Generally, Cushing's is caused by non-cancerous tumors (like...90 or 99% of the time). In the pituitary, which is the kind I have and am most familiar with, pituitary tumors have various classifications too.
There are secreting tumors and non-secreting tumors, meaning they produce hormones or do not.
Tumors that do not produce hormones are generally called "incidentalomas" because they are often found incidentally when having a head MRI, etc. There is some evidence to suggest they are problematic, but for the most part nothing is done about them unless they are big and compressing other tissues, causing problems. They typically are watched periodically for growth and when hormone testing (and lack of symptomology) suggests they are non-secreting, that's about the gist of it.
Cushing's Disease is caused by a tumor producing too much ACTH (adrenocorticotropic hormone). That hormone triggers the adrenal glands to produce too much cortisol in the body (a steroid). You can have multiple tumors, or tumors producing multiple hormones. And tumors that do not produce ACTH but produce other hormones cause other diseases like Giantism in children, Acromegaly in adults, thyroid disease, etc.
Non-cancerous pituitary tumors are called adenomas, and they are also classified by size. Small tumors, under 10mm in size, are called "micro-adenomas."
Secreting tumors generally cause symptoms and illness, and while it can be hard to identify and recognize at first (they can seem random and unconnected), it is those symptoms and the illness that sends you looking for answers. They are often very small, for example, the size of a pencil eraser or even simply the pencil's tip, and are usually micro-adenomas.
Tumors can be larger, though, and when over the 10mm mark, they are called "macro-adenomas." These tumors often cause problems even due to sheer size. The pituitary sits at the base of the brain, right beneath the optic chiasm and between the carotid arteries in a very small space. Macro-adenomas can compress the nerves, arteries, and even the brain causing dysfunction and damage.
There are some more things worth knowing about Cushing's tumor's, so I'll explain a bit further. ACTH-producing tumors (especially if you have cyclic/episodic/periodic disease) are often not a solid mass like one would expect. They can be goopy, stringy, blobby, liquidy masses that are poorly defined (ie, their edges --where they stop and the normal tissue begins-- may not be easy to see). They may have tendrils, like arms stretching elsewhere in the pituitary. They may even be hyperplasia, meaning that instead of being a defined tumor area causing problems, it is more like pre-tumor tissue with many of the individual cells causing the problem. I picture it like mold that is just starting to spread...the seeds can be all over, but you don't always see it till it grows into a more solid green mass. As you can probably imagine, these "tumors" can be hard to find, hard to remove completely, and thus cures don't always come with the first or even second round of treatment. I'll explain treatment options in another post this month.
Another reason this disease is hard to diagnose is that these tumors can be quite difficult to find prior to surgery on MRI's. The MRI of choice is a 3T (3 teslas --the strength of the magnet used to produce the images) machine and done with "dynamic" pituitary protocols. This means that they not only take pictures before and after contrast dye is injected into your blood stream, but also AS the dye is going in. It takes two people to time it right, and they often have a count-down to make sure the timing of the MRI pictures is started as the contrast is injected into your blood stream. The reason for this is that these tiny tumors do absorb the contrast dye, but they do it at a *very* slightly slower pace. The pituitary should enhance at a uniform rate, like a wave washing over the pituitary till it is all a fairly consistent brightness in the picture. The adenomas stay grey longer, while the tissues around them turn white, but if you don't catch it as it is happening, by the time you take a post-contrast MRI, it may have absorbed the contrast and look like normal pituitary tissue by that point. Pretty tricky, huh?
Some 50% of surgery-proven pituitary tumors were not visualized on MRI prior to surgery (ie, the pathology report confirms it was an ACTH-producing tumor, but they couldn't see it before they got in there themselves). There is some testing that can be done to help determine if the illness is pituitary in origin, and can possibly help to determine which side of your pituitary it is on, that will be/is discussed in this month's post about testing.
To untrained eyes (read "most radiologists") these tumors go completely unnoticed even when they are looking for them. My own first MRI was read clean by a radiologist that is actually fairly experienced with Cushing's and works with my endocrinologist frequently so he accepted the report as accurate. A year later when my illness had worsened and I was gathering sufficient lab results to prove not only that I had the disease but that it was also likely pituitary in origin, a neuro-radiologist and my endocrinologist both looked at the old MRI and agreed that there was one, possibly two microadenomas on my pituitary gland. A new MRI was ordered, the same radiologist that had initially read my first "clean" noted one adenoma, and my endocrinologist *kindly* had him compare both MRI's and he admitted to having missed it originally, but that it was indeed there.
Neurosurgeons also are sometimes loath to be tied down with definitive answers, like saying "I for sure see a tumor here" and frequently say things like "possible micro-adenoma" and then give it a size and location. ;) Or they will call it "an area of hypo-enhancement". Rare is the neuro (and generally loved) that will call a tumor a tumor. ;) Mine did. He saw one area and called it as he saw it. The second area he said was likely...and in surgery he explored the whole gland, but no other tumorous area was found. My pathology report did indeed come back with tissue consistent with pituitary adenoma, that tested positive for many hormones, and it was in a pattern found with hyperplasia cells. Pathology reports have their limits, though...remember, if you are cyclic and your surgery is done while in a low, it can skew the results, and if they remove a small amount of healthy pituitary cells with the tumor cells, it can test positive for anything produced by those cells.
Thursday, April 3, 2014
Wednesday, April 2, 2014
Types of Cushing's Syndrome
Did you know there are different types and causes of Cushing's Syndrome?
The first separation is whether it comes from inside the body (endogenous) or outside of the body (exogenous).
Endogenous Cushing's Syndrome is generally caused by tumors on the pituitary glad (most common) which is then called "Cushing's Disease", but it can also be caused by tumors on the adrenal glands or an ectopic tumor (fairly rare, and generally within the lungs, which may be cancerous).
Exogenous Cushing's Syndrome comes from taking steroid medications, generally long-term. Steroids are often used to treat auto-immune diseases and conditions like asthma and skin disorders. They can be quite effective, but with the added steroids your body may begin to show signs and symptoms of Cushing's Syndrome.
Another differentiation in endogenous disease is that it can be florid (ie, your cortisol is always high) or cyclic, periodic or episodic (meaning you alternate from low/normal cortisol to high cortisol and back at varying intervals...sometimes predictable, and these fluctuations can be over days or even years).
It can also present as subclinical, mild, or severe...but that is generally more of a classification of how bad your lab work is and not of how sick you are, or how long or badly you've been effected. You can have mild, cyclic disease and still be unable to function and on death's door.
The first separation is whether it comes from inside the body (endogenous) or outside of the body (exogenous).
Endogenous Cushing's Syndrome is generally caused by tumors on the pituitary glad (most common) which is then called "Cushing's Disease", but it can also be caused by tumors on the adrenal glands or an ectopic tumor (fairly rare, and generally within the lungs, which may be cancerous).
Exogenous Cushing's Syndrome comes from taking steroid medications, generally long-term. Steroids are often used to treat auto-immune diseases and conditions like asthma and skin disorders. They can be quite effective, but with the added steroids your body may begin to show signs and symptoms of Cushing's Syndrome.
Another differentiation in endogenous disease is that it can be florid (ie, your cortisol is always high) or cyclic, periodic or episodic (meaning you alternate from low/normal cortisol to high cortisol and back at varying intervals...sometimes predictable, and these fluctuations can be over days or even years).
It can also present as subclinical, mild, or severe...but that is generally more of a classification of how bad your lab work is and not of how sick you are, or how long or badly you've been effected. You can have mild, cyclic disease and still be unable to function and on death's door.
Tuesday, April 1, 2014
Cushing's Awareness Day is coming up on April 8th!
April 8th is Cushing's Awareness Day, it was Dr. Harvey Cushings' birthday - the man to first describe, diagnose and treat this disease. Many Cushies (as we call ourselves) have participated in a 30-day blog challenge, posting every day for the month of April, sharing more information to educate the public, medical professionals, and hopefully help someone just starting out on this path to skip some of the heartache and time it can take to get answers and a diagnosis.
Cushing's is a disfiguring, disabling, and isolating disease. Most people do not understand the wide-spread, drastic, and devastating effects it can have on your mind, body, and emotions. The fat-bias of our society means that we are continually treated as though we have brought our illness and symptoms upon ourselves. We're just fat and lazy, it couldn't all be caused by a tumor! It can be disheartening and hurtful. Often, those who should be the most supportive do not understand and don't make the effort to learn. Even doctors are rarely helpful, and can be just as biased, uneducated, and even down-right mean.
Because of this, we Cushie's have banded together. We quickly jump to help others in need and develop bonds quickly. Despite the miles between us, we often become very close friends...even family. We help each other understand what is going on in our minds and bodies, give suggestions on how to cope, we empathize, we teach what to expect, and share each others heart aches and especially joys. Few understand the joy and relief that can come from being told you have a tumor on your MRI or need brain surgery! We help each other find doctors who are helpful, educated, compassionate, skilled and TRUE GOD-SENDS. We learn from each other, and in the end, become experts in our disease, knowing more than most doctors. What has amazed me the most is that despite our usually completely drained finances and emotional- and physical- energy, Cushies are some of the most caring and giving people I have "met" in my existence. Not just in the less-tangible ways, but in very real, outwards ways as well. I've received calls, messages, gifts, a fundraiser to help pay for my medication when I had no insurance (From the UK to Alaska!), many offers for housing and car rides while traveling for testing and treatment, and even a travel companion for my next surgery! I am not somehow an unusual recipient --this is the norm. We open our hearts and our lives to each other. The National Organization for Rare Disorders' slogan is SO applicable:
Alone we are rare, together we are strong.
Cushing's is a disfiguring, disabling, and isolating disease. Most people do not understand the wide-spread, drastic, and devastating effects it can have on your mind, body, and emotions. The fat-bias of our society means that we are continually treated as though we have brought our illness and symptoms upon ourselves. We're just fat and lazy, it couldn't all be caused by a tumor! It can be disheartening and hurtful. Often, those who should be the most supportive do not understand and don't make the effort to learn. Even doctors are rarely helpful, and can be just as biased, uneducated, and even down-right mean.
Because of this, we Cushie's have banded together. We quickly jump to help others in need and develop bonds quickly. Despite the miles between us, we often become very close friends...even family. We help each other understand what is going on in our minds and bodies, give suggestions on how to cope, we empathize, we teach what to expect, and share each others heart aches and especially joys. Few understand the joy and relief that can come from being told you have a tumor on your MRI or need brain surgery! We help each other find doctors who are helpful, educated, compassionate, skilled and TRUE GOD-SENDS. We learn from each other, and in the end, become experts in our disease, knowing more than most doctors. What has amazed me the most is that despite our usually completely drained finances and emotional- and physical- energy, Cushies are some of the most caring and giving people I have "met" in my existence. Not just in the less-tangible ways, but in very real, outwards ways as well. I've received calls, messages, gifts, a fundraiser to help pay for my medication when I had no insurance (From the UK to Alaska!), many offers for housing and car rides while traveling for testing and treatment, and even a travel companion for my next surgery! I am not somehow an unusual recipient --this is the norm. We open our hearts and our lives to each other. The National Organization for Rare Disorders' slogan is SO applicable:
Alone we are rare, together we are strong.
Wednesday, January 29, 2014
National Rare Disease Day is coming up in February!
Please show your support and help spread awareness!
To find out more, please visit this link:
Sunday, January 26, 2014
SURGERY!!!
I just realized I have failed to announce my upcoming pituitary surgery!
I will be having my surgery on February 7th at the MD Anderson Cancer Center in Houston, Texas, and Dr. Ian McCutcheon is the neurosurgeon that will be performing the operation. We will be leaving for pre-op appointments (of which there are many) that first weekend in February.
The time is fast approaching! At times I feel a squeeze of anxiety in my chest, but 6 kids and the internet help distract me from dwelling on it. There has been enough logistical planning required that I haven't had a hard time with it yet. I AM excited for surgery, and to see the recovered me, just not the in-between part! ;)
Brain surgery, here I come!
I will be having my surgery on February 7th at the MD Anderson Cancer Center in Houston, Texas, and Dr. Ian McCutcheon is the neurosurgeon that will be performing the operation. We will be leaving for pre-op appointments (of which there are many) that first weekend in February.
The time is fast approaching! At times I feel a squeeze of anxiety in my chest, but 6 kids and the internet help distract me from dwelling on it. There has been enough logistical planning required that I haven't had a hard time with it yet. I AM excited for surgery, and to see the recovered me, just not the in-between part! ;)
Brain surgery, here I come!
Saturday, January 25, 2014
Positive and Negative...Lets talk about illness.
I have an online friend who just recently found out she has Cushing's and is trying to learn about this disease and all it entails, all while quite ill. Interestingly enough, she and I both had similar experiences today. Okay, the method wasn't too similar, but the meaning was pretty close and hit us both rather abrasively. You see, today we were both told that we shouldn't talk about our illness to people we would expect to be caring and supportive.
Some of this stems from ignorance, and I can't blame them too much for that because even doctors are ignorant of this disease, but knowing that still does little to lessen the sting. So, let me explain a few things to those who want to get past the ignorance and move more towards support and understanding:
This disease takes just about everything you've got and then some. Literally. It affects EVERY aspect of your physical body, every system and function, it drains all energy even for simple things, directly affects your emotions, your ability to think and concentrate -everything. If something affected your life that dramatically, and that universally, it WOULD be a part of every aspect of your life as well. Talking about it would be talking about your life, and vise versa. And talking about it is not a measure of your optimism or pessimism, it just *IS.*
Not talking about it does not make it go away, either. In fact, it generally makes things worse for us. Even when someone knows we are ill, they rarely understand how much this disease does effect unless they've somehow experienced it for themselves (as the one ill or a support person). (Think of the worst flu you've ever had, plus bipolar disorder, plus an extra person in weight, plus being 75 years old, plus insomnia, etc...I could keep going but you should get the picture!) Expectations of what you are capable of and what diagnosis, treatment, and recovery should look like are completely different with this disease than surgery and recovery for more common ailments. It is not fast. It is not easy. Even a good outcome can mean lifelong difficulty, and certainly 1-2 years plus of recovery.
To people around me, I may sound like I am pessimistic about my brain surgery because I explain the risk of recurrence and the long recovery time up front. I know I will receive a cure, even if I don't know in what form or timeframe it will come. I talk about the "negatives" because of people who have these false ideas, because I don't want them ignorant. Again, it isn't negative, it just *IS.* I know what to expect and I know that if everything goes perfectly, it will be long and hard. And still, I am THRILLED for the chance of surgery, and the remission that may bring. When those long, hard days of recovery roll in, I do not need uninformed people coming up to me on a difficult day and making judgmental or flippant comments. I don't need to again be blamed for the fact that I haven't recovered yet, some 3 or 8 months after surgery, to have their support withdrawn because I must be faking, or to have them tell me that my daily struggle is inappropriate to discuss with friends and loved ones. In fact, the stress such comments/actions would bring me can be life-threatening in the days and weeks following my surgery.
When you think about it, we don't expect someone who has just found out they have cancer to NOT think/talk/read/research about it, or someone who has just had a child with down syndrome or autism. It is consuming! It is new, and it is big, real and in your face. Even with something positive like pregnancy or childbirth, we women talk about it all the time for a long time, and every time the subject comes up, we talk about it more! The difference is that Cushing's only gets worse as time goes on and steals more of the old self we once had. It is truly like mourning the death of your old self, learning what this new self is and what it requires of us, all while trying to fight for a diagnosis and treatment so we can get to the next self, the post-op, recovering self.
Right now Cushing's IS all but ruling my life. I don't make any excuses for that --it does take over; that is what it does. I also HAVE been fighting back as best I can and have been for a long time. Now that I've got the upper hand, I plan to win! But, for now, this is a big part of my life. If you wish to share a part in my life, you will, sad as it may seem to you, also have to share a part in my Cushing's. If you want to know about my day, you will undoubtedly hear about Cushing's in some way. If you want to understand why I do what I do, you'll have to understand something about Cushing's. If you want to be able to support me, you'll have to know something about what I am going through. It isn't an ultimatum, it is just the way it works.
I can't tell you how many acquaintances and old friends have told me they've looked up my disease, researched it, and asked questions or offered words of support showing that they spent a few moments to learn about what is happening in my life. It is SO touching that they would spend that time, that they would show that love and concern. One friend followed my progress and realized she had the same issues, went to her doctor, and now is well on her way to her diagnosis (She has the lab work proving disease, just has to locate the source now). Who would have thought? I don't take credit for her diagnosis; she did the work all on her own...but I AM so glad that she will get the help she needs now.
Maybe through reading about my illness, there'll be a little spark planted in your mind, so that the when you see an obese person you stop and think that their body may have done this to them and have compassion on them. Or maybe you'll have a friend who shares some odd symptoms one day, and you'll remember a tidbit you learned, and that'll be enough of a nudge to help them find the information and treatment they need.
Life isn't as black and white as it seems. We are all connected more than we'd like to admit sometimes. And, the world can always use a little more compassion.
Some of this stems from ignorance, and I can't blame them too much for that because even doctors are ignorant of this disease, but knowing that still does little to lessen the sting. So, let me explain a few things to those who want to get past the ignorance and move more towards support and understanding:
This disease takes just about everything you've got and then some. Literally. It affects EVERY aspect of your physical body, every system and function, it drains all energy even for simple things, directly affects your emotions, your ability to think and concentrate -everything. If something affected your life that dramatically, and that universally, it WOULD be a part of every aspect of your life as well. Talking about it would be talking about your life, and vise versa. And talking about it is not a measure of your optimism or pessimism, it just *IS.*
Not talking about it does not make it go away, either. In fact, it generally makes things worse for us. Even when someone knows we are ill, they rarely understand how much this disease does effect unless they've somehow experienced it for themselves (as the one ill or a support person). (Think of the worst flu you've ever had, plus bipolar disorder, plus an extra person in weight, plus being 75 years old, plus insomnia, etc...I could keep going but you should get the picture!) Expectations of what you are capable of and what diagnosis, treatment, and recovery should look like are completely different with this disease than surgery and recovery for more common ailments. It is not fast. It is not easy. Even a good outcome can mean lifelong difficulty, and certainly 1-2 years plus of recovery.
To people around me, I may sound like I am pessimistic about my brain surgery because I explain the risk of recurrence and the long recovery time up front. I know I will receive a cure, even if I don't know in what form or timeframe it will come. I talk about the "negatives" because of people who have these false ideas, because I don't want them ignorant. Again, it isn't negative, it just *IS.* I know what to expect and I know that if everything goes perfectly, it will be long and hard. And still, I am THRILLED for the chance of surgery, and the remission that may bring. When those long, hard days of recovery roll in, I do not need uninformed people coming up to me on a difficult day and making judgmental or flippant comments. I don't need to again be blamed for the fact that I haven't recovered yet, some 3 or 8 months after surgery, to have their support withdrawn because I must be faking, or to have them tell me that my daily struggle is inappropriate to discuss with friends and loved ones. In fact, the stress such comments/actions would bring me can be life-threatening in the days and weeks following my surgery.
When you think about it, we don't expect someone who has just found out they have cancer to NOT think/talk/read/research about it, or someone who has just had a child with down syndrome or autism. It is consuming! It is new, and it is big, real and in your face. Even with something positive like pregnancy or childbirth, we women talk about it all the time for a long time, and every time the subject comes up, we talk about it more! The difference is that Cushing's only gets worse as time goes on and steals more of the old self we once had. It is truly like mourning the death of your old self, learning what this new self is and what it requires of us, all while trying to fight for a diagnosis and treatment so we can get to the next self, the post-op, recovering self.
Right now Cushing's IS all but ruling my life. I don't make any excuses for that --it does take over; that is what it does. I also HAVE been fighting back as best I can and have been for a long time. Now that I've got the upper hand, I plan to win! But, for now, this is a big part of my life. If you wish to share a part in my life, you will, sad as it may seem to you, also have to share a part in my Cushing's. If you want to know about my day, you will undoubtedly hear about Cushing's in some way. If you want to understand why I do what I do, you'll have to understand something about Cushing's. If you want to be able to support me, you'll have to know something about what I am going through. It isn't an ultimatum, it is just the way it works.
I can't tell you how many acquaintances and old friends have told me they've looked up my disease, researched it, and asked questions or offered words of support showing that they spent a few moments to learn about what is happening in my life. It is SO touching that they would spend that time, that they would show that love and concern. One friend followed my progress and realized she had the same issues, went to her doctor, and now is well on her way to her diagnosis (She has the lab work proving disease, just has to locate the source now). Who would have thought? I don't take credit for her diagnosis; she did the work all on her own...but I AM so glad that she will get the help she needs now.
Maybe through reading about my illness, there'll be a little spark planted in your mind, so that the when you see an obese person you stop and think that their body may have done this to them and have compassion on them. Or maybe you'll have a friend who shares some odd symptoms one day, and you'll remember a tidbit you learned, and that'll be enough of a nudge to help them find the information and treatment they need.
Life isn't as black and white as it seems. We are all connected more than we'd like to admit sometimes. And, the world can always use a little more compassion.
Tuesday, December 10, 2013
My Cushing's Story
I started writing up a bit of my story because I joined a new support group, and realized it was far too long for an introduction. I decided I would paste it into a document and work on finishing it as my story for the Cushing's-Help website's patient bio's. I'm not sure I'm done...I should probably add my symptoms list for others to be able to relate to themselves, but here is what I've written thus far this afternoon:
I've had random symptoms off and on for years (almost two decades now, from about the age of 15) but didn't realize they were related to illness, or that I had one overarching disease causing them all.
Looking back, the onset of my disease was in my teen years. I gained more than 60lbs in roughly a
year's time without changing diet or activity level. I developed stretch marks that ran from my knees
to my elbows (and everywhere in between!). I started losing my once-thick hair. I developed horrible acne. I went from being an early morning riser to staying up late at night because I was wide awake, and waking often throughout the night. I went from being happy overall to being anxious and depressed for no apparently reason (and medication had no effect on it either). I was told it was either all in my head or all my fault (by varying people, some directly, some implied it) and I internalized that and just assumed I was too lazy and had bad genetics... I TRIED to exercise but would feel so sick afterwards that I couldn't make any gains, I joined a gym and put myself on a diet in high school but none of it made any difference. When I brought up my symptoms to doctors, they could never put it together, often blamed me for them (Just diet and exercise and it'll go away), and sometimes treated me like I was just plain crazy. I still don't go to doctors unless I have to because of those experiences.
After getting married, I had had some complicated pregnancies...but it was more than that. I would get flank pain and drop into "lows" that I didn't understand, complete with feeling cold, diarrhea,
weakness, exhaustion, nausea, loss of appetite, and extreme weight loss (muscle loss, more like it). I
had high cardiac output but low blood pressure and a high pulse rate. I'd go into tachycardia for NO
apparent reason and had all kinds of cardiac monitoring done. My blood pressure was labile, but usually low, and still I'd end up with severe complications. Breastfeeding wasn't going well despite the "mechanics" and flow being there...my babies were never satisfied and I always felt sickly. The
differences were drastic (but a bit graphic to share here publicly). I seemed to get pregnant at the
drop of a hat, but my body seemed unable to deliver on it's own. My body just didn't react like it
should to anything. I even once had an episode post-partum that now I know was likely somewhere between adrenal insufficiency and adrenal crisis. I was left alone to sleep it off (just thinking about it now scares me), but I didn't know any better at the time.
Then about 3-4 years ago I hit this point where I just had the feeling that if I didn't get whatever was
going on under control, I'd end up with something more permanent and dangerous (like cancer or
diabetes). I still got seemingly random symptoms but I had too many of them, and they were getting
worse. I also started to notice that my good days and bad days seemed to come in cycles. 3 days, 3
weeks...I'd be good for a while, then worse for a while, then good for a while. I had already eaten
"clean" and kept myself active, so I decided to try "nutritional balancing therapy" and started taking a
karate class multiple times a week (burns TONS of calories, fyi). They ran some tests for various
vitamins/minerals, and said I had adrenal insufficiency. The diet I was put on was a higher fat (good
fat), higher protein, TONS of veggies diet (basically we just cut out my grains/starches and added in
more fat) but between the diet and the exercise, I became so ill I couldn't get off the couch for about
4 weeks. I had to give up both and it took some time to recover, but I never got back to where I had
been, not even close.
I started studying adrenal fatigue and came to the realization that I had done everything to correct AF
but was still going downhill. I obviously needed outside help. I had tried supplements, diet (years of
it), everything. I became pregnant unexpectedly and was active, even tap-dancing with a major part in a musical at 20 weeks pregnant. I would have these ups and downs that seemed random, but when I finished the musical, I hit a new low and never seemed to come back from it. I just became more and more exhausted. To the point that certain days I could *feel* the energy it took to hold my head up to watch a movie with my kids. The CNM and OB both said I was just depressed and upped my dose of Vitamin D. They wanted me to go on antidepressants, and I refused. I knew the difference between not wanting to do things and not being able to do them. I called a doctor that specialized in Adrenal Fatigue in California after having read through his website, and he basically said that I would continue to get worse, but that he wouldn't treat me because of my pregnancy. No help, no suggestions, just "come see me if you make it out alive" basically.
I had joined an Addison's support group online, and they helped me learn a lot about AI and Addison's, about symptoms, testing, about Hashimoto's, etc. I am SO grateful to these women who supported me and taught me much. They never questioned if I was just depressed or if I was really sick, and they were so kind they WERE the sanity that I needed so desperately. I was getting nowhere with local doctors, my husband believed me and was as helpful as he could be, but it was taking a big toll on us, and when we asked for help from our local church leaders with cleaning our home because I no longer could do it (and my husband was so overwhelmed doing everything by himself), we were threatened as a family and refused help. I was desperate; I was hurting. My whole family was struggling because of this disease and the treatment (and lack thereof) we'd received from doctors and so-called friends.
These Addisonians had been talking a lot about one specific endocrinologist that specializes in
pituitary disorders (Dr. F). In complete desperation, I emailed him, knowing the chances that he'd take me or that I could even get in to see him before delivery (due to travel restriction based on gestation) was unlikely. But I was scared of what a delivery with untreated Addison's might bring (I knew the stats and knew I didn't trust the local OB), so I emailed explaining my situation and sent my current lab work (I had to go to my GP because my OB wouldn't even test my thyroid or iron!). I knew it sometimes took weeks to get a response or get in to see this doctor 3 states away, but I sent the email on February 8th, and heard back via email that same night from his office lady. She was sure he could help me, and suggested I schedule an appointment right away, and was waiting to hear back from him directly. He responded that he did see something amiss in my lab work, and I was scheduled for an appointment and buying plane tickets. My appointment was on Valentine's evening and a friend flew with me because I was too weak to do it alone, and because my brain was too foggy to feel comfortable understanding and responding to everything in the appointment, not to mention I was super pregnant with my 6th child!
I went in SURE I had Addison's Disease, or at least a form of adrenal insufficiency, and even tried to argue that fact. I came out with a LOT of testing for Cushing's Disease. It was, in fact, the low cortisol periods that I was noticing, but it was being caused by periods of high cortisol. You see, the cortisol takes a big toll on your body and overrides the normal feedback system of your pituitary and adrenal glands. While the tumor is actively pumping out ACTH, it can shut down your own pituitary's normal production because the pituitary feedback says there is already too much cortisol in your system. Then, if/when the tumor "kicks off" (who knows why they do this), your pituitary is in a lazy state from not having been working and it can take a while for it to kick back in. This can bring life-threatening lows, but generally it just brings low-cortisol symptoms which are still uncomfortable.
I was unprepared for the change in direction at my appointment. I had the right system and hormones, but I was looking at it backwards, and the more I learned about cyclic Cushing's Disease, the more sense it made, the more things clicked together, and the more I understood about my past and present symptoms. I have cyclic Cushing's Disease. I had read up a little on this about 10 years prior, when my mother-in-law had died from untreated Cushing's (she refused treatment and was a stubborn, intelligent women who got her way). I had read through some information then, with my husband. We had concluded that it was a possibility, but I didn't have enough of the symptoms (maybe half?) and decided that I wasn't nearly sick enough for that to be the problem. How wrong we were! I certainly wasn't as bad as many, but I found that the downhill turns were often sudden and drastic, especially in the more recent years.
At my appointment I was also told I had hypothyroidism. He ordered more of those tests (to get a trend) and an antibody test. I had Hashimoto's Thyroiditis (an autoimmune thyroid disease) and was put on thyroid medication. My ferritin level (stored iron) was so low it was in single digits (he wants it around 60) and he said that had I not been flying home the next morning, he'd have had me in the hospital for IV iron infusions. Needless to say, I was put on iron --lots of it. My vitamin D was still lower than he'd like, despite having been on treatment, so he switched me to 50K iu's of D3 weekly (My OB had chastised me repeatedly for taking D3 instead of D2; Ha ha!).
I had to wait for a while after my pregnancy to allow my body to normalize before doing my testing. I first tested by date (randomly, basically) and got a few marginal highs, but mostly normal test results. My pituitary MRI was read clean. Dr. F told me he didn't know what was wrong, but that it didn't look like it was Cushing's because of the testing. I was not prepared for that, and just ended the conversation. I was emotionally, mentally, and physically exhausted and didn't plead my case. I didn't have the money to test more, even though I was pretty sure I needed it. And looking back, had I asked, he probably would have obliged.
I decided to again try natural healing methods. Nothing worked, and some things (extended juice fasting, for instance) actually made me much worse. Every time I hit another "low", it seemed to become my new normal...and that was scary. I kept losing more energy and strength, more of my mental ability, and each time I couldn't imagine it getting worse, yet it always did. (I still haven't learned this lesson!)
About a year later, after a lot of prayer and thinking, after I'd exhausted most natural treatment
methodologies I felt willing to try, I realized I did indeed need to go back and push for further
testing, and test by symptoms. Mentally and emotionally I was in a much better place, and while I had recovered a bit after my delivery, I had started to again slide downhill despite my best efforts.
I emailed Dr. F to ask about further testing, this time by symptoms, and there was no pushing or
arguing necessary! He gave me more sensitive testing this go round, and told me to test as much as it took. He believed me! It was as if the way just opened up for me this time. I was uninsured, but I applied for the Cushing's Assistance program through NORD (The National Organization for Rare Disorders) and was accepted. They offered to cover the costs of testing, doctor's appointments, and travel needed for the same, that would lead to a diagnosis of Cushing's Disease. I was in public when my husband called and read me the letter, and I started bawling right then and there in the shopping isle. It was an answer to a prayer I didn't even think to voice. I then called to share the news with family and friends and bawled again, scaring yet more customers!
Tracking my symptoms wasn't a very easy task. I went totally OCD on them, and still I was only somewhat successful in my efforts. I could get the overall trend, but the day-to-day was confusing as all-get-out. My testing was also complicated by living in Alaska. I could only turn in tests 4 days a week because they had to fly out to the labs in Seattle, WA and beyond. It took about a month to get each result back. Add to that a head cold that killed my cortisol levels for 6 weeks, and it took me a few months to get sufficient high labs even with my 2-page-wide spreadsheet of symptom data.
In that time, I also made friends on the Cushing's-Help website and Facebook groups. I learned a LOT of things from them, and one friend in particular likes to "read" pituitary MRI's the way I like to "read" fetal ultrasounds. She looked at my previously "clean" MRI and said that in her lay opinion, it was anything BUT normal. As a favor, her neuro-radiologist also took a look at my MRI, and was so kind as to send back pictures with ARROWS of pituitary adenoma's and suspicious areas on my MRI to forward on to my endocrinologist. As it turns out, my doctor hadn't read the disc himself and had just read the report. He looked at the disc and agreed it was not normal, and sent me a message stating I needed a new MRI (it had been over a year at this point and my previous MRI still had some of that post-partum "rainbow" shape to the pituitary) and that it should be read by a neurosurgeon this time around. JOY OF JOYS! This brought me even more hope! He said SURGEON, not just himself...that meant I was getting so close to that diagnosis and surgery clearance --to getting help.
I scheduled my MRI trip (can't do a 3T dynamic here), and decided to schedule a face-to-face with my endocrinologist again while in the same city. NORD paid for the flights, reimbursed me for the cost of my doctor's appointment, paid for the MRI, and paid for my hotel room. My husband came with me this time, and it was the best doctor's appointment I've had in my life. I was still nervous that somehow it wasn't enough, or that the MRI done the day before my appointment would miraculously have become normal again. That was not the case. My MRI showed two adenomas on opposite sides of my pituitary amongst other things, and my 7+ diagnostic-level high labs were sufficient...and it felt AMAZING!
Who knew we'd be so excited to hear I was diagnosed with a deadly disease? That we'd shout for joy and clap our hands at finding multiple tumors in my head? I had a smile that wouldn't go away. The medical student shadowing my endocrinologist hadn't seen the diagnosis side, where patients are so relieved to have an end in sight, to finally be getting help and have a chance at getting better, that they are happy! I also wore my "Does my pituitary gland make me look fat?" shirt to this appointment, so we were joking, taking pictures, and having a grand old time. He gave me permission to share the picture of us, and without prompting pointed to my head for the next picture saying, "It's right HERE!" Dr. F is generally stoic, dealing with very ill people, with a very serious disease, and he is often their last hope at life...so I feel myself privileged to have had the opportunity to see him in-person for my diagnosis appointment, and to see this other side of him. I hope he felt our gratitude as well.
The "pick whose going to cut into your head" decision took a while. I was offered 100% coverage through a quality hospital and with a quality neurosurgeon for anything done at their facility, but the
endocrinologist there wanted me to start my testing process ALL over again with them, at my cost at
home. I was not willing to start over after all that hard work and with as quickly as I was
deteriorating, so I decided to wait till January when the new health coverage laws were in effect and I
could again get insurance without preexisting conditions clauses. I was able to be referred to my
first-choice of neurosurgeon's and am on Ketoconazole to help lower my cortisol while I wait. And it
just so happens that my brother-in-law has recently moved with his family to this large city and offered us a place to stay before/during/after surgery.
Keto has it's benefits and side effects, but I'm glad to be on it while I'm waiting. I feel like it is
giving me some practice for what things will feel like and when to act in the months following my
surgery. I won't get a set surgery date until January 2nd after my insurance is fully in effect, but my
surgery is penciled in for January 24th right now. I'll update after my surgery when I am able.
I've had random symptoms off and on for years (almost two decades now, from about the age of 15) but didn't realize they were related to illness, or that I had one overarching disease causing them all.
Looking back, the onset of my disease was in my teen years. I gained more than 60lbs in roughly a
year's time without changing diet or activity level. I developed stretch marks that ran from my knees
to my elbows (and everywhere in between!). I started losing my once-thick hair. I developed horrible acne. I went from being an early morning riser to staying up late at night because I was wide awake, and waking often throughout the night. I went from being happy overall to being anxious and depressed for no apparently reason (and medication had no effect on it either). I was told it was either all in my head or all my fault (by varying people, some directly, some implied it) and I internalized that and just assumed I was too lazy and had bad genetics... I TRIED to exercise but would feel so sick afterwards that I couldn't make any gains, I joined a gym and put myself on a diet in high school but none of it made any difference. When I brought up my symptoms to doctors, they could never put it together, often blamed me for them (Just diet and exercise and it'll go away), and sometimes treated me like I was just plain crazy. I still don't go to doctors unless I have to because of those experiences.
After getting married, I had had some complicated pregnancies...but it was more than that. I would get flank pain and drop into "lows" that I didn't understand, complete with feeling cold, diarrhea,
weakness, exhaustion, nausea, loss of appetite, and extreme weight loss (muscle loss, more like it). I
had high cardiac output but low blood pressure and a high pulse rate. I'd go into tachycardia for NO
apparent reason and had all kinds of cardiac monitoring done. My blood pressure was labile, but usually low, and still I'd end up with severe complications. Breastfeeding wasn't going well despite the "mechanics" and flow being there...my babies were never satisfied and I always felt sickly. The
differences were drastic (but a bit graphic to share here publicly). I seemed to get pregnant at the
drop of a hat, but my body seemed unable to deliver on it's own. My body just didn't react like it
should to anything. I even once had an episode post-partum that now I know was likely somewhere between adrenal insufficiency and adrenal crisis. I was left alone to sleep it off (just thinking about it now scares me), but I didn't know any better at the time.
Then about 3-4 years ago I hit this point where I just had the feeling that if I didn't get whatever was
going on under control, I'd end up with something more permanent and dangerous (like cancer or
diabetes). I still got seemingly random symptoms but I had too many of them, and they were getting
worse. I also started to notice that my good days and bad days seemed to come in cycles. 3 days, 3
weeks...I'd be good for a while, then worse for a while, then good for a while. I had already eaten
"clean" and kept myself active, so I decided to try "nutritional balancing therapy" and started taking a
karate class multiple times a week (burns TONS of calories, fyi). They ran some tests for various
vitamins/minerals, and said I had adrenal insufficiency. The diet I was put on was a higher fat (good
fat), higher protein, TONS of veggies diet (basically we just cut out my grains/starches and added in
more fat) but between the diet and the exercise, I became so ill I couldn't get off the couch for about
4 weeks. I had to give up both and it took some time to recover, but I never got back to where I had
been, not even close.
I started studying adrenal fatigue and came to the realization that I had done everything to correct AF
but was still going downhill. I obviously needed outside help. I had tried supplements, diet (years of
it), everything. I became pregnant unexpectedly and was active, even tap-dancing with a major part in a musical at 20 weeks pregnant. I would have these ups and downs that seemed random, but when I finished the musical, I hit a new low and never seemed to come back from it. I just became more and more exhausted. To the point that certain days I could *feel* the energy it took to hold my head up to watch a movie with my kids. The CNM and OB both said I was just depressed and upped my dose of Vitamin D. They wanted me to go on antidepressants, and I refused. I knew the difference between not wanting to do things and not being able to do them. I called a doctor that specialized in Adrenal Fatigue in California after having read through his website, and he basically said that I would continue to get worse, but that he wouldn't treat me because of my pregnancy. No help, no suggestions, just "come see me if you make it out alive" basically.
I had joined an Addison's support group online, and they helped me learn a lot about AI and Addison's, about symptoms, testing, about Hashimoto's, etc. I am SO grateful to these women who supported me and taught me much. They never questioned if I was just depressed or if I was really sick, and they were so kind they WERE the sanity that I needed so desperately. I was getting nowhere with local doctors, my husband believed me and was as helpful as he could be, but it was taking a big toll on us, and when we asked for help from our local church leaders with cleaning our home because I no longer could do it (and my husband was so overwhelmed doing everything by himself), we were threatened as a family and refused help. I was desperate; I was hurting. My whole family was struggling because of this disease and the treatment (and lack thereof) we'd received from doctors and so-called friends.
These Addisonians had been talking a lot about one specific endocrinologist that specializes in
pituitary disorders (Dr. F). In complete desperation, I emailed him, knowing the chances that he'd take me or that I could even get in to see him before delivery (due to travel restriction based on gestation) was unlikely. But I was scared of what a delivery with untreated Addison's might bring (I knew the stats and knew I didn't trust the local OB), so I emailed explaining my situation and sent my current lab work (I had to go to my GP because my OB wouldn't even test my thyroid or iron!). I knew it sometimes took weeks to get a response or get in to see this doctor 3 states away, but I sent the email on February 8th, and heard back via email that same night from his office lady. She was sure he could help me, and suggested I schedule an appointment right away, and was waiting to hear back from him directly. He responded that he did see something amiss in my lab work, and I was scheduled for an appointment and buying plane tickets. My appointment was on Valentine's evening and a friend flew with me because I was too weak to do it alone, and because my brain was too foggy to feel comfortable understanding and responding to everything in the appointment, not to mention I was super pregnant with my 6th child!
I went in SURE I had Addison's Disease, or at least a form of adrenal insufficiency, and even tried to argue that fact. I came out with a LOT of testing for Cushing's Disease. It was, in fact, the low cortisol periods that I was noticing, but it was being caused by periods of high cortisol. You see, the cortisol takes a big toll on your body and overrides the normal feedback system of your pituitary and adrenal glands. While the tumor is actively pumping out ACTH, it can shut down your own pituitary's normal production because the pituitary feedback says there is already too much cortisol in your system. Then, if/when the tumor "kicks off" (who knows why they do this), your pituitary is in a lazy state from not having been working and it can take a while for it to kick back in. This can bring life-threatening lows, but generally it just brings low-cortisol symptoms which are still uncomfortable.
I was unprepared for the change in direction at my appointment. I had the right system and hormones, but I was looking at it backwards, and the more I learned about cyclic Cushing's Disease, the more sense it made, the more things clicked together, and the more I understood about my past and present symptoms. I have cyclic Cushing's Disease. I had read up a little on this about 10 years prior, when my mother-in-law had died from untreated Cushing's (she refused treatment and was a stubborn, intelligent women who got her way). I had read through some information then, with my husband. We had concluded that it was a possibility, but I didn't have enough of the symptoms (maybe half?) and decided that I wasn't nearly sick enough for that to be the problem. How wrong we were! I certainly wasn't as bad as many, but I found that the downhill turns were often sudden and drastic, especially in the more recent years.
At my appointment I was also told I had hypothyroidism. He ordered more of those tests (to get a trend) and an antibody test. I had Hashimoto's Thyroiditis (an autoimmune thyroid disease) and was put on thyroid medication. My ferritin level (stored iron) was so low it was in single digits (he wants it around 60) and he said that had I not been flying home the next morning, he'd have had me in the hospital for IV iron infusions. Needless to say, I was put on iron --lots of it. My vitamin D was still lower than he'd like, despite having been on treatment, so he switched me to 50K iu's of D3 weekly (My OB had chastised me repeatedly for taking D3 instead of D2; Ha ha!).
I had to wait for a while after my pregnancy to allow my body to normalize before doing my testing. I first tested by date (randomly, basically) and got a few marginal highs, but mostly normal test results. My pituitary MRI was read clean. Dr. F told me he didn't know what was wrong, but that it didn't look like it was Cushing's because of the testing. I was not prepared for that, and just ended the conversation. I was emotionally, mentally, and physically exhausted and didn't plead my case. I didn't have the money to test more, even though I was pretty sure I needed it. And looking back, had I asked, he probably would have obliged.
I decided to again try natural healing methods. Nothing worked, and some things (extended juice fasting, for instance) actually made me much worse. Every time I hit another "low", it seemed to become my new normal...and that was scary. I kept losing more energy and strength, more of my mental ability, and each time I couldn't imagine it getting worse, yet it always did. (I still haven't learned this lesson!)
About a year later, after a lot of prayer and thinking, after I'd exhausted most natural treatment
methodologies I felt willing to try, I realized I did indeed need to go back and push for further
testing, and test by symptoms. Mentally and emotionally I was in a much better place, and while I had recovered a bit after my delivery, I had started to again slide downhill despite my best efforts.
I emailed Dr. F to ask about further testing, this time by symptoms, and there was no pushing or
arguing necessary! He gave me more sensitive testing this go round, and told me to test as much as it took. He believed me! It was as if the way just opened up for me this time. I was uninsured, but I applied for the Cushing's Assistance program through NORD (The National Organization for Rare Disorders) and was accepted. They offered to cover the costs of testing, doctor's appointments, and travel needed for the same, that would lead to a diagnosis of Cushing's Disease. I was in public when my husband called and read me the letter, and I started bawling right then and there in the shopping isle. It was an answer to a prayer I didn't even think to voice. I then called to share the news with family and friends and bawled again, scaring yet more customers!
Tracking my symptoms wasn't a very easy task. I went totally OCD on them, and still I was only somewhat successful in my efforts. I could get the overall trend, but the day-to-day was confusing as all-get-out. My testing was also complicated by living in Alaska. I could only turn in tests 4 days a week because they had to fly out to the labs in Seattle, WA and beyond. It took about a month to get each result back. Add to that a head cold that killed my cortisol levels for 6 weeks, and it took me a few months to get sufficient high labs even with my 2-page-wide spreadsheet of symptom data.
In that time, I also made friends on the Cushing's-Help website and Facebook groups. I learned a LOT of things from them, and one friend in particular likes to "read" pituitary MRI's the way I like to "read" fetal ultrasounds. She looked at my previously "clean" MRI and said that in her lay opinion, it was anything BUT normal. As a favor, her neuro-radiologist also took a look at my MRI, and was so kind as to send back pictures with ARROWS of pituitary adenoma's and suspicious areas on my MRI to forward on to my endocrinologist. As it turns out, my doctor hadn't read the disc himself and had just read the report. He looked at the disc and agreed it was not normal, and sent me a message stating I needed a new MRI (it had been over a year at this point and my previous MRI still had some of that post-partum "rainbow" shape to the pituitary) and that it should be read by a neurosurgeon this time around. JOY OF JOYS! This brought me even more hope! He said SURGEON, not just himself...that meant I was getting so close to that diagnosis and surgery clearance --to getting help.
I scheduled my MRI trip (can't do a 3T dynamic here), and decided to schedule a face-to-face with my endocrinologist again while in the same city. NORD paid for the flights, reimbursed me for the cost of my doctor's appointment, paid for the MRI, and paid for my hotel room. My husband came with me this time, and it was the best doctor's appointment I've had in my life. I was still nervous that somehow it wasn't enough, or that the MRI done the day before my appointment would miraculously have become normal again. That was not the case. My MRI showed two adenomas on opposite sides of my pituitary amongst other things, and my 7+ diagnostic-level high labs were sufficient...and it felt AMAZING!
Who knew we'd be so excited to hear I was diagnosed with a deadly disease? That we'd shout for joy and clap our hands at finding multiple tumors in my head? I had a smile that wouldn't go away. The medical student shadowing my endocrinologist hadn't seen the diagnosis side, where patients are so relieved to have an end in sight, to finally be getting help and have a chance at getting better, that they are happy! I also wore my "Does my pituitary gland make me look fat?" shirt to this appointment, so we were joking, taking pictures, and having a grand old time. He gave me permission to share the picture of us, and without prompting pointed to my head for the next picture saying, "It's right HERE!" Dr. F is generally stoic, dealing with very ill people, with a very serious disease, and he is often their last hope at life...so I feel myself privileged to have had the opportunity to see him in-person for my diagnosis appointment, and to see this other side of him. I hope he felt our gratitude as well.
The "pick whose going to cut into your head" decision took a while. I was offered 100% coverage through a quality hospital and with a quality neurosurgeon for anything done at their facility, but the
endocrinologist there wanted me to start my testing process ALL over again with them, at my cost at
home. I was not willing to start over after all that hard work and with as quickly as I was
deteriorating, so I decided to wait till January when the new health coverage laws were in effect and I
could again get insurance without preexisting conditions clauses. I was able to be referred to my
first-choice of neurosurgeon's and am on Ketoconazole to help lower my cortisol while I wait. And it
just so happens that my brother-in-law has recently moved with his family to this large city and offered us a place to stay before/during/after surgery.
Keto has it's benefits and side effects, but I'm glad to be on it while I'm waiting. I feel like it is
giving me some practice for what things will feel like and when to act in the months following my
surgery. I won't get a set surgery date until January 2nd after my insurance is fully in effect, but my
surgery is penciled in for January 24th right now. I'll update after my surgery when I am able.
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