Also known as lymphangioleiomyomatosis.
LAM, or lymphangioleiomyomatosis, is a rare lung disease that mostly affects women of childbearing age. In people with LAM, abnormal muscle-like cells begin to grow out of control in certain organs or tissues, especially the lungs, lymph nodes, and kidneys. Over time, these LAM cells can destroy the normal lung tissue. As a result, air can’t move freely in and out of the lungs. In some cases, this means the lungs can’t supply enough oxygen to the body’s other organs.
There are two types of LAM. LAM that occurs sporadically is called sporadic LAM. When LAM occurs in association with a rare disease called tuberous sclerosis complex (TSC), it is called TSC-LAM. Doctors may diagnose LAM with imaging tests such as high-resolution computed tomography (CT) scans and blood tests for vascular endothelial growth factor D (VEGF-D). Other tests and procedures may be needed to diagnose LAM.
Doctors treat LAM with sirolimus (rapamycin), a medicine that stabilizes lung function, treats an abnormal fluid buildup in the lung called chylothorax, and improves overall quality of life. They may also prescribe other medicines or therapies to control other symptoms or complications.
LAM has no cure. It is important to get routine follow-up care because the disease tends to worsen over time. How quickly the disease worsens varies. More than half of women who have LAM will develop a serious condition called pneumothorax, or collapsed lung, that requires immediate treatment. Over time, LAM may cause permanent damage to the lungs and cause potentially fatal respiratory failure. Lung transplant is a treatment option for some women whose lungs have been severely damaged by LAM.
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