Yesterday morning I got up bright and early to find SNOW! Pure white outside. Trees glistening. Perhaps that was the first sign of good things to come? I do love the snow - well, perhaps it's the temperatures that come with the snow that I really appreciate most! Cold weather - my fave!
So I went to the hospital for blood work, a morning ACTH and cortisol and to check on my liver as I'd planned to start up on keto again. I received a call in the afternoon from my nurse.. and.. <drum roll>.. my cortisol finally broke! The last few labs have shown that my ACTH was coming down, but my cortisol was being stubborn and hovering around 900 still (norm 50-300) and this time it came down! My nurse also told me that my liver was finally starting to look better too. My enzymes were all up, and this was the first time I've shown improvement there in a very long time! Wow, good news! So he said to wait until he talked to my endo. Then today I received another phone call back and she said.. "no keto!".. my cortisol was 665 and we're hoping that it's going to continue the downward trend, and so she wanted to just wait and see what happens. She wants me to do a UFC though. :( Boo! I think that could be my most hated test in this disease!! Give me needles, shove me in tubes, just don't make me do a UFC!! :P So as soon as I get the req, that's what I'll be doing. Ho hum.
Yesterday I also received a phone call from John at Dr. Ludlam's office in Seattle where he runs the Swedish Neuroendocrine Program with Dr. Mayberg. I had sent my info pkg directly to Dr. M and apparently he read it and passed it off to Ludlam. Then today I had a phone appt with Ludlam!!! We spoke for 45 minutes, I was quite shocked! He said that Dr. M thought that I was possibly a good candidate for SOM230 the drug that Ludlam is trying to get FDA approval for, however I learned that I couldn't get into that trial. Ludlum started off though by saying he thought my Cushings was mild now. He tried to do a quick conversion of units in his head and said my cortisol is about 26 with the high end of normal there being 21, so I wasn't too much above normal. He said that they usually use UFC's to keep track of level there and doesn't like how Canada and other countries use blood. He asked about my drs and how I'm feeling, what hormones I'm replacing, about why I had radiation first instead of a transsphenoidal, and many other things. One thing he was especially bothered by was the fact that I'm not on GH (growth hormone) replacements. I told him my endo didn't believe in them, plus we also fear making any tumors grow, and he told me about research discrediting that belief. He said I really need to have GH and that my lack of it could be contributing to a lot of how I'm feeling which I've always just blamed on the cortisol. He said he would happily talk to my endo if she was agreeable to tell her the importance of GH.
Then we discussed possible brain surgery. He and Dr. M feel that brain surgery IS possible, but they agree that I've had too much radiation and that the texture of my pit and surrounding area is very altered due to the radiation, however that doesn't make it inoperable, just makes it very high risk and dangerous. He said that he's certain M would be more than willing to go in and try if I wanted, however he could almost give me a 100% assurance that I would end up with a CSF (cerebral spinal fluid) leak which could mean weeks up to months in the hospital. He said I wouldn't be able to travel home if this happened and I would have to stay until they got it under control and this would ultimately mean 100's of thousands of dollars out of pocket for me. He said the hospital doesn't have any type of programs that I would qualify for so the cost would be mine entirely. And, well.. there's no way of that! All that aside, he said there'd still be a chance of M missing some of the tumor, or that he could get it all, I could have a year of feeling somewhat better, and then it would come back again and a surgery may not be possible then, and I'd feel just as I do now. He just really felt that it wasn't worth it, but IN HIS OPINION - NOT AS MY DR, he would not recommend it. In the end, I'm inclined to agree with him, but I still asked to speak with M to hear it straight from the horse's mouth. I wouldn't feel right just taking the info second-hand as the whole purpose of my mission here is to actually hear "yes" or "no" from all of these drs. So now I wait to hear from M, but especially after being turned down my Dr. Q at Hopkins, it's really not sounding like surgery on my brain is a reasonable option anymore. Ludlam said he also believes I've long ago exceeded my lifetime recommended doses of radiation, so doesn't feel that is a viable option either. I guess my neuro was telling the truth - not that I didn't believe him - but I needed reassurance.
So Ludlam's first choice would be a BLA. I told him my concerns about Nelson's and he feels that my risk is no higher than anyone else's at this point. He said my age is a factor, however with the amount of radiation I've had - there was studies and reports saying that radiation makes the environments non-conducive to tumor growth and so I have that in my favor. In some instances, people will have their adrenals out (a major cause of Nelson's) and then have radiation of the pit/sella JUST to avoid Nelson's tumors because the radiation keeps the tumors away. And with the amount I've had, tumors would practically run the other way before trying to settle in my head! He thinks my odds of Nelson's is about 30%. That still seems like an awful lot to me considering that I can't get any surgeon into my head NOW, how would I get one there later if I was in that 30% and actually got Nelson's??? He saw my point on that one, but he said he still tells all of his patients that if pit surgery doesn't work, a BLA comes next. (However, once SOM230 is released, he plans to put SOM ahead of a BLA as his 'algorithm to treatment'). He tells everyone (and it's in his video! http://www.youtube.com/watch?v=jVSdYYmRqvM and you can also see Dr. Mayberg here: http://www.youtube.com/watch?v=-OLLq6gvUCg&feature=results_video&playnext=1&list=PLEE2AFFA4074EE768) that when you treat Cushings, you trade one disease for another - Addisons and possibly a side of Nelson's, but he feels that treating Addison's is on par with diabetes. Diabetics have meds, needles, tests, and drs to deal with, but they lead otherwise normal (or their new normal), most healthy lives. He feels I could and I SHOULD have this too.
I told him about my taking cabergoline right now. I told him how it was pure pure, evil, deathly hell to start taking it, however it's (or it in combo with my radiation - we'll never know for certain which is working because the two can never be separated) allowed me to feel the best I've felt since becoming sick. He said he's tried caber with some patients, and never seen overwhelming results. Some people improve slightly, but never to the point of returning to normal life, and never feeling "good" again. He said that for as much as I think it's working, I wouldn't be looking for help if it really was - good point! He also said that for some reason, other countries, as in non-USA countries report higher instances of it working successfully, but he's never been witness to that finding in his years, and that's why he rarely prescribes it anymore.
He's also been working closely with mifepristone, but his thoughts on this one are pretty much the same as mine. Mifepristone works on the adrenal/cortisol level the same way as how keto works. And for keto he said it was too toxic (to the liver mostly) to use it how I would need to use it. With these drugs, your body basically still will make cortisol, from my understanding, but the drugs just prevent your body from using them. This however will fool your brain/pit into thinking there's not enough cortisol causing your ACTH to rise which is the beast behind my disease now. As he explained it to me, SOM and even caber work centrally on your brain, they work on the receptors to block ACTH production which in turn halts the production of cortisol. So SOM and caber work centrally, mif and keto work peripherally and we were both in agreement that my case calls for one that works centrally otherwise I might as well just have a BLA right now because it would be doing the same thing as mif/keto - making my ACTH go up!
So that being said, the next topic was Pasireotide aka SOM230 aka SOM. I've heard about it, researched it and only recently learned that he was so involved with it. The FDA recently turned them down for release for whatever reasons so it's been held up. I forget exactly, but I think he said it was just this week that they finished signing the paper work to start up another phase 3 trial. Due to the time that's been wasted though, they signed up this time WITHOUT "compassionate use" which means people like me have been excluded from this specific trial - aka, people who've had radiation already. If I didn't have radiation, I could have come right away and been in!!! He said they just signed up two people from out of the country even! Dammit! But he said he's 100% on board with SOM. As mentioned above, he plans to have it as the first course of treatment after unsuccessful pit surgery. In the past year of the trial, 80% of the people achieved effective results and most had that occur after only 2 months of treatment! Sounds as good as I've been reading! He said the biggest concern with it right now however is that it raises blood sugar and by the sounds of it, some people quit the trials because of this. I'm assuming this could be why the FDA isn't gung-ho about it. Another concern is that he pretty much only uses UFC's as a measurement, and some (including my own dr) don't feel UFC's are the best measurements available. But regardless, I think it's MY best option for right now. All meds, all surgeries come with side effects and risks.. I'm just to the point now of deciding which crappy side effects and risks I'm willing to take, because if I avoid all of them and sit back quietly, I'm no further ahead and I'm not LIVING.
Ludlam told me to email John saying that I authorized them to give my info to Novartis though and he would talk to some of the big wigs there to try to get me SOM! Most likely it'll just be to get me into a trial where they have compassionate use. He said there's many trials going on allover the USA, so hopefully there was one a bit closer to me. He said travel costs would still all be mine - I said I would make it happen! If I have to throw myself a damn fundraiser or a social (it's a regional thing here, basically a fundraiser, people buy tickets, you get cheap alcohol for people to buy drinks and get drunk, you ask for prize donations and people buy tickets to win stuff, etc) I will just damn well do it!!! But SOM really is my BEST option for now. I always still have a BLA as an option down the road, and by the end of our call, Ludlam was agreeing with me for the most part. He kept reiterating that "I am NOT your doctor, and this is NOT official advice, your doctors know what's best for you, and I'm just speaking with you because you've asked for help and my opinion and advice!".. I kept saying "I understand! I know! Thank you!".. but he kept repeating it so I knew that his advice was not the law to be followed!
The end result is that he's going to contact Novartis for me, probably by tomorrow or the next day and I should hear from them. Dr. Mayberg will call me to offer his opinion. I told him I would be doing a UFC soon and having a 3T MRI, so he said I could forward those to them. And he thought it would be good (as he thinks it's one of the best tests for Cushings) if I did a dex/CRH test (which I don't even know what that is!) and get those results to him as well. He said he was more than happy to talk to my endo, but has found most endos don't like talking to him, haha!! I'll be emailing my endo with this update and we'll see what she says! She said she was willing to help me get SOM, so hopefully she'll be inclined to listen to what he's got to say, and who knows, maybe I'll get some GH out of it all too!
Good news! I felt quite educated, rejuvenated, and hopeful after we hung up. I feel like a pit surgery probably isn't the ideal thing and that perhaps I should not even bother worrying about trying to send everything to Dr. Jho, Dr. Laws, Dr. Gentili? I'm not sure. I'd still love to hear from McCutcheon since people speak of him like he's the 'god of pit tumors', but so far no response!! I'm pretty certain I want to try SOM230 before trying any type of surgery though. Ludlam also said that for those who stopped taking it, everything reversed (including side effect and initial symptoms) so I feel like it's risk very worth taking! I really really hope I get good news about getting into a trial! I WANT TO GO!!
Tonight I breath a sigh of.. real HOPE! It's my life.. I'm sure as hell fighting!
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Here's some more info about SOM230. This reports 26% positive findings. Perhaps Ludlam over stated, but it's his 'baby' so I forgive him that (or I misunderstand maybe? I dunno!) http://www.prnewswire.com/news-releases/novartis-drug-som230-is-first-medical-therapy-to-show-efficacy-in-a-phase-iii-trial-in-cushings-disease-a-debilitating-hormonal-disorder-103503689.html
And here's another link to some more about SOM230: http://www.novartisoncology.com/research-innovation/pipeline/pasireotide.jsp?usertrack.filter_applied=true&NovaId=2935376892996810584
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