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Diagnosing and Treating Lymphangioleiomyomatosis (LAM)

You should see your doctor any time you have a new, unfamiliar, or worsening symptom, specifically if you are having difficulty breathing. Your doctor can then decide whether your symptoms are related to LAM and whether you can be treated at home or if you require hospitalization.

What to Expect

Once you are diagnosed with LAM, the disease process continues to progress, more slowly in some and faster in others. Treatment may slow worsening, but there is no cure and, eventually over time, the lungs will be replaced by cysts (holes). When this becomes very severe, lung transplant is the only option available.

Expect a close working relationship with frequent follow-ups with your primary care doctor and lung specialist. Other specialists may be involved at varied stages of this disease.

In the early stages of LAM, you can usually do your normal daily activities. You may find it harder to be active later on. Once you are diagnosed with LAM, there should be discussions with your healthcare provider regarding life planning, pregnancy and birth control.

How LAM Is Diagnosed

LAM may be suspected on a regular X-ray, but your doctor will order a CT scan of the chest which show detailed images to identify the cysts. Abdominal ultrasound may reveal tumors in kidney, liver or spleen.

Laboratory tests may be ordered for levels of vascular endothelial growth factor-D (VEGF-D), which may be elevated in LAM. Your doctor may also order breathing tests to assess lung function. Open-lung biopsy (surgical procedure done in the operating room by a surgeon) or bronchoscopy (a flexible scope with a video camera introduced into your airways through your nose or mouth to inspect your lungs, done by your pulmonologist) may be required to look at lung tissue.

How LAM Is Treated

Currently, no treatment is available to stop the growth of the cysts that occur in LAM. Most treatments for LAM are aimed at easing symptoms and preventing complications. The available treatment options include:

  • Oxygen therapy
  • Pulmonary Rehabilitation
  • Removal of air or fluid around the lungs or abdomen to help you breathe better
  • Surveillance and treatment of osteoporosis (weak bones)
  • Standard vaccinations to prevent respiratory infections
  • Bronchodilators (inhalers) to help open airways and improve breathing
  • Medications
    • Sirolimus (rapamycin). The only medication that may be helpful in treating LAM is sirolimus (also known as rapamycin).  This medication may help to improve lung function, reduce symptoms, and shrink kidney tumors. Sirolimus has side effects, some of which can be serious. If you have LAM, talk with your doctor about the benefits and risks of this medicine. Remember that sirolimus therapy may help to control the disease, but is not a cure. 
    • Hormones. Because LAM is occurs only in younger women and tends to get worse during pregnancy, some physicians recommend treatment to reduce the effects of estrogens.  There is no proof that these drugs work, and there are side effects. 
  • Lung transplantation may be considered in advanced cases, as it can improve lung function and quality of life.

When to See Your Doctor

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 symptom.

If you are diagnosed with LAM, you are also at risk of tumors (angiomyolipomas) in your abdominal organs, most commonly seen in the kidney. As these tumors grow, they are at risk of internal bleeding that could be fatal and cause symptoms like abdominal pain and dizziness.

Seek prompt medical attention if you experience worsening of your usual breathing. This could be due to:

  • Pneumothorax
  • Fluid collection around the lung (chylothorax)
  • Blockage of the lymphatic system, which can cause fluid overload inside of the lung

Your doctor can then decide whether your symptoms are related to LAM and whether you can be treated at home or if you require hospitalization.

    This content was developed in partnership with the CHEST Foundation, the philanthropic arm of the American College of Chest Physicians.

    Page Last Updated: August 2, 2019

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