Saturday, April 18, 2015

Testing for Cushing's, Introduction

I started this blog post during last year's Cushing's Awareness Blogger's Challenge in April, and never finished it because it felt like a Herculean task.  I do believe it is important information and it turns out I had the energy to delve into it some today, but I'm going to split it into separate posts because there is a fair amount of information to cover and I only have so much energy and brainpower each day.  Below is the intro and I'll make a separate post on each different type of test in the upcoming days. 

There are various tests used to look for high cortisol levels within the body.  None is perfect and each has it's inherent strengths and weaknesses.  Not all test types will work for everyone, and they won't show a diagnostic high every time you test either, unless you have a more florid form of the disease (ie, always high all the time).  Most of us are diagnosed off of a mixture of these tests, and they often have to be repeated many times.  Here are the most common:

24 hour Urinary Free Cortisol (UFC)
24 hour 17-hydroxycorticosteroids (17 ohc/17ohs)

10 hour Urinary Free Cortisol
Midnight Serum Cortisol
Serum Adrenal Corticotropin Releasing Hormone (ACTH)
Serum Cortisol Binding Globulin (CBG)
Dexamethasone Suppression Tests (Dex)
Inferior Petrosal Sinus Sampling (IPSS)
Imaging such as MRI's and CT's

For most patients, we have to do a series of tests, usually in about 3 different types, before we get our official diagnosis and move on to treatment.  My endocrinologist typically runs 3 or 4 each of 3 different types of tests when suspicion is high for Cushing's (saliva, 24 hr UFC, and either he tags on 17ohc's to each of those 24hr samples or he might order 10 hour UFC's if he suspects you are more cyclic with higher cortisol at night and lower cortisol in the day --it just all depends on your specific case) , along with a midnight serum cortisol and ACTH level after your appointment while in his office, testing of other hormones, and usually he orders an 8am lab to be done when you get home as well (8am serum cortisol, ACTH, and maybe other hormones, vitamin D, and often ferritin as well).

I personally did countless 24hr UFC's, 17ohc's, 10 hr UFC's, Midnight Serums, a handful of ACTH's (midnight and 8am), and a handful of midnight salivary cortisols.  I got many diagnostic highs in every test type *except* for salivary.  (Sad, too, cause they are the easiest to do!)  We don't know why, but patient experience seems to show that it is QUITE common for a patient to test high in some tests and not in others...which tests work best varies from patient to patient and from case to case.

(If I miss something important, please feel free to contact me and I'll update as necessary.)

Friday, April 17, 2015

Growth Hormone Deficiency in Adulthood and the Effects of Growth Hormone Replacement: A Review

This isn't Cushing's-specific, but MANY of us have Cushing's Disease which is caused by pituitary tumors...and tumors are apparently the #1 cause of adult-onset Growth Hormone Deficiency.  We Cushing's patients have those tumors surgically removed, which surgery also can cause a loss of growth hormone production (double whammy, I say), so this is a very appropriate subject for Cushing's Patients to be aware of.  There is a lot of overlap in the symptomology as well, so AGHD needs to be ruled out as the initial cause of symptoms when being diagnosed as well as when a recurrence might be suspected.

So, today I received the Magic Foundation's Friday email which contained an amazing article with LOTS of good information related to Adult Growth Hormone Deficiency and it's treatment (replacement injections).  This article goes through changes to different body systems (metabolism, muscle, bone, skin, body fat, etc) due to AGHD, and then how those things have been affected by GH replacement therapy.  Like I said, there is a TON of interesting information here, but what I found really interesting was how some changes were immediate (happening within days or weeks), some changes didn't really take effect until 3 or 6 months of continuous treatment (and some of those seeing continued improvement up to 3 years later as well), and then there were other more negative changes that happened up-front but returned to normal as your body adjusted over time.  Click on the title below to read for yourself:

Growth Hormone Deficiency in Adulthood and the Effects of Growth Hormone Replacement: A Review

Thursday, April 16, 2015

Nerdy Zebras

We spent the last night of our trip with one of my sisters-in-law, and she pulled out this absolute beauty:

I never was a dedicated fan of zebras, or their stripes, before Cushing's Disease.  (Though admittedly, yellow and blue --the Cushing's awareness ribbon colors-- were my wedding colors!)  But now that I've been diagnosed with rare diseases, I see them everywhere and enjoy their stripyness so much more.  The awareness ribbon for rare diseases is made of zebra stripes and we often call ourselves zebras, too.  There is an oft-heard phrase doctors are taught and then repeat to us patients...patients who couldn't possibly have THAT disease's too rare!

"When you hear hoofbeats, think of horses not zebras." 

A phrase taught to medical students in the late 1940's by Dr. Theodore Woodward, 
professor at the University of Maryland School of Medicine.

Well, many of us got our nerdy glasses on, learned what we needed to know, did what we needed to do, and got our zebra selves to the doctors we needed to see to get our diagnosis and treatment.

We earned our stripes and we wear them proudly!

Tuesday, April 14, 2015

Cushie Friends

On the first night of our trip, we were able to meet up with some fellow Cushie's for dinner ON Cushing's Awareness Day -- Dr. Harvey Cushing's birthday.  It was a blast!  For some perspective, I believe between the 5 of us we've had at least 9 surgeries in an effort to cure Cushing's Disease.  LOVE THESE LADIES!!!

Monday, April 13, 2015

Pushing yourself

I've missed a few days, SORRY!  We were out of town on a crazy-busy trip where I didn't even see a computer (even though we brought a laptop with us!), and I was very, very exhausted and sore when I got home.  I went AI one night on our trip, hard and fast, and that was scary.  I had to stress-dose before I could sleep, wait for it to "wake me up" a bit...I'd really say it was more like it made my head less sludgy and more clear.  Then I went to sleep and slept well. 

So yesterday I just stayed in bed to try to recover from the trip.  If it weren't for my doctor's appointment this morning, I would definitely have preferred to stay in bed as long as possible today.

In relating my experience to my friends, one responded: "Making yourself do stuff is a good reminder of how little your body can actually do without falling apart."  Wow.  I expected the soreness and tiredness, but was shocked at just how sore and exhausted I was.  And, the AI wasn't so pleasant, just sayin'.  All my life I was the kind of person who'd just push to get the job done, but Cushing's eventually made that impossible.  If I push myself, I crash...and it's the kind of crash that can be life-threatening, and even with the proper medication, can take many days to really fully recover from. 

Friday, April 10, 2015

Sphygmomanometer (SFIG-moh-mə-NOM-i-tər)

If you are working on getting your Cushing's diagnosis (cause all that testing IS work), if you are post-op and in recovery, or if you had your adrenal glands removed (BLA), I highly recommend a sphygmomanometer (BP cuff). 

Tracking your BP and pulse can really help you get a feel for when your adrenal hormones are too high or too low.  It isn't a perfect barometer, but it can definitely help.  Many cyclic patients have a bit of a learning curve differentiating high cortisol periods from low cortisol periods when they are first learning about Cushing's and starting to test.  Even post-op you have to learn what "too low" feels like, and it can be somewhat different than the "lows" you feel when you cycle before surgery.

In general, your BP (and sometimes pulse) is higher when your cortisol level is higher.  This is a good time to test if you are not yet diagnosed.

In general, if your BP and pulse is lower, your cortisol level is also lower.

But, as I said, this is NOT a hard and fast rule and it is best to really get a feel for your own individual symptom sets, mixed with signs such as blood pressure and pulse, and to be proactive and use prudent judgment. 

In the case of adrenal insufficiency (if you cycle hard and fast, are post op, etc), things can get really bad really fast, and I always prefer to err on the side of caution.  Though too much hydrocortisone, just like high endogenous cortisol, can cause significant damage over time, a little too much cortisol at one specific time (when AI/adrenal crisis is suspected) isn't likely to do any lasting harm.  Too little in that same situation can become life-threatening quickly, and it often takes your ability to think clearly and act in the process.    

Sometimes people with adrenal insufficiency have what is called a paradoxical response This means their blood pressure goes high even though their cortisol levels are plummeting, as their body tries hard to compensate and stave off adrenal crisis.  It will crash eventually, possibly quickly and dangerously, so it is best to treat it early if you have reason to believe this is the case with you.  In such a case, adding sufficient cortisol will actually LOWER blood pressure and stabilize the patient.  I had a friend show up to the ER with a BP in the 200's/100's, was finally given 100mg solu-cortef, her AI-related symptoms resolved and her BP dropped down to around the 130's/80's.  (If you don't need the extra cortisol, it will raise your BP...she obviously was desperately in need!  Her body put that cortisol to immediate use and started to stabilize itself.)

Regardless, try to get a feel for what "normal" is for you.  Try to pay close attention to your body so you can recognize what might signify a high, or what might signify a low.  At the same time, I've noticed that AI with differing causes for me can also start with differing symptoms.  I have a typical "slow decline" set and a typical "fast crash" one.  I also found that the low symptoms I experienced due to taking a medication that lowerd my cortisol felt different than my natural "stress/overdoing it" lows.  I got used to that too.  But then this year when I added in Growth Hormone replacement, I experienced another completely different set of low symptoms!  It didn't occur to me it wouldn't be like the previous medication's symptoms.  As miserable as I felt, I didn't recognize the symptoms as being from low cortisol till my Endocrinologist brought it up as the likely cause!  DOH!  It won't always be the same, so just listen, listen, listen to that body.

Another form of Adrenal Insufficiency:
  If you happen to also be dealing with aldosterone (another adrenal hormone) or it's synthetic replacement, fludrocortisone, the pulse/BP differences can be a very helpful indicator of sufficient replacement dosage.  Addisonians, BLAers, those with hypopituitarism, and the rare few like me who have had low aldosterone despite high cortisol levels sometimes have to balance both fludro and cortisol...and it can get tricky. 

Higher BP and lower pulse suggests high aldosterone, fludrocortisone over-replacement, or too much fludro and sodium in the diet.  (But fludrocortisone requires a goodly amount of salt intake to function well, so judge wisely.)

Lower BP with higher pulse suggests a need for more salt or too low of an aldosterone/fludricortisone level.

Symptoms of high and low aldo/fludro are actually quite similar.  And another few points worth keeping in mind is that when it is hot outside, you are sweating/active, or you are dehydrated/losing fluids for other reasons, you may need to increase your fludro or salt intake (and fluids, too) to make up for the extra loss.  Some find one works better for them than the other, and how much and which appears to be fairly individual.

*This is just informational, from patient experience.  Please speak with your doctor about how and when to treat symptoms and signs.*