While there is no cure for LAM, living with the disease is much different today than in years past. LAM research has come a long way and has led to some amazing breakthroughs, including an effective treatment for the disease. Living with LAM is different for every patient depending on the progression of their disease. Below is useful information about living with and treating this complex disease.
Coping with Your Diagnosisplus
Receiving a diagnosis of LAM may cause you 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 emotions are normal responses and are all a part of the grieving process. Grief isn’t related just to death; it can be the result of any loss—including the loss of your health and/or the lifestyle you once enjoyed. Understand that you’ll probably grieve because of some of the changes you may face with LAM. Many changes could be far in the future or may not occur at all, so it’s best to stay optimistic, focus on the positives, and try not to dwell on your disease.
You may experience another common reaction to your diagnosis: relief. If you, like many patients, have experienced symptoms such as shortness of breath or lung collapses without your doctor finding a reason for your problems, you’ll likely be relieved to finally have an explanation for your health issues. You’ll feel validated knowing that you didn’t imagine all of these problems and that ever-powerful fear of the unknown you’ve been living with now has a name.
As with any chronic illness, your family and friends will want to provide emotional support for you. And, just like you, they will experience a flood of emotions and will have many questions and concerns regarding your diagnosis. But you need to understand that your closest supporters may not feel any more comfortable in their new roles than you do. Good communication is essential for all of you. Let your loved ones know what you need from them. Doing so enables them to feel helpful and supportive—rather than helpless and powerless—in their relationships with you.
Good communication also helps to ensure that your relationships remain healthy, even in the tough times. In fact, you may find that some of your relationships become even stronger! Being treated differently by friends and family is one of the greatest concerns for many LAM patients. It might help your support people to accept and better understand your desire for things to remain the same if you assure them that when you do need assistance with something, you’ll let them know. A diagnosis of LAM can be difficult, but poor communication will only lead to more difficulties.
In addition to concerns regarding finances, insurance coverage and employment, you may have concerns regarding parenting and marital relations. All of these issues can trigger additional worries and anxieties. To help manage these stresses consider complementary therapies to nurture your body, mind, and spirit. Exercise, acupuncture, massage therapy, yoga and reflexology are just some ways to relax and enhance your coping skills. If the stresses accompanying your diagnosis of LAM become overwhelming, seek emotional support from a friend or relative or from a professional such as a psychologist, a counselor, or a member of the clergy. Learning how to manage stress, anxiety, and depression is necessary in all phases of life, but it’s even more essential in maintaining an optimum level of health now that you’re living with a chronic disease. Be patient with yourself.
When you’re feeling ready to confront LAM on a larger scale, you may want to attend the annual LAM conference—LAMposium. During this conference, you can attend informational sessions to learn more about LAM and specially designed sessions are also available just for your group of support people. Medical professionals, who work with LAM and who are themselves attending concurrent clinical and scientific sessions, give presentations to women with LAM and their families and they are also available to answer your questions. LAMposium is an opportunity to spend time with other women and family members who can relate to what you’re experiencing.
Even though almost all women with LAM have difficulty breathing, it is important to continue exercising. The best way to get started with a training program that is appropriate for you 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 – and not hurting you.
If you’ve never used supplemental oxygen, the staff at pulmonary rehab can advise you if they feel oxygen would help you exercise more safely and effectively. If you already use supplemental oxygen, they will help you learn how much oxygen (liter flow) you need to perform different kinds of activities.
Pulmonary rehab is also helpful if you are listed for a lung transplant. It is important to make sure that you are as strong and healthy as possible in preparation for your surgery and pulmonary rehab will help you to achieve that goal.
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 many other problems. Supplemental oxygen can help make sure your body is getting everything it needs for peak performance.
Oxygen (O2) is an element your body needs to live and to perform all of your bodily functions. In general, when you inhale you take in oxygen, which then passes through your lungs and into your bloodstream through alveoli (small air sacs in the lungs). Once your blood has picked up the oxygen, it’s pumped through the rest of your body. As your body uses the oxygen for its various functions, it releases carbon dioxide (CO2) as a waste product. The carbon dioxide then travels through your blood into the cells in your lungs, up through your lungs, and you breathe it out. If this process is flawed and your body can’t pass oxygen from your lungs into your bloodstream, the oxygen levels in your blood will not be sufficient for your body to function well. If this is the case, you may need supplemental oxygen. This is especially true for people who are unable to obtain sufficient oxygen due to respiratory illnesses like LAM.
How do I know when I need Supplemental Oxygen? It’s important to recognize when supplemental oxygen might be helpful for you. These three tests can help you and your doctor determine if your body is getting enough oxygen.
Arterial Blood Gas (ABG) Test – This test measures the percentage of oxygen and carbon dioxide in your blood. This test is performed by drawing a small sample of blood from an artery, usually near your wrist (radial artery) or the crook of your arm (brachial artery).
Pulse Oximetry (SaO2) – This simple test is used to measure your oxygen saturation or the amount of oxygen in your blood. This test is performed by placing a small clip with a sensor on one of your fingertips. 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) your oxygen levels drop with exertion. As the name indicates, it’s performed by having you walk for six minutes at your own pace, covering as much distance as possible. A pulse oximeter is used to measure your heart rate and oxygen level during the test. If your saturation levels drop significantly during the test, you may be a good candidate for the use of supplemental oxygen during exercise.
Advice When Using Supplemental Oxygen
- Walk slowly and steadily rather than quickly or at various speeds. Pacing yourself can help preserve both your energy and your stamina.
- Ask your doctor about trying a nebulizer if you normally use bronchodilators. You might get an extra boost from this method of O2 delivery.
- Do necessary tasks and harder chores at whatever time of day you feel your breathing is best. You may breathe better early in the day or in the mid to late afternoon when you have more energy.
- Rest when you need to rest.
- Purchase a pinching/grabbing device for picking up items off the floor and for reaching items on high shelves. Tasks that require you to bend over or to raise your arms above your head will make you more short of breath.
A pneumothorax, or lung collapse, is a leakage of air (pneumo) from a lung into the chest cavity (thorax). This air outside the lung abruptly diminishes the negative pressure (vacuum) between the chest wall and the lung, causing the lung to deflate or collapse.
The cause of a pneumothorax in a woman with LAM is thought to be the rupture of a cyst just below the surface of the lung. Often the collapse is only partial but you still might experience some pain and shortness of breath. If a large pneumothorax occurs and collapses a sizable portion (or all) of your lung, you’ll likely have chest, back or shoulder pain and significant difficulty breathing. Most women with LAM will experience more than one pneumothorax during the course of their disease. Because of this, pleurodesis intervention is recommended after the first occurrence of a pneumothorax.
There are two goals when treating a pneumothorax. The first is the immediate removal of air from the space between the lung and the chest wall (the pleural space) so that the lung can re-expand. The second objective is the prevention of recurrent pneumothoraces. If you have a persistent leak and/or have had a pneumothorax previously, your doctor may ask you to consider pleurodesis as a preventative measure. Although pleurodesis isn't a foolproof remedy against future pneumothoraces, it generally diminishes their likelihood and the severity of future pneumothoraces and can significantly improve quality of life.
Pleurodesis is a procedure which adheres the outside of the lung to the inside of the chest cavity to prevent the lung from collapsing. If you have had a pneumothorax previously and/or have a persistent leak, your doctor may ask you to consider 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.
Although pleurodesis isn't a foolproof remedy against future pneumothoraces, it generally diminishes their likelihood. Also, if you have a pneumothorax after you’ve had pleurodesis, the collapse is more likely to be partial, and intervention may not be required. The pleurodesis procedure may be done by a number of different techniques.
A mechanical pleurodesis is performed when a surgeon manually strokes the pleura (membrane enveloping the lung) with a piece of gauze. Mechanical pleurodesis roughens up the pleura so that when the abrasion heals, the lung adheres to the chest wall. Sometimes the procedure is done in combination with some type of chemical pleurodesis.
Chemical pleurodesis involves instilling a chemical irritant into the pleural space which causes adhesion between the chest wall and the lining of the lung. Chemical pleurodesis can be done either through a chest tube while you’re awake in a hospital room or it can be done under general anesthesia via surgery. Sometimes a combination of chemical and mechanical pleurodesis is used.
One of the most common methods of chemical pleurodesis is performed using surgical talc and a chest tube. If a patient already has a chest tube in place, a talc procedure is often recommended. Once the air has been removed from the chest cavity, a talc slurry is instilled through the chest tube into the chest cavity. Alternatively, during surgery talc can be blown into the chest cavity using a bulb syringe, a method called talc poudrage. Suction via a chest tube is used after this procedure to remove any remaining air in the pleural space. Talc pleurodesis or poudrage often produces a burning sensation in the chest as the area heals, but this procedure tends to be very effective. In addition to talc, other chemical irritants such as doxycycline can be used.
If pleurodesis doesn’t work or if you have recurrent pneumothoraces, you may be a candidate for a pleurectomy. This surgery involves stripping off pleura from the inside of the chest wall to promote the fusion of the lung and the chest wall.
To perform the procedures mentioned above, most doctors use one of the following two procedures to gain access to your lungs:
Thoracoscopy or VATS
Video-assisted Thorascopic Surgery (VATS) is performed using a tiny fiber-optic camera (called a thorascope) and is a much less invasive procedure than a thoracotomy. Instead of using one large incision to gain access to the chest, one or more small finger-sized incisions, or ports, are made in your side and used to insert the scope and other surgical instruments. The thorascope transmits images of the inside of your chest onto a video monitor, guiding the surgeon in maneuvering the instruments to complete the procedure. These smaller incisions, unlike a larger surgical incision, typically result in less pain and faster recovery time. For these reasons, VATS has become the preferred method for surgical lung biopsies and other lung surgeries.
Thoracotomy is a general term for open-chest surgery. While the patient is under general anesthesia, the surgeon makes an incision that runs approximately from the front to the back (by the shoulder blades) of the chest in between two ribs. This incision allows the doctor access to the lung. A thoracotomy is used for mechanical pleurodesis, for some chemical pleurodesis procedures and for pleurectomy.
A patient usually requires about a week long stay in the hospital after a thoracotomy but full recovery can take several months. During recovery, the patient should practice deep-breathing techniques to help prevent pneumonia. As with any surgery, bleeding and infection are always risks.
Recovery From Pleurodesis
Proper pain management is important following any of these procedures. Your doctor may prescribe pain medication to help reduce the pain. It is important to stay proactive with your pain management because once pain becomes intense, it can be difficult to manage.
Pain and discomfort with these procedures will likely be intense for the first few days but will generally decrease after a few weeks of recovery. It’s also normal to experience shortness of breath and fatigue for a few weeks following any of these procedures. Resting during this time of healing is extremely important. You may need to take some time off work and will likely be restricted from driving and other activities for a period of time.
Once the healing is complete, there are usually no residual effects. Your breathing, however, may feel strange at first, as if something has changed. Unfortunately, some discomfort or a pulling sensation in the chest area can continue for months after pleurodesis.
Transplant After Pleurodesis
Many women with LAM have undergone successful lung transplants after experiencing pleurodesis. While there is a risk of bleeding complications for patients who had pleurodesis prior to lung transplantation surgery, studies have shown that these complications do not have any adverse effects on the outcome of the transplant. Experts recommend that a pleurodesis procedure is performed for women with LAM after their first episode of pneumothorax.
Although LAM develops differently in each patient, some women have aggressive, fast-growing disease. Some eventually need oxygen therapy. In time, even extra oxygen may not improve your condition and you may need to consider a lung transplant.
A lung transplant is surgery to replace one or both of the diseased lungs with healthy lungs from a human donor. This is usually considered the last resort for irreversible lung failure.
There are strict guidelines to determine which patients are eligible for transplantation. A national waiting list run by the United Network for Organ Sharing (UNOS) matches donors to potential recipients based on shared blood type, lung size, the severity of the patient's disease and likelihood that a new lung will improve the patient's survival.
Benefits versus Risks of Lung Transplantation
Deciding to have a lung transplant is a big decision. It is important to educate yourself about the risks and benefits of this life-changing and potentially life-saving procedure. While lung transplantation won't treat the underlying causes of LAM, it holds the promise of giving you more years of life and improving your quality of life.
Lung transplantation has the same risks as any other major surgery. These may include major bleeding, pneumonia, fluid in the lungs and painful recovery. In addition, transplant patients may reject the new lung(s) and be vulnerable to infection. Other potential problems include blood clots, side effects to medications and an increased risk of certain cancers.
Your body's immune system will identify the new lung as a foreign invader and may try to attack them just as it would the flu or other viruses. To prevent this response, patients are given immunosuppressive (anti-rejection) medications. Rejection of the donated organ is most likely to occur within the first three months. Symptoms of lung rejection may include fever, chills, flu-like aches, shortness of breath and infections. Patients taking immunosuppressive medications following transplantation are less able to fight off infections and viruses. Because the body's natural ability to fight off infections is reduced, it is critical to report any signs of infection to your treating physician right away. Regular follow-up visits, breathing tests, X-rays and other tests are needed to keep an eye on how the body is responding to, and healing from, the transplant.
LAM patients who undergo transplant can help LAM researchers and scientists become more knowledgeable about LAM by donating lung tissue for LAM-related studies. Access to fresh tissue is very important to scientists with open protocols and requires coordination many months in advance. If you are facing a procedure in which tissue will be removed, you may be able to donate your tissue samples to our LAM scientists for research. Please contact The LAM Foundation for more information.
The LAM Foundation Circle of Hope Transplant Group
United Network for Organ Sharing (UNOS)
If you are interested in talking with other women with LAM who have had a lung transplant, please
contact The LAM Foundation to learn more about this support group.
700 North 4th Street
Richmond, VA 23219
Second Wind Lung Transplant Patients Association
American Lung Association
61 Broadway, 6th Floor
New York, NY 10006
American Thoracic Society
61 Broadway, 4th Floor
New York, NY 10006