Never in a million years would I have dreamed I’d be writing a letter like this. All of my life I’d been an active, athletic girl who loved sports, working out, and being physically active. I was a big runner all throughout high school and never had a problem with my weight. In fact, if anything, I was probably too small and people would comment on how “tiny” I was. I was almost 5’4″ and weighed between 110-115 pounds at most. I could eat anything I wanted, never had to worry about dieting, and exercised only because I wanted to, not because I had to.
Shortly after I graduated college in May 2003, I had several stressful events occur which resulted in some major life transitions for me. The biggest of these was selling my house and moving in with the man I am currently engaged to marry. My life started getting back on track again and I was very happy. I noticed I was starting to gain a little weight, and I figured it was probably due to the fact that I had been slacking off a bit (exercise and diet) while adjusting to my new life. So, I decided I needed to start becoming healthier again and I joined a local gym. I hoped to knock off those few extra pesky pounds and get back into shape without any problems. However, this did not prove to be the case. I worked out every day and for hours at a time, but those extra pounds just wouldn’t come off, no matter how hard I tried. I even adjusted my diet to the point where I was barely eating anything and still not losing the weight. I thought it was just a part of getting older and my metabolism changing, since I wasn’t running and working out the way I had in school. I also started noticing other things like my skin changing and having frequent breakouts. This seemed rather odd to me because I never had problematic skin even as a teenager. I was told by the doctor that adult acne was common and was sent home with prescription facial medicine. My menstrual periods started to get lighter and lighter until they finally disappeared altogether. My gynecologist said this sometimes happens when you’re on birth control pills, and I didn’t need to be concerned. So, basically I just learned to live with all these changes occurring to my body, believing all my doctors when they said all of it was normal.
I continued to battle these problems for the next couple of years, unsuspecting that I had a much more serious medical condition. Finally, in December 2006, something happened that made everyone realize something just wasn’t right with me. I went to the doctor to get my prescription refilled for my facial medicine when he took my blood pressure and got this shocked look on his face. He asked if I felt okay, and when I said yes, he responded that I shouldn’t, because my blood pressure was sky high! He immediately said I needed to get some blood work done and when the results came back I was sent to the emergency room and was ultimately admitted to the hospital. During my three day stay I was subjected to numerous tests, scans, x-rays, MRI’s, MRA’s, etc. and was sent home. After two weeks, my doctor told me he suspected I might have something called Cushing’s Disease, and wanted me to visit the kidney specialist I had seen in the hospital. When I told her my symptoms and the prospect of Cushing’s she immediately agreed this was a very real possibility and referred me to a wonderful endocrinologist at John Hopkins University in Baltimore, Maryland.
I met with my endo on January 8th, 2007, and upon looking at me and reviewing my cortisol levels diagnosed me with Cushing’s disease. She wanted to perform several of her own tests to be sure and also to locate the pesky tumor which had not shown up on any of my scans, MRI’s, etc. I was sent home with orders to perform a salivary cortisol test, dexamethasone suppression test, and another 24-hour urine collection. I was also scheduled for an IPSS test which was given a few weeks later. Unfortunately, the test wasn’t performed correctly and came back with “slightly negative” results that “looked like they were trying to be positive”. However, with all other tests coming back strongly positive, the doctors felt very confident the source of my problem was located in my pituitary gland. Surgery was scheduled quite quickly for me and on February 5, 2007, I had transsphenoidal surgery at Johns Hopkins University. The surgeon said he did not see an actual tumor, but took out a lot of “abnormal tissue”, comprising the right side of my pituitary gland. I recovered from the surgery quite easily, was only in the hospital for 3 days, and my only repercussion was a temporary battle with diabetes insipidus. Boy, was that fun! Unfortunately, a week after surgery my endo called to tell me the pathology came back and there was no evidence of any ACTH producing cells in the tissue the neurosurgeon had removed. The surgery was unsuccessful. I was completely distraught. I had just undergone major surgery and was no better off than before. I just wanted this nightmare to be over and to able to live my normal, healthy life again.
My endo said before the neurosurgeon would agree to try surgery again, he wanted another IPSS done with more conclusive results since the MRI’s were still not showing the tumor. My endo was able to get me accepted into a study at the National Institute of Health (NIH), in Bethesda, Maryland. I checked in at NIH on March 12th and had all of the standard tests and blood work repeated again over the next week and they again all indicated a pituitary tumor. However, again my tumor eluded all MRI’s. I had another IPSS test performed correctly by a wonderful doctor and the results were “strongly positive” for a pituitary tumor. I was told I could go home and would be contacted in about two weeks after the doctors met with the neurosurgeon to discuss my case. Weeks went by and I was told nothing had been decided because all of the doctors and surgeons were unable to find time to get together due to vacations, schedules, etc. I started to get impatient waiting, because I was still so sick. I had gained about 40 pounds by this point because I was no longer allowed to exercise due to my high blood pressure. Not that I could have anyway because my muscles had become so sore and tired by this point. It took every ounce of strength I had to walk up the stairs or to the mailbox. Finally my endo was able to contact the doctors at NIH who said they had decided their recommendation was to put me on medicine to ease my symptoms and come back in 6 months for a repeat MRI to see if the tumor would finally show up. If it did, I could have surgery, if not, perhaps radiation. I was very upset by this decision, because I wanted a “quick fix” to my problem, not the potential of months to years with radiation. At first, my endo felt I should still try a second surgery, but then after talking to her boss decided that NIH was probably right in that a second pituitary surgery wouldn’t be the best choice for me right now, since my tumor still was not visible. She put me on ketoconazole and set me up with an appointment to see a radiologist at Johns Hopkins to schedule gamma knife radiation.
I was progressively getting worse and worse. I was tired all the time during the day and couldn’t sleep at night. I was told that’s because my cortisol levels were peaking then. I was eating next to nothing, yet gaining pounds each week. I started losing my hair on my head and gaining it in unwanted places. I was bruising constantly and getting purple stretch marks all over my sides, abdomen, and thighs. I couldn’t fit into anything that buttoned and elastic waists became my best friend. I was so embarrassed to go anywhere and didn’t want to leave my house. I was absolutely miserable. My endo increased my dosage of ketoconazole and although it did help a little, I still suffered tremendously. The doctor couldn’t go much higher on my dosage because she was afraid of possible liver damage.
I met with the radiologist on May 17, 2007. He was very nice and said before he’d agree to give me radiation he’d like me to discuss the possibility of repeat pituitary surgery with the neurosurgeon and get his opinion. I tried to contact the neurosurgeon via e-mail and he told me he’d discuss things with my endo and get back to me. In the meantime, I had a really scary episode which really turned everything around for me. One evening, I was with my fiancé and I noticed my feet starting to swell. When we got back to our house, I put my feet up, but the swelling didn’t go away. I called my endo and after asking me several questions was told to go immediately to the emergency room. I ended up getting admitted with extremely low potassium levels, which was the cause of my swelling. I had an IV drip with potassium all weekend.
I think that episode was enough to convince everyone I was not doing well on all the medicines I was taking and something had to be done soon. Everything flew by pretty quickly from there. The following week, I met with the neurosurgeon to talk about the possibility of repeat transsphenoidal surgery and its chances for success. I also met with my endo and her boss to talk about another option, bilateral adrenalectomy. After weighing all the pros and cons and doing some serious soul-searching, I decided on the adrenalectomy. I knew I’d be on lifelong steroid replacements and would always have to closely monitor my health, but I also knew I wanted to be cured. I did not want to take the chance of a second pituitary surgery being unsuccessful again and then possibly facing an adrenalectomy anyway. I didn’t want the chance of the tumor growing back. I wanted these symptoms gone, for good. I knew with no adrenal glands, my body couldn’t make cortisol and thus I would no longer suffer from the Cushing’s symptoms. I knew by removing my adrenal glands I was indirectly solving my problem, in that I would still have the ACTH producing tumor supposedly in my pituitary gland. I knew there was the possibility the tumor could rapidly grow (Nelson’s Syndrome) and I might have to get it removed at that time, but I was prepared to face it all. I just wanted these Cushing’s symptoms gone forever.
On June 26, 2007, I met with the surgeon who would potentially be doing my adrenalectomy. Once again, I chose to have this surgery performed at Johns Hopkins because I was always very impressed by the skill and knowledge of their doctors. The doctor reiterated the risks of the surgery to me and also the consequences. I signed the consent forms and found out the following day my surgery was slated for that Friday. I could hardly believe it! I had wanted for so long, and it was finally happening. I had to go on a clear liquid diet for the two days before surgery and also do a “bowel cleansing”. I never want to do that again! I did all the pre-op stuff and anxiously waited for Friday morning.
I arrived at the hospital and went into pre-op around 10:15 am. My family and fiancé all came in to wish me luck and I was wheeled off to the operating room. Next thing I knew I was waking up in recovery. I was told everything went fine; the doctor was able to remove both adrenal glands laparoscopically, in about 6 hours. I was taken to ICU for the first night and moved to a regular room for the next three days. I was definitely sore, but made myself get out of bed and walk around the next day after surgery. I had seven small incisions on my abdomen and that was it. I could hardly believe they could pull anything out of those small cuts.
After I got home, I continued to heal nicely. All of my blood work indicates a successful surgery (cortisol levels are zero w/o medicine). I saw physical changes immediately after surgery. I lost 10 pounds in the first two weeks and the puffiness in my face and neck has drastically been reduced. My bruising and high blood pressure has disappeared. My hair is starting to grow again and two days after I came home I got my first period in three years. I never thought I’d be happy to get my period, but I honestly cried. I was able to drop down to my maintenance dose of hydrocortisone (15 mgs daily) fairly quickly and easily with no real problems or scares.
It’s now a little over two months since my surgery and I feel great for the most part. I have a small problem with swelling of my feel and ankles from time to time and I still get tired fairly easily, but that’s the worst of it. I’ve started exercising again, and the soreness I feel after a good workout is a welcome feeling because I know my muscles are rebuilding again. I continue to lose weight without even really trying too hard, and feel confident I will eventually get back down to my pre-Cushing’s weight. The weight isn’t coming off as quick as I’d like it to, but slowly and surely the scales are going down. I know if I stick with it, I will reach my goal.
I truly feel Cushing’s patients are some of the strongest people in the entire world. If we can conquer this disease, there is nothing we can’t do if we just have faith and believe. I’d like to say a special thanks to all my wonderful friends and family who taught me just that. Special thanks to my wonderful fiancé who was by my side every step of the way. Also, to my angel mother who made me never give up and fought this disease for me even when I felt like I couldn’t. The power of love really is a miracle in itself. For all of you still fighting out there don’t give up. When you feel like hiding and giving in to this awful disease, just think about all those people who love you and are in your corner. You’ll realize how lucky you are and you owe it to them to keep fighting. I am speaking from experience when I say it will get better. Don’t give up and don’t give in and when this is all over and you are looking back on it as I am, you will be a much better person for it.
Newsletter: Fall, 2007
An Update: Spring, 2009
When I finished writing this story over a year ago, I was feeling great and I hoped that I wouldn’t have to do any additions, at least not for a while. However, after marrying the man of dreams in August 2008, the man who stuck by my side through all of this, I started developing some frequent headaches. Nothing horrible, but growing ever more persistent. I had been slowly growing a deeper and deeper tan, so much so that I couldn’t go out into the sun for more than a few minutes without a high SPF sun block or my skin would turn REALLY dark. We went to the Dominican Republic for our honeymoon, and people thought I was a native I was so dark by the time we left. I always knew that there was the possibility of me developing Nelson’s Syndrome, but I always hoped it wouldn’t happen.
I pretty much knew going into my MRI at the end of September that my tumor had grown, especially after finding out that my ACTH levels had doubled in a matter of months. Sure enough, when the results of my MRI came back, we were finally able to see the little booger that had been evasive up until now. My adenoma was clearly visible at approximately 8mm located on what was left of my pituitary gland. My new endocrinologist (my former doctor went into research for awhile) along with my amazingly talented neurosurgeon, as well as the radiologists agreed that I should give a second transsphenoidal surgery a try. They felt that with my age, desire to have children, and current condition, it was the best choice for me. The neurosurgeon felt he would have a good chance for success this time, especially since the tumor was now visible. He said that as long as when they got up in there and there was a clear difference between what was normal tissue and what was tumor, he thought it would be very likely the surgery would work and he would be able to remove the tumor. I had grown to really trust my neurosurgeon and believed that this was indeed the right decision for me.
Everything happened pretty quickly, and I was in the hospital awaiting surgery on the morning of October 15, 2008. There was a delay in the start time, as the previous surgery had taken longer than expected and we didn’t have a room. They finally arranged for another room, and I was wheeled on in to have my surgery. I awoke in the recovery room to find my husband waiting there for me to open my eyes. I knew immediately, I just had this feeling that was different from my first transsphenoidal, that everything had been successful. I was thoroughly amazed at how well I could breathe this time around! I wasn’t stuffy at all the way I had been the first time around. I didn’t even have to go to ICU, I went straight to my private room. The neurosurgeons came around the following morning and said that the surgery went remarkably well and I handled it like a champ. They said it didn’t even look like I had had surgery. I told them that it really didn’t feel like I had. They said that because I already had this done before, they used the same pathway, through my nose, and it wasn’t near as intense since the hole was already there. Since I had the same two surgeons both times, they knew already how they had done the first one, so they were familiar with my nose and head. I was up and walking around and everyone – doctors, surgeons, nurses, physical therapists were amazed. Everyone could see that I was ready to go home. I was released early that evening after only a little over 24 hours since my surgery.
The recovery at home was very easy, I was only off work for a few days, just to gain my strength back and make sure everything was indeed okay. My post-op blood work showed a significant drop in ACTH levels indicating that the surgery was indeed successful. My post-op MRI looked great as well, no signs of tumor. Of course, we can’t be 100% sure that the tumor is completely gone, and that it won’t grow back, but that is what we will hope for. In the meantime, I am so happy, healthy, and grateful to be alive and enjoying life. Things really got better for me after I had the BLA. I haven’t had any problems since then, no adrenal crisis (knock on wood!), nothing. I would do it again in a heartbeat. I have gotten back down to my pre-Cushing weight and have never felt better. I will not live each day worrying about what could happen, I’d rather focus on everything good I have right now. …and I’d say, that’s a lot!
I’d like to send my deepest thanks and appreciation to the absolutely wonderful Pituitary Team at Johns Hopkins Hospital. They are some of the most amazingly talented, intelligent, and kind doctors that one could ever wish for. I wouldn’t be sitting here today so healthy and happy without them. I’d like to send special thanks to my endocrinologist, Dr. Salvatori, who always takes such good care of me, and my incredible neurosurgeon, Dr. Olivi, who I trust with my life! You are both my heroes.