Researchers make progress against lung disease attacking women in childbea...( getting married and starting families ...),Health

getting married and starting families, just when their futures look so bright. Women with LAM often struggle to breathe. They experience lung collapses, chest pain, cough, and can become extremely fatigued. Many of them develop a benign kidney tumor. Supplemental oxygen is inevitable, and a lung transplant, which usually buys them only a few years, is the very last resort for survival."

One womans struggle
43-year-old Sheila Egan Addis, a Philadelphia theatrical performer and mother of two, who lives in Center City, started suffering from alternating collapsed lungs when she turned 30. "It felt like a sharp pain in my upper back, like Id pulled a muscle when I tried to take a deep breath," Egan Addis comments. After the third time each lung had collapsed, surgeons went in to restore some of the lung function. Nine years after she began suffering symptoms, a Penn pulmonologist finally diagnosed her with LAM disease. "Finally, it all made sense," Egan Addis adds.

Two years ago, her right lung collapsed again and she was operated on. Egan Addis has since lost some more lung function. She says it does take a toll on a performer in live musical theater, "I don't have the stamina that I used to. I can't do a lot of running around and singing. I have to think about it before taking on a challenging role. The pace of live theater can be very tiring and draining physically."

There is hope
Krymskayas discovery in 2002 revealed that abnormal smooth muscle-like LAM cells invading the lungs are due to the loss of growth control by tuberous sclerosis (a genetic disorder which causes tumors to form in organs) complex proteins - and furthermore, Krymskaya then discovered that growth can potentially be controlled by a drug (FDA-approved Rapamycin), which mimics the function of the missing proteins. Krymskaya made the link between similar basic research found in flies and applied it to humans, opening the door for the first-ever LAM treatment
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Contact: Susanne Hartman
susanne.hartman@uphs.upenn.edu
215-349-5964
University of Pennsylvania School of Medicine
8-Dec-2006

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