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!