My Journey by Rachel Wilson
It’s hard to say when my exact “journey” began with what we now know is Cushing’s Disease. Both my Mom and my doctor believe I’ve had this for years, when I started having period problems, migraines and unexplained weight gain in high school.
I can safely say that I started really noticing/documenting this last round of health issues in the spring of 2007. I can remember my first doctor’s visit regarding the onset of this in spring 2006, but the majority of my health issues really began a year later. I’d always suffered from migraines, but only around my menstrual periods. I took birth control to help with that, and it also helped regulate my periods for the majority of my adolescent and young adult life. In Spring 2007, however, I started experiencing cluster migraines that were debilitating, blinding and just absolutely horrible. I don’t usually have a primary care physician because I’m, for the most part, a really healthy girl. But I started seeing a doctor to pinpoint the cause of the migraine headaches. I had a CT scan which came back negative for any problems. I tried different abortive medications to treat the migraines when I had them (Imitrex) and also preventive medications (Topamax) but nothing helped. I’m not a big fan of popping pills and insisted that I find the cause of the migraines, rather than just masking them with an expensive, non-generic daily pill. I cut back on caffeine, avoided certain trigger foods, changed the lighting in my house and in my office, had my eyes checked and even experimented with different birth control options, since estrogen can be a huge trigger for migraines in women. Those lifestyle changes helped a little, but I was still having them.
The second (and biggest) indicator that something was seriously wrong was the extreme amount of weight gain. I packed on 40+ pounds in less than eight months for absolutely no reason at all. I’ve always been a health nut and getting me to eat fruits and veggies has never been a problem. I also don’t abhor exercise. I stay pretty active for the most part, so it wasn’t food addiction or laziness that was causing the weight gain. I’ve always been a thick girl, but obesity was never a problem. It was never easy for me to lose a lot of weight, but it was possible. I’d go through “bouts” of weight gain and had been doing that since high school. The first time I noticed it was between the summer before my senior year of high school up to graduation. I packed on about 60 pounds. We all blamed it on the stress surrounding my parents’ divorce at that time. I lost some of it, but not all of it, in college. My junior year of college I once again ballooned up for really no reason. Still eating healthy, still working out. I tried some different weight loss programs and lost a little bit, but again, not all. My weight went back down to “thick” girl status rather than “fat” girl status until this last year, when once again, I packed on a hefty amount of weight. I once again started trying to combat the weight gain, but it seemed no matter what I did, I only seemed to gain weight. I tried Weight Watchers and gained 17 pounds! Talk about frustrating. I researched different diets and causes for abdominal weight gain, which is where I noticed most of the weight was accumulating. Insulin resistance was my first self-diagnosis, so I tried the South Beach Diet with decent success. I was able to lose a total of 15 pounds before my body stalled completely, which appeared to be the norm every time I tried to lose weight.
Period problems were also very indicative of some other problem. My primary care physician never seemed too concerned. His answer was for me to “Eat less and exercise more.” Why, thank you, kind sir! He never once tested me for hyperthyroidism (even though my brother had thyroid cancer and my mother had her thyroid removed) or diabetes (even though it runs rampant on both sides of my family). My OB/GYN was much more proactive, though, and worked with me as much as possible. We tried different birth control pills, but even the lowest-dose estrogen seemed to trigger migraines. Since I wasn’t in a relationship, we tried the cold-turkey no-birth control method and my migraines went away for a month. But then the non-stop menstrual periods began. From June through August, I had my period every single day. He tested my ovaries and tested for polycystic ovarian syndrome (PCOS). He determined I did not have PCOS, but in order to stop the bleeding, we decided to use the IUD Mirena. He highly suggested I see an endocrinologist, though, because something was obviously not right.
I had several other seemingly unrelated health issues during this two-year period, too. For one, I had an ankle problem that my orthopedic doctor couldn’t figure out. I really wanted to train for the 1/2 marathon, but found that I could only make it 2 miles before my ankle completely gave out on me. I figured it was an ankle sprain or hairline fracture, but it wasn’t. My doctor was at a loss, gave me a cortisone shot, put me in a boot and treated me for tendonitis, even though he couldn’t figure out the exact problem.
I also went through severe bouts of depression and I’m usually a pretty positive gal. And when I say severe bouts of depression, I mean it. It was horrible. Crying spells, mood swings, lethargy, suicidal thoughts, mean thoughts about myself. One minute I was high, the next minute I was low as I could go. I’m normally a very social person, but I found myself hiding out in my apartment with my cat, unwilling to go anywhere or do anything. I blamed it on other issues (bad relationships ending, bad living situations) and sought help, but it was still a nagging problem.
So was fatigue. I was (and still am) tired ALL of the time. It doesn’t matter how much sleep I get, I’m always tired. I need naps constantly and it’s all I can do to make it through my entire day at work sometimes. I found myself falling asleep at the drop of a hat and most of the time just feeling like a train had hit me. It was very bizarre, especially considering I’m a “sleep Nazi” and always make sure to get the recommended 8 hours of sleep.
Perhaps the grossest (and most annoying) issue was hair growth. It came out of nowhere. Chin hairs, cheek hairs, sideburns. It was like I was growing a beard. My eyebrows became bushier and I began growing a mustache. I even found nipple hairs – talk about disgusting. I’m a chronic tweezer/plucker, so you can only imagine how much fun it is tweezing several times a day trying to get the hair growth under control. No amount of waxing or plucking makes it go away. It just seems to get worse. Let’s not even talk about hairy arms!
Other things I noticed were stretch marks all over my body (especially on my stomach, sides and upper arms). I developed them on my breasts. I started accumulating fat on my back, which disgusted me…and I noticed I had this “knot” (what we now know is a symptom called a “buffalo hump”) in between my shoulder blades. When I would get massages, I’d ask them to work on the knot, but there wasn’t a knot, even though I felt constant tension. The “knot” has became worse over several months, getting bigger and bigger. I felt deformed and absolutely ugly. Acne became a severe problem again like it was for me in high school, and I noticed that small things like bug bites took weeks to heal. I bruised easily, too. If I gave blood, for example, the bruise would take at least two weeks to go away. Bug bites take even longer to heal. And I scar, too, leaving dark spots where those wounds once were. My blood pressure steadily began increasing, even though I’ve always had perfect blood pressure. My face became fatter and fatter. I noticed in pictures from Spring 2007 to more recent pictures a huge difference in my overall body shape and facial shape.
I knew something was up – but I had to convince a doctor of it first.
I had to endure more tests from my quack of a primary care physician in order to see an endocrinologist. He did more “bloodwork” and then determined that I was normal and everything was fine. I told him that I absolutely was not fine and wanted to see an endocrinologist immediately. My mother and brother had both started seeing a younger endocrinologist at the Vanderbilt Eskind Diabetes Clinic in Nashville and spoke highly of her. In fact, my Mom mentioned some of my problems to this woman, Dr. Kathryn Dahir, and she suggested that I make an appointment to see her. So I did.
My first appointment with her was on September 18th. She initially diagnosed me with PCOS, even though my OB/GYN had ruled it out, saying that I had the classic symptoms and we needed to treat it from a clinical standpoint. She put me on Metformin, but wanted to still perform bloodwork and check everything else out to be sure she didn’t miss anything. That’s the first time she thew out Cushing’s Syndrome, saying it was pretty rare and chances are I didn’t have it, but she didn’t want to miss it, either. She rattled off several other things she was going to screen as well. I did bloodwork that day, a glucose intolerance test the next day, and a 24-hour urine collection that weekend.
By the following Wednesday, she was calling me at work with the results. My thyroid was fine and I didn’t have diabetes, so that was extremely good news as I was really concerned about both of those. My urine collection, however, was the reason she was calling. They use that to test for cortisol, and in “normal” tests, the range is 10-20 mcg/liter, with 50 being the range when they start to get concerned. My cortisol level was 75.6, for a total sample of 208. So nearly quadruple what it should have been. She told me over the phone that meant I had Cushing’s Syndrome, and now we had to figure out why. One of my best friends, Carolyn, had diagnosed me with Cushing’s via WebMD several months before, in an effort to lighten the mood when I was having a pity party/meltdown on her sofa. And now I really did have it. Unbelievable.
I researched it left and right and was amazed to realize that all these seemingly unrelated symptoms could all be tied to one culprit. It was relieving but really overwhelming. The following weeks were a blur of bloodwork and other tests to determine what the root cause of my Cushing’s Syndrome was. I did a CRH stimulation test, which was ultimately the wrong test. I didn’t need any more cortisol – hello! They tested my ACTH levels, did another 24-hour urine collection and a dexamethasone suppression test. All of my tests kept coming back with high cortisol levels. The low-dose dexamethasone suppression test was the last test I did. My adrenal glands didn’t suppress the cortisol, which pinpointed a pituitary problem…in other words, Cushing’s Disease.
An MRI performed on October 27th, 2008, confirmed that there was indeed a problem with my pituitary gland. It couldn’t show a clear picture of my pituitary gland, but did show several abnormalities. Sometimes even one or two bad cells can throw your body into absolute distress. Dr. Dahir referred me to another endocrinologist on staff at the Vanderbilt Pituitary Clinic, Dr. Vipul Lakhani.
I met with Dr. Lakhani on November 5th at Vanderbilt and was really impressed. We discussed my medical history in detail and he reviewed all of the tests that Dr. Dahir had run and confirmed my Cushing’s Disease diagnosis. He showed me my MRI and all the possible problem areas, and explained the IPSS procedure and why that was necessary. He was very nice and his bedside manner was great. He was also very honest and discussed all of the realities of this disease with me openly and honestly. There is no cure for this. I can only hope for remission. I’ll be on replacement hormones for a year and a half if all goes extremely well, but after discussing the chances of success, the realist in me knows that I’ll probably be on them for the rest of my life. To quote Dr. Lakhani, “We’re in this for life, my friend – I hope you like me!”
Vanderbilt called me on the way back from the appointment with Dr. Lakhani and already had the IPSS procedure scheduled for November 17th. It’s a pretty invasive procedure and I was fairly nervous about it, but willing to do whatever it takes. The test determines with 100% certainty the existence of pituitary disease. If, worst case scenario, my pituitary checked out fine, this test would them know it’s a tumor elsewhere in my body (malignant) and it’s a whole ‘nother ballgame.
The IPSS procedure at Vandy went fine, except that they discovered I don’t have a jugular vein on my right side. So they couldn’t get an adequate blood sample from my right side. Had they been able to, they would have been able to determine which side of my pituitary gland is the problem. But alas, I’m an anatomical wonder, and my lovely inherited veins prevented them from being able to do that. In any case, they still confirmed it is my pituitary gland giving me all of this grief, so now I’m on my way to surgery.
I met with Dr. George Allen the day before Thanksgiving and was booked for surgery on December 16th, 2008. They had to delay the surgery a week, so my actual surgery happened on December 23, 2008. So, from my first meeting with Dr. Dahir to the day of my surgery was exactly three months. Talk about quick! I am SO very thankful and blessed.
Dr. Allen explained the major complications, which are scary on their face, but I’m wasn’t too worried. He’s performed 1900 surgeries over the life of his career and approximately 500 have been related to the type of tumor I had. I was (and still am) in expert hands. He approached the tumor conservatively. Because of my age and gender, he didn’t want to do anything radical and permanently disable my pituitary function if he could help it. He explained that I will probably be infertile after this operation and that is the most major consequence for women with this surgery. Especially those of us in our childbearing years. I do want to be a mom, perhaps more than anything else, but even that reality was still not enough to make me rethink surgery. I’d rather be healthy…and cross the baby bridge when I get there one day.
I battled steroid withdrawal for the first few months post-surgery, and that was my biggest complication from the surgery. Many of my symptoms were similar to those of Addison’s Disease, but my adrenal glands were functioning. It’s just that my pituitary gland was not functioning properly enough to stimulate them to produce cortisol. I developed many rare complications after my surgery and I literally had to take it one day at a time.
The scariest complication related to my steroid withdrawal was the development of a pseudotumor. Over the course of a few days, I started noticing visual disturbances. I found it hard to park in a parking garage, unable to gauge how much distance I had between other cars so I wouldn’t hit them. My eyes seemed to cross all of the time, and my computer screen was blurry. On a shopping trip with my then-roommate, I ran into displays at stores, and smacked into the end-caps at a department store. I’d thought I’d given myself enough distance to clear them, but I had not. Finally, after watching a movie and seeing multiple Harry Potters on the screen when there was only one, my roommate encouraged me to go get my eyes checked. Something was wrong.
It turned out that my optical nerve was swollen, but why my ophthalmologist couldn’t be certain. When I mentioned I’d had brain surgery a few weeks ago, she panicked a bit, because it could have been a complication. After talking with my doctors, they didn’t seem overly concerned so she sent me home to rest. I woke up, though, to frantic phone calls from my doctors. I guess upon further review, they realized that this could be a serious complication and wanted me to come to the emergency room immediately. It could be bleeding on the brain (no bueno!) or another tumor on my brain that they had missed while concentrating on my pituitary tumor. They just didn’t know. Needless to say I was terrified. After running through a gamult of tests, though, they determined that I’d developed a pseudotumor in my brain and that the increased spinal fluid on my brain was putting pressure on my optical nerve and causing the vision problems. They gave me a spinal tap (and yes, I quoted lines from Spinal Tap while my neurosurgeon performed the procedure) and all was well.
Apparently that’s a rare (drum roll, please) complication of pituitary surgery, but of course it happened to me. After that I began to mend, because my steroid dosage was finally right. I felt good for a couple more months until it all came crashing down again. I was out with my roommate at a bar and having a great time when my stomach started hurting. At first it felt like gas, or maybe like I had to go to the bathroom, but that didn’t seem to relieve the pain. My roommate noticed that I didn’t look right, so she suggested that we go home. By the time we pulled into the house ten minutes later, I was vomiting uncontrollably and in dreadful pain. My entire body hurt. I couldn’t lay on my back or my stomach. A heating pad didn’t work, and I continued to vomit uncontrollably. I didn’t know what was going on. I called the on-call endocrinologist, who suggested I go to the E.R. if I felt like something was wrong, and after Googling adrenal crisises, I was sure that was what was wrong. I’d heard that they could happen, and that they were sudden and horrible things.
My mother came and took me to the hospital, and though I told them I was having an adrenal crisis and needed a stress dose of hydrocortisone, they still made me wait in the waiting room for hours. I laid on the emergency room floor and vomited into a Wal-mart bag. Anyone who knows me knows I wouldn’t be the type to lay on a dirty emergency room floor to vomit, so I must have been in severe pain and completely out of sorts. Others waiting in the ER with me offered to let me go first because I was much sicker than they. Finally, the E.R. staff put me on a gurney in a hallway, but not in a room. My mother was livid. We kept telling them that I needed a stress dose to no avail. No one would listen. Finally, after my mother rattled everyone’s cages and threatened legal intervention if I died from this (which was a very real possibility at this point), I received treatment. I told the E.R. doctor that I had Cushing’s Disease, was having an adrenal crisis, and needed a stress dose. He finally confirmed that with my endrocrinologist, who was appalled that they had made me wait to get that. I could have died.
As soon as they gave it to me, I felt better. My body unseized, and a wave of calm washed over me. And then pain…excruciating pain. Because my body had been so stressed, it had thrown three kidney stones. Great. I had an unsuccessful surgery to remove them, since my kidneys are both too small to pass them and too small for surgical instruments to retrieve them. So I had to walk around with a stint for a month so my kidneys would stretch enough for yet another surgery. Kidney stones are horrid, horrible creatures and I wouldn’t wish them on my worst enemy.
Finally, though, I began to get a little better. At two years post-op, I’d only lost 20 pounds, but the acne was gone, and the extra hair growth started. The “moon face” went away, and the stretch marks lightened. The buffalo hump never completely went away, but it did shrink a little. So I experienced SOME relief. But it was temporary.
I never felt “normal” even after my first pituitary surgery. Something inside of me kept saying it wasn’t gone. Earl wasn’t completely dead. And slowly but surely I started presenting with clinical symptoms of Cushing’s again. Headaches, hair growth, weight going up, blood pressure going up, and the crazy, horrible mood disturbances. I started the testing process again, and I’d get inconclusive results. Sometimes it’d be abnormal, but then a follow-up test would be normal. It was way too emotional for me to handle, because I just wanted this to be over.
So I started searching, and my search led me to Swedish Pituitary in Seattle. Many Cushies have experienced success with their program, so I reached out to them. I emailed the doctor, who scheduled a phone interview and talked candidly with me about my experience with Cushing’s, and my life since pituitary surgery. After five minutes, he felt pretty certain that I was in the middle of a recurrence, maybe even simply just not cured the first time, and wanted to see me in clinic. My best friend and I flew out to Seattle and went through Camp Cushie (I say she went through it with me because she did, even if she didn’t actually do the testing), which is an intensive five-day workup of blood cortisol levels, urine cortisol levels, adrenal function, ACTH levels, etc. It’s exhausting, but more often than not, it catches your body behaving badly. And in this case, it worked for me. I got several high blood cortisol levels, several high ACTH levels, and I did not suppress cortisol on the high dose dexamethasone test. Simply put, I still had Cushing’s, just like I’d known all along.
I scheduled surgery and had my second pituitary surgery in Seattle on August 29th, 2011. Where my first surgery had been very conservative (meaning they didn’t touch my pituitary gland at all), the second surgery was very aggressive. The neurosurgeon took out 80% of my gland, and scraped around the area around my pituitary gland, hoping to get any hyperplasia or bad cells that were causing Cushing’s. Whatever he did worked, because two hours post surgery I crashed, meaning that my cortisol levels dropped to what would normally be detrimental levels. They have to do to that – make your body crash off of the high steroid it’s so used to having, and then they start giving you synthetic steroid. It’s a very controlled, regimented process.
Crashing really does suck, though. You feel like you’re dying. And now that I’m dependent on synthetic steroid since my body no longer makes it naturally, it’s a very fine dance. It is a very hard recovery, and your body has to relearn everything without the existence of your pituitary gland, but unfortunately Cushing’s calls for radical measures to be taken in order to be healed. I still do not have any pituitary function, and I take replacement steroid, thyroid, growth hormone, estrogen and progesterone. I will also start taking replacement testosterone to correct my nonexistent sex drive.
I consider myself VERY lucky for several reasons. One, I found two great endocrinologists that listened to me and attacked this with a tenacity that is unfortunately not employed by several other doctors. So many other Cushing’s sufferers have not been nearly as lucky as me. Second, Vanderbilt Pituitary is the third largest center in the nation for these types of disorders, and it’s in my backyard. I don’t have to travel thousands of miles to find a specialist – they’re right here in my hometown. I did end up going to Seattle for Dr. Ludlam, but even then it was well worth the time and the cost. My family and friends are here in Nashville, so my support network is strong. And while I’m not sure how long I’ve had this, I feel pretty certain that we caught this in at least a somewhat early stage. I have youth on my side – at least that’s what the doctors keep saying.
It’s hard not to worry, and where I was was once steadfast that that God had control of this entire situation, I can now honestly say I’m weary. I know this entire experience will be used somehow to help others – and maybe it already has – but I won’t lie. I’m frustrated. I tell myself that if my experience can help out one other person, it’s totally worth it to me. Because it is. I just have to remind myself of that in my “down” moments. Some of us have to suffer in order for others to benefit. I try to remain positive but lately I’ve had more negative moments than positive. I’m only human.