Pulling At Heart Strings - Health Update

While I did received some bad news this week, so many wonderful things have been happening in my world! ...Wow lets just take a moment to appreciate that. Those are not words I get to type very often! While my health may still be struggling, as it always will be, I finally feel I am on a solid treatment plan that gives me enough energy to at least enjoy a few small victories! My weekly iron infusions have made a huge impact on my overall energy levels and improved my quality of life immensely! Plus I no longer have the coloring of a zombie! lol It feel so good to have iron pumping through me veins again! 

I had another dosing of the Rituxan (Rituximab) which suppresses my B-Cells. This drug is helping to suppress my disease and I FINALLY feel like it is working! With this new more frequent dosing I can almost see and feel the differences in my skin, breathing ability, and organ function! I have been able to start drinking things again which is amazingly satisfying and I believe it is a side product of the drug. I am hoping to work up to small amounts of puree items but that will only come in time if my body is willing. Disease is a tricky and conniving little demon... it may decide that liquid is off limits again at any moment, but lets hope not! I feel my ability to breath has improved slightly too; my doc said my lungs do sound a little less stressed! Woohoo! 

I kinda feel like I live at the infusion center currently; I have made some pretty good friends with the nurses after seeing them every five days! One of the nurses that watched my Port access last time got to use me as her first ever Port access patient! She did so well and got a perfect access!

I had an appointment with my Rheumatologist who was very satisfied with my progress! Between the new drugs, more rigorous treatment plan, and use of oxygen I seem to be really holding my own.

I also had an appoiment with my Gastroenterologist this week. Since my body has decided that SIBO (small bacterial intestinal overgrowth) is my new normal I am officially set to be on antibiotics the rest of my life. While this is not optimal, it is the only way to combat SIBO, and is much better than the alternative -- diarrheaing out all your nutrition and iron, which requires hospitalizations every other week. I will be on a alternating daily schedule of heavy antibiotics: one day on, one day off to tackle this overgrowth and hopefully settle my bowls. This issue is what is causing my serve anemia as my bowls can no longer absorb iron. We are hoping that by counteracting the SIBO with antibiotics on this heavy regiment that my iron will level out so I can discontinue my infusions every five days. As of now we will be stopping the iron infusions until we know if the antibiotics are working; if I still in 6 weeks can't keep up my iron levels we will be starting frequent infusions again. Since all of this seems to be spiraling out of control lately my doctor ordered a "Smart Pill" test that will track my digestive system transit time. I basically just get to swallow a giant pill (which may actually have to be placed through an endoscopy since my esophagus doesn't work) which will decipher where my intestines have slowed/stopped so we can find the right medication to stimulate that particular area of the tract.

Lastly I had an appointment with a Cardiologist to follow up with some palpitations I've been having, and a small murmur that has recently shown up in clinic. This new cardio went over my history, and old Holter Monitors results that track the rythem and beats of the heart; what he found was quite surprising. All of my monitors over the past year have showed multiple episodes of SuperVentricular Tachycardia (SVT.) This can be a serious arrhythmia due to improper electrical activity in the heart causing a plethora of problems including fainting, dizziness, lowered blood pressure, chest pain, and a decrease in cardiac output. The doctor was pretty peeved that these test results were not read properly, and that I was not informed of this issue; SVT clearly correlates with all my symptoms. Luckily, this is an easy problem to fix with a medical procedure called an Ablation. An ablation is preformed by placing a long thin catheter through a blood vessel in your groin into your heart where a piece of the tissue is modified using Radio Frequency, essentially re-wiring the heart to stop beating incorrectly. While this sounds very big and scary, it is supposedly a simple procedure that has very high success rates! He told me that I will feel much better once my heart is re-wired to beat properly! We haven't scheduled this procedure yet, as we want to do a few more tests before we move to this somewhat drastic measure. For now I will re-preform a Holter Monitor for 24 hours, and follow up with him to address the results in two weeks.
As a interesting side note, he expressed to me that my small murmur is due to a "heart string." My  Left Ventricle has a small chord running across it that causes innocent murmurs; apparently it's completely benign and is an abnormality formed at birth.

A very BIG thank you to all the supporters who came out for the Scleroderma 5k last weekend! I cannot begin to tell you how much it meant to me to have so many people walking by my side! Our team had the largest number of members; 35 strong! I am still so blown away by the love I have received from our small community! I walked almost the whole 5k, but alas, my poor body couldn't make it all the way. Thankful my husband Noel made it up for me! ;) Running a 10k in my honor! I love that man so much!

In some other rather exciting news I have been asked to be a speaker at this years Scleroderma National Foundations Patient Education Conference! I will be one of three patients on a panel talking to others about "Living Well with Scleroderma"! I am so excited for this opportunity and beyond flattered that the Foundation thought to ask me of all people! Now I am even MORE excited to attend next month! I would love to meet all of my readers at the conference if you will be attending! Look for my panel in the schedule on Sunday in the Belmont Workshop Room from 11-12pm!

How Illness Taught Me That Broken Things Can Still Be Beautiful

Life is a fragile thing. In an instant it can be destroyed by a circumstance beyond your control; the existence you knew shattered like glass beneath your feet. No matter how hard you attempt to piece back the fragments of that previous life there is no restoring it to exactly the same as before.
I remember my life fracturing moment vividly; it consisted of one hour that changed the entire course of my being. October 21st 2011 I was diagnosed with an incurable, and fatal illness called Systemic Scleroderma. I remember walking into that Rheumatologist office equipped for the worst, but still being unprepared for the news I received. In that hour I was told I would be fortunate to live another ten years -- and that was only if my circumstances were favorable.

At the age of 20 I should have had engagements such as college, relationships, and my career swirling through my mind, instead I had my own mortality. I left that office with pamphlets to read, treatments to start, and a new way of life to begin. I was warned of the path my disease would take, and of the horror I would soon endure. My health forced me to watched idly by as my fellow high school graduates left for college, received their degrees, started budding careers, and even began their families. As they progressed through the natural events of life I was alternatively attached to events such as chemotherapy, long hospital admissions, support group, and never ending chronic pain. Slowly over a course of three years my body began to deteriorate, vital systems starting to shut down, and organs beginning to fail. By the age of 23 I was being kept alive by a surgically placed feeding tube, supplemental oxygen, dangerous chemicals, and a team of specialized physicians.

As a young adult you have so many goals for your life, and you honestly believe you have 100 years to achieve them. Suddenly I was given a sliver of that time, and a body that could not handle even the simplest of tasks. I focused on nothing but reclaiming my shattered pieces, of sweeping up and attempting to place them exactly the same as they had been before. I believed I could attain that pre-diagnosis life if I just situated all the shattered pieces back together -- but I quickly found out that that is not the way life works. Many of my pieces no longer existed, crushed beyond what I could realistically repair. Just like shattered glass I found I could not perfectly align the fragments to resemble the flawless and unbroken product. I however during my journey discover that those splintered pieces could be fashioned into something quite new and extraordinary. While this current finished product may have cracks, protruding edges, and be held together by messy glue, it has certainly never been more dazzling.

True joy is letting go of what you believe your life is intended to be, and instead revering all that it is.  Once I accepted this new existence, alone with every alteration and imperfection, I began to enjoy my life again. I began to enjoy who I was again. I no longer focused solely on recovering what I had lost, but on creating something new. I focused on the small victories and minute satisfactions found in my day to day routine. I took the time to enjoy every breath, every word, and every moment. Although my existence doesn't seem like anything to be cheerful about to the typical observer, I have found so many incredible moments to revel in. My life may not be glamorous, or as long as I intended it to be, but it is one full of simple pleasures and an understanding of how broken things can still be beautiful.

Pumping Iron - Health Update

This last weekend I had the wonderful opportunity to spread some Scleroderma Awareness in Seattle at The Great Wheel which was lit up in Teal for Scleroderma Awareness Month! Our Washington State chapter is such a great group of individuals dedicated to sharing knowledge about this disease.
Advocacy work such as this is truly my passion, and even though not one person was interested in learning more about the disease during their sunny excursion on the pier, I still had a blast! I even met Spider Man ;)

As many of you know I was hospitalized last week due to a quick onset of anemia. I have never before encountered this ailment and my doctors are quite puzzled as to why I have a sudden onset. My Iron, Hemoglobin, and Ferritin, seem to be plummeting out of the blue. We will probably have to do some further follow up such as an internal scope to check for possible internal bleeding as I must be losing all this blood from somewhere in my body. This issue can be quite common in Scleroderma patients so my doctors are not surprised, especially with all the bowl issues I have encountered lately. Unfortunately the SIBO situation has not improved even on the heavy antibiotics so my GI may have to come up with a different game plan in the near future; I see her again at the end of this month.
This week I started my iron infusions, as of now my doctor has ordered them to be received every five days! I am hoping that as I stabilize we can move to a more tolerable frequency such as once a month. I am receiving the infusions at a new center down the freeway from my house so I dont have to trek all the way to the university every five days. This new center is amazing and I am totally in love with all the nurses! Everyone was so friendly and excited to have a patient with a Power Port to practice on! I believe I was their first patient with a Port to arrive at this newly opened center. Luckily, they had a seasoned Port nurse, but all the rest had never accessed one before but were eager to learn and watch! It was rather fun having them all huddled in my small room to get a better look, I felt like I was giving a presentation to a class ;) And you all know how much the teacher in me loves that! The infusion went smoothly. Iron is odd because it looks like tar and leaves an odd caramel taste in your mouth. Luckily the infusions are very short and I am only at the center about an hour, so I was quickly on my way home! I let one of the excited young nurses de-access my port, she was so grateful to have a laid back patient to practice on -- while many people would call this "being a guinea pig" I truly don't think of it that way. I enjoy giving learning opportunities to the awesome medical professionals that take such good care of me. I loved how happy the nurse looked when she pulled out that large needle successfully!

I also wanted to take this opportunity to personally thank each and everyone of you who donated to my GoFundMe account. I have officially raised enough money to purchase a Portable Oxygen Concentrator! I am now free of heavy tanks that hinder my ability to partake in my favorite activities such as swing dancing, hiking, and traveling. I now have the ability to travel on planes, trains, and ferries; no longer locked in to my small hometown. I am so excited to start this new journey of breathing easy! Thank you all from the bottom of my heart! You have literally given me the gift of adventure!

Next week will be a busy one! I have another Iron Infusion, a Rituxan Infusion, and an appointment with my Rheumatologist; however, Saturday is the day I have been waiting for! The Seattle Scleroderma 5k will finally be here! Please contact me by email for more information regarding the walk or visit Scleroderma.org/washington and follow the 'special events' link to the Seattle "Into The Feature" walk! I look forward to seeing all of you there supporting myself, and this amazing cause!

Scleroderma Awareness Month

852 pills & supplements taken.

360 hours of enteral feeds pumped.

268 hours of supplemental oxygen breathed.

15 hours spent in an uncomfortable waiting room chair.

24 hours spent with physicians instead of my family.
4 iron infusion pumped through my central line.

5 hours of drugs infused directly into my heart.

This is what any given month of my life currently equates to. 

Scleroderma (Scl-eh-roh-derm-uh) is greek for hard-skin, but this hell of a disease is so much more than that. Take a moment to imagine having thick concrete slowly poured into your skin, your blood vessels, your organs; horrifically crushing and solidifying you until movement becomes impossible. 

This devastating vandalism of the body is a result of an over production of collagen, a connective tissue protein that holds us all together. When overproduced the collagen becomes thick, fibrous scar tissue throughout the patient, crushing their blood vessels, paralyzing organs, and hardening the skin -Essentially turning you into a living statue. Like many other rheumatic disorders, Scleroderma is an autoimmune disease because the condition triggers the body to make antibodies against itself. Chronic, complex and debilitating, this disease can affects the internal organs with life-threatening consequences. In its systemic forms scleroderma can damage the lungs, heart, kidneys and gastrointestinal tract with grave results. Vascular damage due to a loss of circulation and oxygenation can result in tissue death, severe ulcerations, and even a loss of digits. In many cases, the joints and muscles are affected, resulting in widespread chronic pain and limited mobility. Pain, ranging in severity from uncomfortable to unbearable, is a common characteristic of the disease. 

The skin tightening alone can leave patients physically unable to move their extremities, or in severe cases even entire limbs. These patients bodies are permanently contracted and disfigured leaving them unable to preform simple tasks such as opening a bottle, putting on their shoes, pulling up their own pants, or brushing their own hair. This disease changes everything you knew about yourself, and strips you of the dignity to do the most basics of life's tasks. 

Many patients actually resemble each other because of the
resulting facial features; large rounded eyes, red blotchy
rashes, receded lips, and skinnied noses. 

There are multiple types of Scleroderma: 

• Localized Scleroderma: (Morphia & Linear) Which effects the skin, muscle, and joints, but does not normally effect the internal organs. In very rare cases it has effected the GI tract and brain in patients with 'En Coup De Sabre'. This form is still extremely debilitating, especially in children, as it can hinder their normal growth and development. 
• Sine Sclerosis: Which effects only the internal organs and not the skin.
• Systemic Scleroderma: (Systemic Sclerosis & CREST): Which effects the skin, joints, muscles, vascular system, and internal organs. There are three subsets of systemic scleroderma which effect the organs at different progression rates: limited, overlap, and diffused

Today, there is no way to prevent any type of scleroderma and there is no cure. Treatments are available for some, but not all of the most serious complications of the disease. Current treatments include medications that suppress or modulate the immune system, chemotherapy drugs, vasodilators, Proton Pump Inhibitors, and ACE-inhibitors. Other treatments options include the placement of feeding tubes, the use of oxygen, surgical procedures, or Stem Cell Transplant/therapies. Presently, most treatments act to slow the progression of the disease and limit damage rather than truly arresting the disease or fixing existing fibrosis. In addition, some of the drugs currently in use can have serious side effects. Patients are normally seen by teams of physicians who assist in the management of different organs, and body parts effected. Treatments at best help ease discomfort and prolong life expectancies, though prognosis is usually poor in the systemic forms of the disease ranging anywhere from a few months, to 20 years.

How Does Scleroderma Personally Effect Me: 

Scleroderma is what many call a 'designer disease', it effects each of its victims entirely different. While many of our symptoms and characteristics may be similar, we all truly have unique journeys. 

Scleroderma has effected my skin minimally, leaving me with a thick hide instead of truly hard accustomed to most Scleroderma patients. My fingers are stiff and unable to straighten no matter how hard I push them together; my face, lower legs, and lower arms have also thickened to the point where I feel somewhat like stiff silly putty as opposed to hard plastic like many Scleroderma sufferers. For me this disease has greatly affected my internal organs and vascular system. The soft muscle tissue organs such as the esophagus, stomach, intestines, colon, and respiratory muscles have hardened, leaving them unable to function adequately. Image taking the esophagus, a soft wiggling organ that uses peristalsis to gently move food to your stomach turning to rock; no longer able to push food along in waves as it is so stiff to wiggle or contract. Imagine the stomach no longer able to empty, leaving you feeling like you have food poisoning every time you try to eat, violently vomiting as the body rejects the food it cannot move through its system. My digestive system has literally turned to stone, no longer able to do its job; no longer like a sponge that can absorb nutrients, but a solid mass that nothing can penetrate. Imagine the diaphragm no longer able to expand properly leaving your lungs unable to fill to their full potential; shortness of breath following your every foot step.

My vascular system has been crushed by this extra collagen leaving me horribly under oxygenated in my extremities due to a lack of circulation. My finger tips literally die, turning into ischemic ulceration that must be surgically amputated. Even when I do not feel cold my hands, feet, and even knees, wrists, nose, lips, and tongue will turns a violent shade of purple. This vascular issue is not only found in the visible vessels, but the organs as well leaving breathing difficult when I become cold.

Scleroderma has taken my once active life and left me with a husk of a being, unable to preform simple tasks such as eat, breath, or button my own blouse. I found myself stepping down from a promising career as an educator due to my physical limitations and being placed on State Disability at the young age of 23. I am fed by a feeing tube surgical placed in my stomach, breath supplemental oxygen through cannulas placed in my nose, and am pumped full of dangers chemicals through a Central Line in my chest that keeps me alive another few years. While this disease has literally made my existance a living hell, it has provided me with an incredible view of life one only receives through immense suffering and pain. For more information regarding my diagnosis please visit the 'My Story' page located across the top navigational bar.

My Current set of daily pills, liquid supplementations, and devices.

My tightening skin leaves limited mobility in my fingers.

  

How Does Scleroderma Effect Other Warriors:

My incredible friend Jessica M, and founder of Scleroderma Strong, has bean effected in a much different manner than I; Jessica battles severe skin tightining. Since I do not have much expertise in this area of the disease I asked her to share what it is like to live with this ailment, and to share a few photos to enlighten readers of Scleroderma's physical effects. 

"When I was first diagnosed with Scleroderma, 4 years ago, I had no idea it would advance to the stage I am at now. Originally I was diagnosed with Limited Scleroderma, which means the tightening is limited to certain areas of the body. But as time went on I became worse and advanced to Diffuse Scleroderma, which is a lot of skin involvement, included with some organ involvement. My skin is so tight, it cannot be pinched! Imagine that. The worst area it has affected is my hands, which isn't surprising because my hands were the very first place I experienced tightening, 8 years ago. My fingers are stuck in a claw-like position, making everything impossible to grip. I can barely hold a cup, fork, sandwich, book, turn the steering wheel, etc. Not to mention the ulcers on my knuckles! My wrists barely move forward or backward. My shoulders are so tight, I cannot lift my arms above my head. The skin around my neck is so tight, I can hear it cracking when I turn my head from side to side. It is essentially like I am frozen in place, with little room for dexterity. So day-to-day life can be very difficult! But my family helps me with everything. Without my support system of good friends and family, I wouldn't make it through the day!" 

   

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Woman and men like me, like Jessica, and like so many others battle this disease day in and day out; as she once so bravely put on her website, "your worst nightmare our my reality." Each and everyone of us work to bring awareness to this unknown ailment that strikes our fragile bodies; we fight for our lives, but also for our sisters and brothers battling by our sides -- We are too young to be lost to these disease. 

Being Sick Is Not Glamorous - Health Update

While many pictures of me will show little evidence of illness, let me assure you, being sick is not a glamorous existence. Without make-up, stylish clothing, the concealment of my medical devices, and the repression of specific information regarding certain unpleasant symptoms, my life is actually quite vulgar. Hospital stays like my most recent are the true epitome of my life. This excrussiatingly real existence I attempt to keep out of the light of others because it is very unpleasant and not exactly appropriate conversation to strike up with others. No one truly wants to hear about your leaking body parts, horrendous rashes, or infected stoma drainage that ruins all of your clothes.

Sunday night after another beratement of heart palpitations, explosive diarrhea, weakness, dangerously low O2 saturation, excessive bruising and extreme paleness, I found myself in the Universities ER for the second time in ten days. My ragging case of SIBO had once again depleted my electrolytes, this time causing moderate anemia. My bowel moments had become so frequent that making it to the restroom was not an option. A lovely symptom of Scleroderma is decreased pressure in the different sphincters of the body, suprisingly you have more than just the one; there are actually over 60 in the human body. So when you have diarrhea and Scleroderma, you better have a change of pants. Now are you getting the picture why my life is less than glamorous?

After being admitted for electrolyte and iron infusions my levels had risen to stable and I was aloud to return home on antibiotics after one night in the hospital. Due to this consistent issue with electrolytes, anemia, and reoccurring SIBO I will have to start Iron infusions weekly. I will be working out the details with my GI when i see her in a few short weeks; hopefully I can stay out of the ER until then. 

So when you see pictures of me smiling, looking well put together, or wearing a stylish outfit remember, that is not an authentic representation of my life... and those probably weren't my first pair of pants for the day.