical therapy was entirely neglected after my surgery, which I’m remedying by working with a trainer (and wishing I had done so sooner). But!!! I’m not on any HTN meds, and I’m rarely incapacitated by the aforementioned problems, and those are both very positive changes from the state I was in pre-adrenalectomy.

While elsewhere on the Web there are some negative opinions (and frankly, some flat-out misinformation) about adrenalectomy, I’m keeping this site up in the hopes that my story will present a positive view to balance the negative that’s out there. Perhaps not a perfect view, but a positive one nonetheless.

 

 

What I don’t have, Part Two.

In addition to the previously-posted list, I’ve been thoroughly screened for just about all forms of thyroid disease and diabetes. All negative. I’ve seen the new endocrinologist and aside from that thorough screening, she’s having me test my blood glucose whenever I have symptoms. To recap, the symptoms are primarily: left trapezius muscle spasm, headache, blurred vision, palpitations, dizziness, and elevated blood pressure. So far, when any or all of these have occurred, my blood glucose has been steadily 75-78.

The good news, though, is that I’ve had fewer and fewer episodes of symptoms since beginning to follow the dietary treatment for hypokalemic periodic paralysis. At my last doctor visit of the year yesterday, my serum K+ was 5.0 and my blood pressure was 122/83 – this, from a girl with severe white-coat syndrome! My home average is a bit higher, 133/80, but that’s a running average that includes the days I’ve had, for lack of a better term, episodes.

In order to control the episodes, for now what I need to do is pretty simple:

• Avoid dietary sodium and carbohydrates
• Avoid alcohol
• Avoid decongestants and vitamins/supplements (and possibly other meds as yet undetermined)
• Avoid getting too cold (I bought three new coats!)
• Keep serum K+ in the 5.0-5.5 range (still taking supplements, but going to try to reduce the dose and see what happens)

All of that is really pretty easily done; with my allergies, the decongestant bit might be the biggest challenge and is still being figured out.

So after all that – what do I have? I’m still being told I may never be able to put a name to it. Being a librarian, nomenclature and classification is second-nature to me – I’m trying to let that go for now, but the reality is that there are some practical considerations to this. (What if I have to move, and have to start over with a new doctor? being the main one of course.) But as a wise person once said – I’m not going to jump off that bridge until I get to it.

What I don’t have.

More as a record for my own reference than anything else – here’s what I’ve been tested for and *don’t* have:

• Lupus (blood test)
• Lyme (blood test)
• Sjogren’s syndrome (blood test)
• Carpal tunnel syndrome (EMG and nerve conduction test)
• Ulnar radiculopathy (EMG and nerve conduction test)
• Cervical spinal issues (MRI)
• pheochromacytoma (blood test and 24 hour urine)

Tests I’ve had that have been inconclusive or gone wrong:

• ARR (blood test, inconclusive, slightly high aldo, slightly low renin, but my low salt diet could have that affect)
• 24 hour urine for hyperaldosteronism (one inconclusive, one done wrong)
• Trial of Eplerenone (which should have worked if I had PA again; it didn’t work – it actually *raised* my blood pressure, like most BP meds have historically done)
• Two specialists (nephrologist and endocrinologist) do not believe I have PA in my remaining adrenal

Good times coming up next week:

• Holter monitor
• EKG
• Yet another new endocrinologist (third time’s a charm?)

Current diagnosis:

• Hypokalemia (this is the on-paper Dx)

Current symptoms:

• Sudden shifts in potassium
• Palpitations/irregular heartbeat
• Erratic blood pressure
• Headaches
• Frozen left trapezius muscle, pain and weakness in left shoulder, arm and hand
• Dizziness upon standing too quickly
• and probably entirely unrelated, but so weird that I have to include it: hyperalphalipoproteinemia (HDL 161, trigs 69, and LDL 89, and no, those are not typos)

The most recent doctor’s opinion: “some sort of electrolyte problem, probably genetic, causing potassium shifts.” Something in the category of channelopathies, and he does not believe I’ll ever find someone who can tell me exactly what since the genetic testing for this is inaccessible to a mere mortal like myself. His recommended treatment is to keep on doing what I’m doing and adjust as necessary.

In other words, don’t let it get too low or you’ll die. But don’t let it get too high or you’ll die. Fair enough, I know the low signs well enough and probably will never have to worry about high, but – what happens if/when that no longer works?? I’m planning on living for quite a bit longer, ya know…

THREE (!) YEARS POST-ADRENALECTOMY (AND THEN SOME)

Oh hi… I’m a bit late, nothing unusual there. It’s been three years since my adrenalectomy – two fairly uneventful, and this one a bit of a bumpy ride. Here’s a bit of a recap.

Earlier this year, beginning with a well-intended course of vitamin D supplementation, I began to notice a return of some very PA-like symptoms: low potassium, high blood pressure, headaches, that horrible trapezius spasm, and so forth. After discontinuing the D, things got better, then worse again. BP kept jumping all over the place and K+ kept declining, so I began supplementing 40 mEQ K+ per day. BP leveled out but K was still low (and producing symptoms) so I started to undergo testing for hyperaldosteronism AGAIN. My labs got messed up and testing was inconclusive, so my doctor agreed to skip straight to prescribing eplerenone since that would be the end result of positive testing anyway.

EXCEPT. Eplerenone, like just about every other BP med I’ve ever taken, RAISED my BP. I stuck with it for 6 weeks or so but my K+ kept plummeting so I went back to the 40 mEq K+. BP was often normal (under 140/90 is the cutoff for us mono-adrenals; I was low 130s/80s) but every now and then would spike and K would drop again.

A suggestion on the Yahoo group led me to think about carbohydrate loading in my diet. As I have a long history of watching carbs, combined with intolerances to wheat, dairy, and sugar, my intake of carbs had increased this year – having spent the first three months sick and on antibiotics, there were times that I wasn’t eating much more than rice. And in an effort to eat more dietary K+, I was eating a lot more starchy veggies, potatoes, and legumes. I was, for the better part of the summer, mostly vegetarian – which means far more carbs than usual. DING DING DING DING DING!!! Fire alarms went off.

I turned to Doctor Google to look for anything connecting “carbohydrates” and “potassium.” BINGO: hypokalemic periodic paralysis (PDF). It was, as the saying goes, deja vu all over again: not since the time Doctor Google had diagnosed me with an adrenal tumor had I seen such a perfect, concise description of a condition that covered every last one of my symptoms, from weird K shifts to paradoxical reactions to meds.

That was a month ago. I immediately banished the rice and starchy stuffs from my diet and changed absolutely NOTHING else. My last serum K+, taken the week before, had been 3.8, which was pretty much where it struggled to stay all summer long. After a month off rice and starches, and no other changes, my serum K+ taken yesterday was 4.3.

This does not prove that I have HKPP, which has not been ruled out. If I do have it, it’s an atypical presentation – but then so was my PA in some ways, so that’s no surprise to me. To get a definite diagnosis, I would need genetic testing, which I have access to but not funds for ($6200, people, no insurance help whatsoever). And while I’d like a definite diagnosis, just to know what kind of dragon I’m trying to slay here, to make a long story short, the treatment is to do exactly what I’m doing now: watch carbs and salt, take K supplements, and take more when an episode occurs.

That being said, it seems kinda dumb to spend six grand only to be told “good job, just keep on keepin’ on.” I’ve got a few more tests lined up before the end of the year and maybe, just maybe, those can be used to build a case for insurance to cover the genetic testing, but I’m not getting my hopes up.

I am, though, hopeful that while I may not have all of the answers, I am at least on track toward a solution. I have a new doctor on board and of every doctor I’ve seen this year, something he said has helped more than all of the rest combined. He told me he now knows three mono-adrenals – me, an ex-girlfriend, and another patient – and not a single one of us was problem-free after adrenalectomy.

Framed that way, I’m able to deal with this thing a lot better than I was earlier this year. I think I had unknowingly fallen into the mindset of “tumor out? problem solved!” without actually considering that perhaps the tumor, and my low K+ symptoms, while interconnected, could be separate issues. I think my first diagnosis of low K+ was in my late teens, which would be consistent with HKPP. Of course, the advice was “eat a banana” and it was never looked into until the tumor’s effects landed me in the hospital in my early 40s. Instead of feeling sorry for myself and wallowing in “OH NOES! MY ADRENALECTOMY FAILED!” self-flagellation, I’m slowly coming around to just accepting the fact that for the rest of my life, the rules will be different for me than for most people. There are still many unknowns, and the unknowns are pretty hard to live with, but ya know – living with the unknowns beats the hell out of not living at all.

So hey, I’ll take it.

Just the facts.

Hold your schadenfreude, people!

It has come to my attention that people are being referred here from a message board as an example of “adrenalectomy that failed.”

Can I just say that it’s a bit premature? Because we don’t know that yet. We *do* know that adrenalectomy gave me almost 3 years of “normal” life, so no matter what is wrong with me right now, I don’t call that “failure” necessarily.

Here are the facts:

• I have low potassium. When my potassium drops, my blood pressure rises.
• I am taking potassium supplements. As long as I keep my potassium in “safe” range, I do not have high blood pressure. Last check this evening: 123/76.
• There are a lot of variables at play here. It was not as if I had no other health issues along with a bad adrenal: I’ve had celiac disease all of my life, half of it undiagnosed; as a result, I’ve had long term malabsorption issues (that could affect how my body processes potassium, I am learning). I have unexplained hyperalphalipoproteinemia. I have a 40 year history of polymenorrhea with no explanation. I have cone-rod dystrophy in my left eye. I have really severe fructose intolerance. I’m allergic to dairy and just about every antibiotic there is except for three. Need I go on?

In other words, I’m not a simple case. I’ve got a lot of weird stuff going on and at this point it’s not known if my other adrenal has gone bad. At first it looked like it, but keep in mind that a month of eplerenone DID NOT raise my potassium and DID NOT lower my blood pressure. Neither, for that matter, did spironolactone the summer before my adrenalectomy.

So. Back to the facts:

• I had an adrenal tumor.
• I had it removed.
• My blood pressure dropped and stayed low-normal for nearly 3 years.
• Earlier this year, I got really sick with respiratory and sinus infections that kept reappearing. I took a lot of antibiotics, which messed up my stomach even more than it’s already messed up.
• To counteract this, I started taking a probiotic. I also started taking Vitamin D as labs of the same time showed I was low.
• At some point after all of this – being really ill, having a really messed up stomach (use your imagination!), ingesting unregulated supplements from the health-food store – my potassium dropped and my blood pressure started creeping up.
• I went through some testing; the lab botched up the testing. Rather than do it again, I opted to proceed with medical therapy for hyperaldosteronism, as it appeared to be what I was dealing with.
• That medical therapy, as I noted earlier, DID NOT WORK. In fact, it caused the OPPOSITE effect of what it was expected to do in a case of hyperaldosteronism.

All of that to say, quite simply – nothing is as black or white as it seems. Hyperaldosteronism is a medical condition that is poorly researched; there is no glamour in it, no huge profits to be made by Big Pharma, no great prizes to be handed out for discovering its cure. I’ve met with a half-dozen doctors in as many months and NOT A SINGLE ONE OF THEM knows conclusively what I have; in fact, several of them vehemently insist that my remaining adrenal could not possibly be the problem. And the truth is, although I was skeptical at first, I’m starting to believe them.

So if that’s failure to you, fine – you’re entitled to your opinion. But until someone comes along with conclusive evidence that my remaining adrenal is the culprit, I’m remaining open to any and all possibilities.

The mystery continues.

2 days off of eplerenone and 4 20 mEq doses of potassium later, I can say that I feel (almost) back to normal. Blood pressure is running consistently 120s-130s/70s-80s, just like a normal person. Muscle spasm and headache almost entirely gone.

And while overall that’s good news, the following questions remain:

1. Why did eplerenone, an anti-hypertensive, cause my BP to go up?
2. Why do I keep dumping potassium?
3. Why do I have mildly elevated aldosterone and mildly low renin, if I’m not responding positively to the category of drug typically prescribed to treat this?

And yeah, there are probably more questions. And there are probably no answers in sight for the foreseeable future. And I’m exhausted just thinking about it.

Failed.

After a potassium crash, my doctor increased the eplerenone to 25mg twice a day. Guess what? My blood pressure went HIGHER.

So now we’re doing an experiment: no eplerenone, potassium 20 MEQ twice a day, see what happens.

This just doesn’t seem to get any easier. It’s tiresome. Really, really tiresome.

Drugged.

So it’s come to this: 25mg Eplerenone once a day, and for the time being, no potassium pills. I started on 12.5mg and could tell that that wasn’t enough – this time around, I’m getting a bilateral trapezius muscle spasm, cramps in my feet and legs, and some digestive symptoms when the potassium drops too low. “Too low” for me means below 4.0 – it does not mean “below the lab reference range” which for most labs I’ve encountered is 3.6-5.2. My sweet spot seems to be 4.5-4.8, and keeping it there is a bit of a challenge. When I sensed that it was dropping, my doctor had me up the Eplerenone dose to 25mg – still considered sub-therapeutic for most, but then based on size alone, “most” are not me.

I’ve been on 25mg for 6 days now. The low potassium symptoms have mostly gone away. The first 4 days I had a lot of stomach upset, the 4th day I felt like I was having an out-of-body experience all day – I was sooooooo spacey! Yesterday, the 5th day, I woke up feeling clear-headed and without any muscle cramps, but still had some stomach trouble. Today – so far I’m feeling pretty much like myself all around. Here’s hoping it lasts.

As for hard numbers – we’re not sure at this point why I have this pattern, but my BP is staying in the mid-130s/high-80s range during the day, then daily, as predictable as clockwork, drops to 120s/70s-low-80s after the sun goes down. I’m not checking potassium again until the 20th, unless my symptoms signify a drop – I’m really trying to see if I can rely on “listening to my body” for this rather than weekly or twice-weekly blood draws. I think I’ve lived with this beast long enough that I can sense when it’s approaching, and surprisingly, my doctor is on board with this experiment.

And as for my doctor – for now I’m letting my primary care doctor manage this. As mentioned before, I have little confidence in the endocrinologist’s familiarity with this disease. Not to mention I hate going to his office (grouchy staff, crowded waiting room, expensive parking, a downer all around). And there’s the fact that I just can’t afford him. He bills my insurance company $650 for ten minutes of his time, of which I’m on the line for $150 each visit. I’m not a rich person and this isn’t a designer disease; I need to find a not-rich-person’s way to manage it.

Comedy of Errors.

So this morning? I showed up at the endocrinologist’s office to find out my results from the sodium loading.

And… wait for it…

There aren’t any.

Somebody at UCLA’s Santa Monica Clinical Lab effed up, big time, with my 24 hour urine sample. They checked the metanephrines, of course (which were negative, of course), but…

THEY NEGLECTED TO CHECK THE ALDOSTERONE.

Yes, you read that right.

***bangs head against wall***

The endo wanted me to repeat the test. I told him, HELL NO – It’s been over a week since I completed it and I STILL have a horrendous headache and elevated BP and probably the worst trapezius muscle spasm I’ve had since… ever, really.

I am done. DONE with the testing and the clueless doctors and with being medically interesting. Enough already. As I’ve said for years – my body, my science experiment – and even if it kills me, I am determined to be in charge from here on out.

There are two options, really. It’s simple. First get my potassium back under control (feeding me giant doses of salt pills will do the exact opposite) and hope that takes care of the BP once again. If it does – problem solved. I have hypokalemia – easy peasy, there’s my diagnosis. If that fails, I start taking the dreaded eplerenone. If that works – easy peasy, I have a hypertension diagnosis on my record and nothing more.

And if neither of those solve the problem, then maybe I’ll be willing to revisit this testing stuff. But at this point, I’m ready to just let the damned disease have its way with me, even if it kills me.

Because really, I think the medical establishment’s incompetence is more likely to kill me than anything.

A week in the life.

Now let’s see… where were we? It’s been a hell of a week, but it wasn’t all bad – there was a 10+ mile hike on a very hot 4th of July in there that made me feel like I couldn’t possibly be all that sick. But that was the high point. I’ll take it!

My nephrologist essentially “fired” me, which you may recall he tried to do once before. While I respect him for being upfront and telling me that he thinks my case is beyond his expertise, the alternative he proposes – travel to Mayo Clinic for treatment by experts – has no basis in reality. At least not my reality, which is a reality of not having a lot of spending cash for things like plane tickets and cat sitters and airport parking and, oh, minor detail – doctors outside of my insurance network. So that’s not going to happen, although I am considering the NIH option.

This leaves me at the mercy of the rather arrogant endocrinologist. My primary care doc has had words with him, and has told me to try to not be so “intimidating” the next time I meet with the endo. Ummm… I’m all of five feet two inches tall and weigh less than the average American 12 year old*; how intimidating can I possibly be?! Oh, right. In other words, don’t be so intelligent. Because smart women are SCARY!

So this means I’m going through with the sodium loading test the endo ordered next week. Been there, done that; it’s no picnic but it does give me an excuse for a couple days off work so hey, I’ll look on the bright side. And the results might be interesting, or at the very least, useful. I just wish the process wasn’t so heinous – I have to ingest 6000mg of sodium tablets for three days, the third day of which I have to pee into a bottle full of acid all day. Good times. Or, you wish you were me.

*True story, that. If you want to read about the “average” American child’s weight, here’s a fascinating – and depressing – PDF.