Treating and Managing LAM

How LAM Is Treated

LAM is a progressive disease, meaning it gets worse over time. There is no cure, but treatment can help improve symptoms, prevent complications and maintain lung function. Working with a lung doctor, called a pulmonologist, who has experience treating LAM is vital to finding the most effective treatment plan for you.

Initially, if you have little to no symptoms, treatment intervention may be minimal. When you have symptoms or as your disease progresses, you may be prescribed medication to help manage your condition. Medications may include:

• Sirolimus and everolimus- Also called mTOR inhibitors Ttey help control the abnormal growth of the LAM cells and may help improve lung function and reduce symptoms.
• Bronchodilators- Inhaled medicines which can help relax the muscles around the airways, making breathing easier.

Other treatments may include:

• Oxygen therapy-your provider will decide if supplemental oxygen is right for you. It can help you feel less short of breath, less fatigued, sleep better and have more energy to do the activities you enjoy.
• Pulmonary rehabilitation- a program of education and exercise to increase awareness about your disease can help improve your activity tolerance, muscle strength and quality of life.
• Lung transplant- in advanced LAM or when no other treatment option is able to manage your symptoms, a lung transplant may be recommended by your provider.

Managing LAM

Once you are diagnosed with LAM, it is important to see your specialist regularly so that your condition can be monitored closely. During these visits, your provider may do repeat lung function tests and imaging, such as a CT scan, to determine if your condition is being well managed or if you need adjustments to your treatment plan. You should continue to see your primary care provider regularly, as well as any other specialists that are recommended to help manage your disease. 

LAM can affect many aspects of your daily life. Making lifestyle changes can help you better manage your health and quality of life.

• Physical activity: Talk with your provider about what types of activity are right for you. Staying active can help maintain your strength and lung function, but it’s important to discuss your activity plan with your provider before you begin.
• Pregnancy: If you are considering pregnancy, first discuss with your specialist. Pregnancy can carry additional risks, and your provider can help you understand the safest options.
• Take Steps to Stay healthy: 
  • Ask your provider if you are up to date on your vaccinations
  • Eat a healthy diet
  • Quit smoking and avoid secondhand smoke
  • Talk to your provider prior to traveling by air
• Support Your Mental Health:
  • You may feel overwhelmed at times, and this is completely normal. There are support groups available to connect you with others living with LAM, and other mental healthcare options may also help you manage your symptoms. Talk to provider about support options.

If you have new, unfamiliar or worsening symptoms, talk to your healthcare provider. If you have signs of a collapsed lung, also called a pneumothorax, or serious symptoms such as difficulty breathing, chest pain, coughing up blood or bluish skin/lips/nails, seek immediate medical attention. 

Preventing Complications

When you are living with LAM, you are at risk for several complications. Many people  with LAM will experience at least one pneumothorax (collapsed lung). LAM can cause blockage of the lymphatic system, which can lead to fluid collection around the lungs. Kidney tumors called angiomyolipomas are also common for people living with LAM and need to be monitored as they increase the risk of internal bleeding which can be fatal. Talk to your specialist to better understand the signs and symptoms of any of these complications and seek medical attention immediately if any of these occur.