Anti-rejection drug could help treat a killer lung disease
12 a.m. Friday, April 6, 2007
Sheila Egan Addis has a history of collapsed lungs. They started when she was in her late 20's.
"I thought the first time that I was having a heart attack or something. I didn't know what was going on," Egan Addis said.
Doctors didn't either. It took ten years before they put a name on Sheila's problem.
Fast Facts
- Lymphangioleiomyomatosis, or LAM, is a disorder which causes uncontrolled growth of smooth muscle cells in the lung tissue.
- The condition is sometimes associated with an inherited disorder, called Tuberous Sclerosis. However, about 30,000 to 50,000 people worldwide develop it sporadically.
- There is no known treatment. In severe cases, a lung transplant may be needed.
- Researchers are testing an anti-rejection drug, called Rapamycin, to see if the medication can restore lung function in patients with LAM.
General infromation on Lymphangioleiomyomatosis (LAM) is available from the The LAM Foundation, the American Lung Association, the American Thoracic Society or the National Heart, Lung and Blood Institute.
"It's Lymphangioleiomyomatosis," pulmonary researcher Dr. Vera Krymskaya said.
'LAM', for short, is a rare and deadly lung disease. It occurs when an unusual type of muscle cell invades the lung tissue, eventually blocking the airways.
"Loss of pulmonary function could be devastating for the state of the patient and only usually a lung transplant could save patient's life," Krymskaya said.
If that's not devastating enough, the disease strikes women in their childbearing years. Hormones play a role.
"When women become pregnant, that's often when the disease progresses," Krymskaya said.
There's no cure and Dr. Krymskaya wants to change that. A genetic breakthrough in her research lab at the University of Pennsylvania led to a study with Rapamycin, an anti-rejection drug used to treat transplant patients.
"That trial to treat LAM patients with Rapamycin gave very promising results," Krymskaya said.
Sheila is grateful for the research and that she had her children before her problems started. Now her goal is to see them grow up.
"I don't like to think about, I don't like to think about not being around for them, but yeah, that's, that is really my ultimate priority," Egan Addis said.
A biopsy and a CT scan are critical to diagnose LAM. More studies on Rapamycin are in the works.
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