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Dr. Andrew Rynne
MD
Dr. Andrew Rynne

Family Physician

Exp 50 years

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What is the prognosis for a lymphangioleiomyomatosis suffering patient?

My sister was diagnosed with LAM lung disease , and just received biopsy results today . I am wondering what are the next steps to treating this RARE disease , she is healthy , active and 27 years old ....never smoked a day in her life and never lived with smokers , I have read info . and it seems like the life expectancy of this disease is 10 to 20 years is this true . What are the options for someone her age ?
Tue, 12 Jun 2018
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General & Family Physician 's  Response
Hi and Welcome to 'Ask a Doctor' service .
I have read your question and here is my advice.
Sorry to hear of this sad news, infact lymphangioleiomyomatosis (LAM) generally effects people of this age female in thirty , this is a disease in which muscles in the lung proliferate extensively to make lot of small cyst than can rapture and may cause recurrent pnenothoracis.
Regarding the prognosis , the course of the disease is very variable , however the disease slowly progresses over many years.
You have rightly searched according to the literature 50% people are alive at 10 years , 25 percent are alive at 20 years .
Regarding it management , its always a specialist job. it may include inhaler some hormones, or some chemotherapeutic agent like serlolimus. Lung transplant would be something to be considered at the end. If any avoid smoke ,vaccination , and avoiding estrogen are some other things to be considered.
Best of luck


Hopes this clears your mind. I would be happy to answer if your have any thing else to ask in this regards.
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