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!


  1. You are a real trooper. My husband has gone thru a lot (different disease, some similar tests) and he is a tough old bird of 63. To see your cheery face and know you are so much younger leaves me in awe. God bless.

  2. You give people a reason to have hope n faith