Two Year Post hsct Follow-Up

I wanted to start by apologizing for not keeping everyone up to date, but I promptly stopped myself. I have been reminded time and time again that I never need to apologize for putting my health first. It is a hard pill for me to swallow, but I am learning to adapt. #Sorrynotsorry would be appropriate, right? lol Since the auto-gvhd diagnosis I have been keeping my head low and focusing on getting back to a place of stability post transplant. My team and I worked diligently to sort out my physical and mental health which were quite a mess from August - October. Luckily, we finally began to see positive results from the Rituxan infusions I underwent in October. My physician expressed it would take about a month to see any real changes, and he was right! Late November I started feeling more energy, seeing less rashes, less hair loss, and food became less abrasive again. I am definitely still experiencing some issues that may take longer to recover, such as the mucosal membrane involvement, but it should get better with time. While I am not back to 100% of where I was prior to August, I am in a much, much better place. I can stay awake during the day, my skin and insides don't feel like they are on fire anymore, and mentally, I have clarity. Hallelujah!

This past week I found myself in Chicago to see my transplant team for my two year post HSCT follow-up testing! Seriously, where does the time go?! I have a very love hate relationship with the city and hospital. On one hand, it is where my life began again - where my life was saved. On the other hand, it is where I was brought to near death - physically and emotionally tortured. Being back is a whirlwind of emotions. Tuesday consisted of blood work, an EKG, an Echocardiogram, a Pulmonary Function Test, and a High Res Cat Scan. Wednesday I saw my transplant team for preliminary results! 

Labs: My white blood cell count and ANC are still abnormally low. As I had pretty damaged bone marrow prior to my transplant, they believe this is my new normal. My immunoglobulin never returned to normal either, G, A, and M are all still practically non-existent. Due to my recent Rituxan infusions, my B cells are completely depleted as well. As you can imagine, my immune system is pretty fragile right now - and will be for a while. My autoimmune markers are still titered extremely high, though my antibodies are becoming less concentrated! 

PFT: My FEV, DLCO, and other results are slowly coming back up post the dip this summer/fall. All in all, my results are still well below normal, but are trending in the right direction. 

EKG & Echo: I am currently showing an abnormal QRS-T angle again, which is something I dealt with in the past. Currently, the rest of my heart is functioning pretty well, so we are simply going to keep on eye on my EKGs. My pressures are all within normal limits and my ejection fraction is continuing to improve! My left ventricle is no longer enlarged and I have no pericardial effusion which is a huge plus!  

CT: My imaging showed no new fibrosis, I didn't even have air trapping this time! Everything honestly looked lovely, besides for two new nodules. A 2mm and 3mm nodule were found in my right lobe. Luckily, due to their super small stature, my team wasn't too worried. However, I will have to have a repeat scan in 6 month (here in Seattle) to ensure they are not growing. Nodules are not a super uncommon occurrence in autoimmune patients. We sometimes grow what are called, granulomas, which are little build ups of scar tissue. So technically it is new fibrosis, but a different kind then what my doctors are evaluating for. Typically these only arise as a response to active inflammation, a foreign object, or infection in the lungs - so I don't have an answer for why these new nodules are there right now. I am interested what my specialist here will think - the CT is on the way to him as I type. Are they inflammation caused by the auto-gvhd? Are they simply just my body being an idiot? Are they disease progression? Did I have an infection? Di I inhale something?Who knows! lol Basically, I am on, "active surveillance" until we can verify they aren't growing. 

Dec 2016 / Dec 2017 / Dec 2018

All in all, my results are on the mend after a recent dip in my health due to the auto-gvhd. Things are definitely looking up and I feel like the pep in my step is back. The word of the day: Stable. I am so grateful for another bonus year of life. 

Rituxan Round Two

Rituxan. Prior to my transplant I was on it for a almost two years - it was also used during my transplant. Rituxan is a monoclonal antibody. In layman's terms, it is a drug that targets and destroys a specific type of antibody, in Rituxans case, the protein CD20 which is found on immature B-Cells. B-Cells are an important part of the immune system. Monoclonal antibodies are a huge step in medical history because they have the capability to destroy targeted malignant or malfunctioning cells without destroying all other rapidly producing cells. Meaning, it doesn't have all the horrid side effects of cytotoxic agents (aka chemotherapy) but has a similar, hard hitting function. It is frequently used in cancer and certain autoimmune diseases for this reason. 

My second round of Rituxan to combat the autoGVHD went smoothly. I am one of the lucky patients who really doesn't experience much side effects from the drug. Besides a couple days of diarrhea, exhaustion, and sore muscles - I do just fine. The infusion itself is about 5 hours and is accompanied by steroids, Benadryl, and lots of saline. Rituxan is given via two infusions every 6 months. Now that I have completed my second round, it's time to just sit back and wait for results. My physicians hope is that the drug will reign in the autoGVHD reaction before it gets too out of hand, or before it reactivates my immune system, causing a relapse post HSCT. 

While we await to see results from the drug my team decided to get some baseline pulmonary function tests. Unfortunately, my PFTs have slightly declined since my last assessment in May. My spirometry dropped by 5%, my plethysmography dropped by 11%, and my DLCO dropped by 16%. In the PFT world those are noticeable drops, but also drops that could be within the "margin of error," which is around 10%. Is it a skewed result, is it the autoGVHD in my lungs, or is this signs of a relapse? At this point, my local team is already itching to put me back on low dose oral chemotherapy and steroids. I essentially refused a new treatment plan until I could consult with my specialist - I trust him to make the best, most informed decision out of all my care providers as he has the most knowledge of post transplant patients. I saw him on Wednesday. He spent a good amount of time talking over my symptoms, clinical findings, test results, and general quality of physical and emotional health. Thankfully, he still does not believe my current physical state indicates a relapse post HSCT. However, the autoGVHD is definitely wreaking havoc on much of my body still. Since the Rituxan will take a month or so to really generate the results we need he decided to start me on a low dose biologic agent that will more immediately reduce some of the inflammation in my body. We will also be upping my vasodilators as my Raynauds has pretty seriously increased since all this began. Honestly, his biggest concern was my lack of sleep. The body cannot properly function without sleep. Since September I can count on one hand the number of decent night sleeps I have had. Even with traditional sleeping medication, I just cannot stay asleep. He has ordered a sleep study to asses exactly what's going on. We were hoping the poor sleep cycles were related to my mental health, but even after beginning treatment for my anxiety, the poor sleeping habits have remained. 

Mental health. Something I do not often touch on. Not because I am ashamed, not because it is less important, but simply, because mental ailments absolutely, unequivocally terrify me. A condition of the body seems so much less frightening than losing... yourself. As I am sure you are all aware after reading my last blog post, I was struggling. Not only physically, but mentally. I found myself downright riddled with anxiety, PTSD, depression, and panic attacks. Completely incapacitated by cycling thoughts, frustration, and emotional frailty. This wasn't just sadness and worry - this was an all encompassing black pit I could not escape - I felt trapped in my own brain. Luckily, I have a team dedicated not only my physical, but mental health as well. We began anti-depressants and therapy immediately. After some trial and error, we found a drug that significantly improved my quality of life; Cymbalta. I went from having panic attacks at the grocery store and crippling anxiety seemingly every waking moment - to maybe once every week or two. It's incredible to feel such tangible relief. If you are struggling, please, don't be embarrassed to find help. You are not weak. You are not flawed or inadequate. You are dealing with an ailment that can be significantly improved with proper care. Thank you to my friends and family who helped me through a very rough few weeks.

A full work up, including a high resolution CT of the lungs, a repeat PFT, blood tests, and an echocardiogram are all scheduled for early next month. This will give the Rituxan a bit of time to kick in before we can truly assess how things are going. The good news is, my rashes have significantly decreased post another course of my least favorite drug; Prednisone. I now only have a few dry remnant patches left where the more severe rashes were. So it looks like things are headed in the right direction - hopefully, this will be far behind me soon.

Rituxan Round One

I'm struggling. I would be lying if I said otherwise. I am feeling overwhelmed and crushed by life. I had FINALLY let go of all my fear and doubt, fully embracing this second chance at life I had so apprehensively tiptoed - only to crash back to reality. Crash hard. Like a rocket from space, you know, the ones you see totally disintegrate into flames as they re-enter the atmosphere in movies. Seriously, that ships hull was my mental health. It's a flaming disaster right now. I emailed my poor PCP in tears telling her that I was a constant anxiety ridden mess. Will this cause a relapse? Will this take away everything I have worked for? I feel like I am drowning in open water where no one can hear my cries for help - even though I am surrounded by a beautiful support system and highly competent medical team. Definitely not my brightest moment. I am not sure why the idea of fighting again is so daunting. Why I cannot seem to emotionally process this situation, when I did just fine with an onslaught of dire situations in the past. When I received my diagnosis in 2011 it didn't seem to phase me at all. I was sort of emotionally numb and ready to just fight. Each blow seemed to rolled right off me like water on Teflon. Now, all I feel are sensitive emotional nerve endings and I just want to hide. I can't sleep, I have breakdowns in my car, and I feel like I want to jump out of my skin at all times. Everyone expects this constant bubbly, ready to conquer, Chanel, but I am only human. I too have bad days, and right now, I am having a bad month. Needless to say, I am getting the help I need from my amazing team both mentally and physically.

Wednesday I had my first round of Rituxan to combat the auto-GVHD. Besides some swelling and fluid retention due to my crappy heart not pumping out liquid as it should - It went smoothly. I turned on my favorite ASMR youtube channel and just tried to relax. Honestly, I was so drugged with Benadryl I slept almost the entire four and a half hour infusion. A small bought of nausea, a headache, and some facial flushing were my only reactions, so I was a-okay. I head back for my second infusion on the 31st. Yes, I will for sure be in costume. 

Right now I am simply doing my best to keep my head above water and not get too worried about results before they arrive.

Autologous Graft Vs Host Disease

Epilogue: a piece of writing at the end of a work of literature, usually used to bring closure to the work. 

I have lovingly referred to my journey post HSCT as my epilogue. It marked the seeming end of a complicated medical journey and provided the miracle of closure to the most difficult time of my life. However, I have recently learned that life, similar to works of music or literature, often experience a reprise. A moment where you learn that there was in fact no period as you thought, but a semicolon. While one part of your story had ended, another was just beginning.  

In late July I found my legs and feet covered in painful, raised, red lumps. The lumps felt similar to the Leukocytoclastic Vasculitis I had experienced in the past, prior to my transplant. They burned instead of itched, and eventually faded into hard purple spots that left behind tiny dark scars. However, a skin biopsy showed they weren't Vasculitis but, were another sort of immune system reaction coined by an infiltration of eosinophils and lymphocytes. These lumps continued to pop up over the course of three weeks. By about the fourth week, after a weeks course of prednisone, the rash had faded, but new symptoms arose. I began losing large clumps of my hair while in the shower. Finding noticeably thinner spots where my scalp was clearly visibly. The hair loss was accompanied by a localized red, scaly rash. Particularly my eye lids, shoulders, hands, and neck. It was slightly raised, itchy, and looked similar to white scales in an angry red desert. These rashes ebed, growing and shrinking. Suddenly I was taken by exhaustion 4-5 weeks post my initial leg eruption. No matter how much, or how drugged I slept, the bags under my eyes were intensely noticeable. My eyes and mouth were terribly dry and my energy levels dipped to unbearable. I was stopping my car on the road side to take naps on the short drive home from work. I simply couldn't keep my eyes open. By early September my GI tract had reached an extent of disarray. However, not my typical disarray. This didn't particularly feel like a digestion issue, but rather an issue of irritation. It was as if the entire tract lining from my mouth to anus was on fire at all times. It was causing painful swallowing and regurgitation, plus diarrhea with constant nausea and pain. I found myself upping my dose of Domperidone and Nexium, while adding in Zantac a few times a day to keep any food down. Tums and Preparation-H become a daily staple.

In all honesty, I was in heavy denial regarding this barrage of symptoms. I simply wanted so much to believe in my perfect epilogue post HSCT. I waited to see my doctors in fears they would confirm a problem - and in fact they did. I met with my local Rheumatologist, Digestive Disease Specialist, Cardiologist, Dermatologist, and Scleroderma Specialist. Heaps of blood work was performed along side the biopsies and other tests - abnormalities began to reflect quickly. 

Wednesday, I met with my Scleroderma Specialist, who also happens to be one of the worlds leading researchers in Scleroderma HSCTs. He reviewed my labs, tests, biopsies, and clinical findings with a somber mannerism. I expected bad news, some sort of relapse post transplant perhaps? Alas, that was not the case. He knew exactly what was happening - and was as shocked as I was. Autologous Graft Verses Host Disease (AutoGVHD). In his 19+ years of performing and researching Scleroderma HSCTs he said I am only his second patient to ever present these unique and extremely rare symptoms following an Autologous HSCT. 

"Graft-versus-host disease (GVHD) is a common complication of allogeneic stem cell transplantation (allo-SCT). However, a similar syndrome has been reported in autologous stem cell transplantation (ASCT) as well. The target organs of GVHD in ASCT are the skin, liver and gastrointestinal (GI) tract, which are consistent with those in allo-SCT. Histologic findings from the skin and the mucosa of the GI tract also show similar features." https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5827903/

As you can image Acute or Chronic GVHD is a common occurrence post Allogenic HSCTs since the immune system used is another human beings, but autologous transplants which use your own cells? Almost never. GVHD is an allo-immune response where the donated blood cells view the recipient as foreign, so it attacks the body, causing wide spread damage, particularly in the GI tract, skin, mucous membranes, kidneys, liver, and lungs. Essentially, my new immune cells are attacking me because they see my entire body as a foreign agent. Highly illogical, but can you blame them? If you were forced to inhabit a dysfunctional body as a flawless new immune system, you might overcompensate too. GVHD can happen any time post HSCT. In recent medical literature, autoGVHD has presented as much less progressive than GVHD acquired from an Allogenic transplant. It moves slower, and is less severe overall. However, it is still a serious medical condition that can have fatal consequences if not treated diligently and quickly.

While a similar concept to organ transplant rejection, it is actually quite different. Organ rejection occurs when the recipients immune system sees the donated organ as a foreign agent and attacks its tissue. (Host vs Graft instead of Graft vs Host) This devastating condition typically stays localized to the specific transplanted organ - that is, until the failure of that organ creates the failure of other organs. With GVHD, the entire body is attacked, as blood cells (bone marrow) are a major building block to all human structure. Both are catastrophic conditions that result from the overreaction of an immune system rejecting what it believes to be a foreign agent - whether it be yours or your donors.

So how do we know this is simply not a relapse post HSCT? From what my specialist explained, none of my current symptoms directly correlate with my pre-transplant baseline. A relapse would reflect more typical MCTD symptoms, swollen fingers and joints, new skin tightening, shortness of breathe, etc. He also expressed that my current clinical findings, biopsies, and blood work represented classic GVHD features - and that my case presents identically to the one other patient he treated with autoGVHD. After expressing his sincere regret for my circumstances, he shared his confidence that this would be treatable. We caught it early. We parted ways with a solid game plan and a hug. I will be beginning immediate treatment with Rituxan to combat the autoGVHD. The hope is that the Rituxan will target the cells responsible for the autoGVHD and set me back to a recovery baseline. Yes, I am essentially fighting another autoimmune disease - but this time it's new me fighting old me. 

I am exhausted, uncomfortable, and frustrated - but hopeful. Unfortunately, this turn of events puts me at a much higher rate for transplant relapse. Only time will tell.

To learn more about autoGVHD (which goes by multiple different names in the medical community) please visit the extremely informative link below. You may also read any of the other above mentioned hyperlinks from specified case studies.

Autologous Graft Versus Host Disease: http://www.remedypublications.com/annals-of-stem-cells-and-regenerative-medicine/articles/pdfs_folder/ascrm-v1-id1002.pdf

Experienced Sea Captains & Me

I believe I owe everyone an apology. I have been less than honest with the portrayal of my day to day life on social media. I have painted a picture of perfect health and happiness. Followers, friends, and even family often forget what I went through, and continue to go through because of this. I don't blame anyone but myself for not expressing adequately the reality of my ongoing struggles with disease. I put on a good show, but the reality is I still wrestle daily with my conditions. Let me explain:

When ones life pre-miracle was a constant downwards trajectory, both the highs and lows of recovery all seem so manageable. My lowest day now is still better than a good day pre-transplant. (So why wouldn't I smile?!)  This in no way means I do not continue to suffer - I will deal with daily symptoms from the damage that was permanently done to my body for the rest of my life. It simply means, I function at a level of misery many would buckle under - It's just what I am accustomed to.

Imagine this: If you put a sea captain who survived a typhoon in choppy water it may seem he easily navigates these conditions. In reality, it is still difficult, he has simply dealt with worse and is more equipped to deal with the treacherous water conditions than another captain who has never seen a wave. 

Yes, I smile, I laugh, I explore, and enjoy. My Instagrm posts are filled with excitement and happiness. Simply put, I am thrilled to be alive (even on my roughest day) because it is still so much better than where I was. I was dying. Anything better than that is a miracle to me - I just don't see the point of complaining about my current condition after what I survived. My aching joints, my undigested food, my so-so lungs, and my okay heart, all seem so miraculous! I'm enjoying every moment with renewed vigor because I never know when this bonus life will end. I live on the edge of a knife, constantly awaiting the tip. I do not mean to glorify my recovery or paint a skewed picture of my reality, I am just trying to make the most of this glorious epilogue. 

The truth is I am happy, I do feel good, and I have seen vast improvements in my health. I feel like I was given my life back - but keep in mind that is relative to literally being kept alive by tubes and tanks. While I am now functioning at a level of health that seems "normal" to the outside viewer, I am still struggling. As the experienced sea captain, I am simply used to the turbulent water conditions. Rejoice with me, but also understand my situation isn't what you can simply see from the external view.

My health will still be a life long struggle - However, I now have time to enjoy the ride!

day +411 post hsct update

Time is a funny thing, fluid yet seemingly stagnant for durations. Lately, time has escaped me completely. I find myself crossing off days on my calendar faster than should be possible. Months somehow blended into a year. I remember the days when I would apologies for neglecting my blog for a fourteen day period, yet here I am, writing an update for the first time in five months and seventeen days. I missed writing about huge milestones such as my 1st Stem Cell Birthday, my one year follow up in Chicago, and an abundance of glorious good news. The truth is, for the last 6 months I've been renovating. No, not my house; myself. I've been busy relearning who I am post miracle and reinventing what my life and it's purpose should be. I feel like I am living in a bit of a paradoxical existence. No one can tell me how long I have, or where I'll be next year. Before the HSCT my timeline was indisputable, now it's no longer clearcut. My treatment was experimental; the outcome and the longevity of its effects, unknown. My stability could be life long, or it could be but a fleeting moment. I concurred certain death, but now I'm facing uncertain life. My barring are all off and the crutches I had forcibly come to rely on for so many years are gone. Everything I was accustomed to changed when I was given the blessing of a future I never planned for. Where once I lived minute by minute, free of consequence, I am now faced with planning, goals, and liability - Aspects of life I haven't dealt with in over 6 years! I mean, let's be real, my only goals for the past six years were, "live till tomorrow," and "don't crap your pants in public!" lol It's been a transition that no one really talks about, let alone prepares you for. (Why?! This is what everyone stricken with illness dreams of, right?!) Don't get me wrong, I am in no way complaining about this incredible "bonus life," (it's a hell of a lot better than the alternative!) I am simply stating that navigating the 180 that was my time-stamped existence has been more mentally challenging than expected. It's beautiful and exhilaration and terrifying.

I've decided that this bonus life should not be spent behind the screen of a computer. Consequently, I spend less time on Instagram and my blog. I am out making memories, not content. While social media was an aspect of life that was tremendously helpful and gave me purpose through my trials, I feel it's time to distance myself from the virtual reality I resided in and live more in the moment. In all honesty, I don't have much content these days for the blog. I see my medical team only every few months during this glorious period of stability. (Yay!) I absolutely do not plan to run away forever, (many of my best friends are still on the other side of a screen!) I simply plan to take some "me time" to find out who I am sans tanks, tubes, and social following. I am learning to live without my sole identity being, "that dying girl," and I need some space from that social circle to find out who Chanel is again... My therapist taught me that! lol ;) This means emails may go unread, comments may go unanswered, and updates will become infrequent. Please understand this is not because I no longer care, it's because I now have a job and other responsibilities that diminish my free time. Prior to my miracle I had all day to answer emails, comments, and chat online - as mentioned, my whole life was lived behind a screen! It was my escape. So please, excuse my absence. I appreciate everyone who supported me though my darkest days, and I hope you all can support me during my triumphs - even if that means you don't hear from me as often! I am no longer someone fighting for each moment. My life no longer revolves around hospitals, medical devices, and a looming timeline. I am a (mostly) functioning 26 year old and it feels surreal.

Keep in mind, I still post updates on my Instagram. All be it less, but it is definitely more utilized than my blog these days. Blogging seems quite unappealing after a long day of emailing clients at work. Any-who, since it's been almost 6 months I thought I would update you all on my health/life.

In December I ventured out to Chicago for my one year transplant follow up. I underwent a Cardiac MRI, Chest CT, EKG, PFT, Echocardiogram, and blood work. I saw my Motility Specialist and the researching physician who preformed my HSCT. The Cardiac MRI showed stability of my ejection fraction and no further progression of my interventricular septum flattening or right ventricle dilation. I had a bit of pericardial effusion (fluid around the heart), but not enough to need immediate intervention. My Chest CT showed no new fibrosis, just the typical air trapping due to my weak respiratory muscles. My PFTs showed some major improvements! My FVC, FEV1 and DLCO all increased. These tests deal with my ability to move oxygen in and out of my lungs, as well as transfer it to the blood stream. The DLCO was the most dramatic improvement, with a 18% increase! I now sit right around 75% which is incredible for someone with my conditions. My Echo showed lower pulmonary pressures which is awesome as well! Very minimal, but an improvement none the less! My blood work still reflects a lowered immune system, but is slowly improving. At this time I still can't fight even colds off by myself, but it's a start! My inflammation markers are non existent, but my autoimmune markers are still extremely high. It's very odd to me that the markers haven't changed, but my symptoms have. The doctor explained that these markers never really go away, they simply become dormant until activated again. The researching physician was thrilled with my progress and expressed that I should (hopefully) continue to see improvements, especially through out the next 6 months. He gave me a big hug and seemed sincerely thrilled for me.

I saw my Scleroderma Specialist in January who was also thrilled with my progress. During our last appointment he was bit worried things weren't following the appropriate post transplant timeline; however, during our visit he saw the improvements he hoped for when referring me for the transplant program in 2016. My skin score has reduced dramatically, my lungs and GI tract have greatly improved, and my joints are insanely less inflamed. I went from 30+ medications a day to one and three medical devices to zero. (Happy tears!) I was going through our old clinic notes and it's been amazing to see the vast improvements since I really started to tank a few years ago. My CDAI (Clinical Disease Activity Index) improved by almost 75% from pre-transplant. I left his office with a six month follow up time - that's the longest I have ever gone without seeing him! It used to be every few weeks you guys!

January 3th 2018 my immune system turned one! It's now known as my "Re-Birthday." Noel took me to one of my favorite cheeseburger places to celebrate, and my co-workers got me an entire pint of buttercream frosting from Cupcake Royal! lol They know me well ;)

In non-health related news, Noel and I took our first vacation since I became ill 6 years ago. We went to my happy place; Maui. It was a monumental moment for both os us. No tubes, no tanks, just beach, palm trees, and DOLE WHIP!!!! We honeymooned there 6 and a half years ago - it was one of the last places we experienced "healthy" Chanel. We were both so giddy everyone thought we were on our honeymoon! I tried to spend most my time in the water. The ocean is, and always will be the place I feel whole. We even got to check off one HUGE bucket list item for me; The Lanai'i Cat Sanctuary! It was the most magical day of my life! (Shhh don't tell Noel!) Overall life is amazing right now. I'm gaining strength in my weekly TRX work out class, stretching my vocal chords at monthly open mic nights, and planning for adventures that I never thought I would live to experience.

Hugs,
Chanel