Stephen Cobb's got Conn's Syndrome? Probably, but I go through Adrenal Vein Sampling(AVS) to be sure

[Updated 6/11/13: It's official, I do have Conn syndrome. The nephrologist just called to say my AVS test results were, direct quote: "Stunning!"]

This post is about my potential diagnosis of Conn syndrome and how that could lead to a healthier, more energetic life. If you have high blood pressure, dodgy heartbeat, nasty leg cramps, muscle weakness and chronic fatigue, pay attention: Conn's might be what's ailing you, and it can be cured. (This is of particular interest to anyone who has been told they have "essential hypertension", which means the doctors have essentially no idea why your BP is high.)

That fact that Stephen Cobb may have Conn syndrome is not a typo, Conn's is a medical condition first described by University of Michigan endocrinologist Jerome W. Conn in 1955. I did not have Conn's back then, but I'm pretty sure I have it now, and have had it since at least 2004. A recent CT scan revealed a growth on my left adrenal gland (we start life with two, one on each kidney). This type of growth, usually benign, is called an adenoma. Tests indicate that mine is producing the hormone aldosterone, too much of which not a good thing, as I will explain in moment.

Basically, Conn syndrome is an aldosterone-producing adenoma, and my hat is off to Dr. Conn for discovering this condition without the aid of things like CT scans. I got my doctor to order a CT scan looking for one of these adenomas because I had this quartet of symptoms:
  • High blood pressure, poorly controlled despite multiple BP meds
  • Low potassium, despite years of taking big potassium horse pills every day
  • Atrial fibrillation (as a result of the low potassium or hypokalemia)
  • Fatigue (more than just feeling tired)
If you plug these into Google you will likely see "aldosteronism" in the top results (I did this with "high blood pressure low potassium hormone" and "high blood pressure low potassium fatigue" but without the quotation marks).

Dig into these search results and you learn that aldosteronism, also called hyperaldosteronism, exists when too much aldosterone is produced by the adrenal glands, which can lead to lowered levels of potassium in the blood, also known as hypokalemia. An adrenal adenoma is one of a number of things that can cause primary aldosteronism. I have one on my left adrenal.

So what's the good news? If you confirm that just one of your two adrenal glands is responsible for the aldosteronism it can be surgically removed, giving your body a chance to return to normal, which appears to happen in over 50 percent of cases.
Cure of hypertension occurs in 50%–80% of patients after adrenalectomy for an aldosterone-producing adenoma, and most of the remaining cases show improvement (13,21,22). (Radiographics)
That can mean no more blood pressure pills, no more potassium pills, an end to excruciating leg cramps, a return to a regular heartbeat, more energy, even loss of excess weight, partly through being more active but also, possibly, through reduction of cortisol production (but that topic is too complex for this humble blog post).

The role of AVS

After confirming that my body was producing too much aldosterone through blood and urine tests, my primary care doctor referred me to a nephrologist who ordered Adrenal Vein Sampling (AVS) to confirm that the adenoma was the cause of this and not a general adrenal malfunction involving both glands. (You don't want an adrenal glad removed if that is not going to stop the excess aldosterone production.)

The AVS procedure is tricky, requiring interventional radiology (not invasive radiology, a term I used in error when talking to several people about this). A tube is inserted into a major vein (femoral vein in my case) and threaded to the adrenals where a series of blood samples are taken. The doctor doing this is guided by a live X-ray view of your veins using a contrast. Not only are the veins thin, but the support staff have to get things just right with the labeling of samples and so on. In other words, you want an experienced team doing this if at all possible, otherwise you don't get a result and have to go through it again.

Speaking of "going through it", you will need to set aside a day off work for AVS, followed by a day of taking it easy. That's because a. you will be given a strong anesthetic and b. you don't want the vein to pop open due to sudden movements. I went in early for mine (5:30AM) and expected to be back at work in the afternoon. Nope. Could not drive and was clearly still feeling the effects of the wonderful "twilight zone" drugs they use to sedate you (you don't go all the way under, just into a very nice place, and not at all like Twilight Zone the TV series).

I read one blogger's account of AVS before I went for mine and it was very helpful, although not exactly reassuring. Partly this was due to my (now cured) ignorance of the word "catheter" which I used to think meant only those tubes they stick into the most sensitive organs, but no, it just means any tube, in this case the one that went into my femoral vein in this procedure. To be honest, I never felt a thing.

I also read about getting shaved, but that was only a small patch of groin north of the family jewels. The prep work was mainly the shave, some blood draws, an IV for the contrast, and paperwork. The actual procedure only took about an hour and then I was required to rest horizontally for two hours to let the vein heal up. The good news is that you can eat right away (blessings on the nurse who brought me a turkey sandwich). You will likely feel hungry because you have to fast, starting at midnight the night before (no food or liquids, although you may be able to take your BP meds--ask your doctor--I didn't take mine and so BP read very high on admission, but not too high to prevent it going ahead).

How common is primary aldosteronism? A lot more common than most doctors realize. I think anyone with essential hypertension should get the basic urine and blood tests for this condition to rule it out. Although my cardiologist confessed to knowing very little about it, and both my primary care physician and the nephrologist said they have not seen it very often, consider this statement in 2009, from the The Journal of Clinical Endocrinology & Metabolism:
There is an increasing requirement for adrenal vein sampling, which is driven by the appreciation that primary aldosteronism is far more common than previously recognized (13). Many centers throughout the world are reporting a prevalence of between 5% and 10% in unselected hypertensive patients (48). 
So, check it out...and stay tuned. If I am a candidate for adrenalectomy I will describe the operation and my results for the benefit of anyone else who is going down this road.


Sorry I've been out of touch (my adrenal adenoma is to blame)

Just a quick post to let folks know that I came through my recent medical procedure unscathed (apart from a small hole below the belt line that is healing nicely). For the sake of other folks out there who may need to undergo adrenal venous sampling I am writing up the procedure, the reason for it, and the possible long term prognosis, which is very good.

I also wanted to extend a broad apology to those friends and family with whom my communications in recent years have been less than stellar. I have been beating myself up for some time over this, but recent medical adventures have led me to see things differently:
  • I have been feeling seriously fatigued for several years now, leaving me with little energy at the end of the workday/week to reach out and communicate.
  • I assumed that this lack of energy was due to the demands of a stressful life (dealing with Chey's disabilities, the aftermath of the 2008 financial disaster, the demands of a new job--on which I've been working many evenings and weekends, the stresses of moving 3,000 miles to a new city, and so on).
  • I also thought to myself: "this must be what getting old is like"
  • In fact, it appears that I have been suffering the effects of something called primary aldosteronism due to an adrenal adenoma, of which there will be more blogging later.
The good news is that primary aldosteronism--talk about a disease that needs a catchier name--can be treated and, in some cases, cured. That could mean I get back to 100%! Staying in touch will no longer be such a challenge.

I got the diagnosis in April, however it has taken me a fair amount of time to put the implications into perspective. At first I was hung up on the fact that this should have been diagnosed years ago. Right now I am focusing on the positive, the fact that it was finally diagnosed, and I have good access to some good doctors who can treat it.

Yesterday I took a big step toward treatment by undergoing AVS, which will determine if one of my two adrenal glands is responsible for pumping out excessive amounts of the hormone called aldosterone. Thanks to a CT scan I already know that there is a lump, called an adenoma, on one of those glands. That lump could be the culprit. Oddly enough, that condition, an aldosterone  producing lump on an adrenal gland, is called Conn syndrome.

I plan to write up more of what I have learned about primary aldosteronism. Readers and Googlers may benefit from this because, from what I can tell, this is an under-diagnosed condition. For now, please accept my apologies for sub-par performance in the friend and correspondent department. I hope to be able to do better.

I leave you with a list of primary aldosteronism symptoms that I have been experiencing, some for many years:
  • High blood pressure not well controlled despite multiple medications
  • Chronic low potassium or hypokalemia
  • Abnormal heart rhythm and atrial fibrillation (my heart sounds like my dog's)
  • Sodium retention, increasing blood pressure, causing swollen ankles, legs
  • Muscle cramps and muscle weakness
  • Decreased cardiac output associated with elevated renin levels
If I am lucky, my AVS results will indicate than an adrenalectomy can eliminate these symptoms. Stay tuned!