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What is the treatment of Lymphangiomyomatosis?

By  ,  National Institute of Health
Jan 10, 2013
4.8 / 5(4 Ratings)

Currently, no treatment is available to stop the growth of the cysts and cell clusters that occur in 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:

  • 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


Certain medicines can help open your lungs so that you can breathe better.
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 (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 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. However, studies show that rapamycin helps regulate the abnormal growth and movement of LAM cells.


A study on a small number of women who have LAM and/or TSC found that rapamycin shrank kidney tumors by 50 percent over 1 year. The medicine also seemed to improve lung function in some women who have LAM.


However, the study showed that the medicine had a number of side effects. Thus, more research is 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.


Procedures To Remove Air or Fluid 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 (THOR-...

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