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.
4 iron infusion pumped through my central line.
5 hours of chemotherapy 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
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!"
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.
The three major Scleroderma organizations in North America, The Scleroderma Foundation, The Scleroderma Research Foundation, and The Scleroderma Society of Canada, have created the "Hard Word. Harder Disease." campaign for the purpose of spreading awareness for this horrific disease. If you do one thing this month, please let it be to assist them in their campaign. Wear a pin, post on your Facebook, share this blog post, and take the Pledge! Visit HardWord.org and take the pledge to support Scleroderma Awareness this month! Each and every signature counts as one more person 'Scleroderma Aware.' With awareness comes funding, with funding comes research, and with research comes a cure!