Understanding Scleroderma

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Understanding Scleroderma

Painful autoimmune disease plays havoc with skin

By Serena Gordon

HealthScoutNews Reporter

WEDNESDAY, May 22 (HealthScoutNews) -- 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. 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.

SOURCES: Furst, M.D., Carl. M. Pearson professor of rheumatology,

University of California, Los Angeles, Center for Health Sciences;

Bunis, scleroderma patient, Kensington, Md.; May 21, 2002, presentation,

Digestive Diseases Week annual meeting, San Francisco

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