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In "Project Scleroderma: Beneath the Surface," at the Ritz East Thursday, producer Christy McCaffrey (left) and patient Kelly Ellison.

By Curtis Skinner, Inquirer Staff WriterPOSTED: July 21, 2013Sandy Selfridge was one in a thousand. In April 2009, she was diagnosed with scleroderma, a rare autoimmune disease involving the skin. By September, at age 58, she was gone.
Her death led Christy McCaffrey, her daughter, to produce a documentary about the disease. The 45-minute film, Project Scleroderma: Beneath the Surface, which took two years and $40,000 to make, is narrated by comedian Bob Saget, who lost his sister to the disease. It's slated for a Thursday premiere at the Ritz East Theatre, 125 S. Second St., at 8 p.m.
"At first, it was difficult for me to be sharing something that is obviously so personal," said McCaffrey of Havertown, a hairstylist who partnered with Bill Connell and J.C. Costa of New Pace Productions in Ardmore.
"But this film and my mission is truly not about me, it is about the patients who suffer from this disease," she said.
The documentary tells the stories of patients with scleroderma, a potentially life-threatening disease in which the immune system attacks the skin, gastrointestinal tract, lungs, and heart. It is estimated to affect 300,000 people in the United States, and most commonly strikes women between 45 and 65.
McCaffrey purchased and shipped $100 Sony "Bloggie" cameras to patients around the world so they could share their stories.
"It showed that if you line up 10 different scleroderma patients, you can see 10 different diseases," she said.
Scleroderma comes in two forms, limited and diffuse, with complications ranging from isolated hardening of the skin on the fingers and face to a scarring of the organs, which can be fatal. The 10-year survival rate for the disease is about 70 percent. But it can drop to 50 percent depending on risk factors and the disease's aggressiveness, said Fredrick Wigley, director of the Johns Hopkins Scleroderma Center.
There is no cure, but treatments exist to limit or prevent damage and progression of the disease.
One key to successful care, Wigley said, is a timely and accurate diagnosis. Often, due to its rarity, patients and doctors will miss the signs.
"Once it expresses itself, it's very obvious, but the disease in its early stages can be difficult to diagnose because it has some common symptoms that you see in many patients," Wigley said. "The average time for a patient to come to our center is two years into the disease."
He said that if people start to experience unexplained fatigue, musculoskeletal discomfort, and hardening of the skin - or contract Raynaud's phenomenon, which can constrict blood vessels in the fingers and toes - they should consult a doctor.
While her mother's aggressive form of the disease likely wouldn't have been helped by earlier detection, McCaffrey hopes the documentary can spur others to seek care before it's too late.
"This has been the greatest therapy for me," she said.

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