So here begins my story of my fight with Cushings Disease!
History and Time Line of Symptoms and Treatments:
2002-2004
- Started to see weight gain. My height was 5’10 and I had maintained a weight of approx 180lbs (okay, so I was already chubby, but maybe Cushings had started long before I even know?) for a few years, however my weight started going up and I was about 240lbs and much more now!! (Not saying!)
- I started to have problems with my periods being less regular, more cramping than usual.
- Severe acne after never having any skin problems from early teens until this time.
- I began having problems with my blood pressure and was ultimately put on Spironolactone followed by trying a couple other blood pressure medications which I stayed on until after my first gamma knife surgery.
- I had severe swelling in my feet, ankles and calves to the point that it was extremely painful and sometimes I could not even put on proper shoes.
At this time, I had been under the care of my family physician who treated me for the high blood pressure and instructed me to lose weight in order to resolve my symptoms. When this didn’t work, and I continued to gain and eventually lost my periods altogether, I was referred to an endocrinologist. I went through testing for blood diseases, PCOS and some other basic testing and my endocrinologist at the time told me that I most likely had microscopic cysts giving me PCOS, and again I was instructed to lose weight, stop lying about what I was eating, see a psychologist and exercise more.
December 2005
- Still had all the above symptoms with continued weight gain.
- Now was plagued by severe debilitating headaches. Generally speaking, I’d never had headaches in my life until now. I was seen in the ER where they did a CT scan and I was told that they found “an unusual clump of cells” but was also told that in some people’s brains it just looked this way, and I wasn’t to be concerned. I was treated with IV narcotics and sent home with morphine to control the headaches. No explanation was given for why I was having them, however I personally believe my blood pressure was somehow involved because movement and any change in elevation from laying to sitting or standing exacerbated the pain greatly. The headaches got better for awhile.
- I began having vision problems. I noticed that starting around 4pm my vision would become very blurred. If I had a headache it would be even worse. Around 11pm-2am, I would usually get a bit of a reprieve where I would have extremely clear vision, and then it would start to dull again. I wear glasses all the time, but still had these changes daily. By the summer of 2006, my vision was so bad, I could rarely drive anymore, and when my vision wasn’t completely blurred, I suffered from double-vision. I also began to just see shadows and could no longer clearly identify objects unless they were right in front of my face.
August 2006
- The headaches I’d been having became extreme again. I had to take sick-leave from my job as I could no longer handle the pain, I also had extreme difficulty seeing my computer screen in front of myself, and I was suffering from extreme dizziness to the point that I often felt like I would pass out. I no longer felt safe to drive myself to and from work and eventually I was forced to give up my license altogether.
- My mom came across an article about Cushings Disease and after doing research, I took information to my family physician and asked to start testing for this. After the very first blood test, it was pretty clear that cortisol was my problem. I was sent back to my endocrinologist again with the possibility of Cushings as my diagnosis, at which point she drew 2 am/pm bloods. Then she told me that wasn't diagnostic so I did a 24-hour urine and a saliva tests for cortisol, and all of them came back extremely high. My endo refused my calls after these labs were done, and when I finally had a response from her, it was October that she finally put it into writing that Cushings was now mine. Mine to deal with, mine to fight with, I have Cushings Disease. Now I just needed a referral to a neurosurgeon and that alone was a fight.
December 2006
- I FINALLY received my referral to a neurosurgeon! By this point, I truly feel like I was near death. I could barely care for myself any longer. I had been having a hard time sleeping for a long time, staying awake on average 20 hours a day, however by now I was sleeping 22 hours a day. My mom would wake me up to eat and take meds. The rest of my symptoms included:
o Continued weight gain especially around my mid-section
o Continued debilitating headaches
o Dizziness
o Flushed red skin, my face, chest and arms were always bright red in color
o Excessive sweating and hot flashes regardless of environmental temperature
o Swelling across my whole body, I could barely close my eyes and mouth due to swelling,
could no longer wear rings, necklace, watch or bracelets, could not wear normal shoes because of foot swelling
o Total body pain, especially in my lower back
o Extremely high blood pressure, taking two meds and still unable to control it
o Bruising all over my body with the worst being on my arms and abdomen
o Purple striae on my abdomen, forearms and shoulders
o Severe acne on my face
o Extremely blurred vision or double-vision at all times without reprieve
o Nausea or no appetite
o I had some cuts and sores that were open and would not close for the past year
o No menstrual cycle
o Extreme fatigue and muscle weakness to the point that even brushing my teeth and dressing myself became too hard to do
- On December 4th, 2006, I had my first meeting with my neurosurgeon. I walked into his office , sat down beside him at his desk, he put a hand on my knee and said "I can't believe you're still alive!" Based on my scans and labs, he was shocked that I was still among the living. It was determined then that I was much too sick to undergo general anesthesia in order to have a transsphenoidal surgery, and there was also concern of missing some tumor tissue. We agreed that although it would take longer to see results, that gamma knife was my best option. From the latest scan, it was estimated that I had 30-50 microadenomas on my pituitary, and that it was unlikely that any of my pit could have been saved if a traditional surgery was performed. So I was told I would go for gamma knife surgery. On December 7th, 2006 I went for my first round of gamma knife. I received 27Gy during this treatment. I first started to see results about 3 weeks later when my swelling started to resolve slightly. By approx. 2 months post-op I was able to come off one blood pressure medication and by 3 months I was off the second one and have not required any more since then, although at times my blood pressure has been slightly elevated.
April 2008
- Although some symptoms had improved, I all of a sudden began gaining weight again and feeling worse – lab tests showed that my ACTH and cortisol were rising again. I tried Ketoconazole as we hoped that the radiation would continue to work, and with a bit of help from the keto, that I would continue to have good results, however this wasn’t the case and my numbers all continued to get worse.
November 2008
- It was very clear that I was becoming sicker by this point and my neurosurgeon and endocrinologist agreed that it was time for a serious intervention. My CT scan showed a suspicious spot measuring 4mm, and so I was headed for gamma knife again.
December 2008
- December 11th, 2008 I had my second course of gamma knife surgery. The whole pit and sella were targeted at a dose of 35Gy, I was told that this high of a dose has never been given before, and it was enough to kill a heard of horses! It was sure to work!
February 2009
- I finally began seeing my numbers drop and had started to lose a small amount of weight. No other major changes were seen. My thyroid dropped off which was suggestive of the gamma knife working and I began replacing with Levothyroxine.
April 2009
- Cortisol began rising again. Tried Ketoconazole (up to 1200mg/day), Mitotane (up to 3500mg/day) and a combination of the two. I achieved impressive results with lowering my cortisol however my ACTH began to rise, so we eventually aborted the Mitotane and continued with Ketoconazole.
July 2010 - Present
- Started taking Cabergoline and currently am taking 3.0mg three times a week. I have had mixed results on this medication. My cortisol and ACTH have both come down, then gone back up again and I have not really seen any symptoms get better or go away to date.
Sooo... wishing you hadn't asked yet? Well, I guess you didn't, lol.. but if you've read this far, you must be curious!! The above is part of what I sent in the package to neurosurgeons, 11 in total.
With everything that I've tried.. it hasn't worked. When I first was diagnosed and we discussed treatment options, I was told that once I had gamma knife surgery, it wouldn't be an instant fix, but within a year I would be back to my life. I would be back to work, I would be back to school, I would finish my psych degree and would be living life as though nothing had happened. Boy were they WRONG. A year came and went, and as mentioned above, during that first year post-op, I DID have a few improvements and was hopeful when told, "well, maybe another 6 months, it takes time and gamma knife can continue working for up to 10 years".. so I patiently waited. My employer kept calling every 3 months to check on my progress and I kept extending my sick-leave, still hoping. By the time of the second gamma knife, I began losing a bit of hope and soon my employer stopped calling and I was formally "let go." But I continued to fight.
Now, after all the surgeries, and all the meds, I'm still NOT better yet. I finally mustered up the courage to take things into my own hands a little bit. I made the decision that I didn't want my friends and family standing around my grave site saying "well! she just gave up and threw in the towel!".. no, I want them instead to say "she did everything she could! She fought 'til the bitter end!" I want my friends and family to be proud of me. I want my life to have meant something, even if it was just to teach others how to cope with a horrible disease.
1) Dr. Ian McCutcheon - M D Anderson Cancer Center
2) Dr. Daniel Prevedello - OSU Medical Center
3) Dr. Fred Gentili - Toronto Western Hospital
4) Dr. Alfredo Quinones-Hinojosa - Johns Hopkins
5) Dr. Marc R. Mayberg - Swedish
6) Dr. Jho - Allegheny General
7) Dr. Delashaw - OHSU
8) Dr. Edward R. Laws - Brigham and Women's Hospital,
9) Dr. Marvin Bergsneider - Ronald Reagan UCLA Medical Center
10) Dr. Brooke Swearingen - Massachusetts General
3) Dr. Fred Gentili - Toronto Western Hospital
4) Dr. Alfredo Quinones-Hinojosa - Johns Hopkins
5) Dr. Marc R. Mayberg - Swedish
6) Dr. Jho - Allegheny General
7) Dr. Delashaw - OHSU
8) Dr. Edward R. Laws - Brigham and Women's Hospital,
9) Dr. Marvin Bergsneider - Ronald Reagan UCLA Medical Center
10) Dr. Brooke Swearingen - Massachusetts General
11) Dr. William Chandler - U of Michigan
To be honest, I didn't expect much response if any at all. And when I did hear from anyone, I figured they would be saying the same things I've already been told. My neuro also turned me into a case study even. He personally called me at home one Sunday afternoon to ask my permission. He took my case to a National conference and reported back to me stating that everyone at the conference had agreed with his assessment. I later found out from my endo that he also took me to an international conference with the same results. But anyway, I had to find out and ask for myself, so I sent my packages away on October 21th, 2011. I included a cover later, 4 pages of the above history and time line, my last two sets of labs and my MRI disk from May. Then, I sat and waited!
To be honest, I didn't expect much response if any at all. And when I did hear from anyone, I figured they would be saying the same things I've already been told. My neuro also turned me into a case study even. He personally called me at home one Sunday afternoon to ask my permission. He took my case to a National conference and reported back to me stating that everyone at the conference had agreed with his assessment. I later found out from my endo that he also took me to an international conference with the same results. But anyway, I had to find out and ask for myself, so I sent my packages away on October 21th, 2011. I included a cover later, 4 pages of the above history and time line, my last two sets of labs and my MRI disk from May. Then, I sat and waited!
October 28th, one week later, I heard back from Dr. Gentili in Toronto! He emailed me saying that he thought he might be able to help, but that he needed a 3T MRI and that once I have it I should bring it and come see him in office! HOPE! Real HOPE!
November 1st, I received a phone call from the NP at Dr. Quinones' office - Johns Hopkins! She said she just received my package, read through it and that they're interested in helping me. Dr Q (as she called him!) would look over my MRI more thoroughly and I should hear back th/fri. She asked if I had plans to come down there right away and I told her that if Dr. Q felt he could help me, I would certainly be interested in coming. So at this point, I'm still waiting to hear back.
November 2, I received an email from Dr. Brooke Swearingen - Massachusetts General, he asked for my inital MRI and Labs from before treatment, and he's interested!
November 3, email from Dr. Laws in Boston! This one was less exciting and more of what I expected. "Thank you for sending along your clinical notes and MRI scans regarding your difficult situation with Cushing syndrome. It appears that you continue to have elevated cortisol levels despite two episodes of treatment with the gamma knife You have developed the anticipated pituitary insufficiency otherwise. I believe you know how critical it is to make sure that your hormone replacement therapy is accurately and carefully managed. The MRI studies show a secondary empty sella. It is possible, but not certain, that there may be small amounts of pituitary tumor tissue at the base of the sella. Your situation is such that additional radiation therapy is not a reasonable strategy." He went on to tell me about the clinical trials for mifepristone (I have no interest in this, it's "the abortion pill" and much the same as keto) and for Pasireotide, aka SOM230 which I'm VERY interested in!! He said I should try them and if they didn't work, I would need an IPSS and then he would consider surgery. So overall, it was much like what my local neuro has said and what I expected to hear.
Today I saw my endo. I needed a req for the 3T MRI and she was the only way I could get it. Boy was she pissed off! I never consulted her before seeking out another neurosurgeon's help, and she was deeply offended! She started off saying that she had planned to tell me I was fired but once I explained and sucked up, we came to an understanding!! OY! And she ordered the 3T MRI for me! Yeehaw! Part of it was because she thought I was also in the market to replace her, I think since Dr Gentili - the one who wants to new MRI apparently ONLY works with his own endo on cases like this. I don't know. But in the end, it worked out. I told her that I contacted them all on a whim, not really expecting to get such a response. I told her that this is MY LIFE and I don't want to end up on my death bed with people saying "well, she didn't even try!".. I want everyone to know that I've done everything possible and I told her that if every neuro says "NO!" to me, then my only choice left will be a BLA down the road, but I refuse to go for a BLA until I've exhausted every other option first. I also told her that I KNOW I need to try keto again because I'm too high right now, so that pleased her since she wasn't agreeable to me quitting this past time. And then once we had our little chat, and I apologized and sucked up, I asked if she would still be my endo even if I did get somebody to help me, and she said that so long as I kept her in the loop from now on, she would still be my endo.. and this is good. For all the bumps I've had with her along the way, she is a thousand times better than the endo I left before her, and she's also better than all the endos I hear about. I have a lot of respect for her, and she's been very willing to let me try different things and she's accepted my refusal for a BLA and helped me anyway, even though if she had her way, I would have had a BLA already. So.. I think it's a WIN for team Beth!
So anyway, that's the gist of it! Through this blog, I plan to keep track of things as they progress. Mostly for my own knowledge, but also for anyone interested.
Please feel free to comment and ask questions - I really feel like I don't have anything to hide and I hope somebody will learn from my experiences. I also hope that my journey may save somebody else from experiencing the same things.
This is MY LIFE - I'm going to fight for it!
So anyway, that's the gist of it! Through this blog, I plan to keep track of things as they progress. Mostly for my own knowledge, but also for anyone interested.
Please feel free to comment and ask questions - I really feel like I don't have anything to hide and I hope somebody will learn from my experiences. I also hope that my journey may save somebody else from experiencing the same things.
This is MY LIFE - I'm going to fight for it!
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