By Lisa Dumas –
Rosa (Cox) Kegler-Johnson, one of the co-founders of the Greater Rochester Scleroderma Support Group’s annual “Stepping Out to Cure Scleroderma Walk” that takes place at Seneca Park each year, has passed away due to complications from the disease recently.
And, Kegler-Johnson’s sister, Marilyn Sibley, has vowed to continue her sister’s fight to raise awareness regarding scleroderma throughout the Rochester community.
“As head of the local scleroderma support group, I plan to continue educating the public about scleroderma,” Sibley stated. “Especially the medical community, because they have limited knowledge about the disease. This will help them to diagnose patients earlier, and to determine the best type of treatments for their patients.”
According to the National Scleroderma Foundation’s website, scleroderma is a “connective tissue disease generally classified as one of the autoimmune rheumatic diseases. The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin and other tissues throughout the body is one of the most visible manifestations of the disease.”
An estimated 300,000 Americans presently have scleroderma, while many others may still go undiagnosed.
As a result, Sibley, and other members of Rochester scleroderma support group, which is the local chapter of the National Scleroderma Foundation’s Tri-State Support Groups, said the underlying symptoms of the chronic autoimmune disease are one reason the organization has lobbied federal legislators for additional funding to research scleroderma, which can be challenging for physicians to diagnose and treat.
Sibley said the group also hopes to acquire more specialized treatment options for survivors of the disease, locally.
Consequently, University of Rochester rheumatologist Benjamin Korman M.D. has recently spoken to the organization’s members regarding the university’s plans to address the issue, at Sibley’s request.
“While there are individual providers who have diligently taken care of scleroderma patients in Rochester, the community lacks an organized, multi-disciplinary approach to care, and, as a result, many patients travel long distances for specialty care,” Korman stated. “There are several types of scleroderma, and it can cause many serious complications. Patients require routine monitoring, and treatment includes immunosuppressants and other medications, elevating the need for specialized care. We are working diligently to establish a program that brings specialists in all areas of scleroderma care together to support people with the disease. However, we currently care for these patients in our rheumatology clinic, and are collaborating with specialists from dermatology, interstitial lung disease, gastroenterology, nephrology, hematology, and orthopaedics to provide a coordinated approach to care.”
In addition, to add to the disease’s complexity, minorities have been disproportionately affected by the disease, Korman said.
“African Americans are diagnosed with the disease more frequently, and are at greater risk of a severe form of the disease,” he stated. “This often includes higher incidence of interstitial lung disease, the leading cause of death in scleroderma, as well as advanced and extensive skin and muscle disease, which result in poor quality of life. African Americans are typically diagnosed earlier, yet have greater disease-related mortality. In addition, Native Americans, particularly the Choctaw who are native to Oklahoma, have the highest incidence of scleroderma of any ethnic group.”
The university is also expanding clinical research efforts to advance new therapies for the disease, and its complications, Korman said, as well as enhancing the laboratory research of its molecular mechanisms, correspondingly.
Signs and symptoms of the disease include fingers and/or toes that turn red, white, or blue in cold temperatures, called “Raynaud’s phenomenon;” skin thickening, usually affecting the fingers and hands, and sometimes the whole body; acid reflux; red spots on the face or hands caused by enlarged blood vessels; shortness of breath during exertion; difficulty swallowing; hard nodules under the skin; and abnormal blood tests, which a patient’s doctor may order.
“If someone suspects they may have scleroderma, they should see their primary care physician, and discuss their symptoms,” Korman stated. “And, if there is ongoing concern and not a better explanation for their symptoms, they should see a rheumatologist with knowledge of this disease.”
As for Sibley and the rest of the local support group, she said the organization plans to carry on Rosa’s fight to raise awareness regarding scleroderma, and the group is currently planning its 15th annual fundraising walk, which will take place on Saturday, June 2.
“The walk was Rosa’s pride and joy,” Sibley said. “It was a way to raise funding for research, build a support network and talk with others who shared similar health issues; encourage walk participants to become volunteers in the cause, discover new ways to increase public awareness, and honor the memory of those who have passed on. It was also a way to make new, and long-lasting friendships.”
The Greater Rochester Scleroderma Support Group’s upcoming walk will take place from 9 a.m. to 1 p.m., in Seneca Park, at 2222 Saint Paul St.
Interested individuals may contact 800.867.0885 for additional information regarding the event.