Health News / April 2007
Silence of the LAM
The rare lung disease known as LAM has a complicated name and a tragic history. It is a silent killer that almost exclusively affects women. Lymphangioleiomyomatosis is a progressive, frequently fatal disease, characterized by an unusual type of smooth muscle cell that invades lung tissue. As clumps of cells and cysts grow through the lungs, they destroy normal lung tissue and block airflow, reducing the body’s oxygen intake. More than 7 of every 10 patients are between 20 and 40 when they begin having symptoms. Because most women and many health care providers are unfamiliar with the diagnosis and the disease symptoms, LAM may not be diagnosed until a woman reaches her thirties or forties.
Though only about 675 women in the US have been diagnosed with LAM, scientists believe many more have been misdiagnosed as more common lung diseases. The early symptoms-shortness of breath (especially following periods of exertion), chest pain, and a frequent cough-are similar to asthma, emphysema or bronchitis. Misdiagnosed women experiencing ongoing pulmonary complications are often sent for an X-ray, which rarely reveals LAM. A high-resolution CT scan of the chest, and sometimes the abdominal area, is required for accurate diagnosis. A lung biopsy can also provide information for a diagnosis of LAM. A Standard stress test can show the body’s oxygen levels.
The term “lymphangioleiomyomatosis” comes from the Greek. “Lymph” and “angio” refer to the lymph vessels, and “leiomyomas” refers to the type of cells involved in LAM.
In early stages of the disease, most women can go about their daily activities, but as the disease progresses, they experience abnormal cell growths in other organs, including the liver and brain and can develop large tumors on their lymph nodes.
The most common cause of death from LAM is respiratory failure.
Research still has not yet established a cause or why the disease affects women. There is no cure at present, but earlier diagnosis and support services can improve patients’ quality of life. Medicines like bronchodilators that relax the muscles around the airways make it easier to breath and diuretics are sometimes used to prevent fluid build up in the chest.
Women with LAM have a greater chance of developing osteoporosis than other women and may need calcium and vitamin D supplements as well as drugs that prevent bone loss. Some physicians believe estrogen may play a role in the development of LAM and treat their patients with hormone therapy.
The LAM Foundation, based in Cincinnati, was created by the mother of a 22-year-old women with LAM, and has been the driving force behind the search for effective treatments and a cure. In 1994, Sue Byrnes’ 22-year-old daughter, Andrea, was diagnosed with LAM. Sue immediately turned to medical literature for answers about the mysterious disease that had stricken her daughter and found practically nothing–no LAM studies, no resources, no support groups and, basically, no hope.
Determined to help her daughter and other women with LAM, she successfully lobbied Congress to convince the National Heart, Lung and Blood Institute at the National Institutes of Health to develop an LAM patient registry, including a tissue bank, to help physicians and scientists become more knowledgeable about LAM and stimulate research.
The Foundation encouraged the NHLBI in the establishment of an LAM Protocol, where patients are evaluated every six months at no cost to the patient. The LAM Foundation has formed a model collaboration with the NHLBI, and become the education and support network for women affected by the disease. The Foundation has raised more than $9 million in research funding and contributed to major scientific breakthroughs, including the first evidence of a genetic link to LAM, the identification of an LAM gene and a molecular explanation for abnormal smooth muscle cell growth in LAM. The most promising treatment identified through the work will soon be studied in the first LAM treatment clinical trial. As many as 10,000 women in the US and as many as 250,000 women worldwide may have undiagnosed or misdiagnosed LAM. For women with this terrible disease that takes their breath, the LAM Foundation offers, the first ray of hope. PL
For more information, visit www.thelamfoundation.org or the US Department of Health and Human Services website, www.nhlbi.nih.gov/health/dci/Diseases/lam or call 877-CURE-LAM.
Copyright © 2007 A Woman's View. All rights reserved.
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