LAM Lung Disease
The drug sirolimus (Rapamune) is the first drug approved to treat lymphangioleiomyomatosis. The drug has been found to help improve the lung capacity in patients, allowing them to breathe easier.
In addition, inhaled bronchodilators (albuterol, ipratropium) can help open the airways, reducing shortness of breath in some people. Patients with LAM lung disease should avoid smoking cigarettes as well as second hand smoke.
Because hormones like estrogen seem to be involved in LAM lung disease, treatments that manipulate hormone levels could help some people with LAM. A number of hormone-related treatments have been tested in women with LAM lung disease:
In clinical trials that tested these therapies, some women were helped, but others were not.
People who experience pneumothorax generally must undergo procedures to reinflate the collapsed lung, and prevent its recurrence.
If pulmonary lymphangioleiomyomatosis progresses and becomes disabling, lung transplantation may be an option. Though it's a drastic treatment, most people undergoing lung transplant for LAM lung disease gain improved lung function and quality of life after surgery.
Pulmonary lymphangioleiomyomatosis is progressive, and so far there is no cure. Most women with LAM experience a steady decline in lung function, with increased shortness of breath over time.
However, women vary widely in their experience of living with LAM lung disease. Some rapidly progress while others progress slowly: in some studies, nearly 90% of women were alive 10 years after diagnosis with LAM. However, surviving as long as 20 years after diagnosis is rare.
Researchers are working to identify how muscle cells go awry in LAM. Clinical trials of experimental drugs to treat LAM lung disease are also under way.
LAM Lung Disease (Lymphangioleiomyomatosis)
In this article
- What Causes LAM Lung Disease?
- Symptoms of LAM Lung Disease
- Diagnosis of LAM Lung Disease
- Treatment of LAM Lung Disease
- What to Expect With LAM Lung Disease
Treatment of LAM Lung Disease
The drug sirolimus (Rapamune) is the first drug approved to treat lymphangioleiomyomatosis. The drug has been found to help improve the lung capacity in patients, allowing them to breathe easier.
In addition, inhaled bronchodilators (albuterol, ipratropium) can help open the airways, reducing shortness of breath in some people. Patients with LAM lung disease should avoid smoking cigarettes as well as second hand smoke.
Because hormones like estrogen seem to be involved in LAM lung disease, treatments that manipulate hormone levels could help some people with LAM. A number of hormone-related treatments have been tested in women with LAM lung disease:
- Progesterone
- Tamoxifen
- Synthetic luteinizing hormone-releasing hormones (Leuprolide, Lupron)
In clinical trials that tested these therapies, some women were helped, but others were not.
People who experience pneumothorax generally must undergo procedures to reinflate the collapsed lung, and prevent its recurrence.
If pulmonary lymphangioleiomyomatosis progresses and becomes disabling, lung transplantation may be an option. Though it's a drastic treatment, most people undergoing lung transplant for LAM lung disease gain improved lung function and quality of life after surgery.
What to Expect With LAM Lung Disease
Pulmonary lymphangioleiomyomatosis is progressive, and so far there is no cure. Most women with LAM experience a steady decline in lung function, with increased shortness of breath over time.
However, women vary widely in their experience of living with LAM lung disease. Some rapidly progress while others progress slowly: in some studies, nearly 90% of women were alive 10 years after diagnosis with LAM. However, surviving as long as 20 years after diagnosis is rare.
Researchers are working to identify how muscle cells go awry in LAM. Clinical trials of experimental drugs to treat LAM lung disease are also under way.
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