Sunday, April 28, 2013

Adrenal Insufficiency United

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Since my bilateral adrenalectomy, I've realized very few medical personnel understand adrenal insufficiency and adrenal crisis.  There are many reasons for the above, including a BLA.  Some folks are born with it. Others develop it due to an auto-immune malfunction or trauma.

An organization new to me, but probably not so new, Adrenal Insufficiency United, is making a difference in the education of the aforementioned medical folks.  They have put out a great educational publication and video for EMT's, doctors, and anyone else who needs to understand the problems with treatment of those with malfunctioning or absent adrenal glands.

Their publication about emergency care touches on just about everything plus relates real-life testimonies. They also have a video which gives great instructions on how to give an Act-o-vial solu-cortef injection (below).  Kudos to these folks!  Thank you!

Tuesday, April 2, 2013

Cushing's Awareness Month: Brain Tumors Can Make You Fat

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A while back (years? months?) a group of Cushing's survivors had a discussion about the various steroid replacements available after surgery to treat the disease.  Replacement is usually temporary after pituitary surgery unless the whole pituitary is taken or affected.  After BLA is another story.  A Cushie friend of mine took the data I put together and did a great job analyzing it.  Her story:

This blog entry is going to be a little technical in nature, but by the end I hope to explain why it's important to use bio-identical hormones when dealing with diseases like Cushing's that sometimes require replacement of vital-to-life medications.  Stick around with me on this one, I promise it will be worth it.

This chart demonstrates what a normal diurnal rhythm looks like.  Through most of the night from about 10pm to 6am,  your cortisol levels are negligible.  Around 6am your body starts to wake up, providing.... (Click here to read the rest of her research.)

Monday, April 1, 2013

Cushing's Awareness Month: Living With Stripes

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April 8 is Cushing's Syndrome (Disease) Awareness Month.  April 8 is Awareness Day.  Many "Cushie" bloggers will be sharing this month as often as they can.    What I would like to do is highlight another blog each day this week so you can see what others out there do.

One of my friends, whom I first met several years ago through our common disease and whom I have now met in "real life",  has a fairly new blog called "Living With Stripes".   She shares some great info.    One of her posts starts out like this:

“Our study shows that BLA (bilateral adrenalectomry) for persistent Cushing's disease provides patients with considerable improvement in their Cushing-related symptoms with concordant increase in their quality of life. After BLA, patients may attain the same (or better) quality of life as patients initially cured by transsphenoidal pituitary tumor resection. We think that BLA is a safe and effective treatment of the 10% to 30% of patients who fail initial therapy for Cushing's disease, and should be considered preferentially over other available therapies”

I like that statement!   

Today I am 25 days post-op my BLA surgery and my mantra is ‘slow and steady’.  I thought I would outline this step of my journey for those who may be considering a BLA as treatment for their persistent Cushings.....
To read more, click here...

Friday, May 18, 2012

Korlym: New drug to treat Cushing's Disease

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I know several folks who have recently started taking the new FDA-approved drug, Korlym, to treat Cushing's Disease.  Korlym is a new name for the "old" drug mifepristone and was developed by Corcept Therapeutics Incorporated. 
Korlym blocks the activity of cortisol and is proven to reduce high blood sugar (hyperglycemia), a key symptom of Cushing's. Korlym has a unique way of working. Instead of reducing cortisol levels, it blocks the action of cortisol, thus preventing the effects of excess cortisol.1
Korlym has many side effects and cannot be taken by everyone.  Once the patient stops taking Korlym, she will continue to have Cushing's.  The biologic half-life of Korlym is approximately 85 hours.  If a patient suffers adrenal insufficiency or crisis, massive amounts of hydrocortisone or dexamethasone are needed to alleviate these and will have to be continued for the duration of the drug in his system.

To follow a patient who has just started taking Korlym, you will find her blog here:  Cushing's Disease

Sunday, April 29, 2012

Day 29 of the Cushing's Awareness Challenge: Life goes on

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Life goes on...

Life doesn't stop because one gets a rare illness or is diagnosed with a disease.  However, mine seems to be delineated by before Cushings, after Cushing's,  before BLA and after BLA.  Before Cushing's is a gray area.  I'm not sure exactly when I started getting symptoms.  Some of my symptoms went as far back as childhood but others were more recent when I realized what was wrong with me.  I was 47-48 at that time.  I'm sure I had symptoms of Cushing's (verified by my photo evidence) from the age of 24.

Skipping ahead past those years between ages 47 and 52 when I was going through testing, diagnosis, pituitary surgery to remove the tumor, recurrence, and re-testing/diagnosis though my BLA, I am in the after BLA era.  Does anyone else see her life this way?  I know most folks look at graduation, job, marriage, children, etc. as the defining moments of their lives.  And my children, plus my grand-child, are definitely more important to me, but I still categorize them in the pre-BLA/post-BLA eras.

Isn't it crazy that one event can be so momentous in one's life?  I sit here typing this after a day of being lonely and wishing I was closer to my family and my grandson.  Part of me wants to make the big leap and just "do it". Life is short.  Just do it.   The other, conservative part of me says, "You have to make it to retirement.  You have to have something to live on and you don't want to lose this money."  And once I do this, which I will someday, I know it will be a defining moment and I'll classify it post-move.  I think that's a good thing.  I'm tired of living my life around a disease.

Saturday, April 28, 2012

Day 28 of the Cushing's Awareness Challenge: Getting it right...

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The Cushing's Awareness Challenge is winding down, and I haven't posted every day.  I have tried to post at least twice a week.  I have been so busy with work plus dealing with allergies I haven't had time or felt like posting when I have time.

I do believe my allergies are worse since my BLA.  Perhaps the high cortisol treated them?  I don't know.  I do know this spring allergens are worse in my area than they usually are.  Everything seemed to bloom and spout pollen all at once.

Someone asked me the other day why we are so concerned about awareness for Cushing's.  "Isn't is a really rare disease?"

"No", I said, "It's just rarely diagnosed."  

And there is research to back up my statement.  One recent research article is one you should take to your doctor if you believe you have Cushing's.  It talks about the reality of testing for Cushing's Disease/Syndrome and that it requires a lot of testing.  One can have a lot of normal tests and still have Cushing's.

As I go through my daily life, I see a lot of people who have the signs of Cushing's.  It's a daily conundrum deciding whether to approach a person about it or not.  Many times when I have, I've been met with cynicism or been ignored totally.  Other times, folks want information.  A few times, I've been contacted by these saying either a)  my doctor thinks I'm full of it or b) my doctor thinks you may be right but doesn't know what to do from here.  It's tough, having this disease.  Although there are a lot of textbooks for doctors describing how to test and diagnose, so many of us aren't truly textbook cases.  That's the problem with textbooks.  They are a "one size fits all" type of diagnosis/testing.  We come in all sizes, shapes, and genders.  We don't fit the textbook mold.   Slowly, the textbooks are changing.  Recent research is changing how doctors test and diagnose.  In my opinion, it's going to take another generation or two of doctors to really get it right. Until then, many people won't be diagnosed and treated.

Tuesday, April 24, 2012

Day 24 of the Cushing's Challenge: When the "gold standard" becomes tarnished....

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Urinary Free Cortisol (UFC) testing has long been the "gold standard" for determining the need for more evaluation in the diagnosis of Cushing's Disease/Syndrome (CS). However, recent research belies the paradigm, especially with cyclic/episodic and mild/subclinical CS.

A fairly recent testing protocol, late-night salivary cortisol (NSC), is often touted as a replacement for the late-night serum cortisol. The ease of use at home has made it a practical application for testing cortisol levels. It, too, has limitations in testing for cyclic and/or mild CS.

A third application, the dexamethasone suppression test (DST), is another standard by which practioners evaluate their patients for CS. Again, there are limitations when evaluating cyclic/mild CS.

In a recent study, the full text article examines the three tests mentioned above. They found UFC's were of limited value whe diagnosing "mild" CS.

However, UFC may not accurately reflect the cortisol secretory state in patients with even the modest impairment of renal function (8). In addition, most of the cortisol secreted during a 24-h period is between 0400 h and 1600 h. Subtle increases in nighttime secretion, as may be seen in mild CS, may not be detected or only intermittently detected in a 24-h urine collection.

Notice the majority of the tests fell below the "normal" line on the graph.

In turn, the NSC was more accurate, but there were many "normals" in the results, with multiple repeats with several patients before obtaining a "high" result. The authors speculate this is due to cyclic CS or a "variability around a mildly elevated set point."
Of the 11 patients evaluated, all had surgery, and 10 of the 11 had pathology proven CS. (Sometimes it is hard to get enough sample tissue for a decent pathology with pituitary surgery.)
The DST was evaluated in this same study with those patients who were tested via that means, but not all patients were. However, in another study, the use of the DST was found to be of limited value for those patients with cyclic/mild CS.

These results demonstrate that the great majority of patients with mild and/or periodic Cushing's syndrome suppress to overnight dexamethasone. Since patients with mild and/or periodic Cushing's syndrome are the patients in whom the identification of hypercortisolism is difficult, our results from this relatively small study suggest that this test should no longer be used to exclude these patients from further workup for Cushing's syndrome.
It is important to remember that no one test adequately evaluates a patient for Cushing's. Even more important, multiple tests may have to be repeated multiple times. The authors in the first article emphasize this when they say, "Obviously [NSC and UFC ] may need to be performed several times before the suspected diagnosis of endogenous hypercortisolism can be correctly identified."

Still a third study (Findling, et al) says, "Even more problematic is the interpretation of the results of these tests, particularly if they are not in agreement with each other. This is particularly so in mild Cushing's syndrome; if the symptoms are subtle, the biochemical abnormalities are likely to be subtle as well." This is a very long article, chock full of information.

How important is it to screen for "mild" CS? "Mild" is a misleading term, sometimes more appropriately called subclinical CS. Findling, et al, point out a huge population where CS is generally overlooked and the depressing mortality for those same folks.

Dr. Theodore Friedman, et al, , in their research High Prevalence of Normal Tests Assessing Hypercortisolism in Subjects with Mild and Episodic Cushing’s Syndrome Suggests that the Paradigm for Diagnosis and Exclusion of Cushing ’ s Syndrome Requires Multiple Testing point out no one test is conclusive for testing for Cushing's Disease/Syndrome:

The probability of having Cushing’s syndrome when one test was negative was 92    %  for 23:00 h salivary cortisol, 88 %  for 24-h UFC, 86 %  for 24-h 17OHS, and 54 %  for nighttime plasma cortisol. These results demonstrated that episodic hypercortisolism is highly prevalent in subjects with mild Cushing’s syndrome and no single test was effective in conclusively diagnosing or excluding the condition.

Why is CS generally overlooked, then?  Findling lists many reasons, including a study done by Cartagi, et al, where an extraordinarily large percentage of diabetic patients actually had CS. It is often too easy to pin a diagnosis of diabetes or hypertension without realizing it is a symptom.
The recognition of mild/subclinical and cyclic CS has changed the diagnostic approach. Sadly, too many patients are never seen by those who know that.  And most of all, there really is no such thing as "mild" Cushing's.  It damages the body just as much as "florid".
(For more information on how these tests are done, see Testing 101: Biochemical analysis.
For problems/errors to watch for when testing, see When lab tests don't rate an A+, or even a C-..... )

Kidambi, S., Raff, H., Findling, J.W. (2007). Limitations of nocturnal salivary cortisol and urine free cortisol in the diagnosis of mild Cushing's syndrome. European Journal of Endocrinology, 157(6), 725-731. DOI: 10.1530/EJE-07-0424


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