Understanding Lymphangioleiomyomatosis (LAM): A Rare Lung Disease
If you've been diagnosed with lymphangioleiomyomatosis (LAM) or your healthcare provider suspects you may have it, you may wonder what this rare lung disease is and how it can affect your breathing. LAM primarily affects the lungs and can cause gradual damage to lung tissue over time.
What Is LAM?
Lymphangioleiomyomatosis, or LAM, is a rare disease in which abnormal cells grow in the lungs and other parts of the body. These cells can block airways and lymphatic vessels, as well as damage the walls of small air sacs in the lungs.
LAM occurs almost exclusively in women. This is because abnormal LAM cells have receptors for the hormone estrogen, found in women, which triggers cell growth and multiplication.
LAM is most often diagnosed during adulthood.
What Are the Symptoms?
Symptoms can develop gradually and may include:
Shortness of breath, especially during activity
Persistent cough
Chest pain
Wheezing
Fatigue
LAM can also cause complications such as a collapsed lung (pneumothorax) or a buildup of fluid around the lungs called chylous pleural effusion.
How Is LAM Diagnosed?
Your healthcare provider may use several tests to evaluate your lungs, including:
High-resolution CT scan of the chest
Pulmonary function tests
Blood tests, including a vascular endothelial growth factor-D (VEGF-D) test
Evaluation for kidney tumors called angiomyolipomas
A lung biopsy may be needed in some cases, although it is not always necessary.
How Is LAM Treated?
While there is currently no cure for LAM, treatment can help slow the disease, preserve lung function, and manage symptoms.
Treatment may include:
Sirolimus, a medication that can slow abnormal cell growth and help preserve lung function
Supplemental oxygen when needed
Treatment for complications such as pneumothorax
Lung transplantation in severe cases
Your healthcare provider will determine the best treatment based on your symptoms and lung function.
Living With LAM
Regular monitoring is important because LAM can gradually affect lung function. Pulmonary function tests can help your healthcare provider track changes over time.
You can support your lung health by:
Avoiding smoking and secondhand smoke
Staying physically active as recommended
Taking medications as prescribed
Attending regular follow-up appointments
Discussing estrogen-containing medications with your healthcare provider
Frequently Asked Questions
Is LAM contagious?
No. LAM is not contagious and cannot be spread from person to person.
Is LAM a type of cancer?
LAM is not considered a traditional cancer, but the abnormal cells can grow and spread to different parts of the body.
The Bottom Line
Lymphangioleiomyomatosis is a rare lung disease that primarily affects women and can gradually impair lung function. Although there is no cure, early diagnosis, regular monitoring, and treatments can help manage the disease.
