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 30-40%? I know that seems like a small number still, but to me that's 30-40% 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 (remission), 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 during a transplant are not programmed with disease like the old cells wiped out by the high doses of chemotherapy. 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 improve. I have seen incredible improvements in my digestive tract (nerve damage) and respiratory system (inflammation), however I have seen no improvement in my vascular system (fibrosis). 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/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 - and almost every patient chosen to participate has a poor prognosis. 
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. 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 two 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:
Phase One Videos consists of the first chemotherapy, the harvest, and some pre-testing, while HSCT videos are the actual transplant procedure. They go 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 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, only existing in my vascular system and skin. Many of my physicians in Seattle just kind of blame everything on fibrosis without really taking a look since Scleroderma is in my chart. As it turns out, fibrosis was not the reason my digestive tract was paralyzed, and the reason I was always short of breathe is even more surprising!

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 fibrosis, but as I was explained in Chicago - that is not always the case! Their amazing GI did a head to toe scope of my digestive tract and found no fibrosis or strictures, meaning my paralyzed digestive organs were caused by something else. He explained to me that Scleroderma, and every other connective tissue disease, can cause Autonomic Neuropathy, which leads to decreased digestive ability, among other things. Autonomic Neuropathy is damage to the autonomic nerves as a result of an overactive immune response to connective tissue. Yes, even nerves consist of connective tissue! 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 muscles in my digestive system atrophied due to the nerve damage, and eventually become severely slowed, or 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. I honestly had no idea that my illness effected the nervous system in this way!

My Stem cell Infusion!
My breathing issues are actually somewhat similar, as in they are also a consequence of damaged autonomic nerves. As a brief back drop, in summer 2015 small amounts of fibrosis were found on my CT scan, my DLCO dropped dramatically, and I started de-saturating during 6 minute walk tests. As a consequence I was put on supplemental oxygen. After 9 months of chemotherapy the fibrosis had disappeared from my scans and was afterword ruled as, "inflammation" instead of fibrosis - since fibrosis never fully reverses like that. I was off oxygen for many months when suddenly I started becoming short of breath again, my heart would pounded right out of my chest. I was placed back on oxygen as I could barely walk a hallway without struggling for air. I begged my doctors to do another CT to see if the inflammation was back, but due to my age they decided, "I didn't need to the radiation." Basically, they pulled a 'we don't need to know what the problem is if you already have the answer: oxygen.' I spent months frustrated, and unable to breathe, even at my 2LPM. Chicago did all the proper tests, and even some that Seattle had never preformed! (Always, always get a second opinion!) The team found that my lungs were still clear of inflammation after a CT scan, but when I did my six minute walk test I lost consciousness suddenly, even though my 02 stats stayed within normal limits just before the incident. Obviously my lungs weren't the problem, and neither was oxygenated blood! Meaning I didn't need the oxygen - also meaning I probably never needed it after chemotherapy but was told that was my treatment plan since the issue was never fully looked into. (Again, always, always get a second opinion!) 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 odd condition! 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 any exertion. Basically, 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 an oxygenation 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.

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 actual 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? My new immune cells were 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 food in the hospital, and I haven't had a tube feed since! I CAN EAT AND DIGEST FOOD!!
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 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!