Last fall I wrote the monologue "A Big, Fat Target" based loosely off of a past blog post. After many revisions (and with the help of some friends), it was performed readers-theater-style by Elizabeth Nelson of First City Players in February of 2015. I'm so grateful for all who helped, and especially Elizabeth for adding her unique style to make it come to life. Enjoy!
Thursday, April 2, 2015
A Big, Fat Target
Last fall I wrote the monologue "A Big, Fat Target" based loosely off of a past blog post. After many revisions (and with the help of some friends), it was performed readers-theater-style by Elizabeth Nelson of First City Players in February of 2015. I'm so grateful for all who helped, and especially Elizabeth for adding her unique style to make it come to life. Enjoy!
Wednesday, April 1, 2015
APRIL FOOLS! YOU'RE STILL SICK! Enjoy That Tight Rope You're Walking...
Well, once again it is April, which is Cushing's Awareness month. I have again decided to participate in the blogger's challenge, posting each day this month to raise awareness for Cushing's. I've been mulling over what to say and how to start, with no inspiration or direction coming readily to mind. And, as it turns out, a topic presented itself today, just in time.
I received a phone call from someone I haven't spoken to for probably most of a year. She obviously didn't understand that I am still very sick, and she didn't seem to understand that my husband has to take up all my slack, working full time (soon to include lots of overtime and unpredictable hours), caring for our home, cars, cleaning, cooking, doing things with our 6 kids, shopping, etc, etc.
So here's the deal: I am very ill. I'm STILL sick. I'm still in recovery. I still can pretty much only function enough to supervise kids and make sure they do their school work, chores, don't fight, etc.
For the time-being, it appears my Cushing's Disease is gone. I still have many years of recovering from that ahead of me. BUT, in it's place I now have empty sella and hypopituitarism. Basically about 9 months after my brain surgery, a follow-up MRI showed a new development that wasn't seen on my 2-3 month post-op MRI. I now have spinal fluid pushing downward from around my brain onto my pituitary and turning what should look like a rounded bridge into a smushed U shape. This isn't uncommon after pituitary surgery, and it sometimes doesn't cause problems, but it can kill off pituitary cells, cut off blood supply, etc. There is a chance this is the cause for my Cushing's symptoms finally disappearing 9-12 months post-op. After doing extensive testing that required a trip out-of-state, we know it has killed off the cells in my pituitary that produce Growth Hormone. I am no longer able to produce any at all, even when given injections basically forcing my body to produce as much as it can. I'll trade Adult Growth Hormone Deficiency (AGHD) for Cushing's Disease (CD) gladly, but it isn't simple. And, GH is usually the first hormone whose production is affected following pituitary insult (from a turmor itself, surgery, or apoplexy, etc) so we have to watch and wait to see if this is the start of a cascade. I pray my pituitary is able to keep the rest of it's function, but only time and tests and symptoms will tell.
I'm now replacing GH, with an injection every night. It's been hard getting on the medication, and I will have to slowly try to up my dose till my IGF-1 hormone levels are where they need to be. When one hormone level changes, it affects the rest of them, and they all have to re-stabilize. I've had some awful symptoms because of that, having to drop my dose significantly from where I was started. Apparently GH catabolizes cortisol, and what I thought were further symptoms of Intercranial Hypertension (another rare but known side effect of GH replacement, basically a more sudden form of what is causing my empty sella) my endocrinologist thinks were symptoms of my cortisol dropping dangerously low. It took 4 days for the negative side effects to show up, then I cut my dose in half and things improved, but it took another week or two for it to mostly resolve. It felt awful. It's a month later and I'm not back to that starting dose yet, but I'm slowly inching upwards trying to get where I need to be, trying to keep the negative side-effects at bay. With so many hormones in play, all affecting each other, all with similar symptoms, and with so many factors, it is a confusing mess, but I am determined to make it work.
People ask why it is so important I replace this hormone, because the name "Growth Hormone" is a bit of a misnomer. "You're certainly finished growing by now!" It does far more than just allow children to grow, but it is indeed responsible for that in the young, and your body produces it throughout your life at varying levels because it needs it to function properly. I'll perhaps go further in depth on that in a post later this month, but suffice it to say Growth Hormone directly affects your circulation, bones, muscles, metabolism, and ability to think, sleep, move, and function. Having no growth hormone has made me SO much more physically exhausted than Cushing's ever did...and that was no picnic either! There is no other treatment at this time, and I am not willing to simply not recover.
So, my doctors and I are still trying to figure out all the ins and outs of what is wrong with my body, trying to get it stabilized, and waiting to see how and if my body will recover or if I will continue to get sicker (due to the empty sella and hypopituitarism). We hope I don't lose any more hormone production, but even replacing just the GH is a long process with an expected wait of 6 months prior to seeing improvement.
I'm showing signs of insufficient adrenal reserve, but I still have some adrenal hormone production, further complicating things. These hormones are required for us to stay alive. It's a big deal. And adding stressors to my day is a big deal. If I'm already too low, any stress can send me into adrenal crisis.
I already had to stress-dose once this week, on what appeared to be a low/normal stress day for me. What did I do? I stood up from watching a movie with my family, and suddenly I had a headache, nausea, my head was cold and hot, sweaty and clammy, with tinnitus, sudden sleepiness, and a generalized "swirly, I'm going to die" feeling. I took 10 mg of hydrocortisone and within about 20-30 minutes I was out of the woods. It was around 10pm, and yet because I needed that dose of steroids, I was still able to sleep like a baby (that's roughly half to 2/3rds of a normal replacement dose if I were making none of that hormone on my own, and if I didn't need it all, would have kept me wide awake for hours). Had I not taken that medication, I could have gone into shock, followed by coma and death.
I know that sounds dramatic, but it is true. I've had friends simply not wake up from a nap because they went to sleep too low and died. It's heartbreaking and tragic. So, I have to take medication and stay awake (and not alone, because things can get bad quickly) till I transition from feeling like death to just feeling crappy...Till it's safe to go to sleep. And then I have to watch closely in the morning and days that follow that I'm not still working on a deficit and in danger (though I guess that danger never fully goes away).
IF I were to get so bad that the oral dose wasn't enough or couldn't be absorbed (vomiting, diarrhea, unconscious), I would need my emergency injection of 100mg solu-cortef and to be rushed to the ER. Luckily, I've never had to use it. I've always caught it early enough and responded well to the oral medication. I honestly think my inability to move and do much of anything has protected me in that sense. It would be much harder to recognize all those symptoms early (and I'd probably crash far faster) if I were out and about and active. Trust me, I'd rather BE active and functioning, but I can see the blessing in that, at least.
Whew, didn't mean to go there, but that's the reality of what I'm dealing with every day...still. I didn't have AI issues post-op, in fact, my cortisol levels were still too high. But somewhere around September-November 2014, things changed. I'm now almost 14 months post-op, and seem to be going through some of the low cortisol recovery that most people experience as they wean down from replacements over the year following surgery. I weaned off hydrocortisone fully within a month with no issues.
But now any stressor, whether I am emotionally/mentally able to cope with it or not, can be too much for my physical, autonomic response to handle. It is like I have no reserve, or make just barely enough cortisol to stay safe. I've said this before, but even something enjoyable like laughing and chatting with friends can be too much in a low, because it requires energy from my body. And something stressful yet "fake" and enjoyable like a video game (and I'm talking 1980's nintendo here, not even HALO) elicits my body's stress response and can send me into crisis when I'm already deficient. It's CRAZY, but it is real. I WISH I could just take enough steroid every day to not have to worry about it, but it would cause one of two additional problems. It could cause exogenous Cushing's, with the same symptoms of Cushing's Disease I had brain surgery to treat last year, because it would add more cortisol on top of what my body is already producing. Or it would suppress what is left of my adrenal and pituitary function, essentially turning those processes off giving little to no hope of my body being able to produce those hormones itself in the future.
I mean, honestly, I'd rather it either produce enough or none, so it was less confusing, but I'm stuck in no-man's-land. I'm walking a tight rope of trying to keep myself safe, while not taking over my body's own production. And all of this is a "play it by ear" thing. I have to watch my own signs and symptoms and figure it out on my own. It's a mess. It's exhausting. I just want to feel better.
Oh, I guess I should mention I'm also having a hard time regulating my fludrocortisone (another adrenal hormone) while on and increasing my dose of GH. My aldosterone isn't stable, and that makes me feel icky, can cause headaches too when it's too high/low, nausea, loss of appetite, swelling, dehydration, etc. Add to that that the medicine used to treat my post-op Cushing's damaged my liver and I'm eeking towards diabetes, and it's just...overwhelming.
I know everyone expects a quick diagnosis, cure and recovery, but I didn't win that lottery. I know there are many others out there dealing with this same post-op nightmare that I am, even though it isn't spoken about very much. I am still fighting every day just to keep myself alive, all while trying *not to stress* or overdo it. I don't know if, when or how things will get better. I am not depressed, but it IS depressing, and there are days I don't know how I will continue on. I still have hope that things will get better and I pray that day comes, but for now I just keep slowly pushing forward, because for me there is no other option.
As long as I am on the tight rope, I will keep walking. I am not scared of falling off the rope, but I won't knowingly jump off either. I know there is an end, and though it seems impossible now, I will keep trying till I find it.
I received a phone call from someone I haven't spoken to for probably most of a year. She obviously didn't understand that I am still very sick, and she didn't seem to understand that my husband has to take up all my slack, working full time (soon to include lots of overtime and unpredictable hours), caring for our home, cars, cleaning, cooking, doing things with our 6 kids, shopping, etc, etc.
So here's the deal: I am very ill. I'm STILL sick. I'm still in recovery. I still can pretty much only function enough to supervise kids and make sure they do their school work, chores, don't fight, etc.
For the time-being, it appears my Cushing's Disease is gone. I still have many years of recovering from that ahead of me. BUT, in it's place I now have empty sella and hypopituitarism. Basically about 9 months after my brain surgery, a follow-up MRI showed a new development that wasn't seen on my 2-3 month post-op MRI. I now have spinal fluid pushing downward from around my brain onto my pituitary and turning what should look like a rounded bridge into a smushed U shape. This isn't uncommon after pituitary surgery, and it sometimes doesn't cause problems, but it can kill off pituitary cells, cut off blood supply, etc. There is a chance this is the cause for my Cushing's symptoms finally disappearing 9-12 months post-op. After doing extensive testing that required a trip out-of-state, we know it has killed off the cells in my pituitary that produce Growth Hormone. I am no longer able to produce any at all, even when given injections basically forcing my body to produce as much as it can. I'll trade Adult Growth Hormone Deficiency (AGHD) for Cushing's Disease (CD) gladly, but it isn't simple. And, GH is usually the first hormone whose production is affected following pituitary insult (from a turmor itself, surgery, or apoplexy, etc) so we have to watch and wait to see if this is the start of a cascade. I pray my pituitary is able to keep the rest of it's function, but only time and tests and symptoms will tell.
I'm now replacing GH, with an injection every night. It's been hard getting on the medication, and I will have to slowly try to up my dose till my IGF-1 hormone levels are where they need to be. When one hormone level changes, it affects the rest of them, and they all have to re-stabilize. I've had some awful symptoms because of that, having to drop my dose significantly from where I was started. Apparently GH catabolizes cortisol, and what I thought were further symptoms of Intercranial Hypertension (another rare but known side effect of GH replacement, basically a more sudden form of what is causing my empty sella) my endocrinologist thinks were symptoms of my cortisol dropping dangerously low. It took 4 days for the negative side effects to show up, then I cut my dose in half and things improved, but it took another week or two for it to mostly resolve. It felt awful. It's a month later and I'm not back to that starting dose yet, but I'm slowly inching upwards trying to get where I need to be, trying to keep the negative side-effects at bay. With so many hormones in play, all affecting each other, all with similar symptoms, and with so many factors, it is a confusing mess, but I am determined to make it work.
People ask why it is so important I replace this hormone, because the name "Growth Hormone" is a bit of a misnomer. "You're certainly finished growing by now!" It does far more than just allow children to grow, but it is indeed responsible for that in the young, and your body produces it throughout your life at varying levels because it needs it to function properly. I'll perhaps go further in depth on that in a post later this month, but suffice it to say Growth Hormone directly affects your circulation, bones, muscles, metabolism, and ability to think, sleep, move, and function. Having no growth hormone has made me SO much more physically exhausted than Cushing's ever did...and that was no picnic either! There is no other treatment at this time, and I am not willing to simply not recover.
So, my doctors and I are still trying to figure out all the ins and outs of what is wrong with my body, trying to get it stabilized, and waiting to see how and if my body will recover or if I will continue to get sicker (due to the empty sella and hypopituitarism). We hope I don't lose any more hormone production, but even replacing just the GH is a long process with an expected wait of 6 months prior to seeing improvement.
I'm showing signs of insufficient adrenal reserve, but I still have some adrenal hormone production, further complicating things. These hormones are required for us to stay alive. It's a big deal. And adding stressors to my day is a big deal. If I'm already too low, any stress can send me into adrenal crisis.
I already had to stress-dose once this week, on what appeared to be a low/normal stress day for me. What did I do? I stood up from watching a movie with my family, and suddenly I had a headache, nausea, my head was cold and hot, sweaty and clammy, with tinnitus, sudden sleepiness, and a generalized "swirly, I'm going to die" feeling. I took 10 mg of hydrocortisone and within about 20-30 minutes I was out of the woods. It was around 10pm, and yet because I needed that dose of steroids, I was still able to sleep like a baby (that's roughly half to 2/3rds of a normal replacement dose if I were making none of that hormone on my own, and if I didn't need it all, would have kept me wide awake for hours). Had I not taken that medication, I could have gone into shock, followed by coma and death.
I know that sounds dramatic, but it is true. I've had friends simply not wake up from a nap because they went to sleep too low and died. It's heartbreaking and tragic. So, I have to take medication and stay awake (and not alone, because things can get bad quickly) till I transition from feeling like death to just feeling crappy...Till it's safe to go to sleep. And then I have to watch closely in the morning and days that follow that I'm not still working on a deficit and in danger (though I guess that danger never fully goes away).
IF I were to get so bad that the oral dose wasn't enough or couldn't be absorbed (vomiting, diarrhea, unconscious), I would need my emergency injection of 100mg solu-cortef and to be rushed to the ER. Luckily, I've never had to use it. I've always caught it early enough and responded well to the oral medication. I honestly think my inability to move and do much of anything has protected me in that sense. It would be much harder to recognize all those symptoms early (and I'd probably crash far faster) if I were out and about and active. Trust me, I'd rather BE active and functioning, but I can see the blessing in that, at least.
Whew, didn't mean to go there, but that's the reality of what I'm dealing with every day...still. I didn't have AI issues post-op, in fact, my cortisol levels were still too high. But somewhere around September-November 2014, things changed. I'm now almost 14 months post-op, and seem to be going through some of the low cortisol recovery that most people experience as they wean down from replacements over the year following surgery. I weaned off hydrocortisone fully within a month with no issues.
But now any stressor, whether I am emotionally/mentally able to cope with it or not, can be too much for my physical, autonomic response to handle. It is like I have no reserve, or make just barely enough cortisol to stay safe. I've said this before, but even something enjoyable like laughing and chatting with friends can be too much in a low, because it requires energy from my body. And something stressful yet "fake" and enjoyable like a video game (and I'm talking 1980's nintendo here, not even HALO) elicits my body's stress response and can send me into crisis when I'm already deficient. It's CRAZY, but it is real. I WISH I could just take enough steroid every day to not have to worry about it, but it would cause one of two additional problems. It could cause exogenous Cushing's, with the same symptoms of Cushing's Disease I had brain surgery to treat last year, because it would add more cortisol on top of what my body is already producing. Or it would suppress what is left of my adrenal and pituitary function, essentially turning those processes off giving little to no hope of my body being able to produce those hormones itself in the future.
I mean, honestly, I'd rather it either produce enough or none, so it was less confusing, but I'm stuck in no-man's-land. I'm walking a tight rope of trying to keep myself safe, while not taking over my body's own production. And all of this is a "play it by ear" thing. I have to watch my own signs and symptoms and figure it out on my own. It's a mess. It's exhausting. I just want to feel better.
Oh, I guess I should mention I'm also having a hard time regulating my fludrocortisone (another adrenal hormone) while on and increasing my dose of GH. My aldosterone isn't stable, and that makes me feel icky, can cause headaches too when it's too high/low, nausea, loss of appetite, swelling, dehydration, etc. Add to that that the medicine used to treat my post-op Cushing's damaged my liver and I'm eeking towards diabetes, and it's just...overwhelming.
I know everyone expects a quick diagnosis, cure and recovery, but I didn't win that lottery. I know there are many others out there dealing with this same post-op nightmare that I am, even though it isn't spoken about very much. I am still fighting every day just to keep myself alive, all while trying *not to stress* or overdo it. I don't know if, when or how things will get better. I am not depressed, but it IS depressing, and there are days I don't know how I will continue on. I still have hope that things will get better and I pray that day comes, but for now I just keep slowly pushing forward, because for me there is no other option.
As long as I am on the tight rope, I will keep walking. I am not scared of falling off the rope, but I won't knowingly jump off either. I know there is an end, and though it seems impossible now, I will keep trying till I find it.
Sunday, March 29, 2015
Surviving
My youngest turned three today, and I can't help reflecting on the last few years. I've at once come a long way and gotten nowhere.
I'm still just trying to survive day to day. And in my book there is no "not surviving" option, but I'm sick of it. I want to live, to do things I enjoy, to not feel like crap all the time...to thrive. I keep seeing those meme's and they aren't inspiring me, they keep reminding me of how limited my options are.
I don't have to let my disease win and make me feel hopeless, that is true. I do have hope for a better future. But I generally do feel physically miserable most days, I still cannot function physically, and I function on a significantly limited basis mentally.
It's not exactly enjoyable. My "good days" are simply the days I don't feel like I'm dying. I am stuck. And I'm tired. And while there is hope that things will get better for me in the future, the future isn't here yet.
Wednesday, March 11, 2015
Life's a Stage!
Well, I felt inspired to write a monologue for the stage (First stab at that!) based loosely off of this previous post: How Cushing's Has Broken My Body I sent it to some friends last fall, who really liked it and said there was a new performance by our local theater troop, where they were encouraging local writers and filmographers to submit their work. I let it sit a few months, and in February, was asked if I was going to submit it (I had forgotten, honestly, and almost waited too long!). I made some revisions with the help of a few good friends and fellow Cushies, and sent it in. AND, THEY LIKED IT!
It was a bit long and needed a little more editing (not shocking, I've never even acted a monologue out before), but they really wanted to do it. I was asked to perform it, but even though it is my words, I know I wouldn't be able to remember it. I don't know if I'd have the energy to do it physically and emotionally. And considering that I cried every time I read through during editing (seriously, EVERY time), it was clear I'd probably end up bawling through the entire second half...which would kind of ruin the whole point. lol They decided they would do it reader's-theater style, due to the short notice and it's length, and I am thrilled.
This Saturday it will be performed for a live audience and I plan to be in the audience. I'm so excited! But at the same time, I'm fairly nervous. I kind of bare my emotional soul, about the heartache and judgement and difficulty of living with Cushing's Disease, and I don't know how others will respond. I'll let you know...
It was a bit long and needed a little more editing (not shocking, I've never even acted a monologue out before), but they really wanted to do it. I was asked to perform it, but even though it is my words, I know I wouldn't be able to remember it. I don't know if I'd have the energy to do it physically and emotionally. And considering that I cried every time I read through during editing (seriously, EVERY time), it was clear I'd probably end up bawling through the entire second half...which would kind of ruin the whole point. lol They decided they would do it reader's-theater style, due to the short notice and it's length, and I am thrilled.
This Saturday it will be performed for a live audience and I plan to be in the audience. I'm so excited! But at the same time, I'm fairly nervous. I kind of bare my emotional soul, about the heartache and judgement and difficulty of living with Cushing's Disease, and I don't know how others will respond. I'll let you know...
Saturday, February 28, 2015
Rare Disease Day 2015
Today is International Rare Disease Day, and I'd like to take a minute to share some information about the rare diseases I have, Cushing's Disease, Adult Growth Hormone Deficiency, and Hypoaldosteronism.
All three of these diseases are often misdiagnosed as Chronic Fatigue Syndrome, Fibromyalgia, "Adrenal Fatigue", etc...or doctors fail to see that the problems caused are symptoms of disease, not individual, unrelated diseases in and of themselves. For instance: obesity, depression, anxiety, high blood pressure, diabetes, etc.
I'll post some basic symptoms for each condition (not everyone has each symptom), with a link to further information if anything rings true for you or someone you know. And, feel free to have them contact me. Finding information, support, and doctors who know how to test, diagnose, and treat rare conditions can be the hardest part of this battle. While none of these currently have a lasting cure, all can be treated with drastic improvements in quality of life.
Cushing's Disease
Central obesity with thin arms and legs
Round, red, full face (moon face)
Slow growth rate in children
Acne or skin infections
Stretch marks (especially if 1/2 inch or more wide), called striae, on the skin of the abdomen, thighs, and breasts
Thin skin with easy bruising, most commonly on the arms and hands
Buffalo hump (at the back of the neck)
Bone pain or tenderness
Collection of fat between the shoulders (buffalo hump)
Thinning of the bones, which leads to rib and spine fractures
Weak muscles
Excess hair growth on the face, neck, chest, abdomen, and thighs
Menstrual cycle that becomes irregular or stops
Decreased or no desire for sex
Impotence
Mental changes, such as depression, anxiety, or changes in behavior
Difficulty sleeping at night
Fatigue
Headache
Increased thirst and urination
May present with cyclic/episodic "low" episodes of cortisol that can lead to Adrenal Crisis (shock, hypoglycemia, seizure, coma and death)
Round, red, full face (moon face)
Slow growth rate in children
Acne or skin infections
Stretch marks (especially if 1/2 inch or more wide), called striae, on the skin of the abdomen, thighs, and breasts
Thin skin with easy bruising, most commonly on the arms and hands
Buffalo hump (at the back of the neck)
Bone pain or tenderness
Collection of fat between the shoulders (buffalo hump)
Thinning of the bones, which leads to rib and spine fractures
Weak muscles
Excess hair growth on the face, neck, chest, abdomen, and thighs
Menstrual cycle that becomes irregular or stops
Decreased or no desire for sex
Impotence
Mental changes, such as depression, anxiety, or changes in behavior
Difficulty sleeping at night
Fatigue
Headache
Increased thirst and urination
May present with cyclic/episodic "low" episodes of cortisol that can lead to Adrenal Crisis (shock, hypoglycemia, seizure, coma and death)
Adult Growth Hormone Deficiency
Changes in memory, processing speed and attention
Lack of well-being
Depression
Anxiety
Social isolation
Fatigue
Lack of strength
Fibromyalgia syndrome
Neuromuscular dysfunction
Central adiposity
Decreased muscle mass
Decreased bone density
Impaired cardiac function
Decreased insulin sensitivity
Accelerated atherogenesis with increased carotid intima–media thickness
Increased low-density lipoprotein
Prothrombotic state
Decreased sweating and thermoregulation
Lack of well-being
Depression
Anxiety
Social isolation
Fatigue
Lack of strength
Fibromyalgia syndrome
Neuromuscular dysfunction
Central adiposity
Decreased muscle mass
Decreased bone density
Impaired cardiac function
Decreased insulin sensitivity
Accelerated atherogenesis with increased carotid intima–media thickness
Increased low-density lipoprotein
Prothrombotic state
Decreased sweating and thermoregulation
Hypoaldosteronism
High or erratic pulse/palpitations
Low blood pressure
Piercing headache
Dehydration, thirsty
Cold hands and/or feet (possible numbness or tingling)
Shaky hands
Muscle spasms or cramps
Low sodium
Difficulty tasting salt at normal levels, salt craving
Dizziness (especially upon standing)
Weepiness, despair
Fatigue, sleepiness, needing naps
Increased Urination (especially after lying down an hour or two)
High potassium
High renin
Nausea, anorexia
Can lead to adrenal crisis (shock, hyponatremia and hyperkalemia, seizure, coma and death)
Low blood pressure
Piercing headache
Dehydration, thirsty
Cold hands and/or feet (possible numbness or tingling)
Shaky hands
Muscle spasms or cramps
Low sodium
Difficulty tasting salt at normal levels, salt craving
Dizziness (especially upon standing)
Weepiness, despair
Fatigue, sleepiness, needing naps
Increased Urination (especially after lying down an hour or two)
High potassium
High renin
Nausea, anorexia
Can lead to adrenal crisis (shock, hyponatremia and hyperkalemia, seizure, coma and death)
Monday, February 9, 2015
Shannon and the National Organization for Rare Disorders (NORD)
My friend Shannon had surgery to treat Cushing's Disease shortly after I did (about a year ago now!). That is also about the time we "met" online, and we quickly became good friends. We were able to meet in November for the first time "in real life" and it was like we'd known each other for years.
Shannon has been chosen as a patient partner for Genzyme's Boston Marathon fundraising efforts to support NORD - the National Organization for Rare Disorders. Nord provides funding to help patients with rare diseases travel to see specialists and get the testing needed to finally be diagnosed (and thus, get to treatment). When I couldn't get hope locally, it was NORD that paid for my trip to see my Endocrinologist in Los Angeles ("The Wizard" Shannon speaks of), do the months of serial testing for my cyclic disease, .
Shannon used to run Marathons before being struck by this "most morbid of diseases" so it makes this even more bitter-sweet for her, I'm sure. Her blog post, in her own words (link found here):
Getting diagnosed with Cushing’s is a marathon, not a sprintMakes me cry! True words...
by Shannon Seitz
As a former marathoner myself, I see so many parallels between the marathon and getting diagnosed with a rare disease (Cushing’s in particular). Crossing the finish line of the marathon is such a mix of pain, exhaustion and relief. As strange as it sounds, so was getting diagnosed with Cushing’s disease: a big pile of pain, exhaustion and RELIEF.
Before getting sick with Cushing’s, I believed that getting a strange and scary disease went something like this: a purple horn starts growing out of your forehead, so you go see your doctor. Your doctor recognizes the purple horn and immediately runs the standard test for purple horns. The test has a reference range of normal values between 0 and 10 and your test comes back at 1000! Whoa! Immediately, you are diagnosed with Purple Horn Disease and you get shuttled off to a specialist, who quickly hands you a comprehensive treatment plan. Quick and painful, much like a sprint.
Now I know better. I was planning for the wrong race and I was woefully unprepared for what was coming.
Here is what the race actually looks like.
Mile 1. You start getting some vague and annoying symptoms that you (and your doctors) chalk up to hitting your 40s. You start to gain a little weight in your midsection and start getting acne again for the first time since your teens. You also start to get depressed and have trouble sleeping.
Mile 5. Over time, you stop sleeping altogether many nights. Your doctor puts you on an anti-depressant and tells you to exercise more as it will help you sleep and will get rid of the extra weight which is partly what is making you depressed! But the thing is, you can’t seem to run very well anymore. What used to be a warmup run for you now is impossible. Every run is hard and the harder you try to run, the slower you get. Which is depressing!
Mile 10. You have begun a slow downward spiral of having a harder time exercising, gaining weight, and not sleeping. And despite your best efforts to cut more and more calories, to take all sorts of sleeping aids so you can get even a few hours of solid sleep, and forcing yourself to run even when you are walking more than running…you keep getting bigger and slower and more awake at night (and sleepy during the day). You also start avoiding people. You are gaining weight and ashamed of your appearance, you are too tired to anything but flop on the couch when you get home from work. And you have started getting very angry for no reason. Pretty much anything will throw you into a rage, even in public. And this isn’t like you at all, but avoiding people seems wise.
Mile 15. You have now been struggling over well over a year and you are starting to see as many specialists as you can, because this just can’t be a normal part of turning 40. Gaining 50% of your body weight just can’t be normal. And it just can’t be normal for a marathoner to have escalating hypertension. And it isn’t normal to have to sneak home from work to sleep during the day because you are too tired to stay awake for more than 3 hours at a time. But no one can find anything wrong with you. And most of the specialists you see don’t believe anything is wrong with you, because you don’t have a purple horn. All you have is a set of vague symptoms.
Mile 20. The second wind arrives! Finally a cardiologist that is used to working with runners sees you and says “I think you might have Cushing’s!!” And everything is so clear to you. You read the list of symptoms and you have so many of them – hypertension, rapid weight gain, totally wired at night but not during the day, depressed, a “buffalo hump” (collection of fat at the base of the neck) and so many others. You are so happy because finally you are going to get your magical tests and get sent to a specialist and get sorted out. So your cardiologist runs all the right tests…and they come back mixed. Some are slightly positive, some are negative, and the lab didn’t run some of the others correctly so those are no help at all. But you have your second wind and at least have a direction to pursue now.
Miles 22-24. This is the worst part of the race, physically and mentally. You are now sure this is what you have, Cushing’s, so you make an appointment with an endocrinologist because now that you have a diagnosis that fits and at least some tests indicating it could be Cushing’s, you figure it will just be a matter of a few more tests and you will be set to go. Five endocrinologists later, all you have is a bunch of doctors who still don’t think anything is wrong with you. You have a bunch of MRIs of your pituitary too, but the doctors and radiologists don’t even agree on whether there is actually a tumor in your head or not.
Mile 25. You are starting to give up hope. You are so close to the finish, but so far at the same time and you don’t think you are going to make it. But just in time, you manage to find a group of patients on the internet and they turn out to be your saving grace. They have run this race before. Some of them are running it for the second or third time (you want to be a “one and done” marathoner so this isn’t welcome news). And they tell you this is how the Cushing’s marathon course works for everyone. They tell you that tumors like the ones that cause Cushing’s are not always active and that fluctuating labs (and some positive and negative test results) are common. They look at your MRI and tell you that you DO have a tumor (these educated amazing patients look at more MRIs than lots of doctors). They tell you to fly across the country to see another endocrinologist. They call him the Wizard. And they tell you that if anyone can figure this out, the Wizard can.
Mile 26. The final stretch. You think it is completely crazy to listen to a bunch of sick people on the internet that you have never met and you think it is beyond crazy to fly cross-country to see a doctor with a nickname like the Wizard, but you have exhausted every other option and you are getting sicker by the day. You fly to Los Angeles to meet the Wizard. He spends an hour with you and really listens to you. He doesn’t tell you to eat less or exercise more. He doesn’t tell you that you can’t be that tired or that you couldn’t be restricting your calories and gaining that much weight. He just listens and takes notes. He then runs a battery of tests – you do a cortisol test of one type or another every single day for three weeks when you get home. And just like the patients on the internet told you, some of the tests are negative but many of them are positive too. The Wizard also orders one more MRI and very clearly sees one and maybe two tumors. And you finally get diagnosed with Cushing’s. The race is over, you are exhausted and in pain and your body is a wreck. But mainly, you are relieved that after all this you aren’t completely crazy, that this isn’t just your imagination. Something with a name – Cushing’s – was actually destroying your body.
Post-race activities. The Wizard refers you to a neurosurgeon. You fly to Texas and you have brain surgery to remove two tumors, you recover enough to get on a plane and you fly home. But the story doesn’t quite end there. You have blocked out everything the Wizard and the wonderful patients on the internet told you about recovery, including the fact that another marathon is about to start. You had to block it out – no one is foolish enough to think about the pain of the next marathon when you are just trying to survive the one you are currently running. If you did, you would just lay down in a ditch beside the course and you would never get back up again.
But now that I am on the other side, I can tell you the first marathon is only the beginning.
Tuesday, February 3, 2015
Rabbit Holes: Who knew hairstyles could be depressing?
Wow, well...how to describe the experience I just had? It started out just as a typical evening chatting with friends, that led to a discussion of hair coloring, cuts, etc. Now, I don't consider myself very vain. I don't wear makeup, don't generally dye my hair, put product in it, dress to impress, etc. BUT, this seemingly innocuous conversation (and resultant google image search) went places I didn't expect.
I simply made the comment that I needed a haircut. I have for a good number of months already, but since I rarely leave my home, would need a babysitter, and the cheapest ones are a $40 minimum here already, it just isn't on my priority list. I mean, who is going to see it? My husband, my kids, my mother, and my doctor. They really don't care much about my hair, luckily. But, I am human and I am a woman. I don't WANT to look like a slob, I just don't have the energy and money to make up for the physical lot dealt to me through illness right now.
But, I went ahead and googled short haircuts trying to find a picture of my favorite haircut style I've had in the past (like, 13 years ago). I found something close. An angled bob, though I like mine at a steeper angle and much, much shorter. I don't have enough hair on the top to cut the angle now, so this style isn't an option at the moment.
We even joked about colors...pink, purple, blue. I'm crazy enough, I would totally do that at some point if I had a decent haircut to go with it and it didn't cost a fortune. lol
It wasn't too helpful, honestly. I don't want a boy cut. I have a small head on a huge body, so it just makes me look like a pinhead. And I wouldn't mind some femininity, even if it is more edgy. Most of the styles required a goodly quantity of hair...and many required some length. I have neither.
Whether due to the hashimotos thyroiditis (autoimmune hypothyroidism), the Cushing's Disease, or the AGHD, I have lost a huge quantity of my hair over the years and it is not yet improving. It is VERY thin on top. **SIDE TRACK!!! I TOTALLY have a funny to tell on myself! At my first appointment with my endocrinologist, we were going through my list of symptoms, and he switched to a new symptom asking "You look pretty thin on top?" and my brain simply didn't follow. All that came to me was, "Are you kidding? I have to special order these bras!" Luckily I just sat there with my mouth gaping open, staring at him, as my brain thought those things in silence...and eventually caught up. PHEW! SIDE TRACK OVER** I'm blonde and you can see through to my scalp. Rain doesn't hit my hair, it hits my scalp. I can't use clips because I don't have enough hair to hold them in. I can't wear extensions or a weave because I have nothing to cover it up or "weave" it into. This cuts out a great many styles, because I literally don't have the hair required. (I do have an AWESOME wig, but I have heat intolerance and thus sweat and feel ill...well and extreme exhaustion too, so..)
And, I can't grow it long because it makes me look creepy, really creepy, and almost mullet-ish. My husband would certainly draw a line there, no matter how much comedic effect it would have if I walked around with a mullet! HA HA HA!
So, I started googling things like "haircuts for balding women", "haircuts for thin hair" (these women ALL had sufficient hair, it just wasn't thick...so not the same thing), "pixie cuts on fat women", "haircuts for fat women", etc. Don't start mixing up google image searches of "fat and bald women haircuts" unless you're super brave. I was shocked at the things that popped up. Most of it was completely unrelated to my search topics, most hair related photos were STILL skinny actresses and models, and some things were horribly derogatory towards fat women. Maybe I should have used the word "obese" or "plump" or "round", but really? I currently have a fat body. That is the fact of the matter. I might generally use other language when talking to people unless I know them well, but let's get to the point with google, right? So much for that.
About an hour into searching, I gave up. It was getting too depressing. It was all in fun, supposedly innocuous (or so I had thought), I was getting excited looking at ideas and thinking a new haircut could help me feel a bit more confident, feel a bit better about myself right now, have some form of expression and control, but that just isn't my reality now. I had to stop before I let this bring me into deeper emotions that I have to be careful not to dwell on in my current state.
Is this a bit vain and silly? Probably. Is it minor? Definitely. But, it brought me right back down that rabbit hole, reminding me of what my illness has done to me, my limitations, my inability to change it at present, the "me" the world sees on the outside that is so different from who I am on the inside, my desire to express myself with little means of doing so, and it reminds me that wishing it were different isn't enough to make it so. Don't get me wrong...Not being able to get a good haircut that makes me look and feel great is NOT the end of the world. What makes it so hard is that it reminds me I have no control over my world, and that my world appears so vastly different from the world in which those around me appear to live.
My doctor asks me how I'm doing emotionally at every visit. And I try to tell her. I'm actually doing REALLY well. I've been depressed before. I know what that spiral feels like and this is not it. I am somewhere between accepting of my situation and apathetic, but I AM happy, I enjoy what I can in life right now (though comparatively limited), and I have hope for my future. But the depressive sadness still hits like a sudden downpour, I can't just "will" it not to pop up in my life, and I never seem to know what'll trigger it. It is momentary now, but I can't seem to avoid it. I believe it is completely normal, surely even common for people with debilitating and chronic illness, so that is why I write this...to let others know it's okay talk about it if you need to. It's okay to admit you don't simply "move on" as if there was no life besides the one you live now. We don't have to always hide our hurts, worries, fears, and struggles. Acceptance and overcoming require an acknowledgement...but the trick lies in not staying in that downpour and letting it pass.
I simply made the comment that I needed a haircut. I have for a good number of months already, but since I rarely leave my home, would need a babysitter, and the cheapest ones are a $40 minimum here already, it just isn't on my priority list. I mean, who is going to see it? My husband, my kids, my mother, and my doctor. They really don't care much about my hair, luckily. But, I am human and I am a woman. I don't WANT to look like a slob, I just don't have the energy and money to make up for the physical lot dealt to me through illness right now.
But, I went ahead and googled short haircuts trying to find a picture of my favorite haircut style I've had in the past (like, 13 years ago). I found something close. An angled bob, though I like mine at a steeper angle and much, much shorter. I don't have enough hair on the top to cut the angle now, so this style isn't an option at the moment.
We even joked about colors...pink, purple, blue. I'm crazy enough, I would totally do that at some point if I had a decent haircut to go with it and it didn't cost a fortune. lol
Anyway, my friends suggested pixie cuts and I started actively looking for a hairstyle that would look good on me. In my search, all the pictures were of actresses, singers, models, etc. I don't look like them, so seeing a haircut on them doesn't do me much good in picking out something that would look good on me! So, I started looking at face shapes. I'm so overweight I assumed I'm round, but I snapped a quick picture of me, and even still I'm more of an oval shape. So, I started looking up the kinds of haircuts that are best for that shape.
It wasn't too helpful, honestly. I don't want a boy cut. I have a small head on a huge body, so it just makes me look like a pinhead. And I wouldn't mind some femininity, even if it is more edgy. Most of the styles required a goodly quantity of hair...and many required some length. I have neither.
Whether due to the hashimotos thyroiditis (autoimmune hypothyroidism), the Cushing's Disease, or the AGHD, I have lost a huge quantity of my hair over the years and it is not yet improving. It is VERY thin on top. **SIDE TRACK!!! I TOTALLY have a funny to tell on myself! At my first appointment with my endocrinologist, we were going through my list of symptoms, and he switched to a new symptom asking "You look pretty thin on top?" and my brain simply didn't follow. All that came to me was, "Are you kidding? I have to special order these bras!" Luckily I just sat there with my mouth gaping open, staring at him, as my brain thought those things in silence...and eventually caught up. PHEW! SIDE TRACK OVER** I'm blonde and you can see through to my scalp. Rain doesn't hit my hair, it hits my scalp. I can't use clips because I don't have enough hair to hold them in. I can't wear extensions or a weave because I have nothing to cover it up or "weave" it into. This cuts out a great many styles, because I literally don't have the hair required. (I do have an AWESOME wig, but I have heat intolerance and thus sweat and feel ill...well and extreme exhaustion too, so..)
And, I can't grow it long because it makes me look creepy, really creepy, and almost mullet-ish. My husband would certainly draw a line there, no matter how much comedic effect it would have if I walked around with a mullet! HA HA HA!
So, I started googling things like "haircuts for balding women", "haircuts for thin hair" (these women ALL had sufficient hair, it just wasn't thick...so not the same thing), "pixie cuts on fat women", "haircuts for fat women", etc. Don't start mixing up google image searches of "fat and bald women haircuts" unless you're super brave. I was shocked at the things that popped up. Most of it was completely unrelated to my search topics, most hair related photos were STILL skinny actresses and models, and some things were horribly derogatory towards fat women. Maybe I should have used the word "obese" or "plump" or "round", but really? I currently have a fat body. That is the fact of the matter. I might generally use other language when talking to people unless I know them well, but let's get to the point with google, right? So much for that.
About an hour into searching, I gave up. It was getting too depressing. It was all in fun, supposedly innocuous (or so I had thought), I was getting excited looking at ideas and thinking a new haircut could help me feel a bit more confident, feel a bit better about myself right now, have some form of expression and control, but that just isn't my reality now. I had to stop before I let this bring me into deeper emotions that I have to be careful not to dwell on in my current state.
Is this a bit vain and silly? Probably. Is it minor? Definitely. But, it brought me right back down that rabbit hole, reminding me of what my illness has done to me, my limitations, my inability to change it at present, the "me" the world sees on the outside that is so different from who I am on the inside, my desire to express myself with little means of doing so, and it reminds me that wishing it were different isn't enough to make it so. Don't get me wrong...Not being able to get a good haircut that makes me look and feel great is NOT the end of the world. What makes it so hard is that it reminds me I have no control over my world, and that my world appears so vastly different from the world in which those around me appear to live.
My doctor asks me how I'm doing emotionally at every visit. And I try to tell her. I'm actually doing REALLY well. I've been depressed before. I know what that spiral feels like and this is not it. I am somewhere between accepting of my situation and apathetic, but I AM happy, I enjoy what I can in life right now (though comparatively limited), and I have hope for my future. But the depressive sadness still hits like a sudden downpour, I can't just "will" it not to pop up in my life, and I never seem to know what'll trigger it. It is momentary now, but I can't seem to avoid it. I believe it is completely normal, surely even common for people with debilitating and chronic illness, so that is why I write this...to let others know it's okay talk about it if you need to. It's okay to admit you don't simply "move on" as if there was no life besides the one you live now. We don't have to always hide our hurts, worries, fears, and struggles. Acceptance and overcoming require an acknowledgement...but the trick lies in not staying in that downpour and letting it pass.
*Medical discussion* I am severely overweight thanks to long-term undiagnosed Cyclic Cushing's Disease. It appears I am now in remission from that (just seeing some signs of change in the last few months), but the pituitary surgery required to remove the tumor causing the disease also left me susceptible to other issues...some of which have surfaced (partially empty sella and hypopituitarism). I now have adult growth hormone deficiency (AGHD), which also leads to extreme exhaustion, muscle loss, bone loss, central weight gain, sleep problems, cognitive/memory issues, etc. It's so similar, the biggest difference is that I simply don't get "high cycles" any more (there's more than that, but that is the biggest one I notice now). We are on the path to fix that, but it takes time. I had a couple of months of testing, and now am waiting for my insurance company to pre-approve the $1500/mo daily injections required to replace this very useful hormone I no longer can make on my own. So, hopefully I'll see some improvements over the next few months when that is approved and I can start getting my body back into balance, but I am told to expect the recovery to be a slow process. And there is a decent chance this won't be the only hormone my pituitary will stop being able to produce on it's own. If that happens, I'll have to do more testing and add that hormone's replacement, then work through re-balancing it all again. Only time and testing and symptoms will tell. Hopefully this is it, and fixing this imbalance will make me good to go! (And this isn't the depressing part...this is just what is; I've accepted it, I'm happy it is treatable and I have a good chance at regaining control of my body and my life!)
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