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 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: https://www.facebook.com/pg/thetubefedwife/videos/
Phase One Videos consists of the first chemotherapy, the harvest, and some pre-testing, while HSCT videos are the actual transplant procedure. They range from Day -5 to Day +10. 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 not really thinking straight - 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." I guess I had always figured that 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, ectc, 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 something another mechanism of Scleroderma/MCTD. He explained to me that Scleroderma, especially in overlap, damages the vascular system 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 slightly de-saturating during 6 minute walk tests. As a consequence I was put on low supplemental oxygen - first upon exertion, eventually when walking, and sometimes even at rest. My docs felt I had a firm grasp on when I felt I needed it, and when I felt I didn't. 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 obviously still sucked tremendously, but my lungs were thankfully still clear of 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, I have no fibrosis, and that I didn'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. Also, after closer review I did still have very minimal evidence of the fibrosis found prior to chemotherapy.

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!