Monday, November 21, 2016

Chicago Here I Come! - HSCT Update

I am just going to begin with the disclaimer that this will be my last update for a long while. This Friday, as in four short days from today, I leave to begin my Stem Cell/Bone Marrow Transplant process. The actual transplant will not be until December 29th, however; there is a month long work up before a transplant can begin. A patient must mobilize, create, and harvest their stem cells which is a long, and painful process. To learn more about this process please sift through a few of my older posts from the months of August - October!

The actual transplantation process is about a month as well, so my entire journey (if all goes according to plan) will be right around two months. As I do not see myself typing daily updates during this time, I will be creating video diaries of the entire process. My goal being one video a day for the entire two months. Please understand that goal may be completely ridiculous - and not happen! lol ;) However, I will do my best! If that goal falls through, I will be doing once a week updates here on the blog! The videos will be streamed live from my Facebook page found here: The Tube Fed Wife It may end up being both if I am lucky! So check here, and my Facebook page for updates just in case! Also, please note, I will not actively be replying to messages, or comments during this time! Thank you for understanding!

I have had many people ask for an address to send small care packages to -  up until the actual transplant process (December 29th) I will be in a hotel and not inpatient. Please hold all care packages until that time. After December 29th I will have a room number care packages can be sent to. I will have a Facebook Event Page detailing the address, and what can be sent. The transplant wing has very strict rules on what can, and cannot be sent since the tiniest of germs could kill a patient undergoing a transplant. Things like flowers, or any live plant are a HUGE no, as are homemade snacks! This page will also detail visiting rules and regulations.

As for a short health update - being off immunesuppressors while gearing up for the transplant over the past two months has reeked havoc on my body. All my physicians at this point have been very worried during my appointments, stating I seem to have declined pretty severely in the last few month. My breathing has gotten increasing worse, my joints have become incredibly stiff, and my stamina has become non-existent. My physicians seem to be just as excited as I am at the prospects of this transplant!
I have managed to do some very thorough Bucket List work in the past few weeks though! As the transplant approach, and all the uncertainty it brings, I decided I really wanted to knock off a few big items! I had the opportunity thanks to a wonderful wish granting foundation (Photo Below) to meet my favorite band, I spent a wonderful few days in Southern California, and New York during my last two last speaking engagement, received my fist pair of acrylic nails which I adore, I had my hair dyed lavender, and I even had the chance to protest for a cause I believe in! All the photos for these events can be found on my Facebook page!
I also filmed a short documentary with the incredible team at Project Scleroderma - a non-profit dedicated to sharing the raw journeys of patients battling this deadly disease.


Emotionally, I am having a bit of a rough time with the reality of this tasking process. I am worried - to say I wasn't would be a lie. I think the hardest park of this whole experience will be the length though - I am naturally a homesick person. I get emotional after two days away from my husband and beloved kitty! lol So two month is going to be a stretch for sure! We plan to Skype everyday so hopefully that will help ease my homesickness. Thank you as always for all the support and kind messages, comment, and posts I receive, they brighten my day!

I hope everyone enjoys their Thanksgiving!

Saturday, October 15, 2016

The Countdown Begins - Health Update

I feel like it has been a tremendously long time since I have given a solid health update. I have been so busy with pre-transplant evaluations, speaking engagements, and an onslaught of medical bills, I have neglected my blog a bit! Luckily, I have been pretty decent at giving small updates on my Facebook page - so if you are not already following head on over! I am going to start this update at the beginning of October, to make sure I don't miss any pertinent updates or events.

The first week of October I headed to my Ophthalmologist; my vision has quickly decreased over the last few months, to the point where I can barely see without glasses. Turns out my eyes are extremely dry - like so dry the film over my eye in my doctors words, "looks like a bumpy dessert." lol The film is supposed to be flat and moist, but my dry eye has caused the film to completely deform, leaving me with nearsightedness. My eyes are always noticeably red, and very itchy now. We started artificial tear drops 8 times a day, which still showed no improvement, so we moved to a steroid eye drop twice a day on top of the other drops. I went back a few weeks later to be evaluated and it seems my dry eye is pretty refractory to treatment since it is being caused by my autoimmune disease; my vision/symptoms didn't seem to improve at all on the new drops so my physician suggested placing plugs in my tear ducts in an attempt to keep my eyes moist! ....I have placed things all through my body, but my eye!? Yuck! lol Unfortunately, we wont get to find out if they help since they pose too high of an infection risk with my upcoming transplant. Hopefully the transplant will help the dry eye, or I may actually lose my vision due to the damage and inflammation being caused! Urg.

I saw my local cardiologist to review all my heart testing preformed in Chicago. We reviewed the results of all the evaluations, and he had mostly the exact same viewpoint of the transplant teams cardio. My heart, while it isn't great, isn't in failure! Woohoo! My ejection fraction is lower than normal, and I do have significant electrical imbalances, however; he believes I will be just fine during the transplant. His opinion eased my worry a bit since he is so familiar with my case. We spoke about the new electrical imbalances found while in Chicago and he did express that in a few years I will need to have a defibrillator placed - but that I shouldn't be worrying about it right at this moment.

I also saw my Scleroderma specialist. I had been feeling a bit off that week, but made it to the appointment after an extremely rough morning. I woke up that day with completely locked, and incredibly swollen joints! I was unable to move my writs or fingers in the slightest, and my hands, shoulder, knees, ankles, and elbows looked like someone had inflated them! Everything was bright red and so painful, I was in tears trying to get dressed. When my physician came into the room he took one look at me and sent me for a direct hospital admission! By that point I was unable to walk, or really do anything at all due to the swelling and pain. I was in the hospital for three days while we tried to get the swelling and pain under control. Since I had a fever the physicians all believed I had some sort of transient virus that caused my immune system to become confused and attack my joints. If you recall, autoimmune disease is an overactive immune system that attacks your body -- instead of foreign invaders like it is programmed to do. When autoimmune patients become sick our system goes berserk and causes us even more grief! After a few days of heavy anit-inflammatory meds I was released home.

Regarding my HSCT I have some awesome news! By the grace of God my insurance APPROVED TREATMENT! This means the majority of my procedure costs will be covered! That is a huge 150,000 burden relieved from my shoulders! Unfortunately, there is still an incredible out of pocket costs to receive treatment; copays, a large deductible, necessary prescriptions, months of hotels, eight cross country flights, transportation, etc, all fall onto my family. I will still need to raise at least 25,000 dollars to receive this life saving treatment! We are currently over halfway to the goal funds! Please visit youcarin.com/chanelwhite to donate!
I also received my procedure dates this month. The transplant is broken into two phases, the first phase where you are preparing your body to produce stem cells, and the second phase where the transplant is actually preformed. The first phase is out patient, while the second phase is in patient.

  1. Nov 25th - Dec 18th Phase One:
    1. Nov 28th Pre-Testing - a few procedures to asses my pre-transplant status 
    2. Dec 6th Mobilization - one day of chemo to mobilize stem cells
    3. Dec 11th - 16th Neupogen - shots to over produce/migrate cells to blood from bone marrow
    4. Dec 16th Harvest - harvesting of the stem cells through apheresis
  2. Dec 18th - 25th Six Day Christmas Break at Home
  3. Dec 26th - (Unknown) Phase Two
    1. Dec 28th - (TBD) Transplant
      1. Dec 29th - Jan 2nd Chemotherapy - 5 days of intensive chemo to wipe out immune system
      2. Jan 3rd Stem cell Infusion - stem cells reintroduced through infusion
      3. (TBD) Stem Cell Grafting - waiting period while cells graft to my bone marrow
This is going to be a hellish two+ months so please bare with me as updates on the blog will be minimal. I do, however, want to share the reality of this treatment with the world, and will be doing daily, live video updates on my Facebook page! To support this journey you may make a donation to my transplant fund! You could also attend my super fun Singing For Stem Cells Karaoke Event this Thursday! Come out, sing a song, and save my life! We still need to raise about 10,000 more dollars to cover the out of pocket costs accumulated during this treatment, so why not karaoke for a cause?!


This month I also had the privilege of presenting in Missouri at the Scleroderma Foundations Heartland Chapter Patient Education Day! What a lovely group of people, passionate about bringing empowerment, education, and support to patients! For photos, visit my Facebook page linked above! Next off I am off to New Jersey to present at the Scleroderma Foundations Children's Conference Halloween weekend! As always, thank you all for your support! All of you that have shared my Singing For Stem cells video helped to raise over 11,000 dollars! Thank you for offering me your time, and hard earned money - they have given me a second chance at life!

    Sunday, October 2, 2016

    Hallelujah - Singing For Stem Cells Video

    "And even though it all went wrong, I'll stand before the Lord of Song, with nothing on my tongue but Hallelujah."

    When I first became sick I thought I was stripped of my voice forever - only to realize I could use it in a different capacity. I went from a classical vocalist, to a patient educator after my diagnosis, traveling all over the country to teach seminars on finding the beauty in illness. Now, as I fight for my life awaiting a Stem Cell Transplant, I find myself using my voice to ask for help from others. A voice can do an incredible amount of good, and I hope to continue to use mine after this life-saving treatment to educate, support, and empower patients. Please watch and share my video, or create your own, using the hashtag #singingforstemcells All proceeds benefit my stem cell transplant happening late this fall.




    Crowdfunding - The Thin Veil Between Life & Death

    Did you ever take part in the school fundraisers your junior high might have put on? The ones where you would go around and ask your neighbors, your coaches, and your parents friends to give you money for overpriced items such as magazines, wrapping paper, or cookie dough.  You were embarrassed, but you really wanted that hamburger shaped phone you would win at 30 items sold! Now imagine the stakes were a bit different: you are asking for money with nothing to give in return, and the prize for receiving the right amount - is your life. That is what it's like running a crowdfunding page such as GoFund Me, YouCaring, or GiveForward.

    I cannot tell you the cringe worthy, slimy feeling that shivers down your spine when you must crowdfund from those that already do so much to love and support you in order to simply afford necessary medical treatment. My failing body asks much physically and emotionally from those around me, and now I must drain them financially as well? How is begging the community to open their wallets because I became unexpectedly ill and do not want to die fair to me, or those donating? It leaves me conflicted knowing they are sacrificing so I can live. I feel a bit like a panhandler begging to receive others hard earned money with nothing to give in return. I know that when someone donates to a crowdfunding site they genuinely want nothing more than to see your treatment succeed and for you to lead a happy life lacking disease. However, it still eats me up knowing I have no way to repay their kindness - They are literally giving me life, and all I can do is say, "thank you." 

    I am a patient living the reality of not being able to afford life saving treatment because of its incredible cost. I am a patient who relies on crowdfunding, and the grace of others to keep me alive. You see, I am losing a battle against an aggressive autoimmune disease known as Systemic Sclerosis and unable to work. I am on oxygen, a feeding tube, and receive weekly visits to my home by a nurse for infusions - I am only twenty-five. Rare disease patients like myself often require expensive medical interventions in their lifetime but, over 95% of rare disease have not one FDA approved treatment. Insurance has deemed almost every life altering procedure, treatment, medication, and surgery that would benefit me, "off label," and "experimental." Therefor, the burden of the cost lays solely on the patients shoulder - our lives literally depend on crowd funding, which can deffer our treatments by months. I find myself in this situation currently, instead of receiving immediate treatment, my life saving Stem Cell Transplant will be delayed, simply because insurance doesn't want to invest in a rare patient; I must fundraiser the immense cost on my own. It not only is overwhelming for me, but for my family who has to watch as I slowly deteriorate while awaiting the, "Goal Funds" to be raised.

    High treatment costs, and health insurance inequalities force patients into situations such as bankruptcy, eviction, and even death. These are devastating epidemics that plagues patients throughout the country. We live in a society where lower and middle class individual pass away from treatable illness even though we have advanced medical technology available - they simply cannot afford it. Crowd funding has changes the lives of an uncountable number of patients; it has literally saved many of them from death. However, the fact that crowdfunding is the deciding factor to which patients live or die is a terrifying thought. That being said, thank you to anyone who has ever donated selflessly to help a patient in need. You made a serious difference in someones life that may have otherwise been lost.  

     Chanel's Youcaring can be found at YouCaring.com/chanelwhite

    Wednesday, September 21, 2016

    Singing For Stem Cells Fundraiser

    Further event details available here: https://www.facebook.com/events/1797081953839957/

    I Am Accepted! - HSCT Update

    Since I know this is what you have all been patiently awaiting - after weeks of deliberation between the researching physician, and the head cardiologist, I was approved for the HSCT Clinical Trial in Chicago!

    *UPDATE AT BOTTOM 9/23/16* 

    I received many emails, and assessment results, since I returned home, not all of which contained pleasant news. What was quoted at two days, ended up being a two week debate about my candidacy for the trial due to some discovered cardiac issues. In my last post I had expressed that my "heart and lungs looked good." Unfortunately, upon further review of the final results, I actually have extensive heart damage. 

    I am sure you are wondering how this vast discretion could happen. When I saw the physicians in Chicago not all my tests were completed, and the ones that were only had preliminary results available. This means they honestly didn't have a great picture of my heart at the time of my appointments; however upon further inspection many abnormalities presented. The basis of the findings indicate that I am in Exercise Induced Heart Failure - meaning upon exertion my heart does not pump blood as it should, leading to under oxygenation, fatigue, and irregular heart beats. This was more than likely caused by the my newly discovered Exercise Induced Pulmonary Hypertension - high blood pressure in the lungs causing serious right ventricular strain. This condition has also lead to flattening of my interventricular septum (D-Sign), the wall that separates your chambers. On top of these anatomical abnormalities, my heart has many electrical imbalances, leading to inefficient blood pumping abilities. In a nut shell, my heart sucks doesn't oxygenate my body. It is in really, really rough shape. These conditions put me at serious risk of irregular heart beats, and cardiac arrest at any given moment - which is a terrifying thought. (See Update Below!)
    My lungs however were found to contain no fibrosis! This leads my physicians to believe it was never my lungs that have caused my oxygenation issues (like previously believed) leading to my supplemental oxygen use, but my heart not properly able to oxygenate my blood stream. The scary part is I have been on oxygen about a year, meaning I have probably been in heart failure that long - I am honestly extremely lucky to have not had a heart attack yet! I have felt irregular heart beats and chest pain almost daily through out the year but my local cardiologist always told me my heart was fine! Yeah -- he is in for a surprise (and a strong talking to!) If he would have just run the tests I ASKED for we could have caught this so much sooner :( I will be consulting with him later this month to find out what my local plan of action is, and if I will need a defibrillator of not. 

    So what does all this mean for the transplant?! Well one of the reasons Chicago's Clinical Trial is so special is became they have three protocols for their HSCT patients:
    1. Standard
    2.  Heart Friendly
    3.  Super Safe Cardiac 
      • Yes those are the actual names! lol
    Every other hospital in the country only has one standard protocol. Chicago a few years back realized that all the incidences of patient deaths were caused by heart abnormalities so, they created two protocols for different levels of heart damaged patients. Three years ago there was no "heart friendly" protocols and patients like myself were turned away and told they would have no chance of survival during a transplant. The third option, the "super safe cardiac" protocol, has only been done 11 times out of the hundreds of HSCTs at Chicago. Those patients are extremely sick and have rough transplants due to their failing hearts. Can you guess which protocol I qualified for!? lol Yup the "super safe cardiac" protocol! It is still the exact same procedure I have explained many times before, only I will have a few different drugs that are easier on the heart, and a lower dose of Cytoxan.




    For those of you wondering what comes after the approval the answer is: waiting! lol There is literally mounds of paper work that must be done, and different insurance companies to fight with before we can move forward with the actual procedure. I was given a guesstimate by the transplant team that we may be looking at starting around December! In the mean time - let the fundraising begin! Please visit my YouCaring for updates, and a breakdown for the costs of treatment and such! Also, check out my fundraising even on Facebook: Sing a song and save a life at my Singing For Stem Cells Karaoke event October 20th! 

    Karaoke Fundraiser Event Details: https://www.facebook.com/events/1797081953839957/

    *UPDATE 9/23/16*
    I received a personal call from the transplant teams cardio this morning with some big news: I am NOT in heart failure! It turns out there was a very unfortunate typo by a technician that accidentally switched a result from "normal" to "indication of heart failure."  The result displayed an "Exercise Volume Fraction" result of 23% which indicates severe heart failure, but it should have displayed the words "Extracellular Volume Fraction" which is perfectly normal at a value of 23%. So long story short, while yes I do still have heart abnormalities, I am not in heart failure. The team has decided that I will still be on the "Super Safe Cardiac" protocol due to my young age, as well as my overlapping medical conditions including POTS. 

    Saturday, September 10, 2016

    HSCT Evaluations In Chicago - Health Update

    I am finally back home after what I would probably consider one of the longest weeks of my life! Exhausting would be putting this week of 7am - 430pm testing and procedures lightly. I want to preface this post stating that I do not have an answer yet from the researching physician as to whether I was accepted into the trial. I was told I would get a call "early next week," which I am taking to mean either Monday of Tuesday! What this post will consist of is a short run down of exactly what went down, and a few photos!


    • Sunday
      • My Mom and I arrived late Sunday night since it was so much cheaper to fly than a Monday flight! Luckily, the hospital provides cost efficeint dorm rooms across the street from the facilities for visitng patients and physicians. I suspect the twin size bed was made of solid rock, but other than that the dorm was great! lol 
    • Monday
      • I actually did not have testing this day, but they ask you to fly down at least a day early in case something must be added to your evaluation schedule. We took this day to do a bit of sight seeing and meet one of my wonderful online friends Lisa, and her sister Heidi. Lisa and I connected last year when she contacted me about doing a blog post collaboration. That collaboration turned into a viral awareness campaign that landed us on Cosmopolitans "Best Selfies of 2015" list! Since then Lisa and her sister have been a huge support through my journey with this disease. Meeting her was a huge check off my bucket list! As a resident of Chicago herself she took us to the beautiful Navy Pier for some beautiful views of Lake Michigan, and the giant Ferris Wheel!
    • Tuesday 
      • Testing began bright and early at 7am! First we were sent to pick up a 24 hour urine test to assess my kidneys. This means we had to haul around a giant jug of pee with us for 24 hours. lol It was gross. After we headed to the EKG lab for a quick assessment of the electrical activity of my heart. It evolves attaching electrodes to your chest and laying still for one minute. Then I headed off to get a Cat Scan of my lungs to asses my Pulmonary Fibrosis. A CT is pretty straight forward, and relatively short! I basically just breathe in and out on cue while a giant circle takes high definition x-rays of my lungs. Then we were off to the lab for some insane amounts of blood work. I had 16 tubes taken in one sitting! Thankfully they used my post which was already accessed, so I didn't even have to get poked! Standing up was an odd feeling after that much blood loss. Last test of the day was a cardiac MRI. I had to lay in a very small tube for 2 hours while holding my breath on cue for magnetic images. At three points they injected very cold dye into my veins to get a better picture. It was a longgggggg test! haha But luckily they let me listen to my favorite music. At 430pm we were all wrapped up with day one! After testing I got to head over to see one of my online friends who just started her HSCT, Karen! It was wonderful to talk to her about all her experiences up till this point. 
    • Wednesday
      • Back at the hospital at 7am to pick up a 24 hour holter monitor to asses the electrical activity of my heart. This is much like an EKG but it assesses your heart for 24 hours while going about your normal activities. After we headed to my Pulmonary Function test which assesses my lungs in depth. It consists of completing breathing maneuvers into a small snorkel like tube. Surprisingly, my results were much better than they were at my local hospital - I found out this is because they take their samples from later in the exhale than my hospital. Then we were off to get a Dobutamine Stress Echo and Bubble Study. I was asked to lay on a table while they pumped a medication to make your heart race and took an ultrasound of the heart to assess what it does under great stress. Unfortunately we couldn't get my heart rate to the proper speed so they had to introduce a second medication to make my heart race. I ended up getting very nauseous and dry heaving most the test while my heart rate hit 170. After they shot three syringe fulls of bubbles into my heart to evaluate any holes in the heart. Wednesday afternoon I was finally able to meet with the researching immunologist! From first impressions Dr Burt was a very busy man interested in the facts, and the facts alone. Totally understandable considering how many patients he deals with in a day! We went over my entire history with Scleroderma for the last 5 years, and talked in depth about the unimpressive medications I have tried in the past. He mentioned that my heart looked great and my lungs looked relatively good, but my GI tract, and general autoimmunity was a mess. He stated that he hadn't seen a patient with my "unique manifestations" before, and that if we were to move forward with the HSCT I would be an off label patient - meaning I wouldn't be part of the clinical trial data, and a new protocol would have to be created to cater to my unique needs. He stated that he wanted a few more tests completed to asses my esophageal and small bowel function before I left Chicago, then said "we will be in touch." I definitely got the impression that he found me interesting, but that I would also be a lot of work since I am so different than any other of his scleroderma patients. Last of the day we headed to see the transplant teams Cardiologist. He again stated that my heart was in tip top shape in response to the tests I had completed at that time. His only concern was possible Exercise Induced Pulmonary Hypertension - so he slightly altered my RHC for the next day to asses that possibility. However, he believed I would be a perfect candidate and respond very well to the transplant! His exact words were something along the lines of "with everything you have going on autoimmunity wise this is exactly what you need, to restart you immune system!" He told me he would put in a good word with Burt. By 430pm we were finally back at the hotel again.
    • Thursday
      • We got to sleep in for a brief hour before heading into the hospital! My only procedure of the day was a Right Heart Catheterization which assess pulmonary pressures. This involves placing a 5 French (16 Gage) catheter into the jugular vein, through to the heart, then passes into the pulmonary artery - which is responsible for carrying blood to your lungs. Throughout the test the surgeon will inflate a tiny balloon inside the arteries to asses pressures. This procedure is completely un-sedated, meaning the patient lies on a table wide awake while they go fishing around your insides. The team does use Lidocaine to numb the neck where the catheter is inserted, but that's it. It is a very odd, and very uncomfortable experience. It "tickles" your heart as they told me, though I wouldn't use the word tickle... My RHC was particularly long as we had to assess my pressures not only at rest, but during exercise as well. What typically takes 20 minutes was an hour long test! I had to peddle a bike while they assessed my pressures, and once that was finished they had to see how I would respond to an event such as fluid overload. They then released a liter of fluid into my heart in under 6 minutes. Thankfully the procedure went smoothly and I was just fine. I wont know the results anytime soon, but from what I could hear it sounded like my pressures were a bit above average with exercise which would mean I have Exercise Induced Pulmonary Hypertension. My neck is bruised to hell! lol Directly from the precede room I had to run to my last appointment of the day, a meeting with the Digestive Disease physician for the transplant team. He was 100 percent amazing! He was incredibly knowledgeable and even knew my hometown GI as they went to school together! What a small world. He also told me he thought I would be a prefect candidate. When he left he stated "I am here to be your advocate." It absolutely made my day! He was also able to arrange a specially modified test the next morning right before we got on our flight home that would help assess my esophageal issues. After we decided to do a bit of sightseeing. My Mom and I went to The Hanckock Tower where you can Tilt off the building in a specially uncased glass ride 94 stories above earth! It was a total blast! We also ventured to Lake Shore Drive to visit the beautiful beaches of Lake Michigan. 
    • Friday
      • Thursday morning I got to preform my least favorite test of all time, one I had hoped I would never have to experience again -The Esophageal Manometry. This test assess your esophageal peristalsis, muscle tone of the sphincters, and any impedance caused by a bolus of water. This procedure involves placing a 60mm long, 10 French tube through your nostril, past the lower esophageal sphincter, and into the stomach. The tube stays in place while you are asked to swallow saltwater every minute, but not aloud to swallow in between. This must be done both laying at a 45 degree angle, and sitting up straight. Then the patient must attempt to swallow a bolus of salt water equaling about 200ml. It is just as horrible as it sounds, especially for someone whose esophagus is paralyzed which basically makes it waterboarding. Getting the tube down is definitely thew worst part and involves an intense amount of gagging, horrible sinus pressure, and a few episodes of vomiting. Once the tube is in place it just straight up hurts. My test of course showed that I have no peristalsis or muscle tone - absolutely nothing showed on the screen, which should be illuminated with color. When it came time for the fluid bolus my entire esophagus backed up at about 15 mls resulting in vomited half the water all over myself, and aspirating the other half. A screen that should have been all blue showing complete bolus clearance, was bright purple showing nothing but water retention. Luckily that was exactly what my test was suppose to assess though, so all in all it was very successful! Then came removal of the 60mm which is almost as unpleasant as the entry. I actually got a pretty hilarious video of the removal which you can find on The Tube fed Wife's Facebook page! 

    All in all I felt the doctors definitely received all the proper information to make their decision, and that the entire trip was very successful! Stressful, exhausting, and hectic, but successful! I really feel that they all seemed very interested in my unusual case! Again, I should receive the finale decision regarding my candidacy either Monday or Tuesday! Be sure to follow my Facebook page for the soonest updates! If I receive a yes I will begin my transplant fundraising immedietly, and will ask everyone the huge favor of sharing my YouCaring page to assist with costs. However, even if it's a no I will still be fundraising to support the cost of these evaluation appointments alone! Even after my insurance coverage this week was close to 5,000 dollars - not including my hotel stay, or plane flights! With the help of my incredible support system, we just might raise enough to save my life!



    Saturday, September 3, 2016

    Chicago & The HSCT Update

    Tomorrow I leave for my HSCT evaluations in Chicago at Northwestern Memorial Hospital! As you know from my last post, an HSCT is a Hematopoietic Stem Cell Transplant (autologous), a procedure that will reboot my immune system by introducing an intense of amount of chemotherapy to wipe out the malfunctioning immune cells derived from my bone marrow. This procedure, while often preformed in cancer patients, or other blood related diseases with minimal mortality rates, pose a higher potential risk to patients with serve organ damage in diseases such as Systemic Sclerosis. For that reason we must go through long evaluations to be sure our already frail bodies can undergo such an overwhelming amount of chemotherapy -- and survive.

    The tests, while they assess just about every organ in the body, specifically reveal issues regarding the heart. It has been found that certain malfunctions of the heart lead to about a 99.9 percent chance of dying during a transplant. For this reason patients such as myself must undergo a week of rigorous evaluations before they can be deemed a candidate for the transplant. While these test cannot guarantee survival even if the heart seems healthy, they do give a basic 'road map' for how well you will tolerate the transplant.

    For those of you wondering exactly what all this means, I have written up a short excerpt of what procedures will be preformed next week, and why. Each day I will start procedures around 7am that will go well into the late afternoon.

    • Tuesday
      • CT of the lungs to check the status of a patients Pulmonary Fibrosis
      • EKG to check the electric activity of the heart
      • Blood Tests for just about everything
      • MRI of the heart looking for abnormalities such as a D sign
      • A 24 hour urine test to assess kidney function
    • Wednesday 
      • 24 hour Holter monitor to assess electrical activity of the heart
      • Pulmonary Function Test to assess lung function
      • Dobutamine Stress Test to assess how ones heart will prefome under stress
      • Appointment with Dr. Burt the researching physician
      • Appointment with the cardiologist
    • Thursday
      • Right Heart Cath to assess pulmonary pressures
      • Appointments with the gastroenterologist 

    Many of these tests are actually pretty invasive, such as the RHC in which a catheter is inserted into your neck and fished down into your heart - while you are awake. Luckily, I know what to expect with every test besides the Dobutamine Stress Echo, because I have had them all before of the course of the last few years. Needless to say however, these tests will take a lot out of an individual. At the end of the week, when all the tests have been reviewed, and the physicians have consulted, the patient will typically receive the word whether they are a candidate or not. From that point, if you are deemed a candidate, all you can do is anxiously await insurance approval, or fundraiser the money to begin your transplant! I will do my best to update my family, friends, and readers as I receive results and answers. Thank you to the many people who donated to my Stem Cell Transplant Fund to make this week of evaluations possible!

    Wednesday, August 17, 2016

    Autoimmune Patients And The HSCT

    I have received an influx lately of questions regarding my current treatment plan to receiving an Autologous Hematopoietic Stem Cell Transplant, also known as the HSCT. Obviously, this is a less traveled rout for autoimmune patients, although it is becoming a more popular treatment within the community. I thought it would be helpful to my family, and readers, if I took a post to explain the reasoning behind this decision, and what the process will look like if I am accepted into the HSCT trial.
    What is an HSCT?
    First I will start off by explaining what the name means: autologous means the transplant will be preformed with my own cells, and a hematopoietic stem cell is a cell derived from the blood or bone marrow that creates blood and immune cells. The name literally means my own stem cells will be transplanted to myself. The entire point of a transplant is having the ability to give an incredibly dangerous amount of chemotherapy to a patient without causing death. The dose will obliterate all their bone marrow and immune/blood producing cells; the stem cell reintroduction is simply to save the patient from the brink of death. These new cells start regenerating blood/immune cells the body cannot on its own. It can be compared to wiping a hard drive. Everything is deleted, and then you must hit the reset button. The stem cells will reset the body by creating new immune cells that are hopefully disease free. This is a widely used practice in many cancers and blood disorders. The high dose of chemo successfully eradicated all the diseased cells -- leaving a clean slate. You can see how this process would highly benefit someone with autoimmune disease, as our primary malfunction is overproducing immune cells.

    Who receives it?
    Now that you know a the purpose behind the transplant, you're probably wondering why they are not a more widely used treatment? The truth is this treatment is extremely dangerous, and does carry a heavy mortality rate. The human body is not meant to withstand that overwhelming dose of poison, and a variety of patients are unable to survive the process.
    Th use of HSCTs in autoimmune patients is fairly recent, only coming into play withing the last fifteen years. This process is solely preformed on severe, therapy-refractory autoimmune patients with poor prognosis' that lack any form of alternative treatments -- It is more considered a rescue strategy than an actual treatment in the eyes of medical science. Studies have shown that Systemic Sclerosis patients seem to have the most difficult time with treatment due to the existing heart, kidney, and lung issues. Our already fragile organs give us less chance of success when compared to other autoimmune, or cancer patients who may not have internal organ damage. The process is currently being studied in severe Multiple Sclerosis, Lupus, Systemic Sclerosis, Myasthenia Gravis, ITP, CIDP, and a growing list of others. While patients may have much to gain form this treatment, it is only considered if all other options have been exhausted due to the evident possibility of serious side effects, including death. Currently the HSCT for autoimmune patients is a clinical trial, and is still considered "experimental" by the US. In order to receive treatment you must be accepted into detailed criteria placed by the researching physician. Very few centers around the country take part in these trials, and only accept a limited number of patients.

    What is the process?
    Once accepted into the trial by the completion of detailed evaluations, the patient will undergo "harvest." The patient will receive a weeks worth of medication that forces the stem cells to overproduce, as well as move out of the bone marrow into the blood stream. You can imagine what a very uncomfortable and painful process that would be; it has actually been compared to child birth. Once the stem cells have migrated to the blood stream a central access catheter is placed and the cells are harvested through an apharesis machine. The stem cells are then frozen and stored for the patient. Next comes the chemotherapy conditioning. Patients are given around 10-14 grams of a cytotoxic (cell-killing) agent called Cytoxan over a four day period. (For reference I only had one gram a month when I received it earlier this year.) This powerful drug will obliterate any cell left in the blood or bone marrow. The stem cells are then reintroduced to the patient which will save them from their now desolated body. Slowly, the stem cells will graft and the patients blood and immune cells will start to reproduce; this process can take weeks, or months. The patient must take heavy precaution from any possible infection risk during this time; fresh flowers, fruit, vegetables, plants, and human contact could cause infection the patient would never recover from -- Isolation is a standard precaution. Once the patient leaves the hospital the recovery is far from over. The body will continue to adjust to its new immune system over a period of about two years, through the first hundred days poses the most risk for the patients recovery. There is a long lists of "do's and dont's" the patient must carefully follow once returned home.

    Lingering Questions?
    Q: What is the difference between this, and a Bone Marrow transplant?
    A: Both BMT and SCT are used in autoimmune patients - the only difference is the harvesting process. BMT are harvested directly through bone marrow aspiration and SCT are harvested peripherally.

    Q: Is there a difference between using a donors cells vs your own cells?
    A: Yes! Autologous is using ones own stem cells, and allogeneic is using a donors cells (whether it be BM or SC.) The allogeneic transplant poses a much higher risk of mortality due to the possibility of Graft vs Host Disease. Both have actually been preformed in autoimmune patients, although there is almost no data published regarding the allogeneic transplants since it has only been preformed a handful of times. In theory donor cells would be a better option as they would not be previously diseased, however; medical journals that can be found regarding allogeneic transplants state, "clinical evidence in favor of allografting is sparse and the risks are considerable."

    Q: Are you killing babies?! (I have actually been asked that..)
    A: No. Stem cells are found many places besides umbilical cords.

    Q: How long does all of this actually take?
    A: If I am accepted into the trial I will live in Chicago roughly two months if everything goes smoothly. However the process could be longer, or shorter.

    Q: Does insurance cover this treatment?
    A: No. HSCTs for autoimmune diseases are considered experimental by the FDA and therefor are not covered by insurance. While often, the researching physician can persuade your insurance company to pay for the procedure, many patients are stuck with a 125,000 dollar bill if they want this treatment -- and that doesn't include travel expenses. To donate to my HSCT fund please visit youcaring.com/chanelwhite

    Q: Is it considered a cure?
    A: No. The HSCT doesn't not cure autoimmune patients. While some patients may find themselves in a blissful remission of disease, they are not without the effects of their disease previous to the transplant. While this is a successful treatment for many, some do relapse after treatment as a consequence of their own stem cells being diseased beyond repair, or the donor cells not grafting properly.

    Q: Why would you do it then?
    A: As stated before, this is more of a rescue strategy for those with a poor prognosis than an actual treatment. In many cases the patients undergoing this torturous ordeal are left with the two options: 1. Die or 2. Playing their luck with a HSCT. Again, this treatment is for serve disease unresponsive t any treatments currently available. Given the choice, I want to go down fighting rather than just accept my death.

    Q: Where can I get more information?
    A: To check availability head to https://clinicaltrials.gov/ and simply search your illness, or hematopoietic stem cell transplantation. It may take some digging, but there are trials still recruiting patients. For medical journals on the HSCT and autoimmune patients simply Google "Autoimmune and HSCT."

    Disclaimer: Please remember every patient is different. Some patients bodies may handle a transplant better than others. The disease the patient is fighting also makes a drastic difference in their HSCT experience. I am not an HSCT expert! All of my information is based of information received from my physicians, patient experience, and medical journals I have read. 

    Thursday, August 11, 2016

    A Towering City Of Disappointment - Health Update

    This week I made my promising trek to Los Angeles to visit a prestigious HSCT (Hematopoietic Stem Cell Transplant) doctor at City Of Hope.  A few weeks ago I had contacted this physician by email and he became extremely interested in my case. He asked if I could fly down and meet him in person for in depth consultation. I received a call from a new patient coordinator the next day, received my appointment date, and booked my flight immedietly! Fast forward to Wednesday. I traveled to COH alone because it was a very last minute ordeal. Everything moved so quickly that it didn't give my family adequate time to save for the trip, request work off, or find pet sitters. So, I waltzed into the giant campus by myself, ready to hear some good news from this physician I was so very ecstatic to meet. The paper work said to arrive at 930am for an 11am appointment, so I figured there would be some sort of blood work, testing, or pre-consultation. I arrived promptly and was given a very kind patient advocate, but told there was nothing happening with my care until I saw the physician at 11am.

    Wait what?! They expected my already anxiety ridden self to arrive two hours prior to an appointment and just wait?! Why they do that to patients is beyond me...

    So I waited, and waited, and waited. By 10:30am I was extremely nervous and antsy, and by 11am I was almost in tears. By 11:20am and still no appointment I was having a full blown anxiety attack and trying not to hyperventilate. I know that all seemed to escalate quickly, but when your life is riding on an appointment and you have had nothing but blank time by yourself to think about it, the mind goes to bad places. I finally went up to a desk and asked what was going on and they just said, "they will call you back shortly." By 11:30am I was called back to a room with a physician who obviously wasn't the one I had emailed. RED FLAG #ONE. This physician told me he was on the same HSCT team and that the other physician I flew all the way to California to see, said this doc could see me instead. Not cool. Since I wasn't about to get up and leave I started asking questions. I wanted to know about their former Scleroderma patients and how they fared during the HSCT. He expressed they had only done three and every single one had ended up in the ICU for upwards of 2-3 months, half dead, drowning of blood in their lungs. RED FLAG #TWO He told me that they were able to pull every patient back from the brink of death however, and that they were doing okay now -- Those aren't good odds if you ask me. 

    With in the first few minutes he told me I was a great candidate and fit all the criteria! He actually also expressed that my body wouldn't make it another year waiting for a transplant, and that we needed to move forward as soon as possible. It was an overwhelming YES! However, he then asked when my last appointment was with Doctor X (name withheld) at UCLA -- "Um never." I replied "Why?" He then went on to explain that I cannot have a transplant at COH without being a patient of Doctor X first; that I would have to become her patient if we wanted to move forward. RED FLAG #THREE

    ....You mean to tell me I was supposed to get an appointment with another physician at a totally separate hospital before coming to see the doctor who I didn't even get to see at COH?! And no one told me that?! 

    So even though I was a yes from their transplant team, I couldn't do jack because no one on the team bothered to tell me I needed to first get an appointment with a physician at a totally different hospital. Why the hell did I fly to California then?! The doctor who I was "consulting" with basically just apologized for "wasting my time." and that if I wanted to fly down again in a few weeks to see that other doctor at UCLA I could come back and re-consult the same day at COH. Probably the most unorganized visit I have ever experienced in my life. The entire orchestration was not up to my standards for a hospital that will literally be holding my existence in their hands.

    So lets look at these red flags a bit close:
    1. I never even got to see the physician I specifically booked a flight to meet.
    2. Everyone of their scleroderma patients have been in the ICU on the brink of death.
    3. They weren't even organized enough to realize I wasn't a patient of the physician I had to see before I came to them -- even though they had all my medical records. Did they even read them?

    For these obvious reasons I am ruling out City Of Hope for my HSCT. Don't get me worng, the physician I saw was lovely and kind; he genuinyl wanted to help but, I need a hospital that doesn't have their head up their bum if I am being frank. I am of course disappointed I wont be able to have the transplant closer to home, but I am also very grateful I had this experience to rule out the poor choice. I will not wonder "what if?" I know that this door is shut so another can open. Thankfully I have been accepted at Northwestern Memorial Hospital in Chicago for a consultation. I should receive a call next week to schedule my tests/appointment. Luckily, it's pretty comon knoledge that that if you are a candidate by STAT trial standards you will most certainly meet criteraia for the ASSIST trial! I of course have to get the absolute yes from the physician, but I don't see how I wouldn't be accepted. Woohoo! I am finally moving forward and am ready to bump up my fundraising goal to assist with transplant costs! Your assistance will help fund travel expenses to and from Chicago, housing for the duration of the transplant process, and medical expenses not covered by insurance. As I am not 100% sure of the complete cost currently, the goal may change in the future. 
    Please visit the link to help save my life! YouCaring.com/ChanelWhite

    Wednesday, August 3, 2016

    Why Is The Tube Fed Wife Tube Fed? - Health Update

    The number one question I receive is, "why are you tube fed?" I decided, even though I have that information available on my Feeding Tube page, that I would address it in a blog post. It is actually a really interesting situation, so I never really mind writing about it. This post will just be focused on my esophagus, though I am tube fed for reasons beyond that as well -- such as the dismotility of my small bowel.

    First off I need to explain what Scleroderma does to the esophagus. Most of the esophagus is made of smooth muscle tissue; this tissue creates waves called peristalsis that move food along to the stomach. At the bottom of the esophagus is the Lower Esophageal Sphincter (LES) which is essentially the door to the stomach. Its purpose is to clear esophageal content into the stomach, and keep acid from splashing back into the esophagus. In Scleroderma thick fibrosis builds in place of your smooth muscle tissue causing extreme atrophy, or weakness, of these important muscles. With this atrophy your muscles eventually shut down completely, leaving your esophagus unable to move food without the help of gravity, and in severe cases, weakening so critical the LES can no longer empty the esophagus contents into the stomach properly causing massive back up in the esophagus. Typically these findings can only be found when a patient is lying down flat, though in end stage motility disease they can be found even when a patient is standing straight up.

    This can all be documented by a study called a Fluoroscopy Esophagram, also known as a Barrium Swallow. Basically, it is a giant video x-ray that watches barrium move through your esophagus. I have had many, many, many of these studies over the last four years since my esophageal motility is at such serve end stage involvement. All the radiologists find my imaging incredibly intriguing! lol Today I had the pleasure *insert sarcasm* of having yet another one of these lovely tests done to asses my current situation as I seem to be aspiration more frequently then normal. The radiologist and his team got me all ready for the study and explained the procedure, I expressed to him that I wouldn't be able to do the laying down part of the test and he asked me why. I told him my esophagus has absolutely zero peristalsis to which he chuckle a bit saying "I am sure that's not true! Everybody has some! We will get you better numbers than zero miss." I just shook my head and asked them to start the test standing up. We started imaging with a tiny teaspoon of barium -- then they asked me to swallow. "Swallow honey!" They yelled again, "Swallow!" Well, I was swallowing. "Ohhhh," I hear from the radiology room. "Wow, her esophagus doesn't work at all, are you seeing this?!" I watched as all the interns huddled into the room to view my apparently very exciting failed organ.
    They asked me to attempt to swallow another teaspoon of barium for those who missed it -- that was a mistake. By the second teaspoon I started coughing, burping, and grabbing my chest. "Oh wow, we have to stop," I hear from the other room. My bum food pipe which has no muscle tone was unable to empty that barium into my stomach fast enough and it was now all backed up into my esophagus. We stopped the test and the radiologists and physicians had me sit down to rest while we waited for the ounce of fluid to move slowly into my stomach. Ten minutes later, I finally had passed the fluid from my esophagus to the stomach -- which should happen instantaneously. If you do the math: in order to not aspirate fluid into my lungs I would need to space drinking 1 cup (8oz) of water over an 8 hour period. Insane.


    This explains all my recent aspiration; anytime I consume more than about one ounce of fluid my esophagus backs up almost all the way to my upper esophageal sphincter. All the doctors in the room suddenly handled me with 'little kid gloves' and puppy dog eyes, expressing how sorry they were for the "torture." I just kind of laughed in between coughing fits because I  had already told them how this would end, they just refused to believe me. After that they sent me on my way telling me to never have more than two teaspoons of anything at one time or I would backup my organs and aspirate. Awesome. Somehow my esophagus has gotten even worse then it was two years ago. I had no idea you could get worse than zero. Urg. So if you're wondering why I am tube fed, it is because my esophagus sucks and cannot handle more than an ounce at a time without ruining my lungs -- and they are already pretty good at ruining themselves! lol

    On a happier note I have a date for my first Stem Cell Transplant Consult! August 10th I will meet a physician at City Of Hope in California to evaluate my candidacy for the STAT trial. This isn't a guarantee of acceptance, but simply a meeting to evaluate my condition. If I am accepted I will have to fly back down for rigorous testing to be sure my organs can handle the stress of the transplant.
    I am also happy to share that another physician has taken interest in my case! A doctor in Chicago from the ASSIST Trail wants to evaluate me as well. This isn't my first choice due to its lengthy travel, but I do believe the physician is extremely skilled at his craft -- and I would like to see what he has to say about my case. I haven't gotten a date for my consult yet, but I did submit all my new patient paperwork this week. I even received an email from his nurse that stated the physician was "eager" to see me. Hopefully that means soon!

    I am extremely grateful to have two physicians interested as I now have the ability to compare experiences and make an informed decision on what I feel will be best for my health. I will keep you all updated as things unfold! Thank you to each and every person who shared, or donated to my You Caring Transplant Fund! You have all made it possible to receive the second consult by funding 600 dollars over my goal! Having two options will insure the best possible outcome for my complicated situation. Thank you from the bottom of my heart!

    Wednesday, July 27, 2016

    A Towering City Of Hope - Health Update

    I wanted to start off this post by simply thanking everyone for their uplifting support these last few weeks. Obviously I have some difficult decisions coming up, and knowing I have an army behind me makes it a bit easier! So thank you to everyone of my readers, family member, and friends!

    My body is really starting to feel the lack of immune suppression. I have been off Cytoxan about two and a half months now, and at a very low dose of Prednisone, making my body a breeding ground for inflammation and disease. I can feel myself becoming more hunched from pain, feel the growing stiffness in my joints, and feel the ocean of fatigue hit like a tidal wave. I haven't felt this poorly in a very long time. My world is caving in once again and it's surrounding me in a thick fog. I am becoming increasingly forgetful, less active, and less motivated due to the pain and shortness of breathe that comes with just about everything I do.
    My Scleroderma Specialist prescribed a immunosupressive drug to hold me over until my looming Stem Cell Transplant, but unfortunately I was only able to take it a few days due to severe side effects. The drug caused unimaginable bone pain in my shoulders, and consistent diarrhea that just couldn't be lived with. I saw my Primary Care since I cannot contact my Specialist at this time due to his schedule, and she suggested that for the moment I increase my Prednisone to reduce a bit of the inflammation. A devastating, but useful suggestion. I have worked for over a year to decrease my Prednisone, and in a simple day I lost all my heard work.

    I have also been working closely with my Digestive Disease specialist due to frequent episodes of aspiration. I saw her this week and she suggested I take nothing by mouth, not even water, until I can preform an aspiration assessment at her hospital. Luckily, I am scheduled for next week so the wait wont be terribly long! My mouth is so dry -- I don't know if I'll make it to next week though lol ;) I am trying to be good, but darn! not drinking water is difficult when you have Sjogrens!
    This last week I also had a Stress Induced Echocardiogram which basically just takes a look at if my hearts electrical system functions correctly with physical activity. My heart actually did great, but my lungs -- not so much! After a few minutes on the treadmill my poor oxygen saturation just kept creeping lower and lower. We ended up having to stop the test at about 6 minutes because I just couldn't breathe. Overall, it seems my body is pretty much exactly where I was before Cytoxan, which was not a good place. Last year I was a wreck, and in tears at almost every appointment begging the healthcare system to do something instead of slowly watching my organs fail! Cytoxan was the magic bullet that gave me a taste of life again -- it just didn't stick. That is what is so special about a Stem Cell Transplant though, the procedure sticks!

    Speaking of Stem Cell Transplants -- I owe you all an update! Ever since my Specialist uttered the words I knew it was time to start looking as my body likes to tank fast, and hard. I took to the internet this past week to review criteria and locations for the transplant. There are two trials in the country that run the HSCT on Scleroderma patients, the STAT Trial and The Assist Trial. The STAT Trial is difficult to get into as it has stricter criteria, but is nation wide and preformed at over 8 hospitals. The ASSIST is done solely in Chicago by one physician, and has a more vast criteria with more organ friendly options. Many Scleroderma patients opt to head to Chicago as it is a late stage trial, and has been preformed on hundreds of patients successfully -- the organ friendly transplant is a plus too! I actually seem to fall into the criteria for both trials, so decided to reach out to a few hospitals in the STAT and the hospital in the ASSIST.  I am currently awaiting a response from Chicago, but I did speak with the coordinating nurse who says the lead physician is looking over my intake paperwork and will have a decision on if he wants to see me for an in person consultation soon! Seattle is part of the STAT and preformed at Seattle Cancer Care Alliance. Unfortunately, as I mentioned in my last post, a physician I did not have a good run in with is part of the team. I still contacted the trial since they are so close to my home, and was shocked by the response! After a week of waiting I received an email from the head physician stating he basically thought I was too far gone, and that I would die. The email made it seem like it would be "charity" to even look at my medical record, but I could still send them in. If they look decent enough I could then meet the physicians in person. Well -- that is just not the compassionate response I was hoping to receive. It was actually quite the opposite. I couldn't believe they would feel it was appropriate to make such judgement before even looking at my records or meeting me in person! All I wanted was a consult, and they weren't even willing to meet me. Needless to say, I will not put my life in the hands of a team that has that little bedside manner over email. It was truly disappointing.

    After that response I emailed the next closest hospital, City of Hope. I received an email back in record time -- one hour! The physician was extremely kind and seemed genuinely excited that I had emailed him and shown interest in the trial! We emailed back and forth for a good few hours as I explained to him my situation. By our last email he asked if I could fly out to California and meet him in person! YOU GUYS I HAVE A CONSULT! I am so ecstatic I can hardly contain myself! His team was super fast and called me the very next morning to set up patient registration, and take my basic information for our initial appointment. They are taking care of getting my basic medical records, and I am bringing along all I have to the appointment. Their goal is take make care and treatment as easy on the patient as possible -- and apparently that starts before you  even see a physician! I think I am in love! lol Now, a consult DOESN'T mean I am accepted into the trial, it simply means the researching physician wants to talk about my case in person, and look me over to see if I qualify. I will receive a call Tuesday August 2nd with my appointment date. From our conversation it sounds like he wants it to be as soon as possible! This hospital is definitely my fist choice due to its close proximity to my state, and the obvious understanding that a patient is a person. It will cut travel costs and the length of flights drastically than if I were to go to Chicago. Unfortunately, any physician who is out of state isn't "in network" so my insurance coverage isn't wonderful but, they are contracted with the doctor! So my current need is to come up with the money for travel, and the initial appointment which wont be covered by insurance at all. I have decided to start an online fundraiser to assist with the costs of out of state consultations, and then, if he says yes to the procedure, I will immediately raise the goal of the fundraiser to match the cost of the Transplant! Link to fundraiser found here, or click the Widget on my sidebar!


    I will be sure to keep everyone up to date as the next few weeks unfold. Be sure to follow my Instagram and Facebook for photos and updates as well! I love to document my health journey so I am sure you all will get some fun pictures of California! Hugs!

    Wednesday, July 13, 2016

    The Future Of My Treatment -- Health Update

    I know it's been a really ling time since I posted an update. Things have been a bit rocky and I just haven't been able to write about it all until I felt I had some sort of outcome for my readers, and family. I cannot thank everyone enough for being so invested in my care; it means the world to me to have such an amazing support group from all over the world. I got some big news this week... which I know none of you were hoping to hear but, it is the reality of aggressive autoimmune disease.
    As you know I was pulled off Cytoxan last month due to severe side effects. It had damaged my bone marrow enough that I was not adequately producing WBC's leaving me Nutropenic more often than not. I am actually still quite low in my ANC, WBCs, and platelets almost two months after the initial decline. I also became very ill after my last few infusions, to the point where I was hospitalized after each round. Of course this couldn't continue so we halted the medication; after missing only one round of this life saving drug my body has started to fall apart.

    I was scheduled for a Pulmonary Function Test last week which showed a 25% decrease in my DLCO (the ability to transfer oxygen into my blood stream). This is an indicator that my lung disease, Pulmonary Fibrosis, is active once again. My joints and skin have been on the decline as well, with swelling and tightness now being a daily occurrence. Unfortunately, my GI tract has taken a huge blow as well. I have begun to choke on food again as it becomes lodged in my esophagus, as well as apirating a good portion of my liquids into my lungs.

    I met with my Scleroderma Specialist from UCLA today who was surprised to see such a decrease in health over the short span of a month I have been off chemotherapy. He expressed his worry that my illness is continuing to progress and that it was time to start thinking about the future of my treatment. With the recent developments he wants to start talking Stem Cell Transplants, formally known as Bone Marrow Transplants. This procedure is a long, complicated, and expensive treatment where you receive extremely high dose chemotherapy over a few day period, then receiving life saving healthy stem cells that will graft to your bone marrow -- creating a new immune system. While the treatment is considered experimental in autoimmune disease, it has already proven to be the most useful, life saving treatment available; however, the victory of remission from your disease is only if you live through the transplant. Many weak autoimmune patients will pass away during the process due to organ rupture, and septic shock. You can see why agreeing to this procedure is an extremely difficult decision.

    Since The HSCT is technically an experimental clinical trial it is not preformed everywhere. You must fit specific criteria, at specific hospitals,written by specific physicians. You have to be just the right amount of sick - essentially dying, but not too far gone. It's a very delicate balance. Luckily, there is a trial here in Seattle, but it's run by a physician who I do not trust my life with. (If you want to read that story visit here. lol) The only hope for a Seattle transplant is to get another physician involved which is unlikely, but possible. I could however, have the transplant preformed in California, it would just add travel to my already non existent funds.
    Did I mention the experimental transplants can be over 100,000 dollars? Yeah...
    So, the game plan for now is to try one last line of defense, an immunosuppresant called Tacrolimus, and for my physician to speak to a few of the transplant teams here in Seattle until I see him again in September. The drug is nothing special, and is actually quite dangerous for the kidney, but there really inst any drug stronger than Cytoxan -- and I have already failed the rest, so that's pretty much my only option. If my lung function continues to drop we will start the process of consulting with transplant teams in the area. We already know I qualify the basic criteria, it will just be getting a doctor who is willing to accept my case.

    I know many of you are wondering how I am taking this decision emotionally. To be honest, I am not going to pay it much thought until I see my physician again in September. Why worry about a decision that isn't 100% set in stone? I have always figured a HSTC would be in my future however, so I have already given it a lot of thought, and accepted it as a part of my treatment plan. Yes, I am obviously worried about the mortality rate, but I have also seen this treatment work miracles -- and I am more than ready for my miracle! My decision will come down to two very important factors: location and fundraising. I will keep you all updated as the situation unfolds. I have a few more tests this week and next to hopefully get a good idea at where else the Cytoxan has worn off.

    Don't forget to follow along on Facebook for images you wont find here on my blog! I created a Facebook page last month and completely forgot to announce it formally. It pretty much just has stuff I wouldn't post here -- such as daily photos. Head over and take a look! As an unrelated side note -- who is attending the Scleroderma National Conference in New Orleans this year?! If you would like to meet me, I will be speaking on Saturday and would love to connect with my fellow patients/readers!

    Saturday, June 25, 2016

    To The 'Well Intentioned' Individual Giving Medical Advice

    You are my family, my friends, my social media followers, and sometimes even strangers on the street. I am grateful that you care about my situation enough to share your knowledge in hopes that you will ease my suffering. Thank you for displaying what you believe is kindness and compassion towards my difficult circumstance. I know you mean well, and that you honestly believe you can help.
    There is only one problem...

    You often become abrasive, and lose understanding that this is my battle, my fight, and my body. I have found your relentless comments on every medical update I post, your strong opinions on my social media accounts, and even private messages conveying that you “know how to cure” my incurable disease. You tell me I am making mistakes in my treatment, and that if I were to just “treat the root cause, leave western medicine behind, and take special supplements, oils, and herbs” I would be disease free. You imply that my treatment choices are the reason I am still sick. 
    Your harsh stance makes me uncomfortable, and is not helpful or supportive, but hurtful. 

    Please understand I am open to alternative treatments, but also be aware that I have a fast progressing illness, and taking the time to experiment with “miracle cures” could actually cause my organs to fail.  I know western medicine is full of toxins, and poison, and will destroy my fragile body if my disease doesn’t get to it first, but for now, it is my most viable option. When my health spirals, these drugs keep me alive; my physicians prescriptions keep me with you another day. Please accept that your “cure,” though it may have worked for your cousin, or dozens of people in a online forum, may not be right for me. Every patient is unique, and every body respond differently. While an oil may have relieved your pain, it could cause me to go into anaphylactic shock. While a diet may have improved your quality of life drastically, it could cause me excruciating pain as my body doesn’t properly digest and I am on a feeding tube. While the approach to medicine you swear by may have put your disease into remission, my body may reject it and lose valuable organ function waiting to see results. My treatment choice is literally life or death.

    You see, I am in no way against your treatments, I am actually intrigued by most of them and hope I am stable enough someday to experiment with them. However, they are typically expensive, not covered by insurance, and require in many cases to attend treatment in another state, or county. As many patients battling illness, I am extremely tight on funds and cannot physically, or financially afford your experimental, ‘no guarantee’ treatment at this time — It’s true, not even western medicine is guaranteed, but at least the statistics are in my favor. Possibly, once I am medically stable enough to travel, and have extra money, I can use your ideas to help improve my quality of life — but right now my focus and my funds must be on staying alive


    Next time you feel the urge to give medical advise please ask yourself these three things:
    1. Is my advice physically possible for the patient?
    2. Is it my place to give advice?
    3. Is now the appropriate time to give advise? 


    Then ask yourself this one very important thing:
    1. Am I respecting the patients decision?


    By using these simple guidelines you will take unneeded stress, judgement, and negativity, out of a patients already hectic life. Remember that we are battling our bodies, and if we had the choice we wouldn’t be sick at all. You have no idea how hard of a decision it is to use one evil to fight a greater evil. Hooking up to that infusion full of chemicals is not anyone’s first choice — but sometime it is the only choice that generates the ability to live another day. There will come a time where I may ask for advice, and I know you will be waiting in the wings — for that I am appreciative. 
    Instead of continually pursing me with your opinion on how I should be implementing my treatment, show me support by accepting, and respecting my decisions.

    Friday, June 24, 2016

    A Fork In The Road. Again. -- Health Update

    I am sure you all have been wondering where I've been recently. Honestly, I've just been too exhausted and somewhat defeated to write a post. The last few weeks my health crumbled around me little by little and I shut down from the world a bit. After my hell of an ER visit the night of my last chemo, during which I got a concussion from passing out over the side of the bed that did not have rails up, my body went into full flare up mode. This means my illnesses went into overdrive and attacked my systems because they didn't know how to deal with the stress of a concussion along with chemotherapy symptoms.

    My vasculitis purpura lesions.
    Though I often post about Systemic Sclerosis it is unfortunately not the only serious illness that plagues my body. I have a disease called Leukocytoclastic Vasculitis that very infrequently rears its ugly head. Well, last week it decided it was time to bring some misery into my life, which kept me home bound for a while. Vasculitis is the inflammation of blood vessels so serve it causes them to become necrotic and leak, forming excruciating lesions called Purpura. In my case the purpura always manifest on my lower calves, making walking nearly impossible due to pain. I honestly have no idea how to describe the pain of these lesions -- the closest description would be to imagine the worst bug bite you have ever had, now pretend it was 1000 of them in one spot, then pretend it goes deep into your tissue causing internal bleeding -- but nothing actually bit you. Sounds great right? There really isn't anything you can do besides take steroids to reduce inflammation, though severe cases require Cytoxan treatments, which I already do. Basically my body just hates me and decides that no matter what toxin I throw at it, it still will cause destruction of my major organs. So yeah, that was an adventure. The lesions typically last one-four weeks, then dissipate. I upped my Prednisone for a few days and the pain and swelling seemed to subside a bit, so I am mobile once again, thank goodness!

    I had an MRI last week to take a closer look at my cervical spine due to some decreased sensation in my extremities. I have yet to go over the results with my physician but, from the report I read it all looked pretty straight forward. Between C5-C6 I have moderate Degenerative Disc Disease with obvious damage, as well as straightening of the cervical lordosis. These changes could be due to 16 years of gymnastics injuries, but I guess I will never know! lol I am pretty sure my last MRI of the cervical spine showed something similar. For these reasons I don't believe that is what's leading to the numbness and tingling in my extremities, but I will find out more once I see my doctor next month for a follow up.

    Today I had an Echocardiogram (heart ultrasound) preformed to rule out causes of my shortness of breath, chest pain, and irregular heart beats. My echo had actually improved in a few areas, but declined in a few too. It seems the Cytoxan helped my heart! Woohoo! My Ejection Fraction increased by about ten percent, my pulmonary pressures lowered, and a few of my valve regurgitations improved. I was extremely happy to see the results of this drug improving another vital organ. The only worse off results seemed to be the size of my left chamber, and a slightly prolapsed valve. For some reason my left chamber increased in size, which I am guessing was from the stress of my lungs declining so rapidly last year. It wasn't a huge increase, so nothing to worry about now thankfully. I am not sure why I suddenly have a prolapsed valve, but I will be talking to my physician about it when we follow up next month.

    Last but not least, I got some big news from my Scleroderma Specialist through a phone call yesterday -- you know something is up when they call you at home. Unfortunately my CBC (complete blood count) has been very off the last two months. It seems my bone marrow cant keep up with Cytoxan and is failing to produce my white blood cells and platelets. My poor lab results had giant 'L's next to just about everything, Typically your counts bounces back about three weeks after chemo, but my bone marrow just isn't holding up like it should. Due to this my body is not produce very important white blood cells called Neutrophils which specifically fight bacteria. This is actually a separate condition called Neutropenia and can be very dangerous. It leaves me extremely susceptible to infection, even from my own "healthy" bacteria! Technically someone that is Neutropenic should not be around children, animals, public places, or the elderly -- basically just sit in a sanitary bubble with a mask on.
    So now the big news: since my counts aren't returning to normal between chemo rounds I am being taken off Cytoxan. I am devastated, and happy at the same time. Typically when a chemo patient goes through low WBC the physician has them restart at a lower dose, but we have already done that once and my WBC count still can't keep up, proving the Cytoxan is damaging my bone marrow at unhealthy levels. Hopefully we can find a comparable medicine, though Cytoxan is the top treatment options besides a full blown Stem Cell Transplant. I am currently unsure what the future holds, but trust my specialist and know he will have a plan when I see him next month. Hopefully my body will hold out till then.

    Oh, and in case you're curious. I attempted to take action against the hospital that didn't place up my bed rails, but to my surprise the physician lied in the chart notes stating "patient didn't hit head," thus creating a hearsay situation; skilled physician against sick girl. They did state I "fell to the floor" but followed up with "didn't pass out and didn't hit head" which is all blatantly false. They also never documented my episode of low RR followed by unconsciousness. Basically I am SOL. I have filed for a documentation amendment, as well as called the Washington State Department of Health and filed a formal complaint. Luckily I have proof I hit my head, as well as two physicians verifying a concussion. Hopefully this hospital will be forced to take responsibility for their falsified documents and negligence!

    Thursday, June 9, 2016

    Cytoxan Round Nine & The ER Visit From Hell

    Tuesday I had my ninth round of Cytoxan ...It is incredible the difference a day makes in the life of someone with a chronic illness. Monday I was on the news with the only trace of illness being the thin tubes running from my dress, while Tuesday I looked like death, and ended up in the emergency room with a respiratory rate if 5. (Yes, you read that right. 5 breathes a minute.)
    While flushing my port the oncology nurse mentioned it was a bit harder to flush than my last round, which wasn't a surprise to me as I had noticed that it had been a bit harder to flush over the past week. The blood return in the port was still fabulous so we didn't worry about it much and continued with the infusion. The actually infusion went off without a hitch, though I began to feel the effects of the drug much sooner than I normally do. Once at home I rested on the couch and took the afternoon easy with a light nap. About three hours after returning home I noticed a pain in my neck over my port side; the pain eventually spread to my armpit, and down the right arm. As the pain intensified I started to feel a bit light headed. I figured I possibly had a blood clot that had been dislodged from my port, though it was unlikely; I decided not to take the chance with my life and head to the nearest ER. Noel had left for work only minutes before so I enlisted the help of my kind neighbor who took me to the nearest hospital. Upon arriving at triage the pain had intensified, my breathing was labored, and I was having a hard time standing. The Triage nurse actually caught me before I lost my balance and quickly brought over a wheelchair for me to rely on.

    I explained the situation, jugular/arm pain, increasing dizziness/lightheadedness, and labored breathing. The nurse took one look at me and got on her phone before I left the room, "Hey, get room 16 open immediately; we have a patient that needs to be roomed now." She hung up and told me they were working on getting a patient discharged and I would have the net room. They wheeled me out to the waiting room barley conscious. I waited, and waited. Half an hour passed and a woman complaining of a deep cut finger with a large bandage went into triage and never came back out, meaning she was placed in a room immediately. Why they let me sit in the room while others were placed before me in my condition is beyond me -- finally I was wheeled back to the emergency department, only it was in "the nose bleeds" where there was only one nurses station, no monitoring equipment, and was away from the actually emergency wing. My heart sank. The nurse who wheeled me back asked if I could get up to the bed from the wheelchair and I told her, "I don't think so." She helped me onto the thin table which had no railings up, nor did she put them up as she left the room to fetch a gown. Suddenly I got very nauseous, I was wearing a mask to protect myself from germs and attempted to rip it off in time, but to no avail; vomit came poring out of the mask as I turned my head to the side to finish over the bed.

    Next thing I know I was looking up at the ceiling with three nurses above me frantically calling for help and trying to get me off the floor. After vomiting I blacked out, fell off the bed which had no rails up, and slammed my head on the ground. The nurses got me off the floor, but I was very out of it. They tried to get me changed and explained that we would be moving to a room where they could monitor me more closely. I told them my head hurt -- it really hurt. I found myself in Trama Room 3 after the incident with a lovely nurse who watched me like a hawk. About twenty minutes after the incident I got very tired -- next thing I know I was being shaken awake by my frantic nurse, "Chanel? Chanel! Can you hear me? Wake up honey? Chanel! Can you hear me?" I gasped for air and woke startled from yet another black out. My stats had alerted the nurse who had run in to find me on the cusp of unconsciousness. My respiratory rate had bottomed out at 5 and my heart rate had dropped to a measly 50 bpm  (My average is 89), all obvious signs of a concussion. At no point did the ER department, nurse, or physician address the fact that I had fallen due to negligence and now had a concision; no CT scan was done, no alertness or neurological tests, and no mention of the word "concussion." I lied in the bed and struggled to stay conscious while already fighting the issues I was admitted to address. The physician ordered a basic blood panel, and an ultrasound to check for a blood clot, all of which came back clear of any abnormalities. While I was there I ended up losing just about every bodily fluid without intention. Not only was I dry heaving uncontrollably, I soiled myself in the process. After a few hours they decided to send me home, but only under the supervision of another adult who could "make sure I was arouse-able." I was also told I would have to come back the next day to get my Port placement checked by Interventional Radiology, and would need to schedule an appointment with a cardiologist who could further diagnose my issues the emergency doctor could not..

    Frightened and shocked at this horrible experience, I luckily had a pre-planned appointment with my GI and Neuro the very next day before any of this had happened. My Neuro preformed a quick neurological exam after I complained of the fall on my head and found some obvious abnormalities. I was completely unable to close my eyes and touch my fingers to my nose, along with other simple tasks. "Yeah, it's absolutely possible that you have a concussion," she said. My GI who I saw next addressed my incontinence issues and preformed a rectal exam. She found that the muscles of my anal sphincter where extremely weakened, which she said is an unfortunate complication of Scleroderma. I asked her what I was supposed to do?! I mean for heaven-sakes I am 24 years old and I cant keep pooping my pants every-time I sneeze or walk too fast! She told me she could refer me to Pelvic Floor Therapy but doesn't believe there is much muscle left for the therapist to work with. That was a bummer. Next I headed immediately over to IR who checked my port placement. They said everything looked normal, but didn't dress my needle correctly which lead to blisters the next day. (Yes, my skin is that sensitive.) The next I saw the cardio, who based off the "in office one second long EKG", told me I was probably fine and to head home. I basically expressed that, that wasn't good enough so he settled on coming back for an Echocardiogram next week, as well as an MRI.

    For those of you wondering, I have already contacted the patient relation/safety representative at the hospital regarding the railing incident, and lack of concussion acknowledgment. I have been counciled that I should look into the situation further, and get a lawyer for "medical negligence," but I honestly do't have the energy with everything going on in my life. I am exhausted, miserable, nauseous from chemo, and have a huge headache. What a week.