Living Well with LAM

Everyone’s journey with LAM is different, depending on the progression of the disease. But never think you are alone. In fact, many others have been where you are now. The LAM Foundation has fostered a large and active patient support community that values the opportunity to share their experiences and advice for living a full and rewarding life with LAM. Connect with this community below, and benefit from their knowledge of the road ahead.

Coping with Your Diagnosis

Receiving a diagnosis of LAM may cause a patient to experience feelings of anger, denial, shock, grief, helplessness, confusion, despair, sadness or fear. These feelings are common, and many women experience one or all of them during various stages of the disease. These are normal responses and are all part of the grieving process. Grief isn’t related just to death; it can be the result of any loss—including the loss of one’s health and/or more active lifestyle.

Relief is another common reaction to a LAM diagnosis. Many patients have experienced symptoms such as shortness of breath or lung collapses without doctors determining why – and are actually relieved to have an explanation for these health issues. There is a certain feeling of validation knowing that these symptoms were not imagined – and that ever-powerful fear of the unknown now has a name.

In addition to concerns regarding finances, insurance coverage, and employment, there may be worries regarding parenting and relationships. To help manage these stresses, patients may consider complementary therapies to nurture the body, mind, and spirit. Exercise, acupuncture, massage therapy, yoga, and reflexology are just some ways to relax and enhance coping skills. If the stresses accompanying a LAM diagnosis become overwhelming, it’s important to seek emotional support from a friend or relative or from a professional such as a psychologist, a counselor, or a member of the clergy.

Taking Sirolimus

Your physician may prescribe sirolimus to treat symptoms or complications of LAM. It is the first line treatment and FDA approved for LAM.

Take time to educate yourself about this medication and talk to other members of the LAM community about their experiences starting the drug. There are some side effects, however the many patients taking sirolimus have reported an adjustment period followed by stabilization of side effects and symptoms.

Some common side effects include mouth ulcers, acne, and high cholesterol. Read more about sirolimus in the prescribing guide. 

Pulmonary Rehabilitation

Even though almost all women with LAM have difficulty breathing, it is important to continue exercising. The best way to get started with an appropriate training program is to attend pulmonary rehabilitation. Patients in pulmonary rehab are supervised by trained personnel who monitor oxygen saturation levels and heart rate levels to make sure that the exercise is helping.

If a patient has never used supplemental oxygen, the staff at pulmonary rehab can advise if oxygen would make exercise safer and more effective. For those already on supplemental oxygen, staff can help gauge how much oxygen (liter flow) is needed to perform different kinds of activities.

Pulmonary rehab is also helpful for patients listed for a lung transplant. It is important to make sure that they are as strong and healthy as possible in preparation for surgery.

Supplemental Oxygen

Supplemental oxygen makes a huge difference in the quality of life for many women with LAM. Every tissue in the body needs a constant supply of oxygen to work properly. When these tissues are not receiving enough oxygen, their performance suffers — which can lead to fatigue, poor sleep, and other problems.

Oxygen (O2) is an element your body needs to live and to perform all bodily functions. In general, when a person inhales, they take in oxygen, which then passes through the lungs and into the bloodstream through alveoli (small air sacs in the lungs). Once the blood has picked up the oxygen, it is pumped through the rest of the body. As the body uses oxygen for its various functions, it releases carbon dioxide (CO2) as a waste product. The carbon dioxide then travels through the blood into the cells in the lungs, and then the lungs breathe it out. If this process is flawed and the body cannot pass oxygen from the lungs into the bloodstream, the blood oxygen levels will not be sufficient for the body to function well. If this is the case, supplemental oxygen may be necessary. This is especially true for people who are unable to obtain sufficient oxygen due to respiratory illnesses like LAM.

These three tests can help the patient and doctor determine if her body is getting enough oxygen:

Arterial Blood Gas (ABG) Test

This test measures the percentage of oxygen and carbon dioxide in the blood. This test is performed by drawing a small sample of blood from an artery, usually near the wrist (radial artery) or the crook of the arm (brachial artery).

Pulse Oximetry (SaO2)

This simple test is used to measure oxygen saturation or the amount of oxygen in the blood. This test is performed by placing a small clip with a sensor on a fingertip. A level of 95 – 100% is considered normal but anything over 90% is typically considered adequate. Levels below 90% at rest are usually considered candidates for supplemental oxygen.

Six-Minute Walk Test

This test is used to determine if (and how much) oxygen levels drop with exertion. As the name indicates, the patient must walk for six minutes at her own pace, covering as much distance as possible. A pulse oximeter is used to measure the heart rate and oxygen level during the test. If saturation levels drop significantly during the test, the patient may be a good candidate for the use of supplemental oxygen during exercise.

Advice When Using Supplemental Oxygen

• Patients must walk slowly and steadily rather than quickly or at various speeds. Pacing oneself can help preserve both energy and stamina.
• If a patient normally uses bronchodilators, she can ask her doctor about trying a nebulizer. This method of O2 delivery may provide an extra boost.
• Some patients breathe better early in the day or in the mid to late afternoon when they have more energy. The best time to perform necessary tasks and harder chores is whatever time of day one’s breathing is best.
• A pinching/grabbing device can help for picking up items off the floor and reaching items on high shelves. Tasks that require the patient to bend over or to raise her arms above her head can cause shortness of breath.

Pleurodesis

Pleurodesis is a procedure that adheres the outside of the lung to the inside of the chest cavity to prevent the lung from collapsing. If a patient has had a pneumothorax previously and/or has a persistent leak, the doctor may suggest pleurodesis as a preventative measure. Pleurodesis is usually recommended after the first incidence of lung collapse in women with LAM because patients who have one lung collapse are likely to have additional lung collapses.

Reproductive Planning

LAM may progress more rapidly in the presence of exogenous estrogen and during pregnancy. Patients should stop taking estrogen supplements and estrogen-containing birth control options. Please see this guide for contraception options in patients with LAM. The role of anti-estrogen therapies such as progesterone, Gonadotrophin-releasing hormone (GnRH) antagonists, and oophorectomy, is not clear. They are not routinely recommended for the treatment of LAM. Clinicians sometimes consider hormonal therapies in special situations, such as continued lung function decline despite sirolimus use, and in patients who have recurrent pneumothoraces that are timed with the menstrual cycle.

The risk of pregnancy in LAM has not been rigorously studied. However, case reports and small series have suggested an increased risk of spontaneous pneumothoraces, growth and bleeding in AMLs, and accelerated lung function decline, in pregnant patients with LAM. Although women with LAM may have successful pregnancies, pregnancy may be more of a risk for those with poor lung function. The physician and patient should discuss the risks of pregnancy carefully and decisions should be made on an individual basis. The exact risks associated with sirolimus use during pregnancy are unknown. Generally, most patients and physicians avoid the use of sirolimus during pregnancy. However, some published care reports and anecdotal observations suggest this might be an option for some pregnant patients. Like the overall pregnancy decision, the choice to use sirolimus during all or a part of pregnancy should be made in consultation with maternal fetal medicine specialists.

Air Travel

Many LAM patients are concerned about air travel because of the potential risk of a lung cyst rupturing and causing a lung collapse due to atmospheric pressure changes during flight. The risk of air travel-related lung collapses in women with LAM is approximately 1-2 episodes per 100 flights. This risk is low enough that in most LAM patients, air travel is considered safe. It is reasonable to consider additional factors, including a history of frequent or recent lung collapses, and the overall extent of cardiopulmonary impairment. If patients have a newly diagnosed or recent lung collapse, it is recommended they wait two weeks after complete healing prior to flying. In any patient with unexplained chest pain, shortness of breath, or symptoms suggestive of a new lung collapse, appropriate radiologic testing should be performed prior to air travel.

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Vaccinations

Patients with LAM should maintain appropriate vaccinations. There have been some exciting new developments regarding vaccines, chief among them being the release of data regarding the safety and efficacy of two COVID-19 vaccines that have been granted Emergency Use Authorization (EUA) by the Food and Drug Administration (FDA). Linked below are the latest updates on vaccines and LAM, and COVID-19 guidance.

Vaccination Recommendations

Exercise

We all know that exercise is good for us. But how good? Here, Mary Beth Brown, PT, PhD, discusses the findings of her study called “Exercise & LAM,” including information on blood oxygen levels, and the benefits of a home-based exercise program for individuals living 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.