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Deborah Mielo's Fundraiser:

The LAM Foundation

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BENEFITING: LAM Foundation

Deborah Mielo

THE STORY:

What is LAM?

Lymphangioleiomyomatosis (LAM) is a progressive lung disease that usually strikes women during their childbearing years. Lymphangioleiomyomatosis is pronounced lim-fan-gee-o-ly-o-my-o-ma-to-sis. “Lymph” refers to the lymph vessels and “angio” refers to the blood vessels. “Leiomy” means smooth muscle, “oma” is a tumor, and “tosis,” refers to a disease condition.

LAM is a systemic neoplasm that results in cystic destruction of the lung. It is characterized by an abnormal growth of smooth muscle cells. These cells invade lung tissues, including the airways, and blood and lymph vessels. Although these cells are not considered cancerous, they grow uncontrollably within the lungs. Over time, the cells form bundles in the walls of the airways, as well as the blood and lymph vessels, causing them to become obstructed. These cells produce materials that break down tissue causing the formation of cysts. The delicate architecture of the lungs is destroyed and the airflow is blocked limiting the delivery of oxygen to the rest of the body.

LAM manifests itself in a wide variety of ways, so it is sometimes difficult to diagnose. The difficulty of the diagnosis is compounded by the fact that a lot of the symptoms of LAM are similar to those of other lung diseases such as asthma, emphysema, and bronchitis. Chest x-rays are not usually sufficient to detect LAM, but a high-resolution chest CT scan can detect the characteristic cystic structure of LAM, thus providing an accurate diagnosis, if other manifestations of the disease (e.g., benign kidney tumor, lung collapse, or fluid in the lungs) are present. In some circumstances, a lung biopsy may be needed to provide an accurate diagnosis.

The cause of LAM remains unknown, and although great strides have been made in researching the disease, no cure is yet available. A recently completed treatment trial (the MILES Trial) suggested that a drug called sirolimus, or rapamycin, may improve lung function in some women with LAM.

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