Lymphangioleiomyomatosis (LAM) is a genetic condition that causes the formation of non-cancerous growths in the lungs and other parts of the body. LAM primarily affects women and can lead to worsening breathing issues over time. Treatment often involves medication and supportive care.
While the mutated genes linked to LAM can be inherited from one’s parents, medical researchers are not sure what causes the mutation in sporadic forms of the disorder. LAM results from faulty tumour suppressor genes, which normally prevent cancer from developing by controlling cell growth. LAM does not cause cancer, but the same mechanism that impedes malignant tumours can also stop non-cancerous tumours from forming.
When tumour suppressor genes are mutated, they can have the opposite effect and accelerate the growth of cells. This can lead to the development of cysts within the lungs, as well as the kidneys and lymphatic system.
The symptoms associated with LAM mimic other lung disorders, which means you should seek a formal diagnosis if you notice any of the following:
Most people are diagnosed with the condition between the ages of 20 and 40, and the vast majority of people with LAM are women.
As a chronic condition, the health effects of LAM will continue to worsen and may cause complications like collapsed lungs and pleural effusion (i.e., fluid around the lungs). A medication called sirolimus slows progression of the disorder by reducing tumours in the lungs and elsewhere in the body. Sirolimus was originally developed for people who received organ transplants, as the medication limits the immune system response to prevent rejection.
When breathing issues affect health, doctors can also recommend the following supportive care:
In the event lung damage is already severe and breathing is significantly impacted, your doctor may discuss a lung transplant as the next best treatment option.