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.


  • 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! 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.
  • 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 and a stable pulmonary pressure! 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. 

Wednesday, July 26, 2017

day +204 post hsct update

Remember when I said, "lets hope it isn't gastroparesis?" Well, turns out, it's gastropareis. Bummer. Unfortunately, the digestive issues I was hoping would chalk up to some sort of virus was definitely just a delayed gastric emptying flare, aka gastroparesis. This has lead my team to believe that my systemic sclerosis may once again be active, which would garner a diagnosis of relapse post HSCT. However, I am still holding out hope this is just a bump in recovery since I am so young post HSCT.


  • My gastric emptying study shows moderately delayed emptying. The nerve that tells the soft muscles to move, called peristalsis, is damaged - so it's not sending the correct signal to move food through to the intestines from my stomach. This causes my stomach to hold onto food longer than it should, equaling serve cramping, distention, bloating, vomiting, and nausea. Basically, the food stays undigested in my body until I eject it. Gross. Unfortunately, this requires the use of a feeding tube that bypasses the stomach so I can get proper nutrition. 
  • I saw my GI physician post GES, who wasn't fabulously optimistic I would get to continue my life without a permanent feeding tube. She schedule a GJ feeding tube surgery for about six weeks out, that way we have some time to trial a few medications. She prescribed a med called Domperidone, which essentially moves the stomach muscles for you; it also blocks the receptor in the brain that controls nausea. The medication isn't FDA approved even though thousands of patients use it for gastroparesis. So while I wait for it to ship from Canada, I am trying out Erythromycin - an antibiotic used to speed up gastric emptying. I take it around the clock, but especially before I try to eat anything. Unfortunately, these drugs are subject to the consumer, which means they may or may not work. So far, I have seen a little improvement from the Erythromycin, but not enough to eat an entire meal. Just a light snack, or drink - both of which have to follow the strict gastoparesis diet of low fat/fiber. I have experienced much less nauseas since starting the meds at least, so thats a win! Unfortunately, that means I am stuck with my NJ tube until surgery placement, or until/if the domperidone works. Urg. Chances are I will need the tube, and the domperidone, but in a perfect world, I would be able to eat and not need the tube! 
  • We also scheduled an appointment with my Pulmonologist, Rheumatologist, and Autonomic Specialist. Since my DLCO decreased by 22%. I will be repeating a PFT here with my Pulm, and following up to diagnosis whatever this issue may be. My Rheum will probably throw me back on some immunosuppressive treatments to hold off this damn disease until we have a better option, and my Autonomic Specialist can hopefully get my increased Dysautonomia symptoms under control. I'm exhausted mentally, but physically feeling okay!
  • I spoke at the National Scleroderma Conference in Phoenix last weekend. I always enjoy connecting with other patients, and hearing about all the new medical advances! 

I'm going to be taking a short social media break. I just feel I need to focus on other things right now. A little cleanse/detox from the turbulent online world is good for the soul sometimes. It kind of breaks my heart, since I absolutely LOVE connecting with other patients and my readers - but please understand, this is for my mental health. Transitioning back to the world of chronic illness after have such an incredible, trouble free 6 months has been mentally, and physically tasking - but mostly mentally. I will not be answering messages, or comments until further notice. I am so grateful for the love and support I constantly receive, but right now I just need to focus on what's best for my overall health. I may occasional post a quick update, but won't be divulging into replies. I promise I will be back soon! Thank you for your understanding at this time. 

xoxo Chanel

Wednesday, July 12, 2017

Day +190 post hsct update

The last three weeks have been a bit of an emotional roller coaster. I have been feeling so out of sorts - kind of like I am a puzzle piece that just can't find its place in the picture. I am happy with being a puzzle piece, but I desperately feel left out and confused. Does that make sense? lol Any-who, my body has been an absolute piece of work since mid Jun, so I actually have quite a bit to update. The last week of June I headed to Chicago to see my HSCT team for a six month follow up, and it was a raging disaster. Yikes.


  • The last week of June I trekked out to Chicago to see my transplant team for a six month post HSCT follow up. Remember, I signed my life away for 5 years to participate in this study, which means I have to return to Chicago once a year to update the doctor on my progress for research purposes. I basically got one piece of bad news after another the entire trip. I saw the researching physician, the pulmonologist, and the gastroenterologist. I also had blood work, a PFT, an EKG, and a last minute angio-CT preformed. My blood work showed my autoimmune markers didn't change a bit (ANA still tittering at 1:1280), my DLCO actually dropped 22%, my skin and raynauds somehow worsened, and my GI tract decided to crap out on the trip which resulted in many sleepless nights vomiting undigested food. Now to be fair, everything was going swimmingly for the first 5 months, but for some reason in June all hell broke loose. I started having a lot of nausea/bloating while eating, uncontrolled diarhea, and on the trip started throwing up about 3-4 hours after meals. My fingers noticeably tightened, and I just couldn't get my raynauds under control. To top it all off we, suspect aspiration. Basically, the whole trip consisted of everyone telling me how "unfortunate it was that the effects of my transplant only lasted 6 months." *Insert tears and eyeroll here* I am hoping this is just the "rollercoaster" that is so often talked about and not a relapse!


  • Due to the severity of the digestive issues my transplant team sent me back with loads of test to preform here in Seattle. The tests due to be completed are: an aspiration assessment, a gastric emptying study to asses stomach function, and a small bowel follow through to asses intestinal function. The tests will help us better understand what organs are malfunctioning, and what type of tube I will need moving forward - or if possibly, all these digestive issues are of a more transient nature due to a virus, or possibly even Graft Vs Host. (Fingers crossed for transient!) In the interim, my GI physician placed a NJ (Naso-Jejunal) tube. This is a feeding tube which is inserted through the nose, fished through the esophagus, past the stomach, and into the intestines. Due to my suspected aspiration she wanted a tube that bypassed the possibility of aspiration, while still providing nutrition until we have the situation assessed. Honestly, my digestion by that point had gotten so bad I welcomed the horrible little device. I was so light headed from malnutrition I was unable to drive myself around upon retuning from Chicago! It has given me the much needed nutrition I had been deprived of the previous two weeks. 
  • Monday I had the aspiration assessment preformed in fluoroscopy. The test was done by a speech pathologist who uses an x-ray machine to watch different consistencies of barium pass through the oral, pharyngeal, and esophageal phase of swallowing. Compared to a similar study I had done about two years ago my esophageal muscles had improved greatly! Liquid was able to pass through the esophagus unaided - While I still couldn't pass solids unaided, such as thick pudding super well, being able to pass liquid easily was a huge improvement post transplant! While the test didn't show any barium entering my lungs, my doc explained that it doesn't necessarily mean I am not aspirating. Unfortunately, these kind of tests are limited as food/drink in the "real world" is varied from that in a lab environment. The speech therapist did notice something odd though during the test, a violent Laryngospasm. For reference, the larynx protects the trachea from aspiration. Even though they couldn't see aspiration, my vagus nerve is clearly telling my larynx that I am in danger of aspirating, causing it to abruptly close as a defense mechanism. Yes, momentarily closing my airway. The vagus nerve is part of the autonomic nervous system which regulates unconscious actions such as digestion, heart rate, breathing, etc. The speech pathologist explained to me that I have Vagus Neuropathy, meaning I have damage to that system, causing overreactions. 
Tomorrow I have my gastric emptying study to asses my stomach motility. This is the gold standard test to diagnosis gastroparesis, aka stomach paralysis. This is often a comorbidity of scleroderma, and besides esophageal paralysis, is the number one reason a patient requires a feeding tube. I already feel my motility has improved a bit since we ordered the test a week ago, so here is to hoping all the vomiting and nausea was due to some crazy virus and not gastroparesis! However, gastroparesis can be a "flare" illness, meaning the paralysis can come and go - when caused by, you guessed it, a faulty vagus nerve! Guess I'll find out tomorrow! For more detailed and up to date information don't forget to check out my Facebook page!

Saturday, June 17, 2017

day +165 post hsct update

How insane is it that I will be 6 months post transplant in two weeks?! Im honestly having a hard time processing it! lol I am incredibly amazing how fast time has flown by, and how little I have been in a physicians office! Remember when I had upwards of 16 appointments a month! Yes, sixteen! I have only seen a handful of my doctors in the past six months, totaling about five appointments. Its a truly freeing feeling!

I realize post transplant, my blog posts have become so far, and few between, as I do not have frequent appointments to update my family, friends, and readers with. For this reason, I have been doing most of my medical updates via Facebook Live. To visit my FB page please click the link: I have decided to format my posts a bit differently moving forward, since there will not be as thorough of content. I will do more of the "Buzzfeed" format which consists of bullet points, and lists. Unless of course it is an opinion piece instead of an update. This will cut down on my time spent behind the computer, as well as your screen time as the readers. I would love your feedback on this new transition in the comments below!


  • I started a part time job! I am an office manager for a tech company in Seattle, and I absolute love it! My body holds up pretty well, as my hours are smack in the middle of the day. This lets me continue healthy sleeping habits for optimum recovery, while still venturing out of the house a few days a week to contribute to society! lol It is in the cannabis/tech space, which as you know is a huge passion of mine! It has been such a beneficial treatments for so many of my ailments, including: nausea, depression, anxiety, pain, etc. and is why I truly believe I have ket such a level head through my very difficult diagnosis. 
  • My counts still sucked. My WBC count and ANC were no where near where they should be. The team says I may just be a late bloomer...


  • May kicked of my summer speaking series! I presented an award at the Washington State Scleroderma Foundations Gala which was an absolute blast! I love taking part in this awesome event every year! 
  • I GOT MY PORT REMVED! Yes, you heard me, I am officially COMPLETELY medical device free! My transplant team decided it was safe to remove my port, so I jumped on it as soon as possible! lol The surgery was done wide awake in IR. I got to sort of watch, which was amazing! The numbed my chest with local anesthetic, cut me open, and pulled the thing right out! My surgeon was incredibly nice, and even let me keep it! Plus, I got to listen to the guardians of the galaxy  volume two sound track, which was a major plus! It was emotionally to see each small needle stick it the reservoir - each infusion, each round of chemo, each bag of saline that kept me alive was accounted for. The surgeon who removed it goes; "wow, that was a well loved port!" lol Yes, yes it was! It fearlessly took 3 years of treatment - however, I was not sad to see it go! ;) 
  • I had my first follow up appointment with my Scleroderma 
    specialist who referred me for the transplant. It felt so good to walk into his office without my feeding tube, oxygen, or port! It was especially fun to go over the medications with my nurse and exclaim, "No!" when she asked me if I was sill taking my laundry list of meds! lol I am dow to 5 medications, out of 17 pre-transpant meds! My doctor was still pretty bummed my transplant wasn't able to be preformed in Seattle with a few of his colleagues, but he was honestly just so happy I had it done, period. My darn organs prevented my ability to preform the transplant in Seattle, luckily Chicago accepts pretty heavily damaged organs! I of course was extremely excited to show him my progress, however he was cautiously optimistic, as he believed I may not be as well off as I was demonstrating. He felt I was a bit over-excited and expressing I was better off than I actually was. I assured him that wasn't the case, but upon clinical evaluation, he was right. My skin score was a bit worse in places, and he could still make me throw up in office with the touch of a finger! lol (Seriously, I have a spot on my abdomen that, due to my damages LES, will cause me to vomit when pressure is placed. It isn't cute! ;) He always has the trash can ready though!) He did however acknowledge the vast improvements I have seen, such as my ability to now swallow an array of foods (though not all foods!!), the loosening of skin on my fingers, and my ability to more easily breathe. Like I said though, he is very cautiously optimistic, as he wants to do a full organ rundown before he truly appreciated the benefits. I appreciated his dose of realism, as I probably need it! So in July I have quite a few tests coming up, such as: a bone density scan, a Echocardiogram, and an HRCT. My PFT will be preformed in Chicago during my follow up in June. 
  • I got my first cold and it kicked my ASS - Like royally kicked it. I know my team expressed that "normal people sick" post transplant would suck, but holy hell, I wasn't expecting it to be that bad! I was locked into bed for days, completely able to move. I had snot pouring out of my face, and was hacking a lung at all times! I was actually struggling to breathe so badly one day, I was worried my lung fibrosis was coming back, but it was just phlegm - hallelujah! Luckily I didn't end up with pneumonia, just a killer cold that took 5 weeks to dissipate! 
  • My labs still sucked, though my WBC count was closer to normal. My ANC still didn't want to budge. My team assured my by June they would be better!
  • Really, really horrible hot flashes, terrifyingly severe memory issues, and urinary incontinence began. 


  • June started off with seeing a physician about my hot flashes. Turns out my hormone levels are POST menopausal! Like, not even perimenopausal - my ovaries are that of a 55+ year old woman apparently. My FSH levels were extremely high, and my Estradiol was extremely low, indicated complete ovarian failure. So I guess my body dove into menopause hyper drive and tried blasting out the whole ordeal in a matter of weeks. It is literally awful. My hormones are so out of wack I am bawling one minute, and yelling at everyone I love the next. I ma not a very mentally stable, or kind person at the moment. It is exhausting not feeling like myself. I won't complain about no longer having persons though...  I have an appointment next month to chat about the possibility of hormone replacement. 
  • June 26th - 30th I will be in Chicago for my six month HSCT follow up. I will be preforming a PFT, a bunch of blood work, and seeing the researching physician - the one who saved my life with his groundbreaking trial. He and his team are saving lives that would other wise be lost! I get asked frequently about this trial, and if it would be right for other autoimmune patients. I do not have information on every autoimmune illness Dr. Burt works with, however; I do have information I can pass along to my readers about the Systemic Sclerosis Trial at Northwestern. Click the link here:  The link details the strict qualifications one must meet to qualify for the trial, as well as how to get a hold of the team if you and your doctor believe you are a good fit. Note, this link details the DIScl2011 regimen, and I took part in the ASSIST I regimen.
  • My counts are NORMAL! Happy dance! My WBC count and ANC are in the normal level, all be it low normal, but normal all the same! 
  • P.S. I have so much hair! Lol And by that I mean a very short pixie with no bald spots. 
  • I am dawn to two medications! OMG it feels so good to be dropping pharmaceuticals left and right! 

I am so excited to attend the Scleroderma Foundations National Patient Education Conference next month! I hope to see many of you there - Please come say hello so I can give everyone hugs! lol 

Monday, April 3, 2017

day +90 post hsct update

If you follow me on Facebook, you know I am much better with updates on that platform! lol I am sorry my blog has been the recipient of so much neglect. Honestly, I have been busy living - truly living! I even start a (very) part time job tomorrow guys! Currently, outlets like Facebook, and Instagram seem to be an easier, quicker mode to update my readers. Plus, chemo fog is still incredibly thick, and busting out a blog post isn't as easy as it used to be - darn chemo brain! lol I'll take it over the oxygen and feeding tube any day though! ;)

My last update was way back at day +50 after a few boughts of CMV. I am happy to report the CMV hasn't come back, however, new problems seemed to arise in the past 40 days. My bone marrow stopped producing for close to 3 weeks, which caused my counts (blood levels that indicate my immune system strength) to plummet back to non-existent! It had my transplant team pretty worried. Talk of starting an emergency treatment such as IVIG was being thrown around. Yuck! Luckily, we figured out the low counts were due to a medication I had been taking that, in a small number of patients, can suppress bone marrow production. Since I discontinued the medication my counts have begun to rise! Woohoo! I am still no where near having a normal immune system, but it's better than none at all! I got myself some delicious ice cream to celebrate.

I had the absolute incredible opportunity to film with the BBC for a documentary series called Amazing Humans. The videographer flew all the way from London to film a woman named Sara, who provides free Henna Crowns to patients suffering hair loss. I was lucky enough to be the recipient, and share a bit of my story! Her kind act empowers patients - and turns them into Queens! It was a fabulous day, and my crown was breathtaking! The video recently aired on BBC and has spread to all corners of the internet! Woah - I am in a viral video! lol How crazy is that?!

I recently began writing for an awesome company called Clara Health. I wanted to link a few articles I wrote since I think they are very important for patients! Their website helps connect patients to clinical trials on one, easy to use platform! It's a million times better than, but has all the same information! Super patient friendly - It is amazing. Here is a feature I wrote demystifying a bit of the clinical trial experience, and here is another I wrote about my personal experience as a patient undergoing a clinical trial. If you are interested in branching out of the, "standard care" offered by your clinic, I highly suggest you check them out!

Health wise, I seem to be pretty stable, though some recent stress has caused a bit of a digestive system flare. I've been aspirating a bit, which is never good. Let's hope it all works itself out soon so I don't cause damage to my lungs! My lungs, and POTS still seem to be steady - I still have symptoms, but no where near as severe as they were pre-transplant. My body has been extremely sore, and many of my joints feel as though they are out of place... I have been assured that this type of pain is normal post HSCT. That sucks. I find myself taking Advil often, which I never used to do pre-transplant, so that's saying something! Due to the pain sleep has been pretty non-existent. I see my scleroderma specialist from UCLA next month, and I cannot wait to show him my vast improvements! The transplant has improved my quality of life very much, and I am so grateful for however long this miracle lasts.

Thursday, February 23, 2017

day +50 post hsct update

The first year post stem cell transplant is a roller coaster, and on that roller coaster the first hundred days are the terrifying drop! Those first hundred days are typically when your new immune system either rejects its purpose, or begins to flourish and grow. These days are when you are most susceptible to infections, typically are the most weak, and the days that seem to be the most treacherous. Day +100 is a celebration for any transplant patient, and seems to be when they can take their first sigh of relief - It is the first large milestone in the patients journey. 

Yesterday I reached day +50! Halfway to the dreaded first hundred days. So far my journey has definitely been a roller coaster. The first few weeks home after transplant I ended up with CMV, (Cytomegalovirus) a globally widespread virus that only manifests in severely immunocompromised patients. This virus can be extremely deadly if not caught and treated immediately. Luckily, my transplant team has me preforming weekly blood draws to check my progress, and check for serious viruses such as this. My viral load was pretty high, but my team was able to treat me successfully for the virus with a two week course of anti-virals. The CMV definitely set me back a bit on the recovery time line, lowering my WBC, and confining me right back to the couch! lol About a month post transplant the WBC count should be rising at a steady rate, however, mine was actually dipping, or barley rising at all due to the virus. As of now my counts are on the rise - slowly, and a bit slower than they should be, but still rising. Unfortunately my neutrophils are still at a neutropenic level, so hopefully they will rise soon!

While most of my 50 days have been rough, I have had a few huge victories too! Monday evening I received the okay to remove my feeding tube! My GI physician was definitely hesitant, however I have successfully sustained my weight for a solid 7 weeks without the use of my tube. Between my GI and the transplant team, we decided to move forward with removal. It was so odd to say goodbye to a piece of a equipment that was quite literally part of me! ...though I cant say I was sad to see it go! The site will take a few weeks to heal completely, though the stoma has actually already closed. The scar the tube leaves looks a bit like a second belly button! lol My digestive tract, while I can now sustain my life without a feeding tube, is not perfect. Before transplant my esophagus worked at a whopping 0%, and now my guess would be it works around 20 - 30%? I know that seems like a small number still, but to me that's 20 -30% better swallowing! Honestly, any movement compared to pre transplant feels insanely amazing! So, while you see me shoving my face with whatever I can get my hands on, thinking to yourself, "wow, her digestive system was cured!" Please know that isn't actually the case. I am quickly learning by elimination diet the things I can and cannot have with this post transplant peristalsis. 

I have put a lot of thought into whether or not I would change my blogs name since I am in fact, no longer tube fed! At first I was leaning towards changing my branding, however; the more I think on it, the more I want to keep The Tube Fed Wife. Why? ...Because it has carried me through so much. I want to continue to grow with this title to show others that there is hope! This blog all started to give others a glimpse of  'a day in the life of the tube fed wife,' and I hope by keeping my title it shows that any day can hold a miracle!

The most frequent question I get asked currently is, "so are you cured?" That answer to that question is: no. The transplant was not a cure, but solely an aggressive treatment that hopes to halt progression of my illness, and reverse as much damage as possible. As I have previously explained, hematopoietic stem cells are blood cells, blood cells make up your immune system. The new hematopoietic cells infused post the removal of my old damaged cells via chemotherapy are not programmed with disease like the old cells. This new immune system, created by your own hematopoietic cells, is no longer constantly attacking the body - meaning nerves have time to repair themselves, inflammation begins to dissipate, organs are no longer under siege, and the body can begin to finally heal - instead of constantly trying to fend off its own immune system! The stronger ones new immune system gets, the less evidence of disease should be in the blood. Many patients see antibody markers for disease, and inflammation drop dramatically, and symptoms lessen, or come to a complete halt - ailments such as inflammation would completely subside, however ailment such as fibrosis would not completely improve. I have seen incredible improvements in my digestive tract and respiratory system. The improvements that manifest should be permanent as my immune system has been completely reset, however; similar to cancer patients, relapse of disease is possible even after an immune system reset. Currently stem cell transplants are only FDA approved to treat certain cancers and blood disorders - that is why my stem cell transplant was a clinical trial. The physician was simply using an already FDA approved treatment on a disease it was not currently FDA approved for. Since my diseases have only been studied for the past few years in regard to stem cell transplants the long term results are truly unknown for patients like me. 

Probably the second most common question I get asked is, "how can I get a transplant?" As I mentioned above, stem cell transplants are only FDA approved for certain cancers, and blood disorders. There are a few clinical trails in the US that preform stem cell (aka bone marrow transplants) on specific autoimmune diseases. Your physician would refer you to these programs if they believe it would be an appropriate treatment at your stage of disease, however; you can also browse them on if you're curious. (Search "your disease AND stem cell transplant") Due to the high mortality rate of the transplant, these trials have extremely specific candidate criteria. 

Do be advised that "stem cell therapies" are very, very different than stem cell transplants. I often see/hear patients mistake the two to be the same treatment. "Stem cell therapy" is a non-FDA approved procedure preformed at private clinics where a patients adipose (fat) cells are harvested through liposuction and then, either injected into a problematic joint, or infused in hopes of reducing systemic inflammation.  Effects can last for weeks, to months. A stem cell transplant is a FDA approved medical procedure that involves extremely high dose chemotherapy, and the use of very specific hematopoietic stem cells derived from the bone marrow. This demolishes one immune system, and replaces it with another. The process takes months, and must be preformed by a highly skilled team of physicians and nurses in proper hospital facilities.
....I'm going to be honest with you guys, this post took me hours! The heavy amounts of chemo used for the transplant really takes a toll on your brain. I've had brain fog, but never to this extent. It's almost as though the chemo has put a thick wool blanket over specific areas of my memory, word recollection, and perception skills. I couldn't even remember how to start a new blog post when I logged on today! lol ...And there is a HUGE button that says "New Post" right at the top of the page. Yeah - it's that bad! 

In other exciting news: I have BABY FUZZ on my head! My hair is finally starting to sprout. Even though it's pretty much invisible right now, I am very excited! Woohoo! 

Sunday, January 22, 2017

HSCT Complete! - Health Update

Wow, it felt good to type that title.
The last two months of my life have been absolute, and utter hell. All the alumni patients warn you, but nothing on earth could prepare someone for a stem cell transplant (aka bone marrow transplant). I have never experienced such pain, such nausea, or the wish to just slip away in my sleep. It's so hard to fight when you're that weak. It was a emotional, and physical roller coaster for everyone involved.

After a total of two and half weeks inpatient, 7 days of chemotherapy, 4 blood transfusions, 5 platelet transfusions, and one stem cell infusion, I am happy to say I am recovery at home with a new immune system slowly growing inside me. The process was in two grueling phases that spanned over many months. To type everything that happened would be to write a novel - honestly, don't want to relieve many of those terrifying days. I did try my best to document the transplant through Live Facebook videos which can be found here:
Upon viewing these videos please remember, this was a live, raw, and unfiltered journey - some of which is very unpleasant, and emotionally upsetting. I was EXTREMELY drugged and out of it - so they are a bit disjointed. Please also keep in mind that as they were LIVE, I may not have had all the appropriate information, or misspoke completely, which was normally corrected and updated in a later video or blog post. I am not a healthcare professional and my knowledge stems from my understanding and interpretation of my physicians comments. And if you have been keeping track, physicians change their minds and their interpretations frequently! lol

I learned so many incredible things while in Chicago. The care I received there is unlike anything I have experienced. They actual helped clarify so many of my diagnosis, plus all of their physicians are on the same page so I never felt flung between two care providers opinions... I have such a hard time with that at home and it can lead to some confusing coordination of care! lol After thorough evaluation of my organs, the transplant team noticed minimal fibrosis thought my body. Many of my physicians at my local University just kind of blame everything on fibrosis without really taking a super in-depth look since Scleroderma is my main diagnosis.

The start of chemo.
Let's start with the digestive tract, all of my esophageal manometries, barium swallows, and gastric emptying studies preformed in Seattle have always shown, "classic Scleroderma findings." My physicians just sort of always chalked this up to meant large amounts of fibrosis, but as I was explained in Chicago - that is not always the case! "Classic Scleroderma Findings" can mean the generalized outcome of the organ dysfunction, etc, but not necessarily the medical mechanism of fibrosis. Their amazing GI did a head to toe scope of my digestive tract and found only small amounts fibrosis/strictures, meaning my severely paralyzed digestive organs were caused by another mechanism of Scleroderma/MCTD. He explained to me that Scleroderma, especially in overlap, damages the vascular system more severly, resulting in autonomic nerve damage that causes weakness or paralysis of smooth muscle tissue; this leads to decreased digestive ability among other things. The autonomic nervous system aids in everything from digestion, to respiration, to heart rate, to blood pressure! You can imagine how damage to these nerves would cause mass havoc on the body. The smooth muscles in my digestive system atrophied due to the nerve damage, and eventually become severely slowed, even paralyzed. This lead to my inability to swallow, or digest food that did make it to my stomach; It was also the reason I would soil myself multiple times a day. The muscles too weak, and the nerves too damaged to function. He explained that this is typical overlap classification findings. Patients with only scleroderma have much more fibrosis, while I have more smooth muscle and nerve damage - though small amounts of fibrosis as well. So same outcome, but slightly different findings than a patient with only scleroderma.

My Stem cell Infusion!
My recent breathing issues are actually somewhat similar, as in they are also a consequence of damaged autonomic nerves and smooth muscle tissue. As a brief back drop, a few years into my diagnosis, due to a combination of severe respiratory muscle damage, small amounts of fibrosis and heavy inflammation found on my CT scans, my DLCO dropped dramatically, my FEV was declining, and I started de-saturating during 6 minute walk tests. As a consequence I was put on low supplemental oxygen - first upon exertion, then eventually when walking, and sometimes even at rest. After nine months of chemotherapy the fibrosis had all but dissipated from my scans and the inflammation was non existent. My respiratory muscles still sucked, but improved somewhat. I was off oxygen, besides for heavy activity and sleep for many months when suddenly, I started becoming short of breath again while walking and even occasionally at rest. My heart would pounded right out of my chest. I began using my oxygen again while walking and sometimes at rest. I begged my doctors to do another CT to see if the inflammation & fibrosis was back, but due to my age they decided, "I didn't need to the radiation." We did a treadmill test instead which did show mild desaturation upon exertion - my 6 minute walk test stayed low normal. My team decided to use the supplemental oxygen as needed - however, I felt like I needed it all the time, not just with activity! I spent months frustrated, and unable to breathe. Chicago did all the proper tests, and even some that my local university had never preformed! (Always, always get a second opinion!) The team found that my diaphragm and reparatory muscles were near non functioning, but my lungs were thankfully still clear of most the inflammation and fibrosis, so something else was a miss. When I did my six minute walk test I lost consciousness suddenly, which happens to me occasionally, even though my 02 stats stayed within low normal limits just before the incident. The wonderfully docs in Chicago ran a test called a CPET which looked at how all my systems function together, not just one system at a time. This test gave us a wealth of knowledge regarding my new shortness of breath. Turns out my Autonomic Neuropathy was more wide spread than just my digestive tract, heavily effecting my heart, and brain! In a nutshell the damaged nerves caused a condition called POTS which left my body unable to cope with basically all everyday tasks. It didn't remember how to adjust blood pressure, heart rate, or blood flow when I was talking, walking, standing, or basically using an effort besides just lying down. All that oxygenated blood ended up pooling in my lower body and not going to my brain which would cause Cerebral Hypoxia. My brain in turn would freak out from not getting proper blood flow, leading to a starvation of oxygen, and tell my heart to pump more blood causing it to overwork. Beating upwards of 150BPM by simply walking, causing serious hyperventilation as I gasp for air my brain is telling me I don't have. Eventually my body would just shut down by passing out. Luckily, this was not a worsening lung issue, it was a blood flow issue. The doctors who figured all this out (a team of three to be exact) prescribed special waist high compression garments to keep blood from pooling in my lower body, as well as a few medications to slow the heart rate. (My body hates me! lol) So essentially, while I was properly using the oxygen at times of heavy activity post chemotherapy in 2015, I didn't need to be on it at all times. The transplant videos I made were LIVE, consequently, this information was a bit misconstrued as it was currently unfolding as I shared updates. I had expressed, "they said my lungs were okay, and that I don't need oxygen!" I expressed anger at my team in Seattle which was, in reality, unjustified. Chicago, after reviewing all the test, eventually concluded I still did need the oxygen, just not at all times as I had been using post the 2015 improvement. They had me wean off constant oxygen, only at use under times of physical exertion under their care.

Basically I went to Chicago, and everything I thought I knew about my diagnosis changed. It's incredible what new sets of eyes can do for a patient that fell into stagnant care. While I talk mostly about Scleroderma on my blog, it is only one of many clinical manifestations of Mixed Connective Tissue Disease, my full diagnosis. MCTD is a rare autoimmune, connective tissue disease with features of three distinct diseases, paired with the findings of high U1-RNP antibodies. The three distinct diseases are Scleroderma, Lupus, and Myositis. This is why MCTD patients are extremely unpredictable, and extremely hard to treat - we are made up of so many illnesses, what do you treat first?! While we have manifestations of all three diseases, we are also our own separate entity with our own quirks. Chicago had actually never treated a patient on an MCTD protocol for this reason; however, after reviewing my case, especially with the severe history of scleroderma in the vascular system and digestive system, I was approved to be the first patient treated specifically for MCTD!

The researching physician!
I won't keep you in anticipation, while I barley survived the transplant, it seems so far to be super successful! While fibrosis cannot be fully reversed, nerves can be repaired, inflammation can be removed, muscles can be rebuilt, and the body can begin to finally heal! Why is this? The high levels of chemotherapy completely wiped out my old immune system (bone marrow) essentially erasing disease. The new immune cells transplanted were just babies, not programmed with disease like my old cells. This new immune system, created by my hematopoietic stem cells, is no longer attacking my connective tissue - meaning locations like the autonomic nervous system have time to heal themselves, instead of being contently beaten down. As my cells began to en-graft, we started transitioning to soft, liquids and puree food in the hospital, and I haven't had a tube feed since! I CAN EAT AND (mostly) DIGEST FOOD with proper medication!!
I never, ever thought I would say that again! I can practically feel the peristalsis move down my esophagus, and it's incredible! Food no longer stays stagnant in my stomach, and I no longer soil myself! It's not perfect, but it's enough to sustain a human life! I truly feel like I received a miracle - a miracle I worked my ass off for! lol ;) My shortness of breath has also decreased, and so have my POTS symptoms which is amazing - though that may be attributed to the waist high compression garments and new meds as well. There are still things I feel have yet to changed, such as my joint swelling and pain, but I am told I'll start to notice more and more improvement over the next few years! For now I recover, and grow my new immune system!

I am extremely grateful to my Scleroderma Specialist who referred me to the HSCT program, and of course the team of researching physicians in Chicago that just added so many years to my life! I am also humbled, and overwhelmed with love, by everyone who supported me on this journey through their beautiful comments, kind messages, fundraising, or simply quiet prayers.

For the first time, in a long time, I am so excited for what the future holds!