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Lymphangioleiomyomatosis (LAM): Treatment


Currently, no treatment is available to stop the growth of the cysts and cell clusters that occur in lymphangioleiomyomatosis (LAM).

An experimental medicine, rapamycin (sirolimus), shows promise in shrinking tumors in the kidneys of women who have LAM. Studies are under way to find out if this medicine improves lung function in women who have LAM.

Most treatments for LAM are aimed at easing symptoms and preventing complications. The main treatments are the following:

 

Medicines that Make Breathing Easier
Bronchodilators are medicines that relax the muscles around the airways. This helps the airways open up, making it easier for you to breathe. About 1 out of 4 women who has LAM breathes better with the use of bronchodilators. 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. 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 that prevent bone loss. He or she also may prescribe calcium and vitamin D supplements.

 

Rapamycin
Rapamycin (sirolimus) is the first medicine to be tested as a treatment for slowing or stopping the growth of LAM cell clusters. This medicine was originally developed to prevent the immune system from rejecting kidney transplants, but studies show that rapamycin helps regulate the abnormal growth and movement of LAM cells.

However, more research is still needed to see how well this medicine works in women who have LAM, and whether its risks outweigh its benefits.

 

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 only need oxygen 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 what your oxygen level is and how much oxygen you need.

 

Fluid Removal from the Chest or Abdomen
Several procedures help remove 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. The procedure to remove fluid from the abdomen is called paracentesis.

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 may be done in an operating room using video-assisted thoracoscopy. In this case, you will temporarily be put to sleep for the procedure.

 

Lung Transplant
A lung transplant improves lung function and quality of life in patients who have advanced LAM. Lung transplants have a high risk of complications. These include 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, LAM cells don't seem to stop the transplanted lung from working.

 

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

Hormone therapy can be taken as a pill or with regular shots. Some doctors also suggest surgery to remove the ovaries. This causes menopause and greatly reduces estrogen levels in the body. Unfortunately, at this time, there's no clear evidence that this type of treatment is effective for LAM.

 

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