Pronounced lim-fan-jee-o-lee-o-my-o-ma-to-sis, LAM is a rare lung disease predominantly affecting women, most often occurring between puberty and menopause. In the United States, it is estimated that 1,000 women have LAM.
LAM is characterized by an unusual type of muscle cell that invades the tissue of the lungs. These muscle cells proliferate within the airway walls, causing them to become obstructed, resulting in pulmonary cyst formation. Over time, the muscle cells block the flow of air, blood and lymph vessels to and from the lungs, preventing the lungs from providing oxygen to the rest of the body.
The causes of LAM are unknown.
At the onset of LAM, a patient may experience shortness of breath during strenuous exercise, but as the disease progresses, shortness of breath may even occur during rest. Other symptoms include chest pain, hemoptysis (coughing up blood), chylous effusions (leakage of white fluid into the chest cavity) and repeated pneumothoraces (leakage of air into the chest cavity).
The diagnosis of LAM is difficult because early symptoms resemble those of lung disease. Because the cause is unknown, treatment to each diagnosis has been individual. Many treatments focus on reducing the production or effects of estrogen. Oxygen therapy may be necessary and a lung transplant may be considered as a last resort. Unfortunately, all treatments vary in effectiveness and none have been scientifically proven. A new drug, rapamycin, is currently being tested at the University of Cincinnati. To read an interview with the Scientific Director, Frank McCormack, about rapamycin click here.