WEDNESDAY, May 22, 2002 (HealthDayNews) -- Wendy Bunis couldn't even pronounce scleroderma when she was diagnosed with the debilitating autoimmune disease in 1993, let alone know what to expect.
In fact, most people know little or nothing about the potentially fatal disease. That's one reason the Scleroderma Foundation is sponsoring National Scleroderma Awareness Month in June.
Scleroderma literally means "hard skin," and the disorder causes the skin to thicken and tighten. However, it doesn't just affect the skin; it can also attack the blood vessels, kidneys, lungs and other parts of the body.
About 300,000 people in the United States have the disorder, according to the Scleroderma Foundation, and it strikes women approximately four times more often than men. The disease generally appears in middle age.
Symptoms vary from person to person. However, Dr. Daniel Furst, a professor of rheumatology at University of California, Los Angeles, says Raynaud's syndrome is usually the first symptom of scleroderma.
Raynaud's causes the hands to turn white on exposure to cold; sometimes they also turn blue. As they warm back up, they often become red. Raynaud's can also cause numbness and tingling. Not everyone who develops Raynaud's has scleroderma, but 95 percent of those with scleroderma have Raynaud's, Furst adds.
Other symptoms of scleroderma include swelling; hardening of the skin that causes it to feel like wood or plastic; fatigue; heartburn; shortness of breath; and painful joints.
Bunis says that when her disease was at its worst, she couldn't tilt her head back without her mouth opening because her skin was so tight. The skin on her hands was so taut that they "clawed up." She was also in constant, excruciating pain.
"It was very, very debilitating for the first couple of years," Bunis says.
"Scleroderma can be a devastating disease from a functional point of view," Furst adds.
The most severe form is known as diffuse systemic scleroderma, and Furst estimates that about 100,000 people in the United States suffer from it. Five years after diagnosis, only 50 percent of those with this variation are still alive. Those with the less severe form of the disease have an 85 percent survival rate at 10 years, he says.
There are no lab tests that can definitively diagnose scleroderma, although Furst says they can be helpful in making the diagnosis. A diagnosis is made by ruling out other causes, plus factoring in the telltale signs of thickening skin as well as other symptoms.
Doctors still don't know what causes the disease, but they do know the hardened skin is caused by an overproduction of collagen. Immune-suppressing drugs and anti-inflammatory medications are most often used for treatment, although no one treatment works for all patients, Furst adds.
Furst notes the first three years after diagnosis are the most critical. Once that time has passed, the disease calms down, and some people even see partial reversal in the skin changes.
There's a lot of ongoing research that will hopefully lead to an effective treatment some day, Furst says.
One new treatment aims to control one of the disease's more unpleasant symptoms -- fecal incontinence. It affects as many as one-third of all scleroderma sufferers, says a study presented yesterday at the Digestive Disease Week annual meeting in San Francisco.
The study says a new implantable device, called the InterStim neurostimulator from Medtronic, stimulates the sacral nerve in the spinal cord to help control fecal incontinence in scleroderma patients.
Bunis says despite advances in treatment, scleroderma is still a very difficult disease to live with and it's important to keep a positive attitude. She says when you have a painful, chronic illness like scleroderma, it's easy to get depressed, and she recommends taking anti-depressants if you need them.
"People should not view having scleroderma as a death sentence," Furst says. "This is not a hopeless disease. Progress is being made."
What To Do: To learn more about scleroderma, visit the Scleroderma Foundation or check with the National Institute of Arthritis and Musculoskeletal and Skin Diseases.
