reprinted from Issue 10, Spring 2010 of Frontiers of Medicine (PDF)
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Ken Sovey enjoyed an active life. He exercised daily, never smoked, and always took the stairs when inspecting buildings for his work in insurance claims.
Several years ago, the Santa Cruz resident was puzzled when he couldn’t build up his endurance while running. He knew something was really wrong when he had trouble climbing a flight of stairs. “I thought, maybe this is what it is to get old,” recalls Sovey, now 47. “But I wondered, why am I so out of breath that I can’t even talk?”
His doctors discovered he had lung fibrosis, i.e., scar tissue formation. Sovey came to UCSF’s Interstitial Lung Disease (ILD) Program, one of a few centers nationwide specializing in the treatment of lung scarring. Its members include experts in pulmon-ology, pathology and radiology – including Talmadge E. King, Jr., chair of the Department of Medicine, who spent much of his career investigating different types of ILD.
The term “interstitial lung disease” includes many disorders that cause lung injury and scarring and interfere with breathing. These disorders can be difficult to diagnose, because the most common symptoms are shortness of breath and coughing – symptoms found in most lung disorders. Some patients develop ILD after chronic exposure to environmental agents like mold, while others’ ILD is related to connective tissue diseases such as rheumatoid arthritis. However, for many patients, the cause is unknown.
Many subtypes of ILD are treatable, if a patient is correctly diagnosed early enough. “Knowing what process you’re trying to prevent the progression of has significant management implications,” says Harold R. Collard, MD, director of the ILD Program. “Early, accurate diagnosis gives patients the best chance to respond to therapy.” A multidisciplinary panel of experts confirms the diagnosis of each patient and develops detailed management recommendations.
Connecting Patients with Resources
In January 2009, UCSF physicians determined that Sovey had idiopathic pulmonary fibrosis (IPF), a common form of ILD whose cause is still unknown. Even though IPF does not yet have a cure, UCSF has several clinical trials of experimental therapies that Sovey may participate in.
Sally McLaughlin, RN, MSN, the ILD Program’s nurse, provided Sovey and his local pulmonologist with information to help manage his disease. “An important aim of the program is to help patients keep doing the things they love to do,” says McLaughlin. “We provide resources and ways to help make that happen.”
McLaughlin referred Sovey to pulmonary rehabilitation, a series of exercise and education classes which help patients increase exercise function, reduce breathlessness and improve quality of life. She also explained different oxygen equipment options to Sovey, who uses a portable oxygen concentrator when he runs errands or flies, and uses another machine at home. Physicians also referred Sovey for a lung transplant evaluation. “UCSF told me, we’re on the cutting edge of research, and we’re trying to find a treatment for this,” says Sovey. “But if all else fails, they said I’d be an excellent candidate for transplant.”
The UCSF ILD Program also runs a monthly support group called Living Well with ILD, and hosts a daylong seminar for patients and their families as well as a workshop for medical providers.
Hope for the Future
In addition to outstanding patient care, UCSF is at the forefront of ILD research. “We have learned so much about the biology of scar formation in the last two decades,” says Collard. “I’m very excited about our progress in developing new therapies that target specific pathways in fibrosis. This will provide treatments that are more effective and less toxic than currently available.”
As part of UCSF’s research efforts, more than 1,000 patients are participating in a registry which allows scientists to access their clinical data and blood and tissue samples. The registry is helping researchers, including many pulmonary fellows training in ILD, discover more about the biology and epidemiology of these diseases.
Collard’s dream is to create a UCSF Fibrosis Center, where discoveries about scarring diseases could be shared across disciplines. For example, ILD physicians could collaborate with cardiologists studying scar formation after a heart attack, or gastroenter-ologists studying cirrhosis of the liver.
For his part, Sovey continues to work full-time and exercise daily. “I know they see a lot of people, and the fact that they remember me when I come in means a lot,” says Sovey. “It’s very important when you’re a patient to have a personal connection, because you know they’ll do their best for you. At this point, they’re kind of an extended family.”