Monday, May 26, 2014
So you think you have Cushing's? Here's what to do...
A link with suggestions from people who've been there, done that...and who really do know their biznatch: Cushiwiki This will link you to page two of a three page game plan. Buckle down; it's going to be a bumpy ride but you CAN make it to the other side!
Tuesday, May 6, 2014
Brain Atrophy in Cushing's Disease (Cognitive and Emotional changes on the brain)
"Brain Atrophy" is a scary term, right? But I do use it. When I say my memory is mush, or I get confused, can't remember things, can't focus, etc...I actually mean it! It doesn't make me feel any better to know you forget names too. ;) I forget things like my own age, social security number, the age of my children (all of them at the same time), can't look from a phone book to the phone and remember a number, etc. And if I get interrupted mid-sentence/story/thought, it's just gone...It won't come back for a good 5-50 minutes, so don't bother asking what I was saying! I can't concentrate well enough to play simple songs on the piano or to even read a "for fun" book sometimes (when I used to play complicated pieces and read medical studies), can't concentrate well enough to feel safe driving (even in "Nowhere, Alaska"), and when I try to look at my options and think things through logically, I miss big, obvious things that someone else has to point out to me. I say the wrong word ALL the time (Like "kitchen" instead of "bathroom" --my poor kids and husband!) and cannot be trusted to order food or make travel reservations alone because I mess things up royally. It is a constant, ongoing problem...and I'm 33, not 66.
So when someone, presumably in an effort to make me feel better, won't allow that I do indeed have a real problem, it is actually irritating and I'll throw that term out there. My body metabolizes bones and muscle, AND it shrinks my brain. I WIN! HA HA HA! Oh....wait. I am glad to be corrected when I make an obvious mistake, but PLEASE do it nicely. It is hard enough living with the staggering effects of this disease all on it's own, I don't need it thrown in my face and laughed at too (Yes, this applies to you too, grammar Nazi's! You know who you are!). Forgive me if I cannot find the humor in it at times; I bet you'd find it hard too. I do my best, but public humiliation shouldn't be expected to be funny to the person being humiliated.
Moving on to the medical side, here are some good excerpts from the article/study linked below:
"Cushing's disease is associated with brain atrophy and cognitive deficits. Excess glucocorticoids cause retraction and simplification of dendrites in the hippocampus, and this morphological change probably accounts for the hippocampal volume loss. Mechanisms by which glucocorticoids affect the brain include decreased neurogenesis and synthesis of neurotrophic factors, impaired glucose utilization, and increased actions of excitatory amino acids. In this review, the timing, pathology, and pathophysiology of the brain atrophy in Cushing's disease are discussed. The correlation of atrophy with cognitive deficits and its reversibility is also reviewed."
It makes real, visible changes in our brains. Isn't THAT a comforting thought! (Can you read my sarcasm?) A friend and I were just discussing whether there was a possibility for this to help in faster diagnosis times. Heaven knows we need that!
"Cognitive impairments are common in patients with Cushing's disease and are correlated with elevated cortisol levels. Starkman and coworkers[22] found cognitive deficits in several domains in patients with Cushing's disease, including verbal intellectual skills, learning, and memory. The largest decline in cognitive function in this study was found in measures of the verbal intelligence quotient and verbal learning and recall. These impairments are consistent with the clinical cognitive complaints reported by patients with Cushing's disease.[24] In contrast to dementia, delirium, and aging, which show increased vulnerability across visuospatial measures, verbal functions are most prominently affected in Cushing's disease. The deficits in verbal intellectual skills suggest involvement of the neocortex, whereas the impairments in verbal learning and recall are consistent with the increasingly accepted view that the hippocampus is especially vulnerable to the effects of glucocorticoids.[17,22,27,29]"
It's not just me! And it is interesting to find that it is different in its presentation for Cushing's than for other causes of mental deficits.
"These data highlight the fact that the effects of glucocorticoids are not limited to the hippocampus-atrophy has been documented in the prefrontal cortex and other cortical areas.[1,12] It is also entirely possible that glucocorticoids affect specific areas of the brain differently. For example, in contrast to the dendritic atrophy observed in the hippocampus and prefrontal cortex, glucocorticoids increase dendritic growth in the amygdala.[12] "
Did you just read that?!? So all those emotional changes that we blame on hormones alone, REALLY also are due to changes in the brain caused by the initial excess of cortisol (and the whole cascade of issues that stems from that --because really, all the hormones get thrown out of balance). Can you think or fee" differently than your brain is wired to think and feel? Recognizing the source could help dampen the effects thoughts and emotions can have on our lives, but we cannot simply chose to not think or feel that way, nor can we be expected to gain mastery over it.
"Primate studies using exogenous glucocorticoids show that hippocampal changes are present within 1 year of glucocorticoid exposure. Because of the often insidious onset of symptoms in Cushing's disease, no specific data on exposure duration and brain atrophy are available. Clinical investigations of exogenous glucocorticoid therapy show an exposure duration of as short as 2 to 6 months before changes in the brain and hippocampus are detected.[5,21] Cerebral cortical atrophy has been reported within 6 months of glucocorticoid exposure, even in children.[21] ...For example, Newcomer and colleagues[15] found deficits in verbal declarative memory after 4 days of cortisol exposure..."
Wow. Seriously? 4 days to 6 months is all that is needed to make obvious changes to the brain. No wonder it has gotten progressively worse over the past ?17? years...but most drastically in the past 3-4. But don't worry, there IS some good news:
"Cerebral atrophy has been shown to be reversible in patients with Cushing's disease.[8] Following resection of adrenocorticotropin hormone-secreting pituitary adenomas, hippocampal formation volume has been shown to increase by as much as 10%.[23] The increase in hippocampal formation volume correlates with the magnitude of decrease in urinary free cortisol. In addition, improvements in memory correlate with decreases in cortisol levels as well as with increases in hippocampal formation volume.[9] Age has been identified as a significant factor that influences the speed of recovery. Younger patients regain and sustain their improvement in cognitive functioning more quickly than older subjects. These findings suggest that at least some of the deleterious effects of prolonged hyper-cortisolemia on cognitive functioning and hippocampal volume are reversible."
So, their findings suggest it can in fact be reversed, at least in part...and that it happens faster with younger patients. I think we knew that anecdotally (that the young recovery from this disease faster, in general, than the old). I'm hoping I hit cure/remission while young enough to recover on the quicker side...of course, I've also read articles stating that length of illness plays a large role in it as well. But honestly, I'll take any improvement I can get and be happy for it!
http://www.medscape.com/viewarticle/566312
So when someone, presumably in an effort to make me feel better, won't allow that I do indeed have a real problem, it is actually irritating and I'll throw that term out there. My body metabolizes bones and muscle, AND it shrinks my brain. I WIN! HA HA HA! Oh....wait. I am glad to be corrected when I make an obvious mistake, but PLEASE do it nicely. It is hard enough living with the staggering effects of this disease all on it's own, I don't need it thrown in my face and laughed at too (Yes, this applies to you too, grammar Nazi's! You know who you are!). Forgive me if I cannot find the humor in it at times; I bet you'd find it hard too. I do my best, but public humiliation shouldn't be expected to be funny to the person being humiliated.
Moving on to the medical side, here are some good excerpts from the article/study linked below:
"Cushing's disease is associated with brain atrophy and cognitive deficits. Excess glucocorticoids cause retraction and simplification of dendrites in the hippocampus, and this morphological change probably accounts for the hippocampal volume loss. Mechanisms by which glucocorticoids affect the brain include decreased neurogenesis and synthesis of neurotrophic factors, impaired glucose utilization, and increased actions of excitatory amino acids. In this review, the timing, pathology, and pathophysiology of the brain atrophy in Cushing's disease are discussed. The correlation of atrophy with cognitive deficits and its reversibility is also reviewed."
It makes real, visible changes in our brains. Isn't THAT a comforting thought! (Can you read my sarcasm?) A friend and I were just discussing whether there was a possibility for this to help in faster diagnosis times. Heaven knows we need that!
"Cognitive impairments are common in patients with Cushing's disease and are correlated with elevated cortisol levels. Starkman and coworkers[22] found cognitive deficits in several domains in patients with Cushing's disease, including verbal intellectual skills, learning, and memory. The largest decline in cognitive function in this study was found in measures of the verbal intelligence quotient and verbal learning and recall. These impairments are consistent with the clinical cognitive complaints reported by patients with Cushing's disease.[24] In contrast to dementia, delirium, and aging, which show increased vulnerability across visuospatial measures, verbal functions are most prominently affected in Cushing's disease. The deficits in verbal intellectual skills suggest involvement of the neocortex, whereas the impairments in verbal learning and recall are consistent with the increasingly accepted view that the hippocampus is especially vulnerable to the effects of glucocorticoids.[17,22,27,29]"
It's not just me! And it is interesting to find that it is different in its presentation for Cushing's than for other causes of mental deficits.
"These data highlight the fact that the effects of glucocorticoids are not limited to the hippocampus-atrophy has been documented in the prefrontal cortex and other cortical areas.[1,12] It is also entirely possible that glucocorticoids affect specific areas of the brain differently. For example, in contrast to the dendritic atrophy observed in the hippocampus and prefrontal cortex, glucocorticoids increase dendritic growth in the amygdala.[12] "
Did you just read that?!? So all those emotional changes that we blame on hormones alone, REALLY also are due to changes in the brain caused by the initial excess of cortisol (and the whole cascade of issues that stems from that --because really, all the hormones get thrown out of balance). Can you think or fee" differently than your brain is wired to think and feel? Recognizing the source could help dampen the effects thoughts and emotions can have on our lives, but we cannot simply chose to not think or feel that way, nor can we be expected to gain mastery over it.
"Primate studies using exogenous glucocorticoids show that hippocampal changes are present within 1 year of glucocorticoid exposure. Because of the often insidious onset of symptoms in Cushing's disease, no specific data on exposure duration and brain atrophy are available. Clinical investigations of exogenous glucocorticoid therapy show an exposure duration of as short as 2 to 6 months before changes in the brain and hippocampus are detected.[5,21] Cerebral cortical atrophy has been reported within 6 months of glucocorticoid exposure, even in children.[21] ...For example, Newcomer and colleagues[15] found deficits in verbal declarative memory after 4 days of cortisol exposure..."
Wow. Seriously? 4 days to 6 months is all that is needed to make obvious changes to the brain. No wonder it has gotten progressively worse over the past ?17? years...but most drastically in the past 3-4. But don't worry, there IS some good news:
"Cerebral atrophy has been shown to be reversible in patients with Cushing's disease.[8] Following resection of adrenocorticotropin hormone-secreting pituitary adenomas, hippocampal formation volume has been shown to increase by as much as 10%.[23] The increase in hippocampal formation volume correlates with the magnitude of decrease in urinary free cortisol. In addition, improvements in memory correlate with decreases in cortisol levels as well as with increases in hippocampal formation volume.[9] Age has been identified as a significant factor that influences the speed of recovery. Younger patients regain and sustain their improvement in cognitive functioning more quickly than older subjects. These findings suggest that at least some of the deleterious effects of prolonged hyper-cortisolemia on cognitive functioning and hippocampal volume are reversible."
So, their findings suggest it can in fact be reversed, at least in part...and that it happens faster with younger patients. I think we knew that anecdotally (that the young recovery from this disease faster, in general, than the old). I'm hoping I hit cure/remission while young enough to recover on the quicker side...of course, I've also read articles stating that length of illness plays a large role in it as well. But honestly, I'll take any improvement I can get and be happy for it!
http://www.medscape.com/viewarticle/566312
Friday, May 2, 2014
How Cushing's has broken my body
I was planning to take a decent break after the whole "post every day in April for Cushing's Awareness Month" thing, but I somehow woke up knowing I needed to get this thought (and information) out there...
When my exhaustion comes up or people are around me a while, I continue to be told/asked things like:
"Have you tried walking?"
"If you did a little extra each day, you'd build muscle."
"Stress hormones are the problem? Well, stop stressing!"
"I know you're tired, but if you don't exercise, you'll lose muscle and gain weight."
"Are you counting calories? Not doing much, no wonder you're gaining weight."
I know these questions stem from ignorance about the disease and some people really are wanting to be helpful. But it really is counter-intuitive, I'm not making that up. And when people feel the need to say these things to me it still hurts my feelings a bit even when I remember that. It just reminds me that they haven't learned the basics about my disease, and it still feels as though they are blaming my symptoms on me again...like I'm a fat, lazy, idiot.
Who doesn't know you exercise to maintain and gain muscle? That you lose it if you don't use it? That eating too much (or incorrectly) causes weight gain? That in healthy people, expending energy in the form of exercise can actually increase your overall energy in the long-run?
Seriously, my body is broken. These "normal" systems just don't work.
Cushing's causes the demineralization of bones. Cushies break bones very easily. Ever heard of someone waking up to a broken ankle with no trauma? Yep. Breaking ribs just rolling over in bed? That too. Imagine that isn't an 80 year old, but a 35 or 45 year old. We develop osteopenia and osteoporosis as part of the disease process, and sometimes that is the first time doctors take notice that something abnormal is going on in our bodies. I have a friend who is paralyzed because of broken vertebrae thanks to this disease. It's horrible. And it isn't his fault.
Now, imagine that your body also metabolizes your own muscles. Really. No amount of exercise can stop that, only curing the disease can...and then you have the long, daunting task of trying to rebuild amidst pain, exhaustion, and weakness. I have a supplement that *might* help SLOW it but cannot stop it. This is why people with my disease tend to have skinny arms and legs. The first muscles to go (dramatically, anyway) are the quads and biceps. It becomes difficult/impossible to walk up stairs, to stand unassisted from a squat or to get up from a seated position without using your arms. And as far as the biceps go, one of the first things people notice is the inability to open lids...that requires your upper arm muscles more than you'd think. As an example, I can't open about every 3rd Gatorade lid these days. It is absurd! Something so simple, and I can't do it. And trust me, I open those bottles multiple times a day, every day, yet I am still losing that ability. (I NEED the salt to raise my BP due to the lack of another hormone, that's a diagnosed need and I will die of dehydration without it, so don't even go there; I've already had that lecture from strangers in Wal-Mart or friends at a dinner table in public.)
At the same time, imagine your disease is CAUSING extreme, uncontrollable weight gain. That's one of the most obvious symptoms --it's not the disease, it's a SYMPTOM! It doesn't matter how many calories you cut or how perfect your diet is, the weight just keeps piling on. At very best you can maintain (not lose), but even that isn't possible for most. It centers around your face (moon facies), your neck (sub-clavicular fat pads, buffalo hump, big chin), and your abdomen. It is both firm on top (almost hard like a pregnancy belly) and soft on the bottom and hanging down (pendulous abdomen). You CAN eat low-carb, low-starch, low-sugar to try to slow the onset of diabetes and weight gain, but you cannot stop what the chemicals in your body are doing. Cortisol is directly involved in blood sugar within the body, and it can be catastrophic when either too high or too low. For example, I eat roughly the same diet. It isn't perfect by ANY means, and I have variations, but in a high I gain between .5-2lbs a day and on that same diet in a low, I lose .25-.5 pounds a day. It isn't my diet, it is my cortisol levels...and the highs are outstripping the lows. I'm in a pattern of slow gain, but it is very disheartening to see.
I mean, we need to use reason here. If you are able to continue to exercise (strenuous is NOT recommended, I was flat-out told not to do that anymore), obviously it is a good idea to maintain as much muscle mass and strength as you can. That said, I do NOT gain more energy if I use what I have. Backwards perhaps, but true. I don't get rejuvenation from sleep either...often waking up feeling as tired as when I went to bed (Cushing's messes up your sleep cycles, if you can get sleep). Obviously it is wise to eat well, but remember most of us are dead broke with all these medical bills (and healthy food DOES cost more) and in my case, I am unable to cook any more. I used to cook from scratch and eat a very clean diet, now even the energy to put a frozen meal in the oven, then remove it and dish it up later is too much most days. We know that eating better is good for our bodies. But, the reality is that being perfect in these things will not stop the disease nor it's effects on our bodies. Even if you mean well, please don't say things that suggest we could stop it if we just tried harder or made a little change ourselves --it is hurtful and even if you don't mean to, pushes blame onto us.
Now, I really need to explain the "broken" thing. Our hormone systems (at least in the pituitary) generally work in feedback loops. Parts in the brain take in information about our body's condition, external environment, emotions, etc. It interprets that information and makes judgments about what our body needs more or less of and sends the appropriate signals to other parts of the body (like the pituitary). The pituitary then sends out stimulating hormones (in the case of Cushing's, ACTH - Adrenocorticotropic Hormone) that tell other glands to produce their various hormones for use. Again, in the case of Cushing's, ACTH stimulates Cortisol specifically. When there is sufficient cortisol in the body, it "feeds back" to the brain and pituitary that there is sufficient and the production of ACTH stops/drops.
With Cushing's, that loop is broken. Really broken. There is a tumor getting in the way and throwing everything off. The tumor produces ACTH on it's own, stimulating cortisol production by the adrenals. That in turn shuts down the pituitary from producing it, because there is already enough or too much. It means that our bodies don't react normally to internal nor external stressors. We have FAR too much cortisol at night, when it should be almost non-existent in the body to allow for sleep. We can have low cortisol during the day when we need it to be active. We can be "revved up" like we're running a marathon while sitting on a chair, resting. But it also means that when we are stressed, ill (just a cold, even), in pain, or exerting extra energy, our body does not have the feedback to produce more cortisol to allow us to function appropriately.
This is why even in a high cortisol day, if I overdo it, I can crash so hard I can't keep my eyes open. Why I can become so cold that blankets, warm clothes and a wood stove (on an already warm day) cannot warm me up. Why I can become nauseated (another low symptom) and that can lead to serious danger (Look up adrenal crisis). You see, if I use up whatever my tumor is producing, generally speaking, my pituitary doesn't fill in the gap because it is suppressed (asleep). It doesn't say "She's doing too much, let's produce more hormones so she can get through it." I've noticed this happen in cortisol highs and lows. Generally I'm FAR more careful in a low because I already know my body cannot handle much of anything, but there were times when I would push myself in a high to get a job done, and then I would crash and burn. It was bad news and I didn't understand how dangerous it was at the time (okay, I still do it sometimes, but I REALLY try not to). And sadly, unlike the normal progression of tiring while exerting yourself, it generally didn't come with any warning signs. I was capable, then I wasn't. It was sudden. I cannot tell when I am coming up on that wall, but once I hit it, it is too late. I am ill, not just a little tired. And because sleep only does a little in my Cushing's situation, I don't start fresh tomorrow. Now I'm in the whole...one I might not recover from. This new level of exhaustion at times has become my new "normal." It's bad news, but it's my life. So if I say something as silly as driving in "Nowhere, Alaska" is too stressful, I mean it. It can make me physically ill, and it doesn't mean I mentally stress over it..it is my body recognizing the energy drain. I cannot lose any more of what I currently have...there just isn't enough left.
I know this sounds so negative and depressing. It is. It wasn't my intent to end that way, but the reality is, this disease hurts us so much already, we don't need misunderstanding (even misguided desires to help) causing us more. And seriously, just ask! We would love nothing more than to educate others about what we're going through. We know it is so convoluted and overwhelming and that you can only handle little tidbits at a time, but any understanding is better than none. It can be so hard for us not to do those things we used to, especially things we consider necessary, simple, or things we love and enjoy, and it helps to have someone understand that and not pressure us into doing more than we should or judging us for not reacting like we used to. We still want to be friends and would love nothing more than to help you or go do those fun things with you...we just know our limits. Stick by us and please don't take it personally.
When my exhaustion comes up or people are around me a while, I continue to be told/asked things like:
"Have you tried walking?"
"If you did a little extra each day, you'd build muscle."
"Stress hormones are the problem? Well, stop stressing!"
"I know you're tired, but if you don't exercise, you'll lose muscle and gain weight."
"Are you counting calories? Not doing much, no wonder you're gaining weight."
I know these questions stem from ignorance about the disease and some people really are wanting to be helpful. But it really is counter-intuitive, I'm not making that up. And when people feel the need to say these things to me it still hurts my feelings a bit even when I remember that. It just reminds me that they haven't learned the basics about my disease, and it still feels as though they are blaming my symptoms on me again...like I'm a fat, lazy, idiot.
Who doesn't know you exercise to maintain and gain muscle? That you lose it if you don't use it? That eating too much (or incorrectly) causes weight gain? That in healthy people, expending energy in the form of exercise can actually increase your overall energy in the long-run?
Seriously, my body is broken. These "normal" systems just don't work.
Cushing's causes the demineralization of bones. Cushies break bones very easily. Ever heard of someone waking up to a broken ankle with no trauma? Yep. Breaking ribs just rolling over in bed? That too. Imagine that isn't an 80 year old, but a 35 or 45 year old. We develop osteopenia and osteoporosis as part of the disease process, and sometimes that is the first time doctors take notice that something abnormal is going on in our bodies. I have a friend who is paralyzed because of broken vertebrae thanks to this disease. It's horrible. And it isn't his fault.
Now, imagine that your body also metabolizes your own muscles. Really. No amount of exercise can stop that, only curing the disease can...and then you have the long, daunting task of trying to rebuild amidst pain, exhaustion, and weakness. I have a supplement that *might* help SLOW it but cannot stop it. This is why people with my disease tend to have skinny arms and legs. The first muscles to go (dramatically, anyway) are the quads and biceps. It becomes difficult/impossible to walk up stairs, to stand unassisted from a squat or to get up from a seated position without using your arms. And as far as the biceps go, one of the first things people notice is the inability to open lids...that requires your upper arm muscles more than you'd think. As an example, I can't open about every 3rd Gatorade lid these days. It is absurd! Something so simple, and I can't do it. And trust me, I open those bottles multiple times a day, every day, yet I am still losing that ability. (I NEED the salt to raise my BP due to the lack of another hormone, that's a diagnosed need and I will die of dehydration without it, so don't even go there; I've already had that lecture from strangers in Wal-Mart or friends at a dinner table in public.)
At the same time, imagine your disease is CAUSING extreme, uncontrollable weight gain. That's one of the most obvious symptoms --it's not the disease, it's a SYMPTOM! It doesn't matter how many calories you cut or how perfect your diet is, the weight just keeps piling on. At very best you can maintain (not lose), but even that isn't possible for most. It centers around your face (moon facies), your neck (sub-clavicular fat pads, buffalo hump, big chin), and your abdomen. It is both firm on top (almost hard like a pregnancy belly) and soft on the bottom and hanging down (pendulous abdomen). You CAN eat low-carb, low-starch, low-sugar to try to slow the onset of diabetes and weight gain, but you cannot stop what the chemicals in your body are doing. Cortisol is directly involved in blood sugar within the body, and it can be catastrophic when either too high or too low. For example, I eat roughly the same diet. It isn't perfect by ANY means, and I have variations, but in a high I gain between .5-2lbs a day and on that same diet in a low, I lose .25-.5 pounds a day. It isn't my diet, it is my cortisol levels...and the highs are outstripping the lows. I'm in a pattern of slow gain, but it is very disheartening to see.
I mean, we need to use reason here. If you are able to continue to exercise (strenuous is NOT recommended, I was flat-out told not to do that anymore), obviously it is a good idea to maintain as much muscle mass and strength as you can. That said, I do NOT gain more energy if I use what I have. Backwards perhaps, but true. I don't get rejuvenation from sleep either...often waking up feeling as tired as when I went to bed (Cushing's messes up your sleep cycles, if you can get sleep). Obviously it is wise to eat well, but remember most of us are dead broke with all these medical bills (and healthy food DOES cost more) and in my case, I am unable to cook any more. I used to cook from scratch and eat a very clean diet, now even the energy to put a frozen meal in the oven, then remove it and dish it up later is too much most days. We know that eating better is good for our bodies. But, the reality is that being perfect in these things will not stop the disease nor it's effects on our bodies. Even if you mean well, please don't say things that suggest we could stop it if we just tried harder or made a little change ourselves --it is hurtful and even if you don't mean to, pushes blame onto us.
Now, I really need to explain the "broken" thing. Our hormone systems (at least in the pituitary) generally work in feedback loops. Parts in the brain take in information about our body's condition, external environment, emotions, etc. It interprets that information and makes judgments about what our body needs more or less of and sends the appropriate signals to other parts of the body (like the pituitary). The pituitary then sends out stimulating hormones (in the case of Cushing's, ACTH - Adrenocorticotropic Hormone) that tell other glands to produce their various hormones for use. Again, in the case of Cushing's, ACTH stimulates Cortisol specifically. When there is sufficient cortisol in the body, it "feeds back" to the brain and pituitary that there is sufficient and the production of ACTH stops/drops.
With Cushing's, that loop is broken. Really broken. There is a tumor getting in the way and throwing everything off. The tumor produces ACTH on it's own, stimulating cortisol production by the adrenals. That in turn shuts down the pituitary from producing it, because there is already enough or too much. It means that our bodies don't react normally to internal nor external stressors. We have FAR too much cortisol at night, when it should be almost non-existent in the body to allow for sleep. We can have low cortisol during the day when we need it to be active. We can be "revved up" like we're running a marathon while sitting on a chair, resting. But it also means that when we are stressed, ill (just a cold, even), in pain, or exerting extra energy, our body does not have the feedback to produce more cortisol to allow us to function appropriately.
This is why even in a high cortisol day, if I overdo it, I can crash so hard I can't keep my eyes open. Why I can become so cold that blankets, warm clothes and a wood stove (on an already warm day) cannot warm me up. Why I can become nauseated (another low symptom) and that can lead to serious danger (Look up adrenal crisis). You see, if I use up whatever my tumor is producing, generally speaking, my pituitary doesn't fill in the gap because it is suppressed (asleep). It doesn't say "She's doing too much, let's produce more hormones so she can get through it." I've noticed this happen in cortisol highs and lows. Generally I'm FAR more careful in a low because I already know my body cannot handle much of anything, but there were times when I would push myself in a high to get a job done, and then I would crash and burn. It was bad news and I didn't understand how dangerous it was at the time (okay, I still do it sometimes, but I REALLY try not to). And sadly, unlike the normal progression of tiring while exerting yourself, it generally didn't come with any warning signs. I was capable, then I wasn't. It was sudden. I cannot tell when I am coming up on that wall, but once I hit it, it is too late. I am ill, not just a little tired. And because sleep only does a little in my Cushing's situation, I don't start fresh tomorrow. Now I'm in the whole...one I might not recover from. This new level of exhaustion at times has become my new "normal." It's bad news, but it's my life. So if I say something as silly as driving in "Nowhere, Alaska" is too stressful, I mean it. It can make me physically ill, and it doesn't mean I mentally stress over it..it is my body recognizing the energy drain. I cannot lose any more of what I currently have...there just isn't enough left.
I know this sounds so negative and depressing. It is. It wasn't my intent to end that way, but the reality is, this disease hurts us so much already, we don't need misunderstanding (even misguided desires to help) causing us more. And seriously, just ask! We would love nothing more than to educate others about what we're going through. We know it is so convoluted and overwhelming and that you can only handle little tidbits at a time, but any understanding is better than none. It can be so hard for us not to do those things we used to, especially things we consider necessary, simple, or things we love and enjoy, and it helps to have someone understand that and not pressure us into doing more than we should or judging us for not reacting like we used to. We still want to be friends and would love nothing more than to help you or go do those fun things with you...we just know our limits. Stick by us and please don't take it personally.
Wednesday, April 30, 2014
The Emotional Side of the Magic Convention
I decided that I'd separate off the emotional information and experiences from the more technical ones of yesterday's post. I'll start by sharing some notes I took as Catherine Jonas, LMFT, CMC from The Pituitary Center at Cedars-Sinai Medical Center spoke on "Tools for Coping with my Pituitary Disorder."
I have to say, despite all the stress and exertion it took for me to get to the convention (I had a lot of travelling on my own, plus an MRI and doctor's appointment) and the heat and humidity that made me feel ill while there, I am BEYOND happy that I went. I plan to NEVER travel alone again, maybe for life (ha ha) because of how sick I felt the first few days, BUT it was worth it all. I cannot truly explain how it felt to be there. I had a decent understanding of my disease and the endocrine system (not perfect, just decent) before I went. I did learn a lot of valuable information, and it was so validating to hear it spoken by national experts from large, recognized hospitals and institutions. Still, what I most cherish from my time at the Magic Convention is the time I had with others like me. They are "my people" as my friend JennyZ would say. I cannot express the immediate bond that I felt with those fighting this disease just like me; we really are like family. I felt safe and comfortable with them and that is a HUGE deal. I didn't have to put on pretenses, deal with dirty looks or judgment, didn't have to pretend I had more energy or was happier than I really was, didn't have to stand up for myself and explain my illness...They just got it. I've met my best friends on the internet via Cushing's support groups --and they are a very, very real part of my life. Now I've been able to meet some of them in person, and I pray they will forever stay part of my family. I could talk about this for hours and still not get across the immense feeling of acceptance, friendship, companionship, even relief that I felt there this weekend. I am not alone.
Some funny tid-bits from the convention to shed a little light on what I mean:
- Coping Mechanisms:Fighting SpiritAvoidance/DenialFatalismHelplessness/HopelessnessAnxious Preoccupation
- Most of us fluctuate between these various coping mechanisms, and they can all be useful and worthwhile to get us through a hard situation as long as we don't get stuck in one long-term.
- Other good methods of coping are: problem solving, decision making, seeking info and setting goals.
- Remember to engage in pleasant activities --things that make you happy or bring you joy. Not everything should be about your disease and limitations.
- We often deny ourselves positive experiences and happiness by almost-instantaneous irrational thought processes. It is good to recognize them for what they are so that we do not isolate ourselves and limit our chances for support and enjoyment.
- How to differentiate between rational and irrational thinking:1. Is it based in fact?2. Is it/does it bring an emotion I want?3. Does it help me reach my goals?
I have to say, despite all the stress and exertion it took for me to get to the convention (I had a lot of travelling on my own, plus an MRI and doctor's appointment) and the heat and humidity that made me feel ill while there, I am BEYOND happy that I went. I plan to NEVER travel alone again, maybe for life (ha ha) because of how sick I felt the first few days, BUT it was worth it all. I cannot truly explain how it felt to be there. I had a decent understanding of my disease and the endocrine system (not perfect, just decent) before I went. I did learn a lot of valuable information, and it was so validating to hear it spoken by national experts from large, recognized hospitals and institutions. Still, what I most cherish from my time at the Magic Convention is the time I had with others like me. They are "my people" as my friend JennyZ would say. I cannot express the immediate bond that I felt with those fighting this disease just like me; we really are like family. I felt safe and comfortable with them and that is a HUGE deal. I didn't have to put on pretenses, deal with dirty looks or judgment, didn't have to pretend I had more energy or was happier than I really was, didn't have to stand up for myself and explain my illness...They just got it. I've met my best friends on the internet via Cushing's support groups --and they are a very, very real part of my life. Now I've been able to meet some of them in person, and I pray they will forever stay part of my family. I could talk about this for hours and still not get across the immense feeling of acceptance, friendship, companionship, even relief that I felt there this weekend. I am not alone.
Some funny tid-bits from the convention to shed a little light on what I mean:
- The convention was changed from 2 full days of presentations to 3 roughly half days of presentations because it was too exhausting for us patients in attendance...That tells you something right there!
- It was COMPLETELY normal and acceptable to go take a nap between lunch and dinner. It was NOT considered being antisocial or lazy, but "necessary" to be able to get up for dinner and function the following day.
- Every day (often multiple times a day), we would have to re-ask others' names. It wasn't considered rude --EVERYONE did it. And yes, we wore name tags. LOL!
Tuesday, April 29, 2014
Trip and Information/Quotes from Magic --READ THIS!
Wow, where do I start? I'm home again, and I SO need to rest for a few days (what's new, right? lol). The trip went well, even though I was over-exerting myself in hot weather with little sleep. OUCH. But, it was so worth it! I'm just not going to travel alone again...maybe ever! ;)
So, the big updates are that I had my post-op MRI and follow up appointment (though it did NOT go as planned). My MRI looks better post-op than it did before surgery (good news, right?!) I went from having what looked like a post-op MRI (chunk missing here, blob added there) to having what looks like a normal MRI (perfect little "u" shape). There is no surgical target on my MRI, which means unless something changes (ie, grows) between now and the time I'm finished with my re-diagnosis testing, I am not a candidate for further pit surgery nor either form of radiation therapy. With those MRI findings plus the thrice-repeated "hyperplasia" (in different forms/ways) on my pathology report, Dr. McC says bilateral adrenalectomy (BLA, removal of both adrenal glands) is the next course of treatment for me. I still need a great deal more testing before I have some life-giving glands permanently removed from my body, but now we know where we're headed, how many tests I'll need, and which surgeons to look into. So far I'm considering a surgeon close to home at Virginia Mason in Seattle or a more experienced (in BLA's specifically) surgeon in Wisconsin. It'll be a big change, with my life literally being dependent on the intake of medication throughout the day for survival and the constant risk of adrenal crisis, basically Addison's disease without ANY cushion of poorly-functioning adrenals (cause I won't have any!), but it should bring a lasting cure from Cushing's disease and even with the illness I'll be trading it for, my quality of life should improve dramatically.
On to MAGIC: How on earth do I sum up 3 days of learning, emotions, and fun!? I already had a basic understanding of the pituitary gland's feedback systems, hormones and functions, along with a decent understanding of my disease and it's treatments, but I did learn some new things along with some nuances and interesting points, for sure. I'll go through my notes and share what I learned and found most interesting.
Some great quotes from this weekend:
So, the big updates are that I had my post-op MRI and follow up appointment (though it did NOT go as planned). My MRI looks better post-op than it did before surgery (good news, right?!) I went from having what looked like a post-op MRI (chunk missing here, blob added there) to having what looks like a normal MRI (perfect little "u" shape). There is no surgical target on my MRI, which means unless something changes (ie, grows) between now and the time I'm finished with my re-diagnosis testing, I am not a candidate for further pit surgery nor either form of radiation therapy. With those MRI findings plus the thrice-repeated "hyperplasia" (in different forms/ways) on my pathology report, Dr. McC says bilateral adrenalectomy (BLA, removal of both adrenal glands) is the next course of treatment for me. I still need a great deal more testing before I have some life-giving glands permanently removed from my body, but now we know where we're headed, how many tests I'll need, and which surgeons to look into. So far I'm considering a surgeon close to home at Virginia Mason in Seattle or a more experienced (in BLA's specifically) surgeon in Wisconsin. It'll be a big change, with my life literally being dependent on the intake of medication throughout the day for survival and the constant risk of adrenal crisis, basically Addison's disease without ANY cushion of poorly-functioning adrenals (cause I won't have any!), but it should bring a lasting cure from Cushing's disease and even with the illness I'll be trading it for, my quality of life should improve dramatically.
On to MAGIC: How on earth do I sum up 3 days of learning, emotions, and fun!? I already had a basic understanding of the pituitary gland's feedback systems, hormones and functions, along with a decent understanding of my disease and it's treatments, but I did learn some new things along with some nuances and interesting points, for sure. I'll go through my notes and share what I learned and found most interesting.
- I learned that 15-30 minutes was NOT enough time for even 3 people to share their Cushing's stories.
- In endogenous Cushing's Syndrome (meaning, coming from within the body, not from taking medications), 70% is Cushing's Disease (pituitary tumor), 10% is ectopic, 15% is adrenal, and 5% are "other" --don't ask me?
- 50% of pathology-proven Cushing's Disease cases (ie, they have surgery and the tumor is found and tests positive as an ACTH producing adenoma) were not seen on MRI prior to surgery.
- Adrenals can become hypertrophied from long-term ACTH-driven Cushing's Disease...meaning, they've been forced to produce so much for so long that they kind of just kick into overdrive regardless of the current stimulating hormone production (ie, even after a tumor is removed from the pituitary, your adrenals might not slow down), though it isn't known how common/rare this is.
- Cardiovascular damage is the biggest concern, with 4 times the mortality rate in Cushing's Syndrome compared to the national average.
- Cushing's is really more like a state of Adrenal Insufficiency with Hypercortisolism superimposed upon it. IE, the pituitary's (and post-op, your adrenals') own ACTH/cortisol production is suppressed because of the tumor = adrenal insufficiency/Addison's disease. The tumor is causing excess production of ACTH/cortisol (ie, Cushing's) = hypercortisolism. So when the source of hypercortisolism is successfully removed/treated, your body is left in a state of adrenal insufficiency, requiring steroid replacement therapy and a slow weaning process to "wake" your pituitary and adrenals in the hopes of returning full function.
- It can take up to 5 years for your adrenals to fully wake up.
- Incidence (new cases) .7-2.4/million cases per year in Europe, looks to be higher in the US.
- Prevalence (number of cases, old or new, at any given time) is roughly 60-100/100,000.
- Some co-morbidities (couldn't take notes fast enough to get the whole list): carotid atherosclerosis, coronary artery disease, dyslipidemia, depression, hypertension, diabetes, obesity, etc.
- The pituitary is made from neuro-endocrine tissue with similar receptors.
- Diagnosis is a 3-step process. The first step is ALL-IMPORTANT, because getting the first step wrong can lead to falsely-concordant results in steps 2 and 3.
Step 1: Suspect Cushing's Syndrome without exogenous glucocorticoid use (steroid-based medication). Test for high cortisol, exclude other physiological causes = Cushing's Syndrome diagnosis.Step 2: Confirm high ACTH (helps differentiate causes of Cushing's Syndrome --ie, ectopic, adrenal, pituitary)
Step 3: Establish pituitary source of ACTH via MRI or IPSS = "Cushing's Disease" diagnosis.
- Cushing's diagnosis is like a justice scale -- you add weights for and against the diagnosis based on severity and likelihood. Some tests and symptoms carry more weight than others.
- UFC's that are 3+ times the normal limit are considered "pathogenic", ie, nothing else can really cause levels that high. Having results below that limit does not exclude Cushing's Syndrome, it just means further testing/investigation to be sure there are no other causes.
- Few people have truly cyclic disease, which is easily predictable in it's pattern of high and low/normal cortisol production, and a more preferable term might be "variable" disease or hypercortisolism, because it varies from person to person and day/week/month/year within the same person.
- ACTH has a 15 minute half-life, so samples can become degraded easily.
- Pituitary MRI contrast shows differences in blood flow and tissue density. ACTH-producing adenomas are very similar in tissue density and blood flow to that of healthy pituitary tissue, though not identical, so on a pre- and post-contrast MRI, pituitary adenomas are very, VERY easily missed. The biggest difference (often the only difference) is visible with "Dynamic" MRI protocols, as the contrast uptake (absorption of the dye) in adenoma's is just slightly delayed in comparison to that of normal pituitary tissues. So as the pituitary absorbs the dye and whitens, there might remain a slightly darker grey area (tumor tissue) for a few moments. If the MRI scan is not performed during that "uptake" time, it will eventually also absorb the dye so as to often look indistinguishable from the gland itself.
- There is a 10-35% chance of surgery failure (meaning surgery doesn't lead to remission).
- 5 year recurrence rates in those who did receive surgical remission are as high as 25%.
- 10 year recurrence rates are as high as 44%
- It is said the longer you follow a patient in remission, the higher the rates of recurrence will go.
- Repeat pituitary surgeries (after the first whether successful or not) have a less than 50% remission rate, along with increased incidence of CSF leaks (cerebrospinal fluid) and loss of pituitary hormone production (hypo-pituitarism). 3rd + surgeries decrease/increase those odds, respectively.
- Radiotherapy (Radiation) has a mean reported remission time of 3 years.
- Radiotherapy leads to hypo-pituitarism in 50-60% of cases, with a risk of damage to surrounding structures.
- Bilateral Adrenalectomy (BLA, removal of both adrenal glands laparoscopically) has a risk of Nelson's Syndrome, corticoid deficiencies (Adrenal insufficiency/crisis), higher rate of ER trips.
- BLA-ers have a Quality Of Life (QOL) level that is VERY closely matched to that of Diabetes patients in terms of lifelong follow-up, testing, medications, etc. (As compared to Cushing's Disease which has a QOL lower than that of Cancer patients, with an average 5 year mortality rate untreated.)
- On Korlym, 60% of diabetic patients had a greater than or equal to 25% reduction in their glucose curve, and a 38% reduction in hypertension. Side affects of nausea, fatigue, low potassium, endometrial issues, etc...(Couldn't type fast enough again!)
- On Ketoconazole, roughly 40-50% had normalization of Urinary Free Cortisol labs (UFC's), though 19% of those still had no change in symptoms. Keto shuts off the production of cortisol (synthesis), though hopefully only partly! Side affects: nausea, diarrhea, low cortisol, increased liver enzymes, etc.
- On Cabergoline (used more often for prolactinomas than ACTH-producing adenomas), in studies with 12-30 patients there was UFC normalization in 25-36%, with a small increase in usefulness seen over time. (Works with Dopamine receptors) Side affects of GI issues and dizziness, etc.
- Signifor works on somatostatin receptors specific to pituitary ACTH tumors and not healthy pituitary tissue, so it allows your pituitary to "wake up" and function, though there are similar receptors found in the GI tract (diarrhea, etc as side effect) and pancreas. It causes diabetes because of it's affects on the pancreas, but that is easily treated with insulin and quickly stabilizes (doesn't continue to worsen). 29% initially mildly elevated LFT's that usually resolves itself. Side affects are basically those of steroid withdrawal. In one patient it did cause QT prolongation (heart rhythm changes), so monitoring is necessary to be sure you aren't that "one."
- Increased IGF-1 is seen in Acromegaly.
- Only 15 pituitary surgeries are required to complete a residency and become a Neurosurgeon. It takes MANY more than that to become proficient, and statistics are poor below 200 total.
- Cushing's tumors are often VERY different than other types of pituitary tumors (gooey, milky, etc) and surgeons inexperienced in Cushing's-Specific pituitary surgeries may miss the tumorous tissue completely, or even "scramble it like an egg" thus spreading the tumor tissue unknowingly. It is VERY important to see a Cushing's-experienced surgeon.
- GPS mapping can be used to locate tumors in the posterior-pituitary region during surgery (an instance in which the tumor isn't visible upon opening for surgery).
- Endoscopic dopplers (ultrasound on the end of what looks like a bent, stainless steel chopstick) can be used to locate the carotid arteries before and during removal of the sella turcica bones, dura, etc.
- The carotids sit as "goal posts" on either side of the pituitary gland with the optic nerves above.
- Cushing's patients CAN get pregnant.
- BLA/Addison's is not a reason to deny yourself a family (ie, pregnancy can be quite manageable)
Some great quotes from this weekend:
- "This is about the messiest disease out there." -Dr. Ludlam, Director of "all things pituitary" at Novartis Pharmaceuticals, and previous Chief of Endocrinology at Swedish in Seattle, known to many as the director of "Camp Cushie".
- "I've worked as a patient advocate for many different diseases before...Cancer, etc. You guys have it so much worse. I didn't hate doctors till I saw what you go through; their mindset and biases make it so much harder for you!" -Cushing's Patient Advocate
- "The Alamo has come to symbolize fighting a battle with impossible odds...which some of you do on a daily basis." -Dr. Heaney, Professor of Endocrinology, UCLA
- In speaking about post-op weaning from replacement medications and how awful it makes you feel, "You have to pay that piper." -Dr. Ludlam
- "My mother died of Cancer...and when she was diagnosed she said to me, 'At least I don't have Cushing's.' I'd talked to her about my work...She got it." Paraphrased from Kate, A Cushing's Support Advocate (Nurse) for Corcept Pharmaceuticals.
- "I was not ready to do pituitary surgery when I became a neurosurgeon." -Dr. Kelley,
Neurosurgeon at UCLA, paraphrased from him speaking of the requirements to graduate from residency and the amount really required to become proficient. Kudos to him for stating that the requirements (cases seen) are far too few, and that much more experience is required to become skilled...which he since has, to many patient's appreciation! **UPDATE - Dr. Daniel F Kelly is now the Director of the Brain Tumor Center at John Wayne Cancer Institute. drdanielfkelly@gmail.com ** - "I've had other doctor's tell me that if they had to diagnose and treat Cushing's all day, they'd quit [or shoot themselves]!" -Dr. Ludlam, said jovially about the confusion and long process that is the diagnosis and treatment of Cushing's disease.
- "I haven't seen people suffer so much and be so misunderstood." Kate, Cushing's Patient Advocate
Monday, April 28, 2014
Home, here I come!
Well, it is sure to have been an amazing, exhilarating, educating and enlightening trip...but I will also undoubtedly be exhausted and glad to be home again. Today, I should be flying from Seattle home. PHEW! I'll be checking in tomorrow with updates as to what I've learned and how everything went for the last two days' posts of the Cushing's Awareness Blogger's Challenge. I'm so glad I did it this year even though I haven't always had the energy to go as in-depth and get as down and dirty as I'd have liked (yes, even writing takes a toll on my energy, even though it is very slight --it is more the emotions it drags up). On those days when I just couldn't, I relied on the strength of others to get me through (ie, linking to their posts and previous posts of mine!).
Sunday, April 27, 2014
Magic Convention 2014, Day 3
Hormone Replacement for the Addison’s Patient: Anthony P. Heaney, M.D., Ph.D.Monitoring your medications requires a delicate balance of hormonal treatments. The segment will discuss the importance of lifelong management of your medications and healthcare.
Life after Bilateral Adrenalectomy - A Patient’s Perspective: Philip ButlerA patient’s journey from onset of symptoms, diagnosis of Cushing's disease and treatments leading to bilateral adrenalectomy. Why it is important to research and fight for your life.
Life after Bilateral Adrenalectomy - A Patient’s Perspective: Philip ButlerA patient’s journey from onset of symptoms, diagnosis of Cushing's disease and treatments leading to bilateral adrenalectomy. Why it is important to research and fight for your life.
Cushings: How to stay connected and informed, Facilitated by Stacey Seitz: The last segment of the program will be an open discussion on how, as a Cushing's community, we can impact the lives of others dealing with Cushing's.
AGHD/Hypopit: How to stay connected and informed, Facilitated by Ann Rumrill:
The last segment of the program will be an open discussion on how to find the support and information we may need to manage our pituitary disorders.
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