Lymphangioleiomyomatosis (LAM) is a rare condition that occurs almost exclusively in adult women, but has also been reported in adult men and children with a genetic condition called tuberous sclerosis.
LAM is characterized by development of cysts in the lungs, accumulation of fluid around the lungs and abdominal organs, and development of abdominal tumors. The lung disease worsens over time, causing shortness of breath with activity, low oxygen levels, and, occasionally, a collapse of a portion of the lung (a) condition called a pneumothorax.
Although the cause of LAM remains unknown, recent scientific discoveries have allowed the FDA to approve a medication that slows the progression of lung disease, reduces fluid accumulation, and shrinks abdominal tumors.
Diagnosing LAM can be difficult because it is so rare and because its symptoms are similar to those of other lung diseases. Symptoms of LAM typically include:
- Shortness of breath
- Persistent cough
- Chest pain
- Abdominal cramping or pain.
People with LAM and tuberous sclerosis may also experience seizures, learning difficulties, and brain tumors.
The medical team at Norton Thoracic Institute uses a variety of tests to diagnose LAM. These may include the following:
- Pulmonary function tests to determine how well the lungs are working
- A CT scan to look for fluid collections, abdominal tumors, and cysts in the lungs
- An MRI to look for brain tumors
- Blood tests to look for genetic changes causing tuberous sclerosis and a marker of LAM called vascular endothelial growth factor D
- In rare cases, a biopsy of lung tissue may be needed to confirm the diagnosis when results of other tests are inconclusive.
If you are diagnosed with LAM, the specialists in the LAM Clinic at Norton Thoracic Institute will develop a personalized treatment plan for you. This may include the following:
- Medications to improve breathing, including sirolimus, the first FDA-approved drug for LAM
- Treatment of LAM-related complications, such as a collapsed lung
- Referral to experts in other specialties to treat complications outside the lungs.
Lung transplantation may be indicated in severe cases of LAM. Norton Thoracic Institute has the second busiest lung transplant program in the United States. The Norton Lung Transplant Center is known for streamlined processes that speed up treatment and produce exceptional outcomes.
Because the cause of LAM is unknown, it cannot be prevented. Researchers are working to understand the cause of this disease and develop a cure.
Learn More About LAM Services at Norton Thoracic Institute
To learn more about our services, call (602) 406-8187.
Norton Thoracic Institute has been recognized by the LAM Foundation as an institution that delivers world-class comprehensive treatment for patients with LAM. To learn more about LAM, please visit www.thelamfoundation.org.
To find out more about the multidisciplinary LAM team at NTI, please visit our faculty and staff page.