Treating LAM

There is no proven treatment yet to slow or stop the progression of lung destruction in LAM. Most “treatments” that now exist for LAM are aimed at relieving symptoms and preventing complications of the disease in the multiple-systems and organs that it affects.

Overview

Some women with LAM are now living much longer, and/or being diagnosed later than had previously been the case. The main treatments for the symptoms of LAM are:

Medicines

Some medicines may help open your lungs so that you can breathe more easily. Bronchodilators are drugs that relax the muscles around the airways. As a result, the airways can open up, making it easier to breathe. About 1 out of 5 women with LAM experience the improvement of symptoms with the use of bronchodilators.

Octreotide and diuretics are sometimes used to prevent the buildup of fluid in the chest cavity and abdomen. Octreotide may reduce leakage of lymph into the abdominal or chest cavity.

Osteoporosis

Women with LAM have a greater chance of developing osteoporosis (a condition that causes bones to become weak and brittle) than other women. If you have LAM, your doctor should measure your bone density. If you have lost bone density, your doctor may prescribe drugs that prevent bone loss. He or she also may prescribe calcium and vitamin D supplements.

Estrogen

Physicians who think that estrogen may play a role in the development of LAM may treat their patients with hormone therapy although there is no strong evidence that this may change the course of disease progression.

Oxygen Therapy

If the level of oxygen in your blood is low, you may need oxygen therapy. Oxygen is usually given through nasal prongs or a mask. At first, you may need oxygen only while exercising. It also may help to use it while you are sleeping. Over time, you may need full-time oxygen therapy. Your doctor may give you a standard exercise stress test or a 6-minute walk test to find out whether you need oxygen while exercising. A blood test will 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 abdominal cavities and prevent them from building up in your chest cavity. Removing fluid from your chest or abdominal cavities may help relieve abdominal discomfort and shortness of breath. Your doctor can usually remove this fluid with a needle and syringe. If large amounts of fluid build up in your chest cavity, your doctor may have to insert a tube into your chest to remove it. Removing air from your chest cavity may relieve shortness of breath and chest pain caused by a collapsed lung. Your doctor can usually remove the air with a tube that is inserted into your chest cavity between your side ribs. The tube is usually attached to a suction device. If this procedure doesn't work, or if your lungs collapse frequently, you may need surgery.

If lymph and air leak into your chest cavity often, your doctor may perform a procedure to fuse your lung and chest wall together and remove space for leakage. This procedure is called pleurodesis (ploo-ROD-e-sis). It involves injecting a chemical into the place where the leakage is happening. Your doctor may do it at your bedside, while you are under local anesthesia. It also can be done in the operating room by video-assisted thoracoscopy surgery, while you are under general anesthesia.

Procedures To Remove AMLs

If you have ongoing severe pain or bleeding caused by AMLs, surgery to remove some of the abdominal growths may be helpful. If the bleeding is not too severe, an experienced radiologist can often block the blood vessels feeding the AMLs. This may cause them to shrink.

Lung Transplantation

Surgery to replace one or both of your lungs with healthy lungs from a human donor may be helpful. Survival after a lung transplant for LAM is probably better than survival after a lung transplant for another condition, such as emphysema. Still, lung transplantation has a high risk of complications and there is a global shortage of lungs so, transplant is not assured even if this is a last hope for a patient with LAM. In some cases, doctors have found LAM cells in the new transplanted lungs and other parts of the body, but the LAM cells do not seem to prevent the transplanted lung from working.

Possible New Treatments for LAM

Researchers are now studying several medicines as possible treatments for LAM, including rapamycin.

Rapamycin (sirolimus) is the first drug to show promise as a treatment that may slow the progression of LAM but there is no conclusive evidence at this point. Doctors now use this drug to prevent the immune system from rejecting kidney transplants. Researchers are looking into whether this medicine can reduce the size of kidney AMLs in LAM and tuberous sclerosis complex (TSC) patients and prevent the loss of lung function in LAM. Ideally, studies will test whether given drugs (it will likely take a cocktail of drugs to slow or stop LAM) can prevent or reverse the growth of the LAM cell clusters and cysts in various organs and slow the decline of lung function.