While there is no cure for LAM, doctors treat LAM with sirolimus (rapamycin), a medicine that stabilizes lung function, treats an abnormal fluid buildup in the lung called chylothorax, and improves overall quality of life. They may also prescribe other medicines or therapies to control other symptoms or complications.

You may want to see a pulmonologist doctor who specializes in lung diseases and conditions, especially LAM, to help treat your condition. You may also want to ask your doctor about the latest American Thoracic Society clinical guidelines for diagnosis and management of LAM which include new treatment recommendations.


Your doctor may prescribe sirolimus (rapamycin) to treat your condition, or bronchodilators or oxygen therapy to help you breathe better. Lung function tests can sometimes show whether these medicines are likely to help you.

  • Sirolimus (rapamycin). Studies have shown that sirolimus helps regulate the abnormal growth and movement of LAM cells. Sirolimus stabilizes lung function and improves quality of life, shrinks abnormal kidney and lymph node growths, and reduces abnormal fluid in the lung called chylothorax. The latest clinical guidelines recommend sirolimus for people with LAM if they have abnormal or declining lung function or in some patients with LAM who have pleural effusions containing chylothorax. Sirolimus may also reduce the size of angiomyolipomas and therefore may be a treatment option before procedures that remove or shrink kidney tumors. Sirolimus does have side effects, some of which can be serious. If you have LAM, talk with your doctor about the benefits and risks of this medicine, and whether it’s an option for you.
  • Bronchodilators. If you’re having trouble breathing or are wheezing, your doctor may prescribe bronchodilators. These medicines relax the muscles around the airways. This helps the airways open up, making it easier for you to breathe.
  • Oxygen Therapy. If the level of oxygen in your blood is low, your doctor may suggest oxygen therapy. Oxygen usually is given through nasal prongs or a mask. At first, you may need oxygen only while exercising. It also may help to use it while sleeping. Over time, you may need full-time oxygen therapy.

The latest clinical guidelines, which are based on currently available clinical data, do not recommend the following medicines to treat LAM:

  • Doxycycline, an antibiotic that did not show a clinical benefit when used to treat LAM.
  • Hormone therapies including certain medicines—progestins, gonadotrophin-releasing hormone (GnRH) agonists, selective estrogen receptor modulators (SERMs) such as tamoxifen—or surgical procedures such as . While these therapies had previously been used to treat LAM, newer analyses suggest they do not produce clinical benefit in all patients with LAM. If you are taking any of these hormone medicines, talk to your doctor to see if you need to discontinue their use.

Living With will discuss medicines your doctor may prescribe to help prevent complications such as osteoporosis.

Procedures that remove air or fluid from the chest or abdomen

Several procedures can remove excess air or fluid from your chest or abdomen. Removing fluid from your chest by thoracentesis or from your abdomen by paracentesis may help relieve discomfort and shortness of breath.

Your doctor often can remove the fluid with a needle and syringe. If large amounts of fluid build-up in your chest, your doctor may have to insert a tube into your chest to remove the fluid.

Removing air from your chest may relieve shortness of breath and chest pain caused by a pneumothorax, or collapsed lung. Your doctor usually can remove the air with a tube. The tube is inserted into your chest between your side ribs. Often, the tube is attached to a suction device. If this procedure doesn't work, or if your lungs repeatedly collapse, you may need surgery.

If fluid or air often leak into your chest, your doctor may recommend a procedure called pleurodesis to prevent repeat episodes. Your doctor may inject a chemical at the site of the leakage. The chemical fuses your lung and chest wall together, which removes the space for leakage.

Your doctor may do this procedure at your bedside in the hospital. You will be given medicine to prevent pain. The procedure also can be done in an operating room using video-assisted thoracoscopy. In this case, you will be given medicine to make you sleep during the procedure.

Procedures that remove or shrink kidney tumors

Kidney tumors, or angiomyolipomas, often don't cause symptoms, but sometimes they can cause ongoing pain or bleeding. If this happens, you may need surgery to remove some of them. If bleeding isn't too severe, a radiologist often can block the blood vessels feeding the kidney tumors. This may cause them to shrink.

Lung transplant

Some patients who have severe lung damage due to advanced LAM may be eligible for lung transplants. While lung transplants can improve lung function and quality of life for eligible patients, they have a high risk of complications, including infections and rejection of the transplanted lung by the body.

Studies suggest that more than three-quarters of women with LAM who receive a lung transplant survive for at least 3 years. In a few cases, doctors have found LAM cells in the newly transplanted lungs. However, the LAM cells generally don't stop the transplanted lung from working.