Fighting a disease with an unknown cause Malibu resident Deirdre Roney has lost eight family members to the disease idiopathic pulmonary fibrosis. The first B.I.G. (Breathing is Glorious) Butterfly Ball, in partnership with UCLA, will take place on Oct. 22, at the Jonathon Beach Club in Santa Monica to raise funds for research on the disease. Bridget Graham-Gungoren / Special to The Malibu Times When the fourth person in her family died from the disease idiopathic pulmonary fibrosis, Malibu resident Deirdre Roney said she couldn’t live in denial any longer-she couldn’t “lay tied to train tracks, waiting to be run over.” Roney decided to take action and fight the disease that has taken eight of her family members, including her grandmother, and her mother in 1987, who died within three months of being diagnosed. “It happened that quick,” Roney said. Three of Roney’s uncles died within two to three years of diagnosis; eight of her cousins under the age of nine lost their parents to IPF. “What really seemed to trigger the willingness to face up to fighting this is when I had children-it’s not just about you anymore,” Roney said. Idiopathic pulmonary fibrosis scars the lungs, which results in an inability to properly transfer oxygen and breathe. Healthy lungs repair their own tissue after inflammation; IPF is believed to be a result of a body’s inability to repair its own tissue. “It’s like a scratch that doesn’t heal,” Roney said. “If you scratch your skin, a scab forms and it goes away. With IPF, you scratch your lungs and the lungs scab fast, but it doesn’t go away.” Roney is tested every year, but so far has not shown signs of the disease. “But doctors don’t really know what it looks like in the early stages; we’ve still got a lot of work to do.” About two-thirds of those diagnosed die within five years. There is currently no FDA-approved treatment or cure known for IPF; very little is known about the cause. However, Roney doesn’t let all this get her down. She approaches the facts with a positive outlook. “We need to be models for our children-how we face adversity,” she said. Roney currently sits on the board of directors for the Coalition for Pulmonary Fibrosis, which raises funds for education, and now research for treatments and a cure for the disease. She is also serving as event chair for CPF’s first B.I.G. (Breathing is Glorious) Butterfly Ball, in partnership with UCLA, on Oct. 22, at the Jonathon Beach Club in Santa Monica. (The butterfly was chosen as the logo because the wings look like lungs.) Founded in 2001, CPF has spent the last four years primarily focused on educating families, patients and doctors. “We wanted to teach doctors to properly diagnose; to teach them to refer patients to specialists and to teach them to use drugs that may or may not work-but perhaps give a small chance of slowing progress,” Roney said. Working with doctors and partnering with other nonprofits, to cover wider ground for insurance, legislative and quality of life issues, CPF has generated free educational literature for more than 20,000 patients as well as for doctors. It is also instrumental in setting up support groups for IPF patients. Idiopathic means “unknown cause.” Sometimes doctors believe there’s a link between IPF and exposure to infectious air pathogens like wood dust, gases, fumes, cigarette smoke and viral infections. And while Roney’s family has been greatly affected, genetic disposition has only been found in 10 percent to 15 percent of reported cases of the 83,000 Americans who are afflicted with IPF. (However, the Pulmonary Fibrosis Foundation on its web site, www.pulminaryfibrosis.org, states, “there is a growing body of evidence, which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of pulmonary fibrosis.”) Thirty-one thousand new cases are reported each year. Yet new research indicates that 55 percent of IPF patients may be misdiagnosed because doctors don’t understand the disease. “If we get to a place where we can properly diagnose, then the number is likely to go up,” Roney said. “In three to five years, we hope to have isolated the gene enough to find a test for diagnosis.” One of the critical components of a diagnosis, which usually involves a complete exam with a pulmonologist, pathologist and radiologist to exclude other lung diseases, is the presence of usual interstitial pneumonia, found after a lung biopsy. While each person is affected differently and progression of IPF varies, patients have the general symptoms of shortness of breath and a spasmodic dry cough. They may experience weight loss and fatigue, and possible clubbing (buildup of tissue in the fingertips). Dr. Robert M. Streiter, division chief of pulmonary and critical care of the Hospitalists Division at the UCLA David Geffen School of Medicine, will be honored at the Butterfly Ball with the first Marvin I. Schwarz Research Award presented by CPF. “We researchers need funding that supports us in being more open-minded, to look at IPF in entirely new ways,” Streiter said in a recent interview. The doctor focuses his research on pulmonary fibrosis along with lung cancer, acute lung injury and lung transplantations. “Several areas are promising for IPF patients-there may be targeted therapies for fibrosis in the next five years …” Streiter said. An option today may be a lung transplant to extend life expectancy. Survival rate information varies, but it is believed that the transplant gives a 50/50 chance to live another five years versus an IPF diagnosis of a 70 percent chance to die within five years. IPF doesn’t transfer to the new lungs, so the primary concern would be organ rejection. Roney remains hopeful for a cure but said, “It may not be me who sees it … It requires everyone to join hands and walk together in the same direction-if we do that, we will find a cure.” The B.I.G. Butterfly Ball will include a cocktail reception, sit-down dinner, live auction, a program of speakers and dancing. Individual tickets are $250; additional information can be found at www.coalitionforpf.org RSVP by Oct. 1. As of March 2004, there were more than 3,900 people on the waiting list for lung transplants. Registration to become a donor can be done at www.donatelifecalifornia.org