Rare Lung Disease Leaves Patients with Agonizing Treatment Dilemma

Anna Kramer
Author: Loyola University Health System
Published: April 23, 2014
URL: http://www.medicalnewstoday.com/releases/275751.php

Summary:
      There is a very difficult decision that doctor's have to make when treating patients with the rare lung disease, lymphangioleiomyomatosis (LAM), that causes a growth of  muscle cells that invade the lungs and make it difficult for air to pass through the airways.  The drug, sirolimus, can decrease the progression of the disease and help with the shortness of breathe, but, it often causes fatal complications following a lung transplant that make it difficult for the wound to heal and the attachment points of the lung do not hold. The Food and Drug Administration has issued a warning attached to this drug, so patients on the waiting list for a lung transplant are not allowed to go on sirolimus. The drug has a long half-life, so it stays in the body longer, making it not predictable on the results of the transplant even if the patient is taken off of the drug.  But, there is another drug, everolimus, which does not last as long in the body once the patient stops taking it. A patient who uses this strategy usually doesn't have an issue with the wound healing after the transplant. Members of the LAM foundation agree that more research needs to be done on the rapamycin-class of drugs (the class that includes sirolimus and everolimus). But, because LAM is such an uncommon disease, there are still many unknowns.

Connection: 
       This article connects to our unit on the respiratory system because LAM is a disease that occurs in the lungs. The lungs are a very significant organ that function in breathing. LAMS blocks the airways and makes it so that air can not travel throughout the lungs. This proposes a very serious issue because without the airflow in the lungs, this affects not only the respiratory system, but also the circulatory system. If the lungs can not provide oxygen to diffuse into the blood, the heart won't have sufficient amounts to pump to the rest of the body. The body needs oxygen to make ATP, and without it, the body can not function. The issue that sirolimus causes with the wound not being able to heal is a very large issue for the immune system because it damages the first-response of the immune system and could lead to an infection of the wound if pathogens were to enter.