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This information was reviewed and approved by Gregory P. Downey, MD, FRCPC (2/2/2023).

Lymphangioleiomyomatosis (pronounced lim-fan-gee-o-ly-o-my-o-ma-to-sis), or LAM, is a rare disease found almost exclusively in women of all races and ethnic backgrounds. Out of 1 million women, LAM affects three to eight women. The average age of women diagnosed with LAM is 35, but it can affect women who are between 30 and 70 years old.

Most people with LAM notice that it slowly makes breathing worse over time. There is no cure, but treatments that are tailored to each person help manage symptoms and prevent complications. Some medications can be very effective in improving breathing and slowing lung damage. 
  
LAM Medications

  • Sirolimus (rapamycin or Rapamune®) is the first drug to be approved by the U.S. Food and Drug Administration for the treatment of LAM. Sirolimus can be effective in improving breathing, slowing lung damage and stabilizing lung function, which improves quality of life in patients with moderate to severe LAM. It can slow and stop the growth of LAM cells by preventing them from multiplying and shrinking them.  
  • Everolimus (Afinitor) is not FDA approved, but in clinical trials, it has been effective in improving breathing, slowing lung damage. 
  • Inhaled bronchodilators open the airways by relaxing the smooth muscle around the airways to improve breathing

 
Oxygen Therapy helps people with chronic lung disease normalize the oxygen level in the blood during sleep, rest, activity and during acute illnesses. This helps decrease shortness of breath when you are active and allows you to do more.
 
Procedures including surgically implanted shunts or tubes, drain fluid from the lungs or around the lungs.
 
Supplements such as vitamin D and calcium may be recommended to prevent bone loss from osteoporosis that can be associated with LAM.

 

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