Symptoms of LAM

The symptoms of LAM are varied and can include some (or all) of the following:

Shortness of Breath (Dyspnea)

This is the feeling of being breathless or having difficulty breathing without assistance from supplemental oxygen. Shortness of breath is an extremely common symptom of LAM, especially during exertion.

Chest Pain (Pleurisy)

Chest pain is often caused by inflammation or irritation of the chest wall and the lining of the lungs. Chest pain can be caused by many things including infections, pneumothorax, or scar tissue from previous procedures. Many women with LAM experience chest pain from time to time.

Fatigue

Fatigue is frequently reported by women with LAM and the underlying cause is the focus on ongoing research.

Cough and Wheezing

Cough and wheezing are commonly experienced by many women with LAM.

Other complications

There are a variety of complications of LAM that may or may not affect each person with a LAM diagnosis. They include:

Collapsed Lung (Pneumothorax)

A pneumothorax, or lung collapse, is a leakage of air (pneumo) from a lung into the chest cavity (thorax). This air outside the lung abruptly diminishes the negative pressure (vacuum) between the chest wall and the lung, causing the lung to deflate or collapse.

The cause of a pneumothorax in a woman with LAM is thought to be the rupture of a cyst just below the surface of the lung. Often the collapse is only partial, but you still might experience some pain and shortness of breath. If a pneumothorax occurs and collapses a sizable portion (or all) of your lung, you’ll likely have chest, back or shoulder pain and significant difficulty breathing. Most women with LAM will experience more than one pneumothorax during the course of their disease. Because of this, pleurodesis intervention is recommended after the first occurrence of a pneumothorax.

There are two goals when treating a pneumothorax. The first is the immediate removal of air from the space between the lung and the chest wall (the pleural space) so that the lung can re-expand. The second objective is the prevention of recurrent pneumothoraces. Without treatment, there is a 70% chance of a recurring pneumothorax. With treatment, the risk is reduced to about 30%. Although pleurodesis isn’t a foolproof remedy against future pneumothoraces, it generally diminishes their likelihood and the severity of future pneumothoraces and can significantly improve quality of life. LAM patients taking sirolimus should consult their physician about holding the drug 2-4 weeks after the resolution of a pneumothorax due to wound healing issues.

Patients who have experienced a recent pneumothorax should avoid air travel and consult their physician about the best time to travel.

Kidney Angiomyolipomas

Angiomyolipomas are benign tumors often found in the kidneys of LAM patients. They are made up of three kinds of tissue: “angio” refers to blood vessels, “myo” refers to smooth muscle cells and “lipo” refers to fat tissue. These tumors can vary by patient and depending on the tissue distribution can be more prone to bleeding or other complications.

Lymphatic Complications

LAM cells can block lymph flow causing milky fluid (chyle) to collect in the chest, pelvis and abdomen. These are referred to as chylous plurual effusions (fluid around the lungs) and angioleiomyomas (chyle in the abdomen or pelvis). Occasionally some chyle is coughed up as sticky, whitish substance. Lymphatic manifestations occur more frequently in patients with sporadic LAM (approximately 30-40%) as compared to 10% for TSC-LAM. Drainage procedures might be needed to confirm the chylous nature of the fluid or to relieve symptoms of shortness of breath and abdominal discomfort.

Pleural Effusions(chylothorax)is a leakage and accumulation of fluid into the chest cavity. A chylothorax can be seen on an x-ray and causes shortness of breath and discomfort.

It is important to note that no two women experience symptoms of LAM in the same way. About two-thirds of LAM patients will have at least one lung collapse (pneumothorax) and about one-third will have leakage of fluid into the chest cavity (chylothorax). Angiomyolipomas (benign kidney tumors that tend to be asymptomatic) can be found in about 30% of women with LAM.

This content was created for general informational purposes only. The content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.