Thursday, August 17, 2017

day +226 post hsct update

I am so happy to report good news finally! They say the first year post HSCT is a rollercoaster, and boy were they right! This roller coaster had all my doctors, and myself convinced too. Convinced I had headed down the road to relapse. Little did we know I simply received a different gift than most patients who previously received the transplant. While many patients see an almost complete remittance of symptoms and halted progression, my refectory to treatment case of scleroderma seems to have only slowed. My symptoms are still progressive, however they are greatly slowed compared to their trajectory pre-transplant. I will take that over being dead for sure! lol Not to mention I did see some vast improvements symptom wise, even a reversal of specific symptoms! Yes, some of the symptoms I saw disappear have returned, but we aren't gong to call it a relapse just yet since they are reoccurring at a slow pace. My team is reevaluating at a year post HSCT both in Chicago and here in Seattle. So, lets divulge into the last month shall we?

July 

  • The new medication my Digestive Disease Specialist prescribed for my Gastroparesis (GP) finally arrived; Domperidone. Domperidone is a prokinetic drug, which is fancy terms for a drug that increases gut motility by increasing frequency of muscle contractions, or making them stronger. It is a miracle drug, similar to Reglan, for so many patients in the GP world. Unfortunately, I didn't see much improvement from this drug even though I was taking it three times a day. It works with a very specific receptor in the brain, which lead us to believe maybe my GP wasn't stemming from that specific receptor. After some research we traced back my GP symptoms to when I stopped my antidepressants about 5 months post transplant. Turns out 95% of serration is found in the gut, so a sudden depletion of serotonin could definitely aggravate GP. We decided to start Mirtazapine, a tetracyclic antidepressant which has shown in many case studies to be a very useful drug for GP patients since it boosts appetite, and curbs nausea/vomiting. Well, it worked! It worked almost immediately! I took it in the evening, and woke up able to eat a whole bowl of oatmeal! HAPPY TEARS. My doc explained that since I had been on antidepressants for so many years, I probably had GP for the same amount of time my esophagus had not been working, however no one ever clinical caught it since I was taking a drug that was helping my stomach move by chance. When I stopped the antidepressants 5 months post HSCT, the GP flared. So the GP isn't really a new issue post transplant, it is simply an issue that was going untreated very suddenly. So for now we are postponing GJ feeding tube surgery to see just how well this new drug will support my unhappy stomach! In a few days I should be able to remove my NJ and sustain completely off oral intake! ...I am actually thrilled to be back on an antidepressant. I wasn't doing so hot emotionally the last few months, so it was a welcomed addition.
August
  • I saw my Scleroderma Specialist who seemed happy with my current progress. Last he saw me was in May when things were going horribly awry post transplant - my skin score had increased, my joint swelling had worsened, my lung function decreased, and my stomach crapped out! Luckily, this appoint was full of smiles, and good reports! My Modified Rodnan Skin Score, a test where the physician pinched areas of your skin to test for thickness, showed my skin drastically loosened since last time I saw him! I am now an 8, as compared to 13 in May! My Echocardiogram, also known as a heart ultrasound, reported a stable Ejection Fraction of 60%, and a normal pulmonary pressure of 20%! That is all amazing news! Both results were the low end or normal - and I couldn't be happier! My Bone Density Scan did show some bone loss, with most bones registering around -1.7. This level indicated Osteopenia which can often times be treated with supplements such as Calcium, and Vitamin D. I was prescribed a heaping helping of both. It is something we will have to keep an eye on, but for now, it's manageable. My PFT, aka Pulmonary Function Test, showed comparable results compared to the PFT at my six month follow up in Chicago - which were decreased from pre-transplant. My DLCO was siting at 59% this time around. My doc told me while it is lower than he would like post HSCT, he believes they will rise! While the PFT wasn't ideal, my docs are considering it stable enough to not cause immediate alarm, and to simply repeat the test in the next few months. Because of the improvements made since my last appointment in May, he decided that I did not need to restart immunosuppressive treatment at this time! I am thrilled!
  • I saw my Pulmonologist to review the recent PFTs, and he had the same thoughts as my Scleroderma Specialist. Not ideal post HSCT, but not bad enough to cause immediate alarm. He suggested another PFT, and a CT in October to look for a rise in my DLCO trends, and to rule out any new fibrosis, or infection. It was the first time I had seen him since my transplant and he couldn't believe how good my skin looked! He just kept staring at my hands and saying, "wow!" 

All in all I am doing ten times better than when I stepped on that plane to head to Chicago for my HSCT. Is life perfect? No. Am I healed? No. Is my disease in remission. No. However, my life is absolutely more livable, and my disease has slowed its rapid progression. That I can manage. I am happily working full time, and just moved to a lovely new home. I have two cats I adore, and a husband that loves me. Life is good; I am doing the best I can to live in the now and be present for whatever extra time this treatment has given me. If you see me out dancing, attending events, or simply smiling wearing makeup, it's not because I am "healthy," it's because I savor each precious moment in life. So instead of saying, "I am so happy to see you so healthy," maybe just says "I am happy to see you happy," because that is the simply truth. 

P.S My hair is crazy and I don't know what to do with it! lol It sticks straight up. I am a hedgehog. 

9 comments:

  1. Love the hair it is uniquely you. Yes be happy love your cats, that handsome Noel and enjoy your new place and your job. And love the life you have.
    Love you to bits.
    Linda Maurer Hoffman

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  2. Oh, what great news particularly about the GP and the antidepressants!!! Man, what a catch to actually figure that out - I am SO STOKED for you to have a chance to be tube-free for a while longer and be able to keep eating. Best of luck that things stay as stable for a good chunk of time. <3

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  3. I'm glad you are happy Channel!

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  4. So happy to hear your good news and was very interested in the information on gastroparesis. I was diagnosed with this condition several years ago and have tried multiple medications without success. I have Multiple Sclerosis in addition. I will certainly look into Remeron! Love your outlook and joy in the moment!

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  5. I have followed you ever since I found I was sick. Please, if your stomach issues resolve - will you please look into the Magnesium Advocacy group on Facebook? I think it would help you and maybe stop this for good. I don't want you to enjoy the time you have left - I want you to live the rest of your life like this autoimmune condition isn't a thing anymore. Please please look into it. This is a total stranger saying they care so much. Please give it a shot.

    Lots of love,
    Brooke

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    1. Hello Brooke! Thank you so much for reaching out. I will absolutely look into it! :)

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  6. Wonderful update Chanel! Roller coaster be damned, I'm happy to see you happy. xoxo

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  7. So glad you have had some better news head your way, and some answers resolved. And yay for new starts and happiness!!

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  8. Hi Chanel. I have a friend who also has schleroderma. She is interested in the clinical study in Chicago. Do you know where she could find information about it? Thanks. Emmy

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