Scleroderma is a rare disease that affects between 5,000 and 7,000 people in Australia and has no known cure. Females are more likely to be affected with the disease compared to males at a ratio of five to one.
The Long-Term Prognosis for Scleroderma
Many scleroderma patients, even those with more invasive systemic scleroderma, can expect to have a normal life expectancy.
In Greek mythology, the gaze of Medusa would turn people to stone. In fact, the idea of creatures turning people to stone is fairly universal and appears in numerous cultures. Now, those creatures may not exist, but there is a disease, and while people don't actually turn to stone, they do feel as though they have.
"The easiest way to describe the disease is the thickening and hardening of the skin. "And after your skin turns hard, your organs mimic what your skin is doing and turn to stone as well." In the two years following the diagnosis, Ms Rainbow-Noack's quality of life has plummeted.
This type of scleroderma is typically accompanied by shortness of breath, a persistent cough, and the inability to perform routine physical activities. End-stage scleroderma often causes pulmonary fibrosis and/or pulmonary hypertension, both of which can be life-threatening.
The cause of scleroderma is unknown. However, researchers think that the immune system overreacts and causes inflammation and injury to the cells that line blood vessels. This triggers connective tissue cells, especially a cell type called fibroblasts, to make too much collagen and other proteins.
The first parts of the body to be affected are usually the fingers, hands, feet and face. In some people, the skin thickening can also involve the forearms, upper arms, chest, abdomen, lower legs and thighs. Early symptoms may include swelling and itchiness.
Hard, thickening, or tight skin
Some people develop 1 or 2 patches of hard, thick skin. Others have widespread patches on their body. The hard, thick skin can feel anchored in place. If you have morphea (more-fee-uh), the most common type of scleroderma, the patches may not feel too hard.
Scleroderma is a chronic, autoimmune connective tissue disorder that is primarily characterized by thickening and hardening of the skin and other tissue. In localized scleroderma, the skin is the main organ system involved and muscles and bones may or may not be.
In general, patients with limited scleroderma have a normal life expectancy. Some have problems with their GI tract, especially heartburn; severe Raynaud's and musculoskeletal pain; and a small subset can develop pulmonary hypertension that can be life-threatening.
It would be like undergoing paralysis, being turned into stone make any movement difficult, with rapidly impaired circulation of the blood accompanied by loss of sensation. Large sections of the person's nervous system would be turned to stone.
In geology, petrifaction or petrification (from Ancient Greek πέτρα (pétra) 'rock, stone') is the process by which organic material becomes a fossil through the replacement of the original material and the filling of the original pore spaces with minerals.
Petrifaction, or petrification, defined as turning people to stone, is a common theme in folklore and mythology, as well as in some works of modern literature.
Scleroderma renal crisis is a life-threatening complication of scleroderma. It typically presents with the abrupt onset of severe hypertension accompanied by rapidly progressive renal failure, hypertensive encephalopathy, congestive heart failure, and/or microangiopathic hemolytic anemia.
Gender: It's much more common in women than men. As many as 80 percent of those diagnosed with scleroderma are women. Age: Most localized types of scleroderma show up before age 40, and systemic types of scleroderma are typically diagnosed between ages 30 and 50.
Started early, treatments like phototherapy (light therapy) and medicines that work on the immune system like methotrexate and cyclosporine can help diminish scleroderma.
Avoid foods that may aggra- vate symptoms such as citrus fruits, tomato products, greasy fried foods, coffee, garlic, onions, peppermint, gas-producing foods (such as raw peppers, beans, broccoli or raw onions), spicy foods, carbonated beverages and alcohol.
How scleroderma progresses and changes over time varies greatly among people. Most people experience tight and swollen fingers in the beginning and will likely have Raynaud's phenomenon. Afterwards, it can take months to years for the full extent of the disease to develop.
Scleroderma affects all organs of the body. Skin manifestations are commonly reported, but peripheral nervous system (PNS) and central nervous system (CNS) involvement has been less frequently reported. Neurological manifestations are broad, and it is challenging for clinicians to confirm a diagnosis of scleroderma.
A small percentage of all cases of systemic scleroderma have been reported to run in families; however, the condition does not have a clear pattern of inheritance. Multiple genetic and environmental factors likely play a part in determining the risk of developing this condition.
Lung problems are usually the most serious complications of systemic scleroderma. They are now the leading cause of death in people with scleroderma. Two major lung conditions associated with scleroderma, pulmonary fibrosis, and pulmonary hypertension, can occur either together or independently.
Systemic sclerosis is a heterogeneous, multisystem disease. It can occur at any age, but most patients develop the disease between the age of 40 to 50 years. There is controversial evidence on whether/how the age at disease onset affects their clinical phenotype.
It is the most fatal of all the rheumatologic diseases. Systemic scleroderma is very unpredictable although most cases can be classified into one of four different general patterns of disease (see Classification).
Scleroderma and Pain
One of the biggest sources of pain in scleroderma is the deep tissue fibrosis (caused by excess collagen deposits) that causes permanent shortening of the joints, and tendon friction rubs (a grinding or crunching feeling in joints when they are moved in a certain way).