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. 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 affects 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. 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, such as chemotherapy. 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 the prognosis is usually poor in the systemic forms of the disease ranging anywhere from a few months to 20 years.
