What Is LAM?
LAM, or lymphangioleiomyomatosis
(lim-FAN-je-o-LI-o-MI-o-ma-TO-sis), is a rare lung disease. It mostly affects
women in their mid-thirties and forties.
In 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 grow throughout the
lungs and destroy the normal lung tissue. As a result, air cant move
freely in and out of the lungs. In some cases, this means the lungs cant
supply the bodys other organs with enough oxygen.
Overview
There are two forms of LAM. Sporadic LAM occurs for
unknown reasons. LAM also can occur in women who have a rare disease called
tuberous sclerosis complex (TSC). Women who have TSC often
have a milder form of LAM.
About 6 out of 10 women who have LAM develop
pneumothorax
(noo-mo-THOR-aks), or collapsed lung. In this condition, air leaks out of a
lung and into the space between the lung and the chest wall (the pleural
space).
This condition can cause pain and shortness of
breath. Sometimes one lung will collapse over and over again. Pneumothorax is a
serious condition that usually requires treatment and can sometimes be life
threatening.
Normal Lungs and Lungs With LAM
Figure A shows the location of the
lungs and airways in the body. The inset image shows a cross-section of a
healthy lung. Figure B shows a view of the lungs with LAM and a collapsed lung
(pneumothorax). The inset image shows a cross-section of a lung with LAM.
Many women who have LAM get tumors called
angiomyolipomas (AN-je-o-my-o-li-PO-mas), or AMLs, in their kidneys. Women who
have LAM also may develop:
- Growths in other organs, including the liver and
brain
- Large tumors in their lymph nodes
- A buildup of fluid in their chests, abdomens, or
pelvic areas
Outlook
LAM has no cure. The disease tends to worsen over
time. How quickly the disease worsens varies from woman to woman. LAM may lead
to death from respiratory failure. Lung transplant is a treatment option for
women whose lungs have been damaged by LAM.
Not long ago, doctors thought women who had LAM
wouldn't live more than 10 years following diagnosis. They now know that some
women may survive for more than 20 years after diagnosis.
Doctors have learned a lot about LAM in recent
years. They're now able to diagnose the condition earlier. Support services
also are now available to help improve the quality of life for women who have
LAM.
Researchers continue to explore and test new
treatments for LAM.
September 2008
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