What Is Scleroderma

852 pills & supplements taken.
360 hours of enteral feeds pumped.
268 hours of supplemental oxygen breathed.
15 hours spent in an uncomfortable waiting rooms.
24 hours spent with physicians instead of my family.
5 hours of chemotherapy infused into my heart.
This is what any given month of my life used to equates to. (2015)

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 itselfChronic, 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. Skin tightening can become so extreme that many patients are in somewhat of a body cast unable to simply turn their heads without hearing skin rip and crack on their necks. Contractions such as elbows, knees, and knuckles bust open into sores from being stretched too tightly. Skin color can even begins to change, de-pigmentating, or in some cases hyper-pigmentation leaving a patient looking overly tan.

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 Transplants. 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 expediencies, 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. 
Especially due to my overlap classification I have a different trajectory than a patient battling Scleroderma alone. It by far is the most aggressive condition I am dealing with. 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, lungs, and respiratory muscles are all greatly effected, 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 a tiny PVC pipe; no longer able to push food along in waves. 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. Imagine crunchy fibrosis infiltrating your precious lungs and heart, or 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 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.

January 2017 - After nearing the end of my journey, my doctors and I decided to take the plunge and receive a life saving Stem cell Transplant. My story is now is vastly different, and am happy to say my condition is much less dire than this time last year. I hope to continue to improve in the coming years!