Last weekend was the 2015 National Patient Education Conference held by The Scleroderma Foundation in Nashville Tennessee! I cannot express to you all my excitement as my Mother and I lifted off the tarmac in Seattle. Last year I had been unable to attend due to an emergency surgery so we made a pact that we would attend the conference that upcoming year! After a year of sheer anticipation the conference did not disappoint! I had the absolute pleasure of meeting some of my best friends in the entire world! As a young woman suffering a rare disease I have grown very close with many other individuals suffering this illness through social media, and to meet them in person, to hug them, spend time with them, and actually speak to them was a dream come true! I cannot even explain the immediate, lifelong connections I have sown this past weekend.
We had the once and a life time experience of partying downtown Nashville on our second day, which was actually quite comical! All of us gimpy individuals with oxygen tanks, feeding tubes, wheel chairs, and ports jetting out of our chests while dancing and singing to country music at the top of our lungs. On lookers must have been very confused! lol This was hands down one of the most memorable experiences of my life! We even got to meet the band who took pictures with us after the show!
On the last day of conference I had the great honor of speaking on the "Living Well with Scleroderma" Young Adult Panel which consisted of me, and two other patients. I adore public speaking, but never dreamed I would have the opportunity to speak at a National level! I am still in shock that it happened! I finally feel I know what my calling in life is... to yell the name of this disease from the top of every mountain!
I unfortunately got pretty sick on the last day as we winded down to the last few hours before our flight. My body just isn't used to being off a couch very often. Once the adrenaline wore off I crashed rather hard. I ended up early making it to the airport before I passed out. Luckily I had wonderful travel companions and there were doctors from the conference in the airport if I needed them ;) lol Overall, I wouldn't trade this experience for the world! It was worth the immense body pain and struggle on that last day!
Once I returned home it was back to the daily grind with an early morning appointment to see my Electrophysiologist. This doctor is a specialized Cardiologist that basically deals with the "wiring" of the heart, in very simple terms. I was referred to him for my SVT episodes, but what we decided to treat was something entirely different, and actually the reason behind my SVT! First off let me just tell you that I LOVED this doctor. He started off by being right on time, which is always a good sign, and continued his awesome streak by talking to me like a well informed individual! He could tell I knew my stuff so he didn't use any of that "baby language" as I like to call it ;) After looking over my medical records, and chatting to me about my symptoms he decided the real problem wasn't my heart at all, but my body ability to retain electrolytes. His words were "Im going to be honest, your body is screwy. Extremely screwy!" Which I whole heartedly agree with. He explained that due to my autoimmune issues, severe malabsorbtion, and my reliance on a feeding tube, my body just can't retain its electrolytes and minerals it needs to function properly. This lack of necessary nutrition and absorption is causing severe autonomic dysfunction, meaning my body can no longer regulate things like proper blood pressure, heart rhythm, and heart rate. This dysfunction is whats causing my fainting spells, low blood pressure, tackycardia, and odd heart rhythms. His protocol in this situation is fairly simple, to prescribe a medication that helps the body retain salt and electrolytes! Now, the catch is, it's a steroid and I am already on steroids... which really shouldn't be done, but we have no choice. It's safe, just not ideal. The other catch is that you have to have at least some electrolytes and sodium for the body to hold onto, which I really don't. He basically said he can't compare my baselines to the typical norm because I am truly one of a kind, so we are basically free falling here! Hopefully this pill will work, and if not our backup plan is to start daily saline infusions to keep me out of Hypotension, which is a fancy name for serious low blood pressure. If you all know what the condition POTS is, that's basically what I have with a few other autonomic dysfunctions thrown in. In a blanket term it's called Dysautonimia. Normally POTS is not connected, or due to a connective tissue disease. It is typically a stand alone, idiopathic condition, so he is hesitant to call it that. Again, he said he can't really compare me to others since my body is so out of whack! Overall it was a great appointment and we have a solid game plan! No ablation needed! I can't wait to follow up with him in a month!
Next I was off to my Pulmonologist for a pulmonary function test (PFT). We have been following my pulmonary and diaphragmatic function very closely lately. A typical patient has this test done every six months to a year, whereas I am having it preformed every 4-6 weeks. There are three very important factors we look at on my PFT, the Diffusion Capacity (DLCO) which expresses the lungs ability to push oxygen through the air sacs of the lungs into the blood, the Forced Expritory Volume (FEV1) which expresses the amount of air your lungs can force out, and the Respiratory Muscle Function (PI/PE). This values give my doctors a look at my overall respiratory systems functioning ability, and also a good idea of how my disease is progressing. Unfortunately during this PFT we saw a large decrease in my respiratory functions. My DLCO dropped by almost 20% leaving me now in the mid 60s, my FEV1 dropped 11% leaving me in the low 80s, and my PE/PI stayed within my typical range thankfully, however it only functions at a measly 20%. These numbers make the obvious statement that my disease is progressing and the current treatment regimen I am on is not adequately slowing this disease. The DLCO especially is a huge red flag as any number in the 60s means there is an evident obstruction, or notable Pulmonary Hypertension. While I was in significant alarm seeing this drop my doctor seemed unfazed, stating they were probabaly false readings. I conveyed my belief that they were not as this decreasing pattern has been pronounced over the last year however, he still did not agree. While I trust his expertise, I am very unhappy to have lungs functioning so low, with a progressive decrease I function, especially at the age of 23. For this reason I will be re-preforming the tests in two weeks to verify the results, and getting a second opinion from another doctor who is wiling to preform a right heart catheterization which I believe would be beneficial. I will not longer sit idly by and accept everything my doctors are telling me; this disease needs to be treated more aggressively and I will not rest until I found someone who agrees with that. I made some professional connections while at the Conference this last weekend and am confident these connections will help lead me to better treatment options, even if they are experimental.
While I convoy mostly strength and resilience and this blog I want you all to know that having a disease that you know will take your life much before your time is truly devastating. I have my days when I break down, become distraught, cry, and feel emotional about my circumstances. Constantly being left in limbo about your life is such a difficult existence. I loath relying on medical professionals for my every need; I was once an independent individual with reigns to my world, but declining health has stolen that from me. While I feel I have prepared myself for every blow, and truly accepted the destructive nature of this illness, sometimes it is still hard to embrace. Each day is another hurtle, another tempest, another period of time spent trying to pick yourself up again. No matter how adjusted to bad news you feel you are, sometimes it just still hurts. I have said before that life with this illness is like a vase being repeatedly smashed. I know that my beautiful, perfect vase will be destroyed and I have prepared myself. I know that no matter how hard I try to frantically place those broken pieces back together I will never be able to place them exactly the way they were before; it would be a lost cause. I feel with this knowledge the blows become easier to take, each smash doesn't leave me lost. I have learned that even with my shattered pieces I cans till construct something beautiful, though it may be different, it is still functional and pleasing to me. Yes.... I know my vase will be mashed and I am prepared... but that doesn't mean it doesn't hurt watching those pieces splinter. It doesn't mean that glue I used to reconstruct it was dry of tears, or that the tape I used wasn't wet from sobbing. I am a positive person, and a strong individual, but I am here to let you know that accepting and embracing every emotion is never a bad thing, even if that emotion is pain, fear, hurt, or the acknowledgment of an unstable future.
This upcoming week I have many appointments and procedures as usual. I start with a Smart Pill Capsule Study to time my digestive function on Monday morning, plus a Rituxan Infusion, and a Dermatology appointment later in the week. Before I sign off on this post I wanted share a wonderful article one of my beautiful Scleroderma Sisters, and fellow Chronic Illness Blogger wrote after a conversation we had last month. I am honored to work with this individual; the Scleroderma community is so blessed by her knowledge and willingness to share.
To visit our Feature: You Won't Believe The Story Behind These Two Faces
To visit her Blog: Comfortable In My Thick Skin