Sandra Raia doesn’t visit her daughter in Jersey City much, partly because the many hours flight for Dallas, TX has become a wearying experience, but mostly because she can no longer stand the cold. Contact with cold aggravates her debilitating disease, scleroderma. Raia’s daughter, Michele Dupey of Jersey City, noted recently that June was scleroderma awareness month. But most people haven’t a clue as to what scleroderma is or how many people have been stricken by this under-reported and often misdiagnosed disease. Raia, like many people who suffer the disease, didn’t know at first what was happening to her when it struck. Scleroderma is an autoimmune disease of the connective tissues that affects an estimated 150,000 to 500,000 people. It is caused by an over-production of collagen – a substance that creates elasticity in the skin. The word actually means “hard skin” in Greek because it tends to create red, hard glossy patches on the face, torso and other areas of the body. Raia said that repeated abnormal swelling on her fingertips caused her to lose her fingerprints. Her fingertips are often cracked and bleeding. She said she noticed something wrong about six years ago, a kind of odd feeling in her hands. “Then my hands began to bleed and I almost lost my fingernails,” she said. “I didn’t understand what was going on.” This condition is sometimes called Raynaud’s phenomenon and is often an early sign of scleroderma. Blood simply doesn’t flow to the small capillaries of the fingers. According to the American Autoimmune Related Diseases Association, a person is likely to have subtle symptoms for several years before doctors can diagnose scleroderma. Often the color of the skin changes, and people feel numbness and pain in the fingers, toes, nose. Changes in the esophagus can cause difficulty in swallowing. “Characteristically, neither the patient nor the doctor feel that these early complaints are of much consequence,” said an AARDA report. Many of the symptoms seem obvious only when looking back after diagnosis. The skin on the face may seem tighter. Joints may become inflamed, painful or unable to bend easily. A person may suffer shortness of breath from very mild activity. Raia has had to be rushed to the hospital three times because of impaired breathing. While Raia later got a special blood test that showed she suffered the disease, diagnosis is usually made over a period of time. It is a disease that can attack organs, because all organs are surrounded by connective tissues. So that in effect, it shuts down one organ after another in a kind of slow and painful death. “The valves in my heart are under attack,” Raia said. “I already have a leak in one of the valves.” A New Jersey exile Raia lives in Dallas because she can no longer standing the cold weather in New Jersey or New York, where she lived most of her life. Cold is the most common and important factor that provokes attacks. Those who suffer from Raynaud’s phenomenon have to avoid the cold and keep warm all the time. “My hands used to turn almost black from the cold,” Raia said. “I know some women have lost fingers.” For this 1948 graduate of fashion design, the disease seemed to strike at the center of her talents. “I used to be a designer, but I’ve had to stop everything,” she said. “I couldn’t work any more. I was always in pain.” As far as anyone can tell, the disease has no cure, and even treatment has a limited effect, often designed around the variety of an individual patient’s symptoms. Traditional anti-inflammatory drugs including steroids have had only limited success, although new versions may be slightly more effective. For the most part, doctors treat the symptoms, not the disease. “I take heart medicine and I go for pain management,” Raia said. For Raia, the worst part of the disease is the nearly constant exhaustion, although she suffers breathing problems and continued charley horses in her hands, legs and sides. Raia said that for her and most of the others who suffer the disease, there is no immediate future. They are medicated against the most immediate pains and try to stay comfortable, while knowing that the disease continues to attack. Raia, said that, however, she doesn’t let the disease stop her from performing simple, daily routines. “I’m a pusher,” she said. “When I get up, it takes me a while to get going. But once I get going, I try to accomplish as much as I can. The worse thing to do is not do anything. That’s worse for the body, for the mind.” “I used to be a designer, but I’ve had to stop everything.” – Sandra Raia
Struggling against scleroderma; Ex-New Jersey native talks about the disease’s effect
