How LAM Is Treated
There is no cure for LAM, but treatment can help ease symptoms, prevent complications and disease progression. Working with a pulmonologist who has experience treating LAM can help you find the treatment that is most effective for you.
Early on, this may be minimal, especially for women whose disease is progressing slowly. The most common treatment includes taking a medication called sirolimus. Sirolimus may help to improve lung function, reduce symptoms and shrink any kidney tumors. Sirolimus has side effects, some of which can be serious, which you should discuss with your doctor when making treatment decisions. Inhaled bronchodilators can also to help open airways and improve breathing.
Hormone therapy was often used to treat LAM in the past, but it now recognized to be ineffective and may potentially cause harm.
As the disease progresses, LAM patients may need other forms of treatment in conjunction with their medication. Oxygen therapy, which increases the amount of oxygen delivered to your lungs and into your blood, may be used occasionally at first, but will eventually be needed full-time. Pulmonary rehabilitation may also be a good idea for people with LAM. Both of these treatments greatly improve patients’ quality of life. When LAM causes advanced disease, a lung transplant may be the only option.
In the early stages of LAM, you can usually do your normal daily activities. You may find it harder to be active as the disease progresses. LAM is a chronic disease, so you may have symptoms or require treatment for the rest of your life.
Once you are diagnosed with LAM, there should be discussions with your doctor regarding life planning, pregnancy and birth control. Expect a close working relationship with frequent follow-ups with your primary care doctor and lung specialist. Other specialists may be involved at various stages of this disease.
You will require multiple repeat lung function testing and imaging after your diagnosis. If your lung function becomes very poor, you will need to go through a process for evaluation for transplantation if your doctor feels you are eligible. Transplantation requires taking multiple medications, as well as regular testing and doctor visits for life.
After the diagnosis is made, your doctor will arrange for routine follow-up visits to monitor your condition. However, you should see your doctor any time you have a new, unfamiliar or worsening symptoms.
If you are diagnosed with LAM, you are at risk for several complications. More than half of the patients with LAM experience at least one pneumothorax (collapsed lung). This will require a procedure to re-inflate the lung and may happen more than once. LAM can cause blockage of the lymphatic system, which can lead to fluid collection around the lungs. Kidney tumors are also common for LAM patients and will have to be monitored as they increase the risk of internal bleeding that could be fatal. Seek prompt medical attention immediately in any of these situations.
Reviewed and approved by the American Lung Association Scientific and Medical Editorial Review Panel.
Page last updated: March 12, 2020