Hearing that a loved one has been diagnosed with LAM can have a devastating effect on family and friends. They may ask themselves several questions: "What do I say?" "How can I help?" "How can I support?" "Who can I ask for help?"

Some also may feel guilt that they did not see the "signs" sooner. For example, at work, colleagues may have noticed that their friend could not keep up on the lunch hour power walk or became short of breath quickly.

Family members may also feel guilt as they were not helping with chores and Mom was not able to finish everything.

These feelings are natural and to be expected. Once the initial feelings of shock lessen, you will find that there are many ways you can help your family member/friend who has been diagnosed with LAM.

Some of the ways are:

BE UNDERSTANDING: Many women with LAM will have 'ups and downs'. Some women will get depressed, anxious, frustrated and/or angry due to the changes that are happening in their lives. Some women with LAM may have to recover from a collapsed lung; spend months in hospital or have to leave their jobs and go on disability. Others may have to decrease their workloads or get help to take care of the children or household duties.

LISTEN: Encourage her to talk and be prepared to listen.

HELP OUT: For example, find out how your family member/friend with LAM is managing with the housework or with the children, or other activities. Try to be available to help out as best you can. It is sometimes very hard for the woman to "give up" some of the activities, so you may need to "go slow" and allow her to decide what she can and cannot do. Over time, as she becomes more comfortable with having LAM, she will feel more at ease reaching out for help.

ADJUST: Some women with LAM get short of breath when walking. Remember to slow down and pace yourself to your family member/friend.

BE SENSITIVE: Women with LAM cannot do things such as moving furniture or carrying heavy parcels. Try to remember to drop her off in front of the store and then go park the car. Realize that she may not be able to go on that hiking or bicycle trip that you are planning.

HOLD A FUNDRAISING EVENT: One of the best ways you can help is to raise funds for LAM research to find a treatment and a cure. Any type of fundraising activity-- from making a donation, letter-writing, or hosting an event, to helping her with her efforts to fundraise will be helpful and supportive. It sends the message that you want the best for your family member/friend and want her to live a quality life. Contact us or the LAM Foundation to see how this can be done.

BE INCLUSIVE. When your friend/family member has an illness such as LAM, she needs to feel included in her circle in friends/family. Please make a special effort to invite her to the usual social activities and don't exclude her because she has an illness. Don't forget to email if that is what you used to do, or call to say "hi"! It may be easier for her to email back rather than talk on the phone.

One point that is evident about LAM is its wide variability. LAM affects all women differently but can be "categorized" in two main ways: 1) by location of her problem and 2) by the seriousness and extent of the disease. LAM ranges from mild to moderate to severe and can progress very slowly to very rapidly. No one knows yet why LAM is so variable.

Collapsed lung (Pneumothorax)
A pneumothorax means a collapsed lung with air in the space between the lung and the chest wall. On one hand, about 60% of women with LAM have had at least one pneumothorax; on the other hand that means that 40% of LAM women have never had one.

Some LAM women may have only one pneumothorax but others may have many collapses involving both lungs. If you have had one collapse, you are at risk for another. But not everyone will have another lung collapse. For most women, the collapse is treated and the lung function may return to normal or near normal once the lung is re-inflated.

Chylothorax
A chylothorax means that there is a collapsed lung and lymph fluid in the space between the lung and the chest wall. It is less common than pneumothorax but is harder to treat.

Shortness of Breath (SOB)
Women will go to their doctor because they feel short of breath particularly during exertion or climbing stairs. This will vary considerably depending upon circumstances. For example, if a woman is very active athletically, shortness of breath may be more evident to her.

For non-exercisers, they may blame the shortness of breath on being 'out of shape' or needing to exercise. Mostly, women will compare themselves to other women of similar age. Again, this can be misleading as other women will also claim the need to exercise more. At some point, if the shortness of breath continues to get worse, women will seek medical attention. Here is where women will tend to be misdiagnosed as there are many causes of shortness of breath. Hopefully a physician will order pulmonary function tests (PFTs) to determine if lung function is the reason for the shortness of breath.

Pneumonia/multiple infections
Women, especially younger women, who have had unexplained pneumonia or multiple bronchitis or chest infections may seek medical advice. Since pneumonia is unusual in younger persons, the physician may do a series of tests to determine the cause of the pneumonia - especially if there have been multiple episodes or pneumonia that is not getting better quickly. This will lead to the diagnosis of LAM in some women.

Kidney problems
LAM women may have various problems with kidney tumours. Unfortunately, some women with LAM have had a kidney removed after doctors have found large tumours not recognizing that this is a sign of LAM. Other women have been more fortunate in having a nephrologist or urologist who recognizes that this tumour might be LAM and orders a CT-scan of the chest.

The combination of kidney tumour and cysts in the chest are the most telling signs of LAM and make the diagnosis. In most instances, but not everyone, women with large AMLs (angiomyolipomas - kidney tumours due to LAM) tend to have mild disease in the lung and vice versa (those with more severe lung disease tend to have smaller AMLs) Not everyone with LAM has an AML.

Abdominal LAM
Although uncommon, some women have abdominal LAM as their major problem. This may involve a lot of fluid build up in the abdomen that may impede digestion and may cause other symptoms and problems. The fluid often has to be drained meaning multiple medical interventions. The fluid may be chyle (lymph fluid).

Abdominal tumours
Abdominal tumours, due to LAM in various locations in the abdomen, are often found. Mostly, these are insignificant and do not cause any problem. Sometimes these lead to some bloating of the abdomen. In some instance, these tumours may lead to unnecessary medical investigations.

Testing for other problems
Some women with LAM have been diagnosed because they have had medical imaging for other conditions. For example, a CT-scan of the abdomen often captures the tip of the lung in the image. In these instances, the radiologist notices that there is something going on in the lung.

Other presentations
These vary considerably. Here is one example. One LAM woman was diagnosed with LAM after she had a blood clot in her eye. Since this is a very unusual situation for a young person, the physician investigated further. It was noted that she had high hematocrit, i.e. more red blood cells than is normal.

Her physician then investigated the reason for the high blood cell count as one reason for high hematocrit may be hypoxia (low blood oxygen). In response to hypoxia the body makes more red cells. One reason for hypoxia is poor lung function as is found in LAM. High hematocrit can lead to blood clots since the blood is 'thickened' and can more easily clot.

Of course the patient also had shortness of breath but since her lungs "sounded clear" when the doctor listened it was not felt that lung disease was present. She just felt she was "out of shape"!

Just because you were diagnosed with LAM doesn't automatically mean that you will need to use oxygen. Many women with LAM have mild lung disease and don't need supplemental oxygen and will never need it. Some women may not need oxygen now but may need it in the future.

Here are a few things to consider when you are making the difficult decision on whether or not to use supplemental oxygen. First you must speak with your doctor; but here are a few issues to consider.

When to use oxygen?
This will depend on each woman's individual situation. However, in general, if your oxygen saturation (see below) is less than 90% then you should consider using oxygen. If your oxygen saturation level is more than 90% when you are sitting or lying down but decreases to below 90% when you are walking around, you should consider using oxygen.

Other women may only need oxygen supplementation for exercising or for flying.

How do I find out if I need oxygen?
To find out if you need oxygen, you will need an assessment. This can be done at a pulmonary rehab program or at a hospital. You can be tested sitting and standing and walking around and also when you exercise.

Sometimes, your oxygen levels fall when you sleep; while this is normal for most people, for women with LAM, your saturation may fall below 90% during the night. A night time assessment can be done in which you wear a pulse oximeter probe (attached to a recorder) around your finger while you sleep.

How much oxygen do I need?
The amount of oxygen will vary from person to person. In general, you will need to use sufficient oxygen to keep your saturation above 90%; preferably 93-95% or better. This needs to be discussed with your doctor.

Note that most oxygen systems are adjustable; you may need more or less flow depending on what you are doing at the time. Many women with LAM purchase their own portable pulse oximeters to help them calibrate and monitor their oxygen requirements (costs about $600).

LAM is different from other illnesses in which people require oxygen; LAM women usually require varying oxygen flows, while in other illnesses such as COPD (chronic obstructive pulmonary disease), the flow rate is stable. Insurers, airlines and oxygen providers may not be aware of the variable demands for oxygen for LAM patients.

What is oxygen saturation?
Oxygen saturation is the percentage of hemoglobin (the part of the red blood cell that carries oxygen) in the blood that is saturated with oxygen. Lower saturation means that the hemoglobin is 'carrying' or has less oxygen. Oxygen saturation is measured by a pulse oximeter that measures oxygen in the blood in one of your fingers. Oxygen saturation is also called "O2 sat" and is measured in percentages. Normal oxygen saturation is above 95% but most people without lung disease will be around 96-99% depending on age (some lung function is lost for everyone as they get older).

Why do I need supplemental oxygen at all? What will happen if I don't use it?
Supplemental oxygen is needed to keep your O2 saturation about 90%. Several things can happen if you are hypoxic - i.e. have O2 saturation less than 90% for long periods of time. At first the body will try to compensate by making more red blood cells. This will work for a while but over longer periods of time and worsening hypoxia, the body may make too many red blood cells and the blood is too 'thick'. This can lead to blood clots which can be dangerous.

Secondly, being hypoxic can lead to being overly fatigued and tired all the time.

This can be a major cause of poor quality of life.

Another issue that can be attributed to hypoxia is heart problems. With lung diseases, the amount of oxygen in the blood is lessened. The heart receives a signal from the brain to work harder to pump more blood through the lungs to get more oxygen. This can work for a while, but in some instances, the heart can no longer keep up the demand. The physiological reaction of the heart is to get larger. This allows the heart to work harder to pump more blood. But there is a limit to how much the heart can enlarge (medical term is hypertrophy). This can lead to heart failure and other heart problems such as pulmonary hypertension. Using supplemental oxygen is believed to help prevent these problems.

How do I arrange for oxygen delivery?
Since each province has slightly different requirements and systems for the delivery of oxygen, we cannot provide any specific information to you. Once your doctor decides that you should have supplemental oxygen, he/she will begin the arrangements to have oxygen supplied to you.

LAM Canada will publish information on new oxygen delivery systems and technologies in our newsletter. Once a woman registers with LAM Canada they will automatically receive the newsletter.

Can I fly with oxygen?
This is a relatively new area of concern for patients with supplemental oxygen. If you are planning to fly it is important that you plan early and check with the Airlines, as each company has their own policies. Some will provide oxygen while others will allow only certain technologies. There is activity going on (i.e. lobbying MPs and government officials) to design laws and practices that will allow for air travel with oxygen.

If you have any other oxygen-related questions, please do not hesitate to contact us at LAM Canada.