A Look at Lymphangioleiomyomatosis (LAM) - The Fatal Lung Disease

Rare and fatal lung disease Lymphangioleiomyomatosis (LAM), predominantly affects women of all races (This lung disease rarely effects men) and of childbearing age (LAM has been reported in patients aged 12 to 70 years old), first described in medical literature by Von Stosesel in 1937. The disease characterterized by an unusual type of muscle cells that invade the tissue of the lungs (including the airways), blood and lymph vessels (Part of the lymphatic system that produce and transport lymph fluid from tissues to the circulatory system. Critically important maintain a healthy immune system). These cells are not considered cancerous. Eventually, these muscle cells grow into the walls of the airways, and blood and lymph vessels, causing them to become obstructed. This blocks flow of air, blood and lymph vessels to and from the lungs. Thus preventing the lungs from providing oxygen to the rest of the body. At least fifty percent of patients develop kidney tumor called Angiomyolipoma. Approximately, 2.5 percent of patients with tuberous sclerosis (genetic disease that causes benign tumors to grow including within the lungs) develop disease that is identical, which occurs in LAM. Symptoms associated to LAM includes: Coughing, difficulty breathing (dyspnea), especially during periods of exercise or exertion, chest pain and can become extremely fatigued. In worst cases, complications lead to collapse lung or fluid accumulation around the lungs (pleural effusion). This could potential lead to chronic respiratory failure, and inevitable require supplemental oxygen. Fortunately, the survival time has increased to more than 20 years after diagnosis.