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Key Points
- LAM is a rare lung disease that mostly affects
women in their mid-forties. It is sometimes seen in older women as well. It
almost never affects men.
- In LAM, clumps of cells and cysts grow throughout
the lungs. Over time, they destroy the normal lung tissue and block the flow of
air. They also reduce oxygen intake by the lungs.
- More than 1 out of every 3 women with LAM
develops growths called
angiomyolipomas (AMLs) in their kidneys.
- There are two forms of LAM: sporadic LAM, in
which the disease comes on for unknown reasons, and LAM that appears in people
with a rare inherited disease called
tuberous sclerosis complex (TSC).
- Doctors do not yet know what causes LAM. The
recent discovery of similarities between LAM and TSC has begun to provide some
valuable clues about the genes involved in both conditions.
- The symptoms of LAM include shortness of breath
(especially following periods of exertion), chest pain, and a frequent cough,
sometimes with bloody phlegm.
- LAM can lead to other serious conditions,
including a collapsed lung, blood in the urine, and abdominal swelling.
- To confirm or rule out LAM, doctors usually do a
chest x ray,
lung function tests, blood tests, pulse oximetry,
high resolution CT scans, and
lung biopsy.
- Doctors have not yet found any treatments to slow
or stop the growth of the cell clusters or cysts that are the main features of
LAM. As a result, most treatments are aimed at relieving symptoms and
preventing complications. These treatments include inhaled bronchodilators,
oxygen therapy, removal of air or fluid from your chest or abdominal cavities,
and lung transplantation.
- Several medicines are now being investigated as
possible treatments for LAM, including rapamycin.
- Most people with LAM can usually continue to be
active in the early stages of the disease. If the condition becomes worse, they
may not be able to be active, and they may need oxygen full time.
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Living With Links
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