The survival time following diagnosis is uncertain, as the disease seems to be highly individual. It had been reported to be less than 10 years, but new reports show patients living more than 20 years after diagnosis.
Diagnosis:The diagnosis of LAM can be difficult because many of the early symptoms are similar to those of other lung diseases, such as asthma, emphysema, or bronchitis. Often the person first goes to the physician complaining of chest pain and shortness of breath that was caused by a pneumothorax. Some patients first consult their physician because of shortness of breath upon exertion. There are a number of tests the physician can do to gather information about LAM:
Treatment: Because LAM affects almost exclusively women of childbearing age, it has been thought that the hormone estrogen might be involved in the abnormal muscle cell growth that characterizes the disease, just as it is in the growth of smooth muscle in the uterus in a woman's childbearing years. Although there is no firm evidence that there is a relationship between estrogen and LAM, the treatment of ther disease has focused on reducing the production or effects of estrogen. The response to treatment has been highly individual, and no therapy has been found to be effective for all LAM patients. Oxygen therapy may be necessary if the disease continues to worsen and lung function is impaired.
For LAM patients with severe disease, lung transplantation is an established therapy. One year survival following transplant is approximately 70 percent, and 3-year survival is approximately 50 percent.
Effect on lifestyle: In the early stages of the disease, most patients can go about their daily activities, including attending school, going to work, and performing common physical activities, such as walking up a hill. In more advanced stages, the patient may have very limited ability to move around and may require oxygen full-time. Patients with LAM should follow the same healthy lifestyle recommended for the general population, including eating a healthy diet, getting as much exercise as they can, as well as plenty of rest, and, of course, not smoking. Traveling to remote areas where medical attention is not readily available or to high altitudes where the blebs can expand and rupture should be considered carefully before undertaken.
In patients with normal lung function, there is probably no increased risk associated with pregnancy. However, in patients with compromised lung function, pregnancy is not advised. There do not appear to be complications associated with oral contraceptives, but this issue should be discussed with the patient's pulmonologist and gynecologist.
Last Editorial Review: 5/13/2016
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