How Is LAM Treated?

Currently, no treatment is available to stop the growth of the cysts and cell clusters that occur in LAM. Most treatments for LAM are aimed at easing symptoms and preventing complications.

The main treatments are:

• Medicines to improve air flow in the lungs and reduce wheezing
• Oxygen therapy
• Procedures to remove fluid from the chest or abdomen and stop it from building up again
• Procedures to shrink angiomyolipomas (AMLs)
• Lung transplant
• Hormone therapy

Medicines

Medicines That Help You Breathe Better

If you’re having trouble breathing, 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.

Lung function tests can sometimes show whether these medicines are likely to help you.

Medicines That Prevent Bone Loss

Women who have LAM are at risk for a bone-weakening condition called osteoporosis (OS-te-o-po-RO-sis). This is in part because many LAM therapies block the estrogen action needed to keep bones strong.

To prevent osteoporosis, your doctor may measure your bone density. If you have lost bone density, your doctor may prescribe medicines to prevent bone loss. He or she also may prescribe calcium and vitamin D supplements.

Rapamycin (Sirolimus)

Sirolimus was originally developed to prevent the immune system from rejecting kidney transplants. However, studies have shown that the medicine helps regulate the abnormal growth and movement of LAM cells.

Research suggests that sirolimus may shrink tumors in the kidneys of women who have LAM.

A recent study funded in part by the National Institutes of Health showed that sirolimus also helps stabilize lung function, reduce symptoms, and improve quality of life for people who have LAM.

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.

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.

A standard exercise stress test or a 6-minute walk test can show whether you need oxygen while exercising. A 6-minute walk test measures the distance you can walk in 6 minutes. An exercise stress test measures how well your lungs and heart work while you walk on a treadmill or pedal a stationary bike.

You also may need a blood test to show your blood oxygen level and how much oxygen you need.

For more information, go to the Health Topics Oxygen Therapy article.

Procedures To Remove Air or Fluid From the Chest or Abdomen

Several procedures can remove excess air or fluid from your chest and abdomen. These procedures also help prevent air or fluid from building up again.

Removing fluid from your chest or abdomen may help relieve discomfort and shortness of breath. The procedure to remove fluid from the chest is called thoracentesis (THOR-ah-sen-TE-sis). The procedure to remove fluid from the abdomen is called paracentesis (PAR-ah-sen-TE-sis).

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 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 collapse often, you may need surgery.

If fluid and air often leak into your chest, your doctor may inject a chemical at the site of the leakage. The chemical fuses your lung and chest wall together. This 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 To Remove or Shrink Angiomyolipomas

AMLs 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 the tumors.

If bleeding isn't too severe, a radiologist often can block the blood vessels feeding the AMLs. This may cause them to shrink.

Lung Transplant

Lung transplants can improve lung function and quality of life in patients who have advanced LAM.

However, lung transplants 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 and other parts of the body. However, the LAM cells don't seem to stop the transplanted lung from working.

For more information, go to the Health Topics Lung Transplant article.

Hormone Therapy

Estrogen is thought to play a role in causing LAM. Thus, your doctor may want to treat you with hormone therapy that limits the effects of estrogen on your body. Hormone therapy is given in pill form or as injections.

Some doctors also suggest surgery to remove the ovaries. This causes menopause and greatly reduces estrogen levels in the body.

Unfortunately, at this time, no clear evidence shows that this type of treatment works for women who have LAM.

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