For me, the initial onset was at once very drastic and noticeable, and yet unrecognized. Over the span of roughly a year: I gained roughly 60 pounds (which couldn't be lost by diet and exercise), my hair went from thick to thin and wiry, my acne became horrendous (and untreatable, even by the "harder" drugs they made), I went from being a morning person with no real sleep issues to being unable to fall asleep at a reasonable hour and waking often throughout the night, I developed bright red stretch marks all over my body (spanning literally from my elbows to my knees, front and back, from a combination of the weight gained and the hormones thinning my skin so that it literally was "ripping" as the weight was gained), I went from being a happy and cheerful person to being suddenly anxious and depressed (with no reason for it and no medical treatments had any effect, either), and I started bruising easily and getting sick more often.
I was a teenager at that time. My father had died a couple years before and I had also switched from playing a lot of sports (basketball, softball, even competed in jump rope at a state level) to taking music lessons a few years earlier too. When the anxiety and depression hit, everyone assumed it was because of my father's death a year or two before. When the weight gain hit, it was assumed that it was because of the change in habits a few years before also. I was just fat, lazy, and choosing to be unhappy. It is something I'm still trying to un-learn because it became a part of who I am. The un-learning process is made ever harder by others who have no understanding of this disease STILL treating me as if that is the case. If there is something wrong with my health, it is because I am overweight. I am overweight because I overeat, don't know how to eat healthily, or didn't exercise. I am losing muscle mass because I'm lazy. I can't sleep well at night because I'm not active enough during the day. And not being able to fall asleep at a reasonable hour at night is because I haven't learned to turn off my thoughts.
None of this could be caused by a disease, a tumor...it must be my fault. I didn't know any better and accepting that blame internally has led me to develop a skewed and incorrect sense of who I am and of my self-worth. In some ways I think it is a blessing that I don't know what the "real" me is like --the adult me without this illness. Because of that, when I do receive my remission/cure, I hope I will only see the benefits and improvements that treatment has brought. Many look back and see that they won't get back to that healthy "self" they were before, and it is a source of grief. But on the other hand, I also don't know who I am without this disease. Some might consider this overly dramatic, but imagine a disease that affects over 70% of your body systems...that directly affects your emotional, physical and mental states. There is still a "you" in there, but how would you identify that "you" and separate it from your thoughts, emotions, physical body, and actions? I dare you to give THAT a try! (And if you happen to find a good answer, let me know!)
This disease has changed my ability to think, remember and concentrate (literally shrinking my brain). It has changed my emotional reactions due to the major fluctuations in hormones (even my zen-master friend was unable to control his anger while the steroids raged through his body, but awoke from surgery a different man --a calm man). It has changed how I relate to others and my ability to form relationships (In too many ways to be able to list here, some internal and some by the way others react to me). It has changed my physical abilities through bone and muscle wasting, a complete lack of energy, and uncontrollable weight gain...not to mention drastically changed my personal appearance. It has changed my ability to sleep at night (my cortisol is often too high at night, messing up the circadian or diurnal rhythm, while being normal or even low during the day when I should have more energy to be wide awake).
Can I get my physical body back? Yes...mostly. I might have to trade this untreatable disease for a treatable, life-long one. And while I expect great improvement no matter which surgery method brings a cure for me (another pituitary surgery or bilateral adrenalectomy), there will be some permanent damage that just cannot be undone.
Can I get my emotional self back? Yes and no. I can become emotionally healthy for sure, but there will always be a rememberance of the lessons learned by the hardships I have gone through.
Can I get back my mental self? I sure hope so! I know many are again able to concentrate and remember, but I've seen data suggesting that some damage (ie, brain atrophy) is permanent. But hey! Thinking clearly and remember things is a major improvement!
I'm trying to be realistic in my expectations. I know I'll get major improvements some day, and I REALLY look forward to that. I've missed out on so much, and my children and spouse have missed out on so much. No matter what form my remission takes, I look forward to truly being able to participate in their lives, to live.
Saturday, April 5, 2014
Friday, April 4, 2014
To our fallen Cushies...
To those that have gone on before us and bravely fought this fight...those who may have lost their lives in the battle against Cushing's Disease: we love you, our hearts break for your families and friends, and you have left a permanent imprint upon this world and our lives. Because of you, many of us have been spurred on to fight harder, to keep moving, to love more, to be more forgiving...
to live.
Cushing's did NOT win for now you are free.
to live.
Cushing's did NOT win for now you are free.
Thursday, April 3, 2014
Cushing's Tumors
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.
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.
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!
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